key: cord-0067425-mraun6oe
authors: nan
title: Abstracts der 59. Jahrestagung der Österreichischen Gesellschaft für Kinder- und Jugendheilkunde 2021: Pädiatrie komplex
date: 2021-09-09
journal: Monatsschr Kinderheilkd
DOI: 10.1007/s00112-021-01292-9
sha: 6d4e944b9b9b7d78d597b9ce3b0b6b44468f73b2
doc_id: 67425
cord_uid: mraun6oe

nan

Immunreaktion auf SARS-CoV-2 bei Kindern. Die entzündlichen Reaktionen können alle Organe und Gefäße betreffen, insbesondere vital gefährdend bei begleitender Myokarditis und Koronararterienaneurysmen. Ziel dieser Untersuchung ist die Beschreibung von Symptomen, Diagnostik und Therapie bei Kindern mit PIMS mit Fokus auf die kardiovaskuläre Manifestation und Folgen einer kardialen Beteiligung. Patienten und Methoden: Für diese retrospektive Studie wurden die Daten (klinisch, Labor, radiologisch) von Patienten im Alter 0-18 Jahren mit PIMS seit März 2020 in 6 Wiener und 3 niederösterreichischen Kinderabteilungen evaluiert. Einschlußkriterien waren die PIMS-WHO Kriterien von 2020. Es wird eine Zwischenanalyse präsentiert, da aktuell noch nicht alle Zentren über ein EK Votum verfügen. Introduction: Despite their importance discharge letters often lack content and form. Accurate, comprehensive transfer of information across the healthcare interface is essential to ensure consistency between planned and provided treatment, as well as to ensure patient safety. However, deficits in communication are widely reported. Low quality in discharge letters is associated with patient risk and adverse outcome (e. g. medication related harm). Today, only few systematic approaches to improve the quality of discharge letters written by young residents have been conducted. Patients and methods: To provide an overview about current content, structure, weaknesses, expectations and most important aspects of an "ideal discharge letter", seven explorative qualitative semi-structured expert interviews have been performed, which allowed in-depth discussion and exploration of these topics, guided by pre-defined questions but also allowing exploration of individual aspects. The experts were residents and senior physicians from different pediatric divisions of the Dpt. of Pediatrics and Adolescent Medicine, Medical University of Vienna, as well as experts from other pediatric centers and pediatricians from the outpatient sector, respectively. Data were analyzed through qualitative content analysis. Results: The results have been compared with existing guidelines and standards and a checklist based on the content analysis and literature search has been created. The items of said checklist have been rated by 15 residents and senior physicians to assign a score for every subcategory. To evaluate the applicability of the checklist 10 discharge letters have been chosen randomly and scored by two evaluators. The reliability of the checklist was assessed, and revealed a good inter-rater reproducibility (Evaluator 1: Median 60.9 % vs. Evaluator 2: Median 55.2 %). The achieved scores (Median 59.2 %) were arranged from 29.1 to 86.9 %. Discussion/conclusion: We were able to generate a checklist that allows a grading of discharge letters and which enables us to compare the current status with the target status. The achieved scores showed the need for improvement. It is planned to implement the content of the checklist into an interactive training platform for residents. The process is going to be evaluated scientifically by comparing letters produced before and after the intervention by means of the created checklist.

FV02-05 "Training culture" -Eruierung der Bedürfnisse und Herausforderungen in der pädiatrischen Ausbildung Hellmann A. 1 Einleitung: Die Ausbildung zum Sonderfach Kinder-und Jugendheilkunde stellt, neben dem Aneignen von spezifischem Wissen und speziellen Fertigkeiten, die Assistenzärzt* innen im klinischen Alltag immer wieder vor neue Herausforderungen. Dazu zählen zum Beispiel Rotation auf andere Abteilungen, Nachtdienstsituationen, aber auch der Umgang mit Todesfällen. Um die Hürden aber auch Unterstützungsmöglichkeiten der lernenden Ärzt* innen sowie deren Bedürfnisse in Bezug auf die Ausbildung systematisch zu erfassen, eignen sich Qualitative Forschungsansätze besonders gut. Die Perspektiven zu personen-, kontext-und prozessbezogenen Aspekten der klinischen Ausbildung sollen durch qualitative Interviews ganzheitlich erfasst und wissenschaftlich aufbereitet werden. Patienten und Methoden: Als Basisdaten dieser prospektiven qualitativen Studie diente das Transkript des Needs Assessment der Pediatric-Day-One-Competencies an der Wiener Universitätsklinik für Kinder-und Jugendheilkunde, das durch offene, semi-strukturierte Interviews mit 16 Assistenzärzt* innen im Rahmen der Task Force Ausbildung erhoben und für diese Studie einer qualitativen Inhaltsanalyse unterzogen wird. Als theoretical framework der Analyse dient das Person-Process-Context Modell ( PPC) nach Hiemstra & Brockett (2012), welches im Bereich Medical Education den Self-Directed Learning ( SDL) Ansatz unterstützt. Es erfolgt eine Zuteilung der durch die Interviews herausgearbeiteten Themen zu den PPC-Sphären -aber auch deren Schnittmengen.

Ergebnisse: Wir erwarten durch unsere Ergebnisse neue Erkenntnisse zur clinical learning environment aber auch vor allem durch die Schnittmenge zwischen Prozess und Kontext bzw. Person und Kontext im Bereich SDL zu erlangen. Durch die Definition sogenannter barriers and facilitators aus Sicht der Assistenzärzt* innen sollen Verbesserungsansätze innerhalb der Ausbildung aufgezeigt werden. Schlussfolgerung/Diskussion: Um die Bedürfnisse und Herausforderungen der ärztlichen Ausbildung hinreichend analysieren zu können, sind qualitative Studien von großer Bedeutung. Auf Basis der Ergebnisse dieser qualitativen Untersuchungen sollen gezielte Interventionen von Seiten der Organisation zur Verbesserung der klinischen Ausbildung in der Pädiatrie gesetzt werden. Einleitung: Patienten mit einer pränatal diagnostizierten kritischen Aortenstenose ( CAS) und sich entwickelndem hypoplastischem Linksherzsyndrom (eHLHS) haben weiterhin eine ausgeprägte Morbidität und Mortalität. Das Ziel dieser Studie war es unsere 20-jährige Erfahrung mit intrauterinen Dilatationen der Aortenklappe bei Feten mit CAS und eHLHS inklusive frühem und "mid-term" postnatalem Outcome zu analysieren und Selektionskriterien für intrauterine Aortenklappendilatationen zu aktualisieren. Patienten und Methoden: Bei dieser Studie handelt es sich um eine retrospektive Studie mit Einschluss aller Feten seit 2001, die aufgrund einer kritischen Aortenstenose eine intrauterine Aortenklappendilatation an unserem Zentrum erhalten haben. Hierfür wurden Echokardiographien und Patientenakten auf ventrikuläre und valvuläre Dimensionen, sowie hämodynamische Daten und Outcome analysiert. Eine CART (Classification and Regression Tree) Analyse wurde durchgeführt, um die prädiktive Kapazität der analysierten Parameter zu analysieren. Ergebnisse: Zwischen Dezember 2001 und September 2020 hatten 103 Feten insgesamt 125 Aortenklappendilatationen an unserem Zentrum. 87,4 % aller Feten hatten einen technisch erfolgreichen Eingriff. Der technische Erfolg pro Patienten war in der rezenten Periode höher (96,2 % vs. 78 %, p = 0,0133). 78 Feten wurden nach erfolgreichem Eingriff lebend geboren und erhielten eine weitere Behandlung. Ein biventrikuläres Outcome im Alter von einem Jahr konnte bei 56,4 % der lebend geborenen Patienten erzielt werden und war signifikant höher verglichen mit dem biventrikulären Outcome einer Kohorte mit natürlichem Verlauf in der Fetalperiode (56,4 % vs. 23,7 %, p = 0,0013). Eine Entscheidungsbaum Analyse zeigte eine hohe Sensitivität und Spezifität von 93,44 % und 94,44 % hinsichtlich postnatalem biventrikulärem Outcome. Die beste Erkennungsrate für eine biventrikuläre Zirkulation im Alter von einem Jahr wurde durch eine Kombination von "Cutoffs" für die Variablen RV/ LV Ratio und Vmax des MI Jets erreicht. Schlussfolgerung/Diskussion: Intrauterine Dilatationen der Aortenklappe konnten mit hoher Erfolgsrate und akzeptablem Risiko durchgeführt werden und führten im Vergleich zum natürlichen Verlauf zu besserem biventrikulärem Outcome. Die Ratio zwischen rechtem und linkem Ventrikel in Kombination mit Druck-Schätzungen des linken Ventrikels konnten eine erfolgreiche biventrikuläre Zirkulation mit 93,44 % Sensitivität und 94,44 % Spezifität vorhersagen.

Interventioneller "reverser Potts-Shunt" bei einem Säugling mit schwerer pulmonaler Hypertension und verschlossenem Ductus arteriosus Kitzmüller E., Beichl M., Albinni S., Michel-Behnke I.

Medizinische Universität Wien, Klinik für Kinder-und Jugendheilkunde, Dept. für Pädiatrische Kardiologie Einleitung: Der "Reverse Potts Shunt" ist ein palliatives Verfahren zur Dekompression des drucküberlasteten rechten Ventrikels bei schwerer pulmonaler Hypertonie ( PH). Wir berichten über den ersten Fall einer interventionellen Erzeugung eines "endogenen" Reverse-Potts-Shunts durch Stenting eines bereits verschlossenen Ductus arteriosus bei einem 32 Tagen alten männlichen Säugling mit supra-systemischer PH. Patienten und Methoden: Der Knabe entwickelte in der ersten Lebenswoche eine schwere pulmonale Hypertension welche trotz Therapieeskalation eine ECMO Therapie für 10 Tage notwendig machte. Danach traten trotz intensiver medikamentöser Therapie pulmonalhypertensive Krisen auf, welche eine Entwöhnung von der Beatmung unmöglich machten. Trotz Verabreichung von Prostaglandin E1 konnte echokardiographisch der Ductus nicht nachgewiesen werden. Trotzdem entschlossen wir uns eine Katheterintervention durchzuführen. Es wurde über einen perkutanen femoralen arteriellen Zugang komplikationslos und erfolgreich ein 4 mm Koronarstent, in den zuvor auch angiographisch nicht nachweisbaren, aber schlussendlich mittels Coronardraht passierbaren Ductus, implantiert. Dadurch wurden die pulmonalhypertensiven Krisen nun klinisch deutlich besser toleriert, sodass der Knabe nach kurzer Zeit von der Beatmung entwöhnt werden konnte. Ergebnisse/Schlussfolgerung/Diskussion: Interventionelles Stenting eines bereits verschlossenen Ductus arteriosus zur Erzeugung eines "endogenen" Reverse-Potts-Shunts bei einem Säugling mit schwerer PH und drohendem RV-Versagen ist technisch machbar und wirksam.

Normwerte aortaler Elastizitätsparameter gesunder Kinder und Jugendlicher Röhl H., Makic B., Meinel K., Kurath-Koller S., Köstenberger M., Burmas A., Grangl G., Schweintzger S., Gamillscheg A., Baumgartner D.

Klin. Abteilung für Pädiatrische Kardiologie, Department für Kinder-und Jugendheilkunde, Medizinische Universität Graz Einleitung: Die arterielle Elastizität beeinflusst den Verlauf vieler kardiovaskulärer Erkrankungen (Marfan-Syndrom mit Aortendilatation, -dissektion und -ruptur, arterielle Hypertonie, Aortenisthmusstenose, diabetische Vaskulopathie …). Zu deren Messung stehen mehrere Methoden zur Verfügung. Für die im Kindesalter geeigneten Methoden fehlen oft Referenzwerte Gesunder. Ziel dieser retrospektiven, ultraschallbasierten Studie war es, Referenzwerte für die aortalen Elastizitätsmarker Distensibiltät und Stiffness Index β bei gesunden Kindern und Jugendlichen zu erheben und mit einem pathologischen Kollektiv zu vergleichen. Patienten und Methoden: Wir untersuchten 116 Patienten (60 weiblich, 56 männlich) im Alter von 10,7 ± 6,1 Jahren mit normaler Linksventrikelfunktion nach Ausschluss einer Aortopathie, einer arteriellen Hypertonie und eines hämodynamisch wirksamen angeborenen Herzfehlers im Rahmen einer ambulanten kinderkardiologischen Routineuntersuchung mittels Echokardiographie. M-Mode Bilder der Aorta ascendens und descendens wurden aufgenommen und der Blutdruck am rechten Oberarm oszillatorisch dreimal gemessen. Nach bekannten Formeln berechtneten wir mit Hilfe einer Konturfindungssoftware die Distensibilität und der Stiffness Index β der Aorta ascendens und descendens. Die Ergebnisse stellten wir nach Alter und Geschlecht getrennt dar. 30 Patienten mit ei-ner Aortenwurzeldilatation bei Marfan-Syndrom, Ehlers-Danlos-Syndrom oder Loeys-Dietz-Syndrom wurden einer nach Alter und Geschlecht gematchten gesunden Kontrollgruppe gegenübergestellt. Ergebnisse: Die Distensibilität Gesunder zeigte sich mit dem Alter abnehmend, der Stiffness Index β im Gegenzug mit dem Alter zunehmend. Die Distensibilität der Aorta ascendens der 30 Patienten mit Aortopathie war gegenüber der Referenzgruppe signifikant erniedrigt, der Stiffness Index β signifikant erhöht. In der Aorta descendens zeigte sich bei den Patienten ein nicht signifikanter Trend zu einer verminderten Aortenelastizität. Schlussfolgerung/Diskussion: Die vorliegenden Referenzwerte für die nicht invasiv bestimmte Distensibilität und den Stiffness Index β stellen die Grundlage für eine Interpretation der Messwerte bei Patienten mit unterschiedlichen Aortopathien dar. So können die genannten Elastizitätsparameter als Marker die Diagnostik mancher Aortopathien erleichtern und im Verlauf zur Therapiesteuerung herangezogen werden. Introduction: Heterotaxy is defined as an abnormal arrangement of inner organs across the left-right axis of the heart. Prenatal differentiation of the two main types of heterotaxy can be challenging: left-atrial isomerism ( LAI) and right-atrial isomerism ( RAI), each with distinct, but highly variable clinical features. We evaluated structural and functional abnormalities, as well as outcome, in our cohort of fetuses with prenatal diagnosis of heterotaxy. Patients and methods: Retrospective analysis of fetal outcome after prenatal diagnosis of heterotaxy from 1998 to 2019 in a tertiary referral center. Prenatal imaging and perinatal outcome data collected. Long-term outcome including postpartum clinical evaluation and surgical interventions were assessed for surviving infants. For terminations of pregnancy, stillbirths and late deaths, autopsy reports were reviewed. Results: Forty-six fetuses with heterotaxy and cardiovascular pathology, of which 28 (58 %) had LAI and 18 (38 %) RAI, were included. In 7/28 fetuses (25 %) in the LAI group, parents chose termination of pregnancy, 3 fetuses were lost to follow up and one pregnancy is still ongoing. In the RAI group 5/18 pregnancies (27 %) were terminated and one fetus was lost to follow up. Arrhythmia was only present in the LAI group: 8/28 (29 %) fetuses with LAI had third degree AV Block, of which three developed hydrops Overall 29 fetuses (63 %) were live born (17 LAI, 12 RAI). Eighteen (18/29, 62 %) newborns survived the first year, and 15/29 (51 %) patients were alive at the time of data collection at a median age of 5.4 years (6 months to 16 years). Biventricular anatomy was present in 8/17 (47 %) patients in the LAI group Univentricular palliation was more common in the RAI group: 10/12 patients underwent at least the first step of palliation, 5/12 (42 %) children with RAI have reached the Fontan procedure stage after palliative surgery. One neonate ( RAI) was found to be inoperable, and one child had a biventricular corrective surgery. Discussion/conclusion: RAI is almost invariably associated with complex cardiac malformations, giving these children generally a worse prognosis compared to LAI. In this study, fetuses with RAI and complex cardiac disease had a slightly more favourable outcome than previously described, once they survive the first year of life.

Topic: Hämatologie und Onkologie FV03-05 Essential thrombocythemia in Austrian children and adolescents Novak W. 1 , Cseh A. 1 , Crazzolara R. 2 , Jones N. 3 , Dworzak M. 1 , Kager L. 1 1 St. Anna Children's Hospital, UKKJ/ MUW; 2 Department of Pediatrics, MU Innsbruck; 3 Department of Pediatrics, PMU, Salzburg, Introduction: Essential thrombocythemia ( ET) belongs to the BCR-ABL1 negative myeloproliferative neoplasms and is an extremely rare disease in pediatrics. Diagnostic and treatment algorithms are well established for adults with ET, but not for younger patients. Patients and methods: We performed a retrospective cohort analysis on data collected between 2009 and 2021 in the Austrian EWOG registry. The diagnosis of ET was established using the WHO criteria. All patients had undergone bone marrow analyses, PB counts, molecular genetics ( BCR-ABL1, JAK2, CALR and MPL), and acquired von Willebrand syndrome ( AVWS) analysis. Results: 14 patients (median age of 12 years, range 0.3-16 years, female/ male 7:7) with ET were identified. Median follow-up was 26.5 months (range, 1-117 months). A JAK2 V617F mutation was detected in 7 patients, a CALR type 1 mutation in 1 patient, while no MPL or BCR-ABL1 mutations were detected. Acetylsalicylic acid was given to 6, and cytoreductive treatments to 7 patients [4 anagrelide, 3 pegylated interferon (of which 2 received ropeginterferon alfa-2b)] and one patient was temporary switched to hydroxyurea. We observed severe complications in two untreated female adolescents with JAK2 V617F ET: one developed Budd-Chiari syndrome and underwent successful liver transplantation, and one presented a recurrent sinus venous thrombosis. Neither secondary development of higher-grade myelofibrosis nor clonal evolution were observed. Although 8 patients (57 %) developed an AVWS, none presented with serious bleedings. Discussion/conclusion: Our results further support recent findings that young patients with ET often have AVWS and can develop life-threatening complications, especially if they carry a JAK2 mutation. While for asymptomatic patients a watchful-waiting strategy is yet recommended, JAK2 positive pediatric patients may benefit from early cytoreductive therapy. Ropeginterferon alfa-2b (a novel long-acting interferon), was used for the first time in patients aged < 18 years. Its use in two symptomatic female patients with JAK2 V617F positive ET lacked a clear hematologic response. However, there was no thrombotic event during the observed treatment period, the symptoms (headache) declined, and the medication was well tolerated.

Topic: Neonatologie FV03-06 Erhöhtes Risiko für zerebrale Hypoxie innerhalb der ersten 15 Lebensminuten bei gesunden Reifgeborenen mit pränataler Nikotin-Exposition Wolfsberger C., Bruckner M., Schwaberger B., Mileder L., Pritisanac E., Höller N., Urlesberger B., Pichler G.

Abteilung für Neonatologie, Universitätsklinik für Kinder-und Jugendheilkunde, Medizinische Universität Graz Einleitung: Rauchen während der Schwangerschaft ist ein globales prävalentes Problem, welches mit zahlreichen prä-, peri-und postnatalen Effekten und Risiken assoziiert ist. Das Ziel der Studie war es zu analysieren, ob maternales Rauchen während der Schwangerschaften bei gesunden Reifgeborenen einen Einfluss auf die zerebrale Oxygenierung (crSO2) und Gewebssauerstoffextrakti-on (cFTOE), gemessen mit Nahinfrarot-Spektroskopie ( NIRS), unmittelbar nach Geburt, innerhalb der ersten 15 Lebensminuten, hat. Patienten und Methoden: Sekundäre Outcome-Parameter prospektiver NIRS Beobachtungsstudien, die an der Abteilung für Neonatologie Graz durchgeführt wurden, wurden analysiert. Inkludiert wurden gesunde reife Neugeborene, ohne Atemunterstützung innerhalb der ersten 15 min nach Geburt, wenn Informationen bezüglich des mütterlichen Rauchverhaltens während der Schwangerschaft bekannt sind. Die NIRS Messung wurde mit dem INVOS 5100C Monitor durchgeführt. Reifgeborene, deren Mütter während der Schwangerschaft nicht geraucht haben (Kein-Nikotin Gruppe) wurden entsprechend ihres Gestationsalters (± 1 Woche), Geburtsgewichtes (± 100 g) und Hämatokrit-Wertes (± 5 %), mit gesunden Reifgeborenen, deren Mütter während der Schwangerschaft geraucht haben (Nikotin Gruppe) im Introduction: During ventilation and endotracheal intubation success rates are low and falling due to decreasing clinical opportunities. Therefore, simulation trainings have been implemented to teach these skills to unexperienced clinicians. Several studies suggest that the use of feedback devices can further support the teaching effect, however it has not been studied before, which specific teaching setup is most beneficial for trainee performance. Patients and methods: This is a randomized controlled trial. We aim to include 171 participants in this study (medical students, 3rd-6th year). Participants take part in a simulation-based training, which simulates a planned intubation of an infant using a video-laryngoscope as well as manual ventilation before and after, which is recorded by a respiratory function monitor ( RFM). Participants are randomized into three groups with different teaching setups. In group A both devices are not visible for supervisor and participant, in group B they are only visible for the supervisor and in group C for both supervisor and participant. The primary endpoints are the number of attempts for a successful intubation and the percentage of ventilations within the tidal volume range. Results: As of today, we have collected data from 75 participants. We identified significant differences in the percentage of tidal volume within the range between all groups (group A to B p < 0.001; group A to C p < 0.001, group B to C p = 0.002).

We established significant differences for intubation attempts between group A and groups B (p 0.002) and C (p < 0.001), until now we could not identify statistical differences between groups C and B (p 0.190). Discussion/conclusion: Based on our results, the most effective teaching method regarding the tidal volume seems to be the teaching setup of group C. We expect similar results for the intubation data once all data is collected. It can be hypothesized that similar effects can be expected when using these teaching methods within a clinical setting. These results could be used to better design future teaching scenarios during simulation trainings as well as in clinical settings. Ergebnisse: SPES-2 umfasst einen Elternfragebogen (Ausfüllzeit ca. 5 min), der anhand einer Wortschatzliste (37 Items) das expressive Vokabular erhebt, zudem werden das Auftreten von Wortverbindungen, Elternsorgen und soziodemographische Informationen erhoben. Lediglich bei Kindern mit Auffälligkeiten laut Eltern (ca. 14 %) wird eine ärztliche Überprüfung des Wortverstehens (3-5 Min) durchgeführt. SPES-2 prädiziert bis zum Alter von 3 Jahren anhaltende Sprachentwicklungsprobleme und erlaubt eine Triagierung in (i) unauffällig, (ii) nur expressiv auffällig und (iii) gemischt expressiv und rezeptiv auffällig. Im ersten Fall (i) wird lediglich ein Kontrollscreening im Alter von 3 Jahren empfohlen, bei (ii) eine Elternberatung zu Sprachförderung in der Familie mit entspr. Unterlagen durchgeführt und bei (iii) erfolgt eine Überweisung zur multiprofessionellen Abklärung. Zudem liegen erste Daten von SPES-2 mit Übersetzungen in häufige nicht-deutsche Erstsprachen in Österreich vor. Die finale Version von SPES-3 besteht lediglich aus einem Elternfragebogen, der die Strukturbeherrschung (Grammatik) und eine Beurteilung des Wortschatzes umfasst. (Ausfüllzeit 5-10 min). Beide Sprachscreeninginstrumente wurden von den Kinderärzten hinsichtlich ihrer Praktikabilität (zeitlich, Akzeptanz durch Eltern) und Sinnhaftigkeit überwiegend positiv beurteilt. Die sehr zufriedenstellenden Daten zur Validität und Reliabilität der Instrumente werden präsentiert. Schlussfolgerung/Diskussion: Angesichts der Testgüte der Screeningverfahren als auch der nach umfangreichen Praxiserprobungen ermittelten Machbarkeit wird eine Umsetzung in der kinderärztlichen Praxis im Rahmen der Mutter-Kind-Pass Untersuchungen im Alter von 2 und 3 Jahren empfohlen. Introduction: Mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy ( MOGHE) is a newly described, rare histopathologic entity detected in resected brain tissue of patients with refractory epilepsies. Here, we present new clinical insights into this intriguing entity. Patients and methods: Retrospective data analysis of 45 pediatric patients histopathologically diagnosed as MOGHE with difficult to treat epilepsy who received epilepsy surgery at our center. Results: Young age at onset of epilepsy with average of 2 years and 47 % of onsets within the first year of life. Epilepsy was difficult to treat in all cases with an average of 7 anti-seizure medications and a high seizure burden with daily seizures in 90 % of patients. 53 % of patients had infantile spasms at the beginning of epilepsy. 91 % of patients had multiple seizure types. The most frequent seizure types were tonic seizures in 84 % of patients. Re-evaluation of preoperative MRIs showed age-related characteristic features of MOGHE with increased laminar T2 and fluid attenuated inversion recovery ( FLAIR) signal at the corticomedullary junction in younger patients and reduced corticomedullary differentiation due to an increased signal of the adjacent white matter in older patients. Average age at surgery was 8.6 years. Unilobar resection was the most common surgical approach in 58 % of patients. Postoperative seizure control according to Engel classification was 64 % Engel class 1, 4 % Engel class 2, 16 % Engel class 3 and 11 % Engel class 4. Discussion/conclusion: To the best of our knowledge, this is the largest cohort of pediatric patients with MOGHE in which we could confirm most of the clinical characteristics mentioned in the first description of this entity by Schurr et al. 2017. Furthermore we add additional demographic, clinical and radiological features of this entity in order to expand the clinical spectrum of MOGHE. Characteristic clinical and MRI features can allow correct preoperative diagnosis and influence the surgical approach and thereby postoperative outcome. Pediatric epileptologists working in the field of presurgical evaluation should be aware of this new entity in difficult to treat pediatric epilepsies.

Blood parameter analysis in pediatric MOG-antibodyassociated disorders Introduction: MOG-antibody-associated disorders ( MOGAD) are acquired inflammatory conditions clinically mainly presenting with acute disseminated encephalomyelitis, optic neuritis, transverse myelitis or neuromyelitis optica spectrum disorders ( NMOSD)-like phenotypes. The majority of pediatric MOGAD patients experience only a single clinical attack. Persisting high antibody titers against myelin oligodendrocyte glycoprotein ( MOG-abs) are rare and seem to be associated with a multiphasic disease course. Despite their easy availability and low costs, blood count parameters have not been investigated systematically in pediatric MOGAD yet. Patients and methods: Blood parameters of 270 pediatric patients from multiple centers in Austria and Germany with a total of 776 timepoints were included in our study. 62 patients had MOGAD (186 timepoints), 10 aquaporin-4 antibody (AQP4-ab)-positive NMOSD (60 timepoints), 56 multiple sclerosis ( MS) (209 timepoints), 128 other neurological disorders ( OND) (305 timepoints) and 14 were healthy controls ( HC) (16 timepoints). We evaluated differences between these groups and different time points (clinical attack, acute treatment, remission). Analyzed parameters included complete blood count, neutrophil-to-lymphocyte ratio ( NLR) and platelet-to-lymphocyte ratio ( PLR). Results: A first data extraction showed elevated leukocyte counts and higher NLR at onset and during relapse in children with MOGAD compared to children with MS, OND, or HC. Highest NLR and PLR at onset and during relapse were found in patients with AQP4-abs. During acute treatment, leukocyte counts were higher than during clinical attack in every group except for MOGAD. Similarly, NLR was higher in all groups except for the monophasic MOGAD group. PLR rose in children with AQP4-abs, MS and OND during acute treatment, but remained constant in those with MOG-abs. In remission, all groups showed lower leukocyte counts compared to onset. While NLR and PLR decreased in MOGAD patients, NLR remained high and PLR even increased in AQP4-ab-positive NMOSD patients between clinical attack and remission. Discussion/conclusion: First results showed differences in leukocyte counts, NLR and PLR between the included groups as well as within the groups between onset/relapse and remission. We hypothesize that certain blood parameters might help clinicians to distinguish between different entities of neuroinflammatory disorders. Further analysis of the data will test potential associations between blood parameters and disease activity. Introduction: In respiratory distress syndrome, exogenous surfactant administration is one of the major therapeutic strategies used in premature infants to reduce mortality and morbidity. Many neonatology centers worldwide perform minimal invasive surfactant application in these patients, in accordance to current guidelines and consensus recommendations, using small-diameter catheters for endotracheal intubation and surfactant injection. Patients and methods: In this single-center, open-label, randomized controlled trial, preterm infants requiring surfactant administration after birth, using a standardized minimal invasive protocol, were randomized to two different modes of endotracheal catheterization: Flexible charrière-4 feeding tube inserted using Magill forceps (group 1) and semi-rigid catheter (LISAcath®) (group 2). Primary outcome was duration of laryngoscopy. Secondary outcomes were complication rate and vital parameters during laryngoscopy. Results: Between February 2019 and April 2020, 31 infants were included in the study. Mean gestational age and birth weight were 27 + 6/7 weeks and 1009 g; and 28 + 0/7 weeks and 1127 g for group 1 and 2, respectively. Mean length of laryngoscopy was similar in both groups (overall and adjusted for weight or gestational age) 61.1 s and 64.9 s (p 0.78). Laryngoscopy was interrupted six times in group 1 (38 %) and four times (27 %) in group 2. Longer laryngoscopy was associated with lower oxygen saturation with lowest levels occurring after failed laryngoscopy attempts. Intraventricular hemorrhage (> grade 1) occurred in both study groups, while bradycardia (hear rate <100 bpm) was exclusive to group 1 (n = 4; 27 %). Discussion/conclusion: This study showed no difference in laryngoscopy duration in endotracheal catheterization when comparing semi-rigid and flexible catheters for minimal invasive surfactant application in preterm infants. Preliminary data on nurses and medical students, as well as published in-vitro trials showed laryngoscopy was performed more readily with the use of semi-rigid catheters. In our study however, experienced neonatologists were able to perform endotracheal catheterization using both semi-rigid and flexible catheters at similar rates and ease, in vitro and in vivo. Due to the small sample size, minor differences may be undetected, but our results suggest that a flexible feeding tube can easily be used for minimal invasive surfactant application, with similar complication rates but lower rates of bradycardia than semirigid devices.

Immune memory and affinity maturation after vaccination in patients with propionic acidemia Introduction: In patients with propionic acidemia ( PA) all routine vaccinations of early childhood are recommended, however data on vaccine response, i. e. specific IgG concentrations and avidity maturation, after measles, mumps, rubella ( MMR) and diphtheria/tetanus vaccinations in these patients are missing. Patients and methods: Our study aimed to analyze vaccine specific IgG concentrations and avidity maturation after MMR and diphtheria/tetanus vaccination. Results: All PA patients had protective IgG concentrations for at least one tested antigen. High relative avidity indices for measles and/or rubella were measured in 6 of 10 (60 %) of PA patients according measles and/ or rubella. Discussion/conclusion: Our study revealed a sufficient immune response and memory function of the immune system in patients with PA and suggests that PA does not induce any striking effects on memory function after re-vaccinations during later life. Introduction: Osteogenesis imperfecta ( OI) is a heterogeneous genetic disorder also known as "brittle bone disease". The phenotypic spectrum varies from minimally increased fracture rates to perinatally lethal bone fragility. Affected children and adolescents face a variety of burdens throughout their daily lives. Pediatric OI patients are affected both by direct burdens of disease and numerous medical procedures during critical periods of development. The goal of this study was to characterize the quality of life ( QoL) of children and adolescents with OI using patient-and proxy reported outcome measures. As secondary aim, associations of QoL with parameters of disease affection were analyzed to identify possible QoL-modifying factors. Patients and Methods: Patients with genetically confirmed OI were included in a cross-sectional approach between May 2016 and May 2019. Patient-and proxy reported health related quality of life ( HRQoL) was assessed by using the German version of the Pediatric Quality of Life Inventory PedsQL™ to assess 4 core dimensions of health as delineated by the World Health Organization ( WHO) (physical, emotional, social and educational QoL). Anthropometric, biochemical and radiological data was analyzed retrospectively from clinical records. Results: 25 children with OI (f:m = 10:15, deforming: non-deforming type 8:17) were included. There was no difference between total QoL and individual life quality dimensions (physical, emotional, social, educational QoL) between the types of OI, sex, established bisphosphonate treatment or migration background. Regarding QoL determining factors, sitting height Z-scores exhibited a strong negative correlation with physical functioning in both children with deforming and non-deforming OI (p < 0.001). Discussion/conclusion: In this single-centre paediatric cohort, patients with deforming and non-deforming types of OI reported a comparable affection of total and specific QoL. Our data indicated that physical and social functioning of patients with OI revealed a strong association. Sitting height z-scores were a strong predictor of QoL, indicating an important role of vertebral affection on life quality. Introduction: X-linked hypophosphatemia ( XLH) is a rare metabolic bone disease which is caused by inactivating mutations in Phosphate-regulating neutral endopeptidase, X-linked ( PHEX). Due to dysregulation of Fibroblast growth factor 23 ( FGF-23), increased systemic levels of FGF-23 lead to chronic renal phosphate wasting and to impaired activation of 25OH-Vitamin D (25OHD). As a result, patients suffer from multiple musculoskeletal symptoms such as long bone deformities, short stature, bone pain, muscle weakness, dental abscesses, and fatigue.

Since data on quality of life ( QoL) in children and adolescents are sparse, this study aimed to characterize main QoL domains in a pediatric single center cohort by a well-established age-specific self-and proxy reported questionnaire. By determining baseline disease affection of XLH patients, we aimed to find relations between the surrogate markers of metabolic affection in XLH and QoL. Patients and methods: This cross-sectional, single center study included 16 (f: m = 10:6) children, adolescents, and young adults with confirmed XLH. The data-set consisted of data from 2018 and 2019. The German version of Pediatric Quality of Life Inventory (PedsQL™), a standardized questionnaire to self-assess health related QoL, was performed as part of regular clinical visits. Biochemical, anthropometric, and therapy-associated parameters were collected at the time of inclusion. Comparison with a healthy and an osteogenesis imperfecta ( OI) cohort were assessed descriptively. Correlations were investigated by calculation of Spearman coefficients. The level of statistical significance was set at p < 0.05. Results: BMI SDS and the numbers of osteotomies showed a negative correlation with physical QoL in the investigated pediatric XLH cohort ( BMI SDS: p = 0.033; osteotomies: p = 0.049). Disease specific factors such as age, alkaline phosphatase ( ALP) or the rickets severity score ( RSS) did not predict QoL. Overall, mean QoL of the investigated XLH patients was comparable to a pediatric OI cohort. Discussion/conclusion: BMI and the numbers of osteotomies are associated with physical QoL in the XLH cohort. Despite of impaired physical and educational function, the cohort showed normal scores in social function and emotional well-being. 

Nutritional rickets in a resource-rich country: the risk of alternative diets Tischlinger K.

Introduction: In resource-rich countries, nutritional rickets is a rarely seen entity in children, a condition caused by insufficient intake of calcium or vitamin D. Children born in Austria receive prophylactic oral Vitamin D through the national prevention program, since neither breast milk nor formula milk contains sufficient amounts, nor is extensive sun exposure recommended in infants. Mineralization of growth plate chondrocytes and bone osteoid requires sufficient supply with calcium and phosphate. In deficiency situations, typical growth plate disturbance occurs (rickets) and the stability and hardness of bone decreases (osteomalacia) resulting in leg bowing Patients and methods: Here we present a case of calcium deficiency rickets due to inadequate nutritional supply of milk and inconsistent vitamin D supplementation in the first years of life. A girl, 2.5 years of age, first presented to our department with motor delay. Classical signs of severe rickets were seen on radiographs and biochemically, with high alkaline phosphatase of 1320 U/l (108-317 U/l) and a parathyroid hormone of 230 pg/ ml (15-65 pg/ml). Serum calcium and phosphate remained within the reference range and 25OH Vitamin D3 at 20 ng/ml (>20 ng/ml), the typical constellation of calcium deficiency rickets. The girl had been exclusively breastfed until the age of 2.5 years of age and then put on a strict vegetarian, partly vegan diet resulting in severely reduced dietary calcium intake. In addition, the mother had not adhered to the national recommendation of infant vitamin D supplementation. Treatment of nutritional rickets consisted of oral calcium supplementation, therapeutic doses of cholecalcif-erol and a change in diet resulted in rapid laboratory, radiologic and clinical improvement. Results/discussion/conclusion: Nutritional rickets in first world countries remains rare due to strict adherence to national prevention programs and adequate nutrition, and functioning child surveillance programs conducted by community paediatricians. Nonetheless, there are well-known risk groups including immigrants, people with dark skin and those on low calcium diets. As such, the substantial morbidity in this child caused by the alternative diet (malnutrition) could have been completely avoided. This case serves as a reminder to regularly monitor adequate feeding and adherence to Vitamin D supplementation during child surveillance visits in infancy.

P02-05 Auswirkungen des Sportverhaltens auf die Stoffwechselkontrolle von Kindern und Jugendlichen mit Typ-1-Diabetes mellitus in Kärnten und der Steiermark Rader L. 1 , Jasser-Nitsche D. 2 Einleitung: Typ 1 Diabetes mellitus (T1D) ist eine der häufigsten Stoffwechselerkrankungen im Kindes-und Jugendalter. Es besteht die Evidenz, dass regelmäßige Bewegung den BMI reduziert und das Auftreten von Dyslipidämie sowie Hypertonie verringert und somit zur Prophylaxe von Spätkomplikationen des T1D beiträgt. Ob ein Zusammenhang zwischen der körperlichen Aktivität und dem HbA1c-Wert bei Typ-1-Diabetikern besteht ist jedoch noch nicht genau geklärt, da relevante Studien kontroverse Ergebnisse liefern. Diese Arbeit beschäftigt sich damit, ob sich ein positiver Effekt der körperlichen Aktivität auf die Stoffwechselkontrolle, anhand des HbA1c-Wertes, erkennen lässt. Die Kernfrage lautet, ob das Ausmaß der sportlichen Aktivität eine Senkung des HbA1c-Wertes, sowie Reduktion der Insulindosis, berechnet in IE/kg/d, mit sich bringt. Patienten und Methoden: Bei der durchgeführten Studie handelt es sich um eine Querschnittsstudie, bei welcher mittels auf Selbsteinschätzung basierter Fragebögen die Häufigkeit der Sportausübung von Kindern und Jugendlichen mit Typ-1-Diabetes mellitus erhoben wurde. Hierbei wurde die Auswirkung des Sportverhaltens auf die Stoffwechselkontrolle, gemessen anhand des HbA1c-Wertes, sowie die Insulindosis analysiert. Die Datensammlung fand im Zeitraum von Juni 2020 bis März 2021 an der Diabetesambulanz der Univ. Klink für Kinder-und Jugendheilkunde Graz und der Abteilung für Kinder-und Jugendheilkunde des LKH Villach statt. Die Studienpopulation umfasste 184 Kinder und Jugendliche mit T1D (106 männlich, 78 weiblich) im Alter von 6-18 Jahren (M: 13,22, ± 3,15). Ergebnisse: Der mittlere HbA1c-Wert in % lag in den verschiedenen Gruppen der Sporthäufigkeit bei Nie: 8,25 ± 1,75; 1-bis 3-mal pro Monat: 8,04 ±1,55; 1-bis 2-mal pro Woche: 7,65 ±1,08; 3-bis 4-mal pro Woche: 7,85 ± 1,61 und mehr als 4-mal pro Woche: 7,16 ± 0,89. Die mittleren Insulineinheiten pro Kilogramm pro Tag lagen bei Nie: 0,76 ±0,24; 1-bis 3-mal pro Monat: 0,78 ±0,23; 1-bis 2-mal pro Woche: 0,82 ± 0,27; 3-bis 4-mal pro Woche: 0,81 ±0,24 und mehr als 4-mal pro Woche: 0,68 ±0,25. Die Sporthäufigkeit hatte einen signifikanten Einfluss auf den HbA1c-Wert (p < 0,05), auch nach Adjustierung möglicher Einflussfaktoren wie Geschlecht, Alter, Therapieform, Diabetesdauer, Ausbildung und BMI-SDS. Ein Einfluss auf die Insulindosis ( IE/kg/d) ließ sich allerdings nicht erkennen (p > 0,05). Schlussfolgerung/Diskussion: Den Ergebnissen dieser Fragebogenstudie zufolge scheint die Sporthäufigkeit von Kindern und Jugendlichen mit T1D einen Einfluss auf die Stoffwechselkontrolle, gemessen am HbA1c zu haben. Ein Einfluss auf die Insulindosis ließ sich anhand der Daten jedoch nicht erkennen. Die Ergebnisse bestärken die Meinung, dass regelmäßi-ge sportliche Aktivität durchaus einen positiven Einfluss auf das Management des T1D von Kindern und Jugendlichen haben kann.

Aldosterone deficiency and resistance: The different faces of renal salt loss Laurer E., Tischlinger K., Montero-Lopez R., Wimleitner M., Hörtenhuber T., Högler W.

Introduction: Heritable conditions causing aldosterone deficiency (hypoaldosteronism) or target-organ resistance (pseudohypoaldosteronism, PHA) can lead to life-threatening salt-wasting crises in early life. Prompt evaluation and correct interpretation of aldosterone and renin are crucial to guide differential diagnosis and further testing.

Here we report on the similarities and differences of two neonates presenting with salt-wasting: Patient (P1) due to PHA type 1A and, Patient 2 (P2) due to aldosterone-synthase deficiency type 2 ( ASD). Patients and methods: Both patients were admitted in their first month of life for failure to thrive, dehydration, with features of severe hyponatremia and hyperkalemia. They had normal external genitalia and no hyperpigmentation. Whilst 17-OH-progesterone and ACTH-test were normal, renin was markedly elevated. P1 (female) had substantially elevated serum aldosterone concentrations, while P2 (male) had low-normal aldosterone. Both patients were initially treated with intravenous NaCl, salbutamol-inhalations, glucose/insulin infusions and fludrocortisone. P1 also received oral and rectal resonium, but was unresponsive to fludrocortisone. Thus, PHA type 1A was suspected in P1 and later on confirmed by genetic testing (heterozygous NR3C2 mutation: c.2306T>C). Further management for P1 consisted of oral resonium (0.3 g/kg/d) and NaCl (5.7 mmolNa/kg/d) with decreasing demands incrementally. P2 had a history of neonatal sepsis and developed an E.coli sepsis during admission. He was initially presumed to have acquired hypoaldosteronism secondary to a critical illness. He was discharged on oral NaCl only (9 mmolNa/kg/d) and followed-up at a peripheral hospital, where he was weaned off NaCl. He was readmitted at one year of age with hyponatremia and hyperkalemia and persistent failure to thrive. Reevaluation revealed elevated urinary aldosterone-precursors and a decreased serum aldosterone/renin ratio (0.05 pmol/mU; reference range 1-72 pmol/mU), which was suggestive of ASD. Fludrocortisone and oral sodium were restarted leading to prompt stabilization. Genetic testing confirmed ASD type 2 (homozygous CYP11B2 mutation: c.554C>T). Results/discussion/conclusion: Salt loss in neonates is an endocrine emergency, requiring tertiary endocrine care. While renin and aldosterone are markedly elevated in patients with PHA, as in our case, aldosterone may remain inappropriately normal in ASD type 2. Thus, an aldosterone/renin ratio < 1 pmol/mU should prompt further investigation to avoid delay in diagnosis and life-saving treatments.

P02-07 "Treatment induced neuropathy" ( TIND) -Eine seltene Komplikation bei einem Jugendlichen mit Typ-1-Diabetes Introduction: Skeletal deformities cause difficulties in diagnosis and lead to uncertainty in the becoming parents and caregivers. Bowing of the lower limbs include a heterogeneous and large group of conditions, which might seriously affect body function or could even lead to neonatal death and may be caused by genetic, endocrine and metabolic derangements. Maternal diseases, such as hypoparathyroidism, with the potential to affect the bone metabolism of the unborn child are often not taken into consideration. The current recommendation in pregnant women with hypoparathyroidism is limited to oral calcium supplementation and administration of vitamin D analogues. Patients and methods: The course of pregnancy in a 36-year-old woman with postsurgical hypoparathyroidism was uneventful until the third trimester. The woman was under regular endocrine checkups, receiving standard treatment including oral calcium and vitamin D. At 30 weeks of gestation, bowing of the long bones of the lower extremity, a femur and tibia length below the 5th centile (49.1 mm and 41.1 mm) and poor fetal growth were detected. On suspicion of skeletal dysplasia, amniocentesis was performed. Molecular genetic sequencing for skeletal dysplasia, including COL1A1/A2 genes was unrevealing. A small for gestational age female neonate (birth weight 2310 g), was born at term, and the baby was clinically completely unimpaired. On physical examination, bowing of the lower limbs was noticeable, and the legs were crossed at rest. Laboratory findings showed a highly elevated parathyroid hormone (161 ng/l in the cord blood) indicating neonatal hyperparathyroidism, but normal calcium and phosphate levels. Detailed analysis revealed maternal calcium levels below the reference range of 2.15-2.55 mmol/l during pregnancy. The girl was discharged home in good condition at day six of life receiving substitution of 800 units of vitamin D daily. The parathyroid hormone levels declined to normal within 2 months. Results: We present a case of fetal compensatory hyperparathyroidism with intrauterine hypocalcemia causing bowing of the lower limbs in a newborn. Discussion/conclusion: The case demonstrates that the management of pregnant women with hypoparathyroidism is challenging. Achieving normocalcemia is important for both mother and child. Further therapeutic options like recombinant human parathyroid hormone must be evaluated.

Fallbericht: exzessive Hyponatriämie bei einem 8 Monate alten Säugling -Addison-Krise

Zoubek P., Pauer S., Hinterleithner R., Wagentristl H.

Einleitung: Die primäre Nebenniereninsuffizienz ist durch eine Verringerung oder Ausfall der Produktion von Gluko-und meist auch von Mineralokortikoiden bei einhergehender ACTH-Erhöhung gekennzeichnet. Im Großteil der Fälle liegt der Erkrankung eine genetische, im späteren Alter auch eine Autoimmunadrenalitis zu Grunde. Jedoch können auch perinatale beidseitige NN-Blutungen oder Infektionen zu einer potenziell letalen Addison-Krise führen. Die Diagnosestellung erfolgt aufgrund der unspezifischen Symptomatik zumeist verzögert. Patienten und Methoden: Wir berichten über den Fall eines 8 Monate alten männlichen Säuglings, welcher auf der Durchreise von Rumänien in seine deutsche Heimatstadt in der Eisenstädter Kinderambulanz aufgrund rezidivierenden Erbrechens, Durchfällen und seit zwei Wochen bestehender reduzierter Nahrungsaufnahme vorgestellt wurde. Der Gewichtsverlust wurde mit 1200 g angegeben. Mit der Befundkonstellation einer Hyponatriämie von 117 mmol/ l, Hyperkaliämie von 5,7 mmol/ l, Hypochloridämie von 89 mmol/ l, Blutzucker von 75 mg/dl, einem Basendefizit von 7,4 mmol/l, einer Serumosmolalität von 230 mosmol/l und negativen Infektparametern wurde der Patient unter dem Verdacht einer viralen Gastroenteritis mit hypotoner Dehydratation aufgenommen. Das klinische Bild war darüber hinaus gekennzeichnet von Lethargie, allgemeiner Schwäche, Bewegungsarmut, reduzierter Flüssigkeitsaufnahme und Fieber bis knapp 40°. Die Ausscheidung von Natrium im Harn war deutlich gesteigert. Trotz hoher Zufuhr von parenteralen Natriumchloridinfusionen kam es nur zu einer geringfügigen Besserung der Hyponatriämie. In der Abdomensonographie zeigten sich beidseits deutlich hyperechogene, verwaschen imponierende Nebennieren. Anhand der Klinik und der Laborbefunde wurde die Verdachtsdiagnose einer Addison-Krise gestellt, die nach Einlangen des morgendlich abgenommenen Hormonstatus ( ACTH > 2000 pg/ml, Cortisol im Serum 0,9 µg/dl, Aldosteron 19 pg/ ml, Renin-Konzentration im Plasma 3021 µIU/ml) gesichert werden konnte. Unter oraler Therapie mit Hydrokortison und Fludrokortison gelang nun sehr rasch der Ausgleich des Serum Natriumgehaltes. In Folge blieb der kleine Patient afebril und es kam zu einer rasanten Besserung des Allgemeinzustandes, der Spontanmotorik und des Ess-und Trinkverhalten. Introduction: Persistent Müllerian Duct Syndrome ( PMDS) is a rare disorder of sex development (46XY DSD), characterized by the presence of müllerian derivates, uterus and Fallopian tubes in otherwise phenotypically normal males. Bi-allelic LOF mutations in AMH ( PMDS type I) or AMHR2 ( PMDS type II) genes cause this condition. In most cases, the diagnosis is made incidentally during laparoscopic orchidopexy or inguinal herniotomy. Patients and methods: A 10-day-old boy, product of a consanguineous marriage, was referred to our institution for right inguinal hernia and bilateral testicular maldescensus. During surgery, the right testicle was located inside the hernial sac and a rudimentary uterus with tubes was found. After repositioning, the testes were located in a pseudo-ovarian position. Both spermatic cords were palpable. A gonadal biopsy demonstrated normal testicular tissue. Because of normal weight gain, normal electrolytes, adrenal and sex hormones, and a normal male AMH (>46 ng/mL), PMDS was suspected. Molecular genetic testing revealed a homozygous AMHR2-Mutation c.24G>A, confirming PMDS. Results/discussion/conclusion: Resistance to anti-müllerian hormone due to mutations in its type 2 receptor (AMHR2) is a rare recessive endocrine condition. By incidentally diagnose, other forms of 46XY DSD such as congenital adrenal hyperplasia should be excluded rapidly. Infertility in PMDS is a frequent complication, both due to alteration of spermatogenesis and variations in the sperm duct. The risk of malignancy is increased. Depending on the source, 5-33 % of patients with PMDS without orchidopexy experience malignant testicular degeneration. Early orchidopexy is protective, but PMDS itself has been suggested to be a contributing factor to gonad malignancy. Malignant degeneration of müllerian derivatives has also been described, although as a much less frequent phenomenon. Treatment of PMDS is surgical and aims to increase fertility and reduce the risk of malignancy. Orchidopexy is recommended, and in the absence of conclusive published results, hysterectomy is a joint decision of the care team and family. There are currently no guidelines on the long-term follow-up for screening for testicular cancer. As such, PMDS requires management in multidisciplinary teams consisting of pediatricians, endocrinologists, surgeons, and psychologists. Introduction: Mitochondria produce cellular energy via oxidative phosphorylation ( OXPHOS), mediated by respiratory chain complexes I to IV and ATP synthase (complex V). Mitochondrial respiratory complexes have been shown to decline with age in several tissues. As the intestinal epithelium is a tissue with a high energy demand, the aim of the present study was to establish whether the expression profile of OXPHOS subunits in the intestinal mucosa changes during the aging process. Patients and methods: Biopsies of intestinal mucosa with no evidence of endoscopic or histomorphologic abnormalities, taken from 55 patients (mean age 42 years, age range 4-82 years; 62 % female), were divided into four age groups (4-19, 20-39, 40-59, ≥60 years). Sections from different intestinal segments (terminal ileum, ascending colon, and sigmoid colon/rectum) were stained immunohistochemically ( IHC) for subunits of OXPHOS complexes I-V and the voltage-dependent anion-selective channel 1 protein (VDAC1, porin), a marker of mitochondrial mass. Scores for IHC staining were determined by multiplication of the staining intensity and the percentage of positive cells. In addition, the numbers of intestinal crypts staining positive, partly positive, and negative were assessed. Results: The average protein expression levels of OXPHOS subunits increased continuously from childhood onward, peaked in persons aged 20-59 years, and declined thereafter. This was seen for complexes II to V in the terminal ileum, complexes I to V in the ascending colon, and complexes I to IV in the sigmoid colon/rectum. Across all age groups, no effect of age on expression of the porin subunit VDAC1 was detected. The number of complex I-and IV-negative crypts in different intestinal segments increased with age. Discussion/conclusion: The protein expression levels of OXPHOS complexes increases from childhood onward and declines in elderly individuals, while the numbers of crypts with partial or complete loss of expression of complexes I and IV increase continuously with age. These data suggest that the continued reductions in the levels of mitochondrial OXPHOS complexes in crypts might be compensated in adulthood, but that, ultimately, reduced expression levels occur in persons aged 60 years and older. These findings raise two important questions: first, can the process of aging could be delayed through (pharmacological) intervention of mitochondrial pathways, and second, pathophysiologically, are these findings associated with disorders of the intestinal mucosa, e. g. inflammation?

P03-02 Expression of oxidative phosphorylation complexes and mitochondrial mass in pediatric and adult inflammatory bowel disease Introduction: Inflammatory bowel disease ( IBD), which includes Crohn's disease ( CD) and ulcerative colitis ( UC), is a multifactorial intestinal disorder but its precise etiology remains elusive. As the cells of the intestinal mucosa have high energy demands, mitochondrial activity may play a role in IBD pathogenesis. The present study aimed to evaluate the expression levels of mitochondrial oxidative phosphorylation ( OXPHOS) complexes in IBD. Patients and methods: 286 intestinal biopsy samples from the terminal ileum, ascending colon and rectum from 124 patients (34 CD, 33 UC and 57 controls) were stained immunohistochemically for all five OXPHOS complexes and the voltage-dependent anion-selective channel 1 protein (VDAC1 or porin). Expression levels were compared in multivariate models including disease stage ( CD and UC compared to controls) and age (pediatric/adult). Results: Analysis of the terminal ileum of CD patients revealed a significant reduction of complex II compared to controls, and a trend to lower levels was evident for VDAC1 and the other OXPHOS complexes except complex III. A similar pattern was found in the rectum of UC patients: VDAC1, complex I, complex II, and complex IV were all significantly reduced, and complex III and V showed a trend to lower levels. Reductions were more prominent in older patients compared to pediatric patients and more marked in UC than CD. Discussion/conclusion: This in-situ investigation revealed age-and inflammation-related significant changes of energy homeostasis in the intestine of IBD patients. In summary, we found lower levels of expression of OXPHOS complexes, more pronounced in UC than CD. Most of the observed changes might be attributable to diminished mitochondrial mass, as indicated by low VDAC1 levels. In addition, we hypothesize that the pronounced downregulation of complex I in UC might have an etiology apart from reduced mitochondrial biogenesis. Therefore, complex I might be of specific pathogenic relevance for UC. Further research is needed to evaluate the impact of OXPHOS-targeted therapeutic agents such as antioxidants in IBD.

Leberzyste und pulmonale Rundherde -Zystische Echinokokkose und andere Bösartigkeiten Einleitung: Zystische Raumforderungen der Leber sind wegen vielfältiger, oft dringend therapiebedürftiger Differentialdiagnosen im klinischen Alltag eine Herausforderung. Ist radiologisch keine eindeutige Diagnose zu stellen, ist eine weiterführende Diagnostik unabdingbar. Patienten und Methoden: Ein 17-jähriger, bisher gesunder, Jugendlicher wurde aufgrund neu aufgetretener Bauchschmerzen seit einigen Wochen und deutlich palpabler Resistenz im rechten Oberbauch vorstellig. In der initialen Sonografie des Abdomens fand sich eine 8,5 × 7 × 8 cm große zystische Läsion, die im MRT bestätigt und in 1. Linie als zystische Echinokokkose interpretiert wurde. Nach Zuweisung an ein hepatologisches Zentrum ergaben sich aus der erweiterten Anamnese regelmäßige Verwandtenbesuche im Kosovo. Eindeutige radiologische Hinweise ("Wasserlilienzeichen") fehlten, dennoch wurde eine Echinokokkose mit Zystenstadium CE3/4 in Betracht gezogen. Im Thoraxröntgen zeigten sich überraschend intrapulmonale Rundherde, die sich im Thorax-CT bestätigten. Morphologisch wurden sowohl ein pneumonische Infiltrat als auch eine pulmonal-arterielle Embolie in Erwägung gezogen. Alle Ergebnisse umfangreicher Laboruntersuchungen (Inflammations-, Infektions-, Autoimmunitäts-Parameter und Tumormarker; CEA, AFP, β-hCG) blieben unauffällig. Nach interdisziplinärer Diskussion erfolgte eine thorakoskopische Biopsie eines pulmonalen Herdes. Eine Biopsie der Raumforderung in der Leber schied wegen intraoperativer Rupturgefahr aus. Makroskopisch fielen nekrotische Lungenareale auf. Während der initiale histopathologische Befund deskriptiv "akuter hämorrhagischer Lungeninfarkt mit massig myxoider schleimähnlicher Substanz" lautete, fanden sich in der weiteren Aufarbeitung maligne Zellen, die schließlich zur Diagnose eines undifferenzierten myxoiden Sarkoms führten. Eine Therapie laut CWS-Guidance für metastasierte Weichteilsarkome wurde initiiert und bei Therapieansprechen von >33 % nach 4 neoadjuvanten Chemotherapieblöcken wurde eine zentrale Resektion der Leberläsion durchgeführt. Introduction: Chronic low-grade inflammation plays a pivotal role in dysregulation of central pathways involved in lipid and glucose metabolism in obesity leading to metabolic disease. MicroRNAs (miRNAs) are known to fine-tune gene expression upstream from known regulators of meta-Abstracts bolic homeostasis. We aimed to assess the relationship of circulating miR-NAs with inflammatory modulators and metabolic disorders in pediatric obesity. Patients and methods: From a pediatric cohort with severe obesity (n = 109), clinically thoroughly characterized including diverse routine blood parameters, oral glucose tolerance test and liver MRI, a panel of 16 circulating miRNAs was quantified using qRT-PCR. Additionally, markers of inflammation TNFα, IL1 receptor antagonist, procalcitonin, CRP and IL-6 were measured. Results: We identified a strong yet unrecognized relationship of miRNAs 34a, 122 and 192 with both obesity-associated inflammation and metabolic disease. Concentrations of miRNAs 122 and 192 correlated with serum adiponectin that links adipose tissue inflammation to glucose metabolism. Several miRNA levels including miRNAs 34a, 93, 122, and 192 were statistically significantly differing between individuals with prediabetes, impaired glucose tolerance, metabolic syndrome, or non-alcoholic fatty liver disease compared and the respective controls. Additionally, miRNA 192 was significantly elevated in metabolically unhealthy obesity. Discussion/conclusion: An unfavorable miRNA pattern related to obesityassociated inflammation and comorbidities is already present in children and may be used to distinguish metabolically healthy from unhealthy pediatric patients with obesity. Moreover, these changes in epigenetic regulation could potentially be involved in the etiology of obesity-linked metabolic disease in children and adolescents.

Determinants of hyperglucagonemia in pediatric nonalcoholic fatty liver disease Introduction: Health systems are not only confronted with the growing worldwide childhood obesity epidemic, but also associated comorbidities. These subsequently cause variations in distinct metabolic pathways, such as hepatic fat accumulation, which leads to non-alcoholic fatty liver disease ( NAFLD). The aim of this evidence map is to systematically evaluate the literature and to identify research gaps on the glucagon induced amino acid turnover and its metabolic interaction with NAFLD. Patients and methods: A systematic literature search was conducted in April 2020 to identify research using three electronic databases. Two independent reviewers screened titles and abstracts, according to prespecified eligibility criteria, as well as full-text articles. Quantitative studies of either humans or animals, at all age, were included. Studies were required to contain at least two of the main research areas, being glucagon, amino acid metabolism and NAFLD. Data was independently extracted by two reviewers and summarized according to study design, publication year, comorbidities, age, sex, sample size, intervention, study duration and control group. Conflicts were resolved by discussion or a third reviewer. Results: Twenty-nine references were finally included. The publication years dated back until 1965 and showed a great increase from 2012 to 2020. All studies were carried out among adults, only 1 study focused on adolescents. The most common comorbidity was obesity. In total there were 13 animal studies and 16 human studies. The study designs in the human experiments differed immensely. The hyperinsulinemic-euglycemic clamp and the oral glucose tolerance test were the most used experimental methods to evaluate metabolic changes. Thirteen studies focused on metabolic effects due to NAFLD, however only 2 studies focused on the interaction of NAFLD, glucagon and the amino acid metabolism, both being non-human studies. The other 14 studies focused on metabolomics, beta cell function or just on one topic of the research area and not as an interaction on one another. Discussion/conclusion: Research on the interaction of NAFLD, glucagon and the amino acid metabolisms in human studies is sparse and completely lacking in pediatrics. Furthermore, longitudinal studies such as studies focusing on hyperglucagonemia independent of diabetes but related to NAFLD present an unambiguous research gap.

Parents' perspectives on prevention and therapy of obesity in children and adolescents Geiersberger S., Weghuber D.

Universitätsklinik für Kinder-und Jugendheilkunde/Uniklinikum Salzburg-Landeskrankenhaus/Pädiatrische Gastroenterologie, Hepatologie und Ernährung/Uniklinikum der Paracelsus Medizinischen Privatuniversität Introduction: The prevalence of overweight and obesity in children and adolescents has increased in recent years. The perspectives of parents can provide deep insights into the living environment and lifestyle of the family. This enables a further development of the preventive and therapeutic measures. The aim of this study was to identify the parents' perceptions regarding prevention and therapy in children and adolescents. Patients and methods: As part of a mixed method, a survey based on standardized questions was distributed to parents (N = 81) at the same time when guided interviews with parents (N = 4) of children and adolescents with obesity were performed. For the quantitative part parents were recruited via quota sample. Parents of children and adolescents who had already completed a weight management program participated in the qualitative interviews. The data of the survey were analysed by using SPSS, the guided interviews were transcribed and thematically analysed according to Kuckartz. Results: Parents think that preventive measures should increase health promoting behavior and decrease unhealthy behavior in their children and adolescents. Parents identify the high consumption of media devices, the low cost of unhealthy food, inadequate physical education in schools and food advertisements as the main barriers in the living environment. Parents see themselves in a role model as well as in the role of a motivator towards their children and adolescents with the aim of changing the family's lifestyle. A continuous support through the interdisciplinary team is convenient. Discussion/conclusion: The results provide multiple insights into the perspective of parents according to prevention and treatment of children and adolescents with obesity. In cooperation with an interdisciplinary health team the Advanced Practice Nurse could support as well as advise parents and address their needs. Further research is required to guarantee an ongoing effect in the prevention and treatment of obesity in children and adolescents. Das Event-free survival nach 5 Jahren lag bei 89 ± 5 %, nach 10 Jahren bei 80 ± 7 %. Dreißig Patient* innen (57,7 %) entwickelten im Verlauf tumor-oder therapiebedingte Spätfolgen (schwere Spätfolgen, n = 6). Diese betrafen Gehör (n = 14), kardiovaskuläres System (n = 8), Leberfunktion (n = 7), Nierenfunktion (n = 6), Harn-/Stuhlverhalten (n = 4): Vier Patient*innen waren durch eine anhaltende Schmerzsymptomatik beeinträchtigt. Bei 4 Patient* innen kam es im Verlauf zu psychischen Problemen. Acht Patient* innen hatten zum Zeitpunkt der Untersuchung Fertilitätsprobleme oder gaben einen unerfüllten Kinderwunsch an. Die objektiv erhobenen Befunde führten in den meisten Fällen nicht zu einer Beeinträchtigung der subjektiven Lebensqualität. Introduction: β-thalassemia is known as an autosomal recessive disease with a high prevalence in malaria endemic regions. Heterozygous carriers, mainly have a clinically asymptomatic phenotype. However, already in 1973 Weatherall described a type of β-thalassemia inherited in an autosomal dominant ( AD) manner. The pathology underlying AD β-thalassemia is related to a highly unstable β-chain variant in the heterozygous state. These unstable β globin variants precipitate in erythroid precursors causing ineffective erythropoiesis and are almost undetectable in hemoglobin (Hb) electrophoresis. AD β-thalassemia is characterized by moderate anemia with splenomegaly (thalassemia intermedia phenotype) and does not show a distinct geographic distribution. Patients and methods: In three members of a Polish family, who clinically had a chronic microcytic anemia (Hb 6-9 g/dl; MCV 69-74 fl, reticulocytes 1-3 %) not caused by iron deficiency, Hb analyses via HPLC and Sanger sequencing of the β-globin gene ( HBB) were performed. Clinical pertinent data (transfusion requirements, growth, development, etc.) were analyzed retrospectively. Results: In Hb analysis no abnormal chains were detected, but HbA2 (3.5-4.6 %) and HbF (6.6-12.4 %) levels were elevated. Sequencing of HBB revealed a novel deletion of one nucleotide(-C) in exon 3 at position 374 causing a frameshift in the coding DNA sequence in all 3 affected family members. The deletion results in a variant β-globin which gives rise to a modified amino acid sequence of 20 hydrophobic residues at the carboxyterminus. This mutation leads to instability of the elongated β-globin. Together with an excess of alpha globin chains, which derives from the inability of the β-chains to form tetramers, these β-globins precipitate causing β-thalassemia intermedia in the heterozygous state. While the children (male, 5 years; female, 9 years) never required blood transfusions, the father (male, 38 years) received approximately 10 blood transfusions during puberty. The children have splenomegaly, but normal growth and development. Discussion/conclusion: In undiagnosed cases of chronic microcytic anemia not caused by iron deficiency, a genetic sequencing of HBB should be considered, even though the patients' origins are not suggesting β-thalassemia. Background: Pyomyositis, usually considered tropical disease, is increasingly common in non-tropical countries. Methods: Description of diagnosis, management and therapy of three almost simultaneous cases in Austria. Results: Pyomyositis was diagnosed by MRI and abscess aspiration. All three infections were due to methicillin sensitive Staphylococcus Aureus. Under appropriate prolonged antibiotic therapy, all three patients had a complete resolution. Conclusion: Pyomyositis may be added to the differential diagnosis of febrile children with unexplained pain in the musculoskeletal system even in non-tropical countries. Die Erziehungsberechtigten wurden schriftlich zur Teilnahme an einer Befragung mittels standardisiertem Online-Fragebogen eingeladen und danach telefonisch kontaktiert sofern die Online-Beantwortung nicht durchgeführt wurde. Ergebnisse: Wir präsentieren die ersten Ergebnisse von derzeit 778 Befragten von der 1267 Kinder im Alter von 0-14 Jahre umfassenden Stichprobe (Rücklaufanteil 61,4 %). Bei insgesamt 108 (13,9 %) Kindern bestanden vier Wochen nach der SARS-CoV-2 Infektion noch Beschwerden, die als mit der Erkrankung in Zusammenhang stehend wahrgenommen wurden. Bei 60 (7,7 %) Kindern waren nach mindestens 3 Monaten noch Symptome vorhanden (0-4 a: 5,1 %; 5-9 a: 3,5 %; 10-14 a: 10,7 %). Am häufigsten waren dies Müdigkeit (2,4 % der Befragten), Störungen des Geruchs-und/oder Geschmacksinns (1,8 %) und Konzentrationsschwäche (1,7 %). Bei 12 Kindern (1,5 %) bestand nach mindestens drei Monaten noch Kurzatmigkeit und bei 9 (1,2 %) Kindern mangelnde körperliche Belastbarkeit. Im Vergleich zu Kindern ohne LZB waren Kinder mit LZB älter (medianes Alter 10 vs. 12 Jahre); die Geschlechtsverteilung bei beiden Gruppen war vergleichbar. Schlussfolgerung/Diskussion: Bei fast 14 % bzw. 8 % der Befragten wurde einen Monat bzw. 3 Monate nach der SARS-CoV-2 Infektion weiterhin Symptome beobachtet. Mangelnde körperliche Belastbarkeit, welche bei betroffenen Erwachsenen oft als typisches Symptom von "Long COVID" berichtet wird, wurde nach 3 Monaten lediglich bei knapp über 1 % der Kinder beobachtet. Diese Studie beruht auf Angaben der Erziehungsberechtigten. Es ist nicht auszuschließen, dass einige der beobachteten Symptome auch andere Ursachen hatten und Kollateralfolgen der Pandemiekontrollmaßnahmen ebenfalls eine Rolle gespielt haben. identifying key demographical factors such as age, sex, race, height and weight in order to identify any common features in these patients. For the purpose of this review we have included patients with at least one undetectable viral load (Abbott m2000) while they are not on antiretroviral treatment. Results: In our cohort of 88 HIV 1 infected children and young people seen in our Family Clinic in North Manchester General Hospital 4 patients (5 %) were identified as EC including 3 females and a male all of black African descent. The patients' ages ranged between 11-16 years with a mean age at diagnosis of 7-8 years. None have currently initiated antiretroviral therapy. All patients demonstrated detectable viraemia before undetectable viral loads were achieved over time periods ranging from 7-26 months. Of our 4 patients 2 remain undetectable at the last available reading. Whilst CD4 counts varied considerably between patients (484-1051 × 106/L at the initial measurement) each showed a progressive decline over the recorded time period despite absent plasma viraemia. The patient with the highest CD4 counts (averaging 973 × 106/L over seven years) demonstrated the longest period of undetectable viral load. Discussion/conclusion: Within our cohort of HIV infected children and young people we have identified a subgroup of 4 patients (5 %) within those who are long term non-progressors, who manage to clear plasma viraemia during the observation period. This rare phenomenon has been investigated and various explanations will be discussed. It has been postulated that these patients may hold the key for a possible HIV Cure. Das mediane Alter der positiv getesteten Kinder und Jugendlichen war im Primärversorgungszentrum 2,1 Jahre ( IQR 0,4 bis 3,0 Jahre), und im Tertiärzentrum 1,6 Jahre ( IQR 0,5 bis 9,5 Jahre). Neun Tests waren positiv; in den anschließenden PCR-Tests zeigten sich CT-Werte unter 30. Die Positivitätsrate im pädiatrischen Primärversorgungszentrum betrug 2,2 %. Die Univ.-Klinik für Kinder-und Jugendheilkunde testete im selben Zeitraum bei 1445 Patient* innen 5,0 % der Fälle positiv auf SARS-CoV-2. Dies entspricht einem statistisch signifikanten Unterschied in der Positivitätsrate (p = 0,01). 

The spectrum of left ventricular hypoplasia is broad. It varies from mild left ventricular hypoplasia to the full extent of hypoplastic left heart syndrome. The evaluation of the degree of hypoplasia regarding the left-sided heart structures is very challenging, especially during fetal life. The aim of this study was to determine fetal echocardiographic factors that have prognostic value for the postnatal clinical outcome, including the type of repair (uni-or biventricular). Patients and methods: Monocentric retrospective data analysis of fetal echos from 2008 till 2020. We included all fetuses with borderline left-sided heart structures. Fetuses with antrioventricular septal defects were excluded. Primary outcome variable was the need for postnatal intervention (atrioseptostomy, catheterization and/or surgery), secondary outcome was the uni-or biventricular repair. Maternal hyperoxygenation was used in 4 cases. Results: 46 fetuses with borderline left ventricles were included in our study. Measurements of fetal left ventricular and aortic arch parameters were obtained. Additionally, pulsed doppler pattern of pulmonary venous flow and size of the foramen ovale were included. A logistic regression was performed to evaluate factors associated with early intervention or biventricular repair. There was no specific correlation between any of the parameters and the postnatal outcome. From the 46 included patients 31 ended up having a biventricular circulation and 15 received univentricular palliation. In our study we also describe 5 patients with a primary univentricular repair that-after staged left ventricular recruitment procedureunderwent a biventricular conversion. 1 out of the 4 cases that received maternal hyperoxygenation showed an effect. Discussion/conclusion: Fetuses with a borderline left ventricle have a reasonably good outcome. The need for early neonatal intervention can not always be predicted by fetal echocardiography. As has already been demonstrated in preceding studies, pulmonary vein flow pattern may be a useful predictor of the degree of left atrial hypertension and hence outcome of the fetuses with borderline left heart structures, especially in cases with poor visualization of the foramen ovale. The optimization of the predictive value of fetal echocardiography not only helps in parent counseling and planning of perinatal management, but may also help improve the field of fetal intervention. Introduction: Fetal counselling in complex congenital heart disease can be a challenging task. Congenital corrected transposition (ccTGA) is a rare cardiac disease and outcome can vary from surgical/intervention free survival to early heart transplant. The objective of the study was to evaluate ccTGA cases in a tertiary referral center to help counselling parents. Patients and methods: Retrospective study of cases with ccTGA without complex cardiac disease diagnosed between 1984 and 2019 in a tertiary referral center. Results: The search identified 32 patients in the database of which 18 met the inclusion criteria. (ccTGA with two patent AV valves). 7/18 (39 %) children were diagnosed prenatally. Associated cardiac lesions were ventricular septal defects in 94 % (17/18), Ebstein's anomaly in 27 % (5/18), coarctation in one child, and pulmonary stenosis of varying degree in 6 children. Primary higher degree heart block was noted in 3 neonates of which all had a pacemaker implantation in the first 4 weeks of life. A pulmonary artery banding was necessary in 7 children. 1 child received a ductal stent. Double Switch and subsequently pacemaker implantation was performed in 5 cases. 2 children underwent a heart transplantation at the age of 4 years and 13 years. 5/18 (27 %) children didn't have any intervention so far. The overall survival in our study is 100 %. Discussion/conclusion: Our data suggest that fetal counselling must be guarded. However our data give a better outlook than recently described in the literature. Morbidity is very much dependent on associated cardiac lesions and the presence of primary rhythm disturbances.

Prävalenz und klinischer Verlauf der peripartalen Kardiomyopathie ( PPCM) in der Steiermark Gollmer J.

Medizinische Universität Graz, Abteilung für Kardiologie, Graz, Österreich Einleitung: Die peripartale Kardiomyopathie ( PPCM) ist eine seltene Form der akuten Herzinsuffizienz unklarer Ätiologie. Das Spektrum der klinischen Präsentation ist breit und umfasst leichte Verläufe mit vollständiger Erholung ebenso, wie schwere Verläufe mit hoher Mortalität. Da typische Symptome wie Luftnot und periphere Ödeme auch ohne PPCM in der Schwangerschaft vorkommen können, besteht die Gefahr der Unterdiagnostizierung. Die Prävalenz der PPCM scheint regional stark zu variieren und ist von 1:100 Geburten in Nigeria bis hin zu 1:4.000 Geburten in den USA beschrieben. Ziel der Studie war die Untersuchung der Prävalenz und des klinischen Verlaufs der PPCM in der Steiermark. Patienten und Methoden: Das Krankenhausinformationssystem der Krankenhausgesellschaft der Steiermark beinhaltet die Krankengeschichten von über 85 % der akuten Hospitalisierungen der Steiermark (ca. Patienten und Methoden: Fallbericht: Wir berichten über einen 36-jährigen Patienten mit Fontan-Zirkulation unserer EMAH(Erwachsene mit angeborenen Herzfehlern)-Ambulanz, welcher sich aufgrund von seit einer Woche bestehenden Bluthustens und eines verminderten SpO2 um 80 % (normalerweise 90 % in der Heimmessung) in der Notfallambulanz vorstellte. Auskultatorisch imponierte die Lunge beidseits unauffällig. Es bestand kein blutiger Harn oder Stuhl und kein Fieber. Im Labor zeigte sich nur ein geringgradig erhöhtes CRP um 14 mg/L bei einem Hämoglobin um 17,8 g/dL. Trotz bestehender Antikoagulation mit Acenocoumarol lag die INR mit 1,5 in der Norm. In der arteriellen Blutgasanalyse war der pO2 auf 47 mmHg reduziert. Bezüglich der Langzeitanamnese wurde ein Jahr zuvor interventionell eine aortopulmonale Kollaterale verschlossen. In einer CT-Angiographie des Thorax zeigten sich lediglich kleine intrapulmonale arteriovenöse Fisteln im Mediastinum. Auffällig waren jedoch mehrere Kavernen mit Kommunikation zum Bronchialsystem insbesondere im Bereich des rechten Ober-und Mittellappens. In einer daraufhin mittels Bronchoskopie gewonnenen Sputumkultur konnte eine Infektion mit Mycobacterium kansasii nachgewiesen werden und somit die Diagnose einer Tuberkulose gestellt werden. Eine Therapie mit Ethambutol-Hydrochlorid, Rifampicin und Clarithromycin wurde etabliert, die Therapie mit Acenocoumarol pausiert und durch ein niedermolekulares Heparin ersetzt. Aufgrund des ausgeprägten Lokalbefundes im Bereich des rechten Oberlappens wäre eine Lobektomie indiziert gewesen. Von einem operativen Eingriff musste aufgrund eines drohenden sog. Fontan-Failure Abstand genommen werden. Die eingeleitete perorale Therapie wurde für insgesamt 18 Monate durchgeführt, worunter die Hämoptysen letztendlich sistierten. Ergebnisse/Schlussfolgerung/Diskussion: Obwohl Hämoptysen bei Pa-tientInnen mit Fontan-Zirkulation zumeist durch aortopulmonale Kollateralen bedingt sind, bleibt die offene Tuberkulose eine wichtige Differentialdiagnose. Dieser Fallbericht eines 36-jährigen EMAH-Patienten mit Fontan-Zirkulation verdeutlicht die Komplexität, aber auch die Schwierigkeiten, in der Diagnostik, Therapie und Nachsorge von PatientInnen mit angeborenen Herzfehlern. Interdisziplinarität ist daher für eine optimale Behandlung dieser sensiblen PatientInnengruppe essentiell. QTc-Zeit wird damit nicht erreicht. Überlegungen bzgl. einer Verödung des linksseitigen Gangion stellatum bzw. Implantation eines ICD finden statt. In Anbetracht der fraglichen Effektivität der medikamentösen Therapie und der Ganglion Stellatum Verödung sowie des hohen Risikos einer malignen Arrhythmie und eines SCD bereits im Säuglingsalter, wird bei dem Patienten im Alter von 6 Monaten ein ICD implantiert. Schlussfolgerung/Diskussion: Calmodulinopathien sind seltene lebensbedrohliche Arrhythmien, die bereits im frühen Säuglingsalter tödlich verlaufen können. Insbesondere die alleinige medikamentöse Therapie ist offensichtlich nicht immer ausreichend. Diese Tatsache hat uns zur frühzeitigen Implantation eines ICD bewogen. P06-08 "Holiday heart syndrome" -Eine Rhythmusstörung im Trend?

Nößler N., Kurath-Koller S., Schweintzger S., Köstenberger M., Meinel K., Burmas A., Grangl G., Gamillscheg A.

Klinische Abteilung für Pädiatrische Kardiologie, Univ. Klinik für Kinder-und Jugendheilkunde, Medizinische Universität Graz Einleitung: Während die Vorhofflimmerarrhythmie ( VHFA) im Erwachsenenalter die häufigste Arrhythmie darstellt, ist sie im Kindes-und Jugend-alter eine Rarität. Im Kindesalter wird die VHFA meist sekundär im Rahmen von kongenitalen Herzfehlern oder postoperativ bei myokardialer Narbenbildung nach korrigierender Herz-Operation beobachtet. Über eine isolierte VFHA ohne organische Ursache in dieser Alterskategorie existieren nur wenige Fallberichte. Patienten und Methoden: Wir berichten über einen 16-jährigen Patienten, welcher sich mit Thoraxschmerzen und Palpitationen präsentiert. Weitere Symptome wie Atemnot oder Fieber zeigen sich nicht und ein Trauma oder eine rezente Infektion können anamnestisch ausgeschlossen werden. Der Patient berichtet jedoch über ausgiebigen Alkoholkonsum in der Nacht vor Beginn der Symptomatik. Das 12-Kanal EKG zeigt eine VHFA mit einer durchschnittlichen Frequenz um 160 Schlägen pro Minute. Echokardiographisch findet sich eine gute biventrikuläre Funktion bei normaler kardialer Anatomie. Laborchemisch sind alle kardialen Parameter bis auf ein erhöhtes BNP (brain naturetic peptide) von 350 pg/ml i. e. L. im Sinne einer anhaltenden Tachykardie unauffällig. Durch synchronisierte elektrische Kardioversion (100 J, entsprechend 1 J/kg) gelingt die Konversion in einen normofrequenten Sinusrhythmus. Auf eine transösophageale Echokardiographie bzw. Antikoagulation wurde aufgrund der kurzen Anamnese verzichtet. Ergebnisse: Die Prävalenz einer isolierten VHFA vor dem 30. Lebensjahr wird in der Literatur mit 0,05 % angegeben. Während ältere Patienten meist asymptomatisch sind, berichten junge Patienten häufig über Thoraxschmerzen und Palpitationen. Als Risikofaktoren für die isolierte VHFA werden Alkoholkonsum, Adipositas und exzessiver Ausdauersport diskutiert. Wichtig bei Vorliegen einer isolierten VHFA ist der Ausschluss einer zugrunde liegenden Erkrankung, wie z. B. Brugada-Syndrom, Long-QT Syndrom oder Kardiomyopathie. Das Holiday Heart Syndrom wurde erstmalig 1978 definiert und beschreibt das Auftreten einer VHFA im Zusammenhang mit Alkoholkonsum bei ansonsten unauffälliger Diagnostik. Hierbei ist bislang keine Schwellendosis des Alkoholkonsums definiert. Das Holiday Heart Syndrom kann individuell unterschiedlich im Rahmen von chronischem Alkoholkonsum oder auch nach Binge-Drinking auftreten. Schlussfolgerung/Diskussion: Bei Jugendlichen mit der seltenen Diagnose einer VHFA muss bei nicht fassbarer organischer Ursache differentialdiagnostisch an ein Holiday Heart Syndrom gedacht werden. Alleinig die absolute Alkoholkarenz gilt als präventive Maßnahme.

Cardiac resynchronization therapy in an infant with heart failure due to mechanical dyssynchrony after right ventricular pacing Karas T., Prandstetter C., Tulzer G.

Introduction: Cardiac resynchronization therapy is a well established therapeutic option in the adult patient population. It is commonly used to improve the clinical outcome in adult patients with poor left ventricular ejection fraction ( LVEF < 35 %), a prolonged QRS duration (> 150 ms) and patients with NYHA Functional Class III and IV who remain symptomatic despite stable and optimized pharmacotherapy. The underlying causes for electric activation delay between different ventricular wall segments are usually either a bundle branch block pattern or conventional right ventricular pacing. The pediatric patient population is much more heterogenous and recommendations for CRT are less well defined. Patients and methods: We report the case of a 4-year-old infant with congenital heart disease after correction of a complete atrioventricular canal and mitral valve replacement who required permanent pacemaker treatment because of consecutive post-operative atrioventricular block Type III. During the follow up the patient developed progressive and dilated cardiomyopathy due to chronic right ventricular pacing.

After installing CRT the patient showed significant improvement in echocardiographic assessment with stable clinical conditions following discharge and during follow up. Discussion/conclusion: Permanent right ventricular pacing is a possible cause for dilated cardiomyopathy in postoperative atrioventricular block or congenital heart block in children as well as in adult patients. The attempt of correct positioning of the ventricular leads during pacemaker implantation can reduce the risk for development of DCM due to mechanical dysynchrony caused by right ventricular pacing. Comparable to the adult patient population CRT shows to be an effective therapeutic treatment option in children as well. . Die gemessenen relativen Änderungen des Totalen Hämoglobins wurden in CBV umgerechnet, und der zerebrale Gewebsoxygenierungsindex (cTOI) erhoben. Zusätzlich wurden mittels Pulsoxymetrie Herzfrequenz ( HR) und arterielle Sauerstoffsättigung (SpO2) ermittelt. Zwei Gruppen, basierend auf dem Geburtsmodus, wurden verglichen: Vaginalgeburt ( VG) und Sectio caesarea ( SC). Der Zeitpunkt der Abnabelung unterschied sich entsprechend dem routinemäßigen Vorgehen an unserem Zentrum: Etwa 60 s nach der Geburt in der der VG-Gruppe, <30 s in der SC-Gruppe. Ergebnisse: CBV: In der gesamten Studienpopulation zeigte sich ein medianer Abfall des CBV von 0,82 ml/100 g Gehirngewebe in den ersten 15 Lebensminuten (p = 0,006). In der VG-Gruppe zeigte sich ein stärkerer CBV-Abfall im Vergleich zur SC-Gruppe, wobei statistisch signifikante Unterschiede (p < 0,05) zwischen den Gruppen in Lebensminute 2, 5 und 13 nachgewiesen werden konnten. cTOI, HR, SpO2: Im Verlauf unterschieden sich cTOI, HR und SpO2 der beiden Gruppen. Beim cTOI zeigten sich signifikante Unterschiede mit niedrigeren Werten in Minute 2 und höheren Werten in Minute 4 und 5 in der VG-Gruppe im Vergleich zu SC-Gruppe. Die VG-Gruppe zeigte eine signifikant höhere HR bis Minute 4, und signifikant höhere SpO2 bis Minute 5. Schlussfolgerung/Diskussion: Der schnellere cTOI-, HR-und SpO2-Anstieg bei VG-Neugeborenen verglichen mit SC-Neugeborenen könnte durch verhältnismäßig schnellere Zunahme der "Oxygen delivery" konsekutiv zu einer stärkeren zerebralen Vasokonstriktion und somit stärkeren Abnahme des CBV führen. 

Einfluss von "physiological-based cord clamping" ( PBCC) auf die kardiorespiratorische Stabilität von Frühgeborenen innerhalb der ersten 24 h nach Geburt Wolfsberger C., Höller N., Prethaler E., Pichler G., Urlesberger B.

Klinische Abteilung für Neonatologie, Universitätsklinik für Kinder-und Jugendheilkunde, Medizinische Universität Graz Einleitung: In rezenten Studien konnte gezeigt werden, dass physiological based cord clamping ( PBCC) mit einer stabileren kardiozirkulatori-schen Adaptation im Rahmen der postnatalen Erstversorgung assoziiert ist. Das Ziel der Studie war es den Einfluss von PBCC auf die kardiorespiratorische Stabilität innerhalb der ersten 15 Lebensminuten und der ersten 24 Lebensstunden zu untersuchen. Patienten und Methoden: Frühgeborene mit einem Gestationsalter ( GA) <32 Wochen und einem Geburtsgewicht ( GG) von <1500 g wurden in diese retrospektive Analyse inkludiert. Frühgeborene, die seit Juni 2020 mit PBCC an der NICU Graz erstversorgt wurden ( PBCC-Gruppe), wurden 1:1 mit Frühgeborenen der Kontrollgruppe (Abnabelung nach 30 s), die zwischen 2014 und 2021 an der NICU Graz erstversorgt wurden, gemäß ihres GA (± 1 Woche) und ihres GG (± 100 g) gematcht. In der PBCC-Gruppe erfolgte die initiale Versorgung, auf einem mobilen, gewärmten Erstversorgungstisch, an der intakten Nabelschnur, entsprechend den lokalen Richtlinien wie in der Kontrollgruppe. Routine-Monitoringparameter (Herzfrequenz, Sauerstoffsättigung, Atemfrequenz, mittlerer arterieller Blutdruck) wurden jede Minute innerhalb der ersten 15 min sowie jede Stunde innerhalb der ersten 24 h verglichen. Zusätzlich wurde die jeweils erste postnatale Blutgasanalyse verglichen. Ergebnisse: 44 Frühgeborene wurden inkludiert ( PBCC n = 22; Kontrollgruppe n = 22). Medianes GA und GG der PBCC-Gruppe betrugen 28 (26-29) Wochen und 917 (700-996) g und in der Kontrollgruppe 28 (26-29) Wochen und 880 (739-1078) g. In der ersten postnatal durchgeführten Blutgasanalyse [Abnahmezeit PBCC-Gruppe Median ( IQR): 90 (60-120) min; Kontrollgruppe: 100 (90-120) min] zeigte sich ein höherer pO2-Wert in der PBCC-Gruppe [69,0 (46,9-74,5) mmHg] verglichen mit der Kontrollgruppe [47,2 (41,7-59,0) mmHg] (p = 0,04*), ohne signifikanten Unterschied in der verabreichten FiO2-Konzentration. In Hinblick auf Monitoringparameter konnten keine weiteren signifikanten Unterschiede zwischen den beiden Gruppen beobachtet werden. Schlussfolgerung/Diskussion: In dieser Studie konnten bei PBCC in Kombination mit einem mobilen, gewärmten Erstversorgungstisch keine Unterschiede in Routine-Montoringparameter sowohl im Rahmen der Erstversorgung, als auch innerhalb der ersten 24 Lebensstunden beobachtet werden. Jedoch zeigte sich in der PBCC-Gruppe ein höherer pO2 in der ersten Blutgasanalyse, hinweisend auf eine bessere Oxygenierung.

Einfluss von pCO 2 auf die zerebrale Oxygenierung bei stabilen Früh-und Reifgeborenen 15 min nach Geburt Wolfsberger C., Bruckner M., Schwaberger B., Mileder L., Urlesberger B., Pichler G. Introduction: Neonatal managements are a special focus of academic discussion, leading to mostly inconsistent results about what is best for the preterm infant. Therefore, national and international guidelines on the care of these infants often do not commit themselves to stringent recommendations. There is a need for evidence-based knowledge regarding the use of certain managements in extreme prematurity. The aim of this study was to assess the mode of delivery, surfactant administration, intubation for surfactant administration and the initial form of mechanical ventilation of preterm infants in Germany, Austria and Switzerland, in 2018, and to compare this data. Patients and methods: An online-based survey, containing eight questions, was sent to neonatologists of perinatal centers in Germany, Austria and Switzerland, taking care of preterm infants < 32 gestational weeks ( GW). Preterm infants were classified into the subgroups "26-31 GW", and "< 26 GW". In country and group comparisons, the rates of cesarean section, surfactant administration, intubation for surfactant administration and the initial ventilatory setting were analyzed. Results: The study included 2,051 German, 548 Austrian and 676 Swiss preterm infants, distributed to 46 perinatal centers. Austria (89.61 %) has significant more cesarean section rates for the 26-31 GW subgroup than Germany (78.30 %) and Switzerland (81.61 %). Germany (85.99 %) and Austria (80.33 %) have significant more cesarean section rates than Switzerland (60.95 %) for the < 26 GW subgroup. Germany (48.90 %; 90.66 %) and Austria (49.78 %; 95.08 %) both have significant more surfactant administration rates for both subgroups than Switzerland (42.03 %; 74.29 %). Intubation rates for surfactant administration are significantly different between all three countries for the subgroup < 26 GW (Austria: 20.65 %; Germany: 68.18 %; Switzerland 100 %). National differences in the use of these managements exist. The initial form of mechanical ventilation varied from Synchronized Intermittent Mandatory Ventilation ( SIMV) in Germany (57.89 %) and Synchronized Intermittent Positive Pressure Ventilation ( SIPPV) with Volume Guarantee ( VG) in Austria (42.86 %) to SIMV with VG and SIPPV with VG in Switzerland (both 40 %). Discussion/conclusion: The mode of delivery, surfactant administration, intubation for surfactant administration and the initial form of ventilation vary between the three countries and within the countries themselves, despite an identical state of scientific knowledge. 

Hypoxic-ischaemic encephalopathy-microRNA expression profiles as a prognostic marker for neurological outcome Patients and methods: This study is a retrospective analysis of prospectively enrolled subjects admitted to NICU Innsbruck between January 2017 and December 2018. The patient group included asphyxiated newborn infants with a gestational age of ≥36 weeks fulfilling the diagnostic criteria of moderate/severe HIE according to Sarnat & Sarnat. Subjects routinely underwent neurological follow-up by means of neuropaediatric examination, general movement assessment and Bayley Scales of Infant Development III® during their first year of life. Healthy full-term infants served as controls. 48 h after birth, dried blood spots were collected from venous blood. Samples were analysed by means of the FirePlexTM particle technology for multiplex miRNA assay (Abcam FirePlex Service Lab, Cambridge, USA). Results: MiRNA expression profiles were analysed from 42 neonates from the following groups: healthy controls (n = 34), asphyxiated infants suffering from moderate/severe HIE with good neurological outcome (n = 6), and asphyxiated infants suffering from moderate/severe HIE with poor neurological outcome (n = 2). Infants with poor neurological outcome showed a significant decrease of hsa-mir-125b-5p and hsa-mir-16-2-3p, and a significant increase of hsa-mir-24-3p and hsa-mir-532-5p in comparison to healthy controls. Furthermore, they showed a significant increase of hsa-mir-145-5p compared to infants suffering from moderate/ severe HIE with good neurological outcome (Mann-Whitney U test, significance Bonferroni-corrected: all p < 0.05). Discussion/conclusion: Infants with poor neurological outcome following HIE show alterations in miRNA expression profiles in comparison to case infants with good neurological outcome and healthy controls. Hence, miRNA expression profiling is a promising novel approach for predicting, within a few days of birth, the neurodevelopmental outcome of infants suffering from HIE and may facilitate clinical management and counselling of parents. Additional studies are required.

The effect of DHEA and DHEA-S on activated-caspase-3 apoptotic cell death in the healthy and injured neonatal mouse brain Saurer J., Bergerweiß S., Urbanek M., Huber E., Kiechl-Kohlendorfer U., Griesmaier E., Posod A.

Medizinische Universität Innsbruck/Department für Pädiatrie II Introduction: Neonatal hypoxic-ischemic encephalopathy ( HIE), a consequence of perinatal asphyxia, still poses a therapeutic challenge for neonatologists. A promising treatment option are the hormone dehydroepiandrosterone ( DHEA) and its sulfate ester DHEA-S, due to their neuroprotective properties. This work evaluates a potential protective effect of DHEA and DHEA-S in neonatal mouse pups suffering from hypoxic-ischemic brain injury. Patients and methods: This experimental study examined activated caspase-3-dependent apoptosis in the brain of 87 mouse pups. In a first step, a toxicity study in healthy animals was conducted. Thirty-nine mouse pups were randomly assigned to one of the following treatment groups. Every individual received a single intraperitoneal injection of either PBS (phosphate-buffered saline, control), PBS/ DMSO (dimethyl sulfoxide, "solute control"), different concentrations of DHEA (0.1 µg, 1 µg or 10 µg per g bodyweight), or different concentrations of DHEA-S (0.1 µg, 1 µg or 10 µg per g bodyweight). In a second step, 48 animals were subjected to hypoxic-ischemic brain injury. Therefore, the right common carotid artery was ligated and cut, and the study animals were placed in a hypoxic chamber filled with 8 % oxygen and 92 % nitrogen for 17 min. Subsequently, they were randomly assigned to the above-mentioned treatment groups. Mouse brains were histologically processed and immunohistochemically stained. Coronal brain sections were assessed via light microscopy and caspase-3 positive cells quantified in various brain regions (grey matter, white matter, hippocampus, striatum, thalamus, hypothalamus).

In the toxicity study, significantly lower numbers of activated caspase-3-positive cells were found in animals treated with DHEA 10 µg/g bodyweight in grey matter (p = 0.036, vs. PBS). No other statistically significant difference was detected. In animals subjected to hypoxic-ischemic brain injury, DHEA and DHEA-S did not affect caspase-3 activation. No sex-specific differences were detected. Discussion/conclusion: In the toxicity study, 10 µg/g bodyweight DHEA seems to reduce neuronal apoptosis in grey matter compared to controls. Since this result was only found in one region of grey matter, the clinical relevance of this finding is uncertain. Neonatal mouse pups with hypoxic-ischemic brain injury did not benefit from a single intraperitoneal injection of various concentrations of DHEA or DHEA-S. Further studies are required.

When synchronized isn't synchronous-an experimental benchmarking study on the efficiency of SIMV in verylow-birth weight premature infants Auer-Hackenberg L., Haselmann C., Brandner J., Hofstätter E., Stroicz P., Wald M.

Universitätsklinik für Kinder-und Jugendheilkunde, Uniklinikum Salzburg/Landeskrankenhaus, Division für Neonatologie Introduction: Synchronized ventilation is the standard tool used to achieve respiratory autonomy in premature infants. It supports a patient's ability to spontaneously breathe by providing intermittent, mechanically controlled respiration. The assisted ventilation is regulated by frequency settings which dictate the interval at which the ventilator becomes sensitive to an infant's breath and responds with an assisted breath. Patients and methods: Using a neonatal active lung model ( NALM), this in-vitro benchmark study investigated how well synchronization works in the "synchronized intermittent mandatory ventilation" ( SIMV) mode of several ventilators. The efficiency of each ventilator was tested under two conditions: the first required that the ventilator be set to a constant ventilation rate while the NALM was adjusted to frequencies equal to and below this rate. The second condition varied the ventilator frequency while the NALM rate was held constant. Correctly triggered breaths were counted and displayed as a percentage (%) of each potentially triggerable breath. Results: Performance among devices significantly differed, ranging from a low 38.9 to a max of 71.7 % under the first condition, while showing an increase ranging from 70,7-102.5 % under the second condition. Discussion/conclusion: At high SIMV frequencies, synchronization between patient and ventilator becomes increasingly limited. Despite their identical functions as ventilators, SIMV algorithms of various manufacturers deliver ventilation rates with significantly different degrees of synchronization; not only in comparison to each other, but also with respect to their own ability synchronize breaths under various conditions typical of preterm lungs. Background: Periventricular leukomalacia ( PVL) may develop pre-, peri-, and postnatally. PVL has far-reaching consequences for the premature infant and its family. The prevalence of PVL in premature infants weighing less than 1500 g at birth has been reported with 3-5 % [1] . The hallmark of PVL is a disruption of cerebral perfusion leading to cerebral hypoxia.

The resultant loss of neurons, astrocytes, and other glia cells leads to the development of cerebral cysts. Both diffuse and focal cystic lesions have been described. Neurodevelopmental impairment and epilepsy are common sequelae. Aim of this study: At the Elisabeth Children's Hospital Oldenburg, a retrospective analysis of all known cases of PVL diagnosed during the period of 01/2016 thru 12/2020 was conducted. Cases were categorized according to the most likely time of insult (pre-, peri-, and postnatally). Methods: Cases of PVL were extracted by means of a database search for the specific international classification of diseases ( ICD) code. Pregnancies and postnatal events were reconstructed and cases were categorized as either pre-, peri-, or postnatal complications. Risk factors for each category had been predefined. Results: Eight patients met search criteria. Three cases each were assigned to the groups of prenatal and perinatal complications. Two cases were categorized as postnatal complications. Motor and neurocognitive development, as well as cerebral ultrasound findings were documented. Conclusions: When compared to international standards, the prevalence of PVL in premature infants of less than 1500 g at birth was below average. The cases included represent a heterogeneous group of patients showing a balanced number of pre-, peri-, and postnatal complications. Therefore, structural deficits at the University Hospital of Oldenburg are unlikely. All patients exhibit symptoms of neurodevelopmental impairment emphasizing the importance of high quality diagnostics and clinical management before and after birth to prevent the occurrence of PVL. In an experimental setup, the dead space of several flow sensors with different dead space and various adapters were tested how to keep the dead space as small as possible. Patients and methods: The experiment was conducted with the continuous flow ventilator Sophie, connected to a pneumotachograph and a test lung, simulating a 1000 g infant. Four different flow sensors from Stephan GmbH with various adapters and Microstream CO2 sampling were tested: A, B, Double, and the new Neo pneumotachograph for preterm infants. The dead space could be determined by flooding the lung with CO2 and measuring the CO2 elimination time using a capnograph. The measurements were carried out twenty times with each combination. Mean elimination times (± SD) were compared. Results: Mean CO2 elimination time was 27.4 s (± 0.05 s) with and 28.4 s (± 0.05 s) without the dead space minimizer for the A Sensor, 32.4 s (± 0.06 s) and 36.5 s (± 0.13 s) for the B Sensor, 27.5 s (± 0.05 s) and 27.5 s (± 0.08 s) for the Double Sensor and 30.4 (± 0.15 s) without for the Neo Sensor because it is not compatible with it. If no dead space minimizer was installed, the standard adapter for the endotracheal tube was used as a reference. Using the Microstream CO2 sampling, mean elimination time was 29.3 s (± 0.1 s) for the A, 36.1 s (±0.05 s) for B, 28.5 s (± 0.05 s) for Double and 31.3 s (±0.07 s) for the Neo pneumotachograph. Discussion/conclusion: CO2 elimination time was significantly lower with the dead space minimizer for A and B Sensor, not significant for Double, and not assessable for Neo Pneumotachograph. CO2 sampling leads to a slight increase in dead space, which is relevant for extremely preterm infants. However, the effect does not appear to be so great that a justified use can be considered.

Condensation-an underestimated risk in the ventilation of extremely preterm infants Introduction: Measuring the correct tidal volume is playing an increasingly important role in mechanical ventilation of extremely preterm infants. Companies have developed several flow sensors based on a pressure differential. Condensation in the pneumotachograph can compromise the measurement accuracy that leads to hypoventilation of the infant. In an experimental study, three commercially used flow sensors were compared to determine how much they are affected by condensation and whether they provide consistent values over a long period. Patients and methods: The measurements were carried out five times with each pneumotachograph, tested for two hours. The experiment was conducted with the continuous flow ventilator Sophie (Stephan GmbH) connected to a pneumotachograph and a test lung, simulating a 1000 g preterm infant. Three pneumotachographs (A, B, Neo PNT) were exposed to simulated ventilation for two hours. The tidal volume measured by the flow sensors every two seconds was recorded by the Sophie Respirator and saved in log files. Based on these files, the progress of the tidal volume over time was compared. Each trial was repeated five times with identical settings. Results: The measurement data showed an increase in the measured tidal volume of 0.5 ml for two flow sensors after 10 min and 4 ml after two hours. The third sensor was able to maintain a constant measurement of tidal volume over two hours. Discussion/conclusion: Condensation can significantly affect the measurements of the pneumotachograph. The measured tidal volume values may be incorrectly high due to the increasing differential pressure in pneumotachographs. Therefore, the measured values may also be questioned.

Beeinflusst die Verwendung eines HEPA-Filters die Qualität der nichtinvasiven Beatmung von Neugeborenen? Eine randomisierte Cross-over-Frühund -Neugeborenensimulationsstudie Introduction: Despite the progress in medical care of preterm infants and sick newborns, prematurity and perinatal asphyxia are still associated with a high risk of neonatal cerebral damage. Causal therapies are currently lacking. The neuroactive steroids dehydroepiandrosterone ( DHEA) and its sulfate ester DHEA-S are a promising option to reduce microglial cell activation in the wake of perinatal brain injury.

In this experimental study we assessed microglial cell activation in 87 neonatal mouse brains, which were either healthy or injured by hypoxia-ischemia. In a first step, conducted as a toxicity study, 39 healthy mice were randomly assigned to one of eight treatment groups and received an intraperitoneal injection of either phosphate-buffered saline ( PBS), PBS+ dimethyl sulfoxide ( DMSO,-"solvent control") or DHEA or DHEA-S in different concentrations. In a second step, 48 animals were subjected to hypoxic-ischemic brain injury by ligation of the right common carotid artery and subsequent exposure to a hypoxic environment. Study animals were then randomly assigned to the above-mentioned treatment groups (n = 6), stratified by gender (1:1). All brains were histologically processed and coronal brain sections were immunohistochemically stained with microglial specific Anti IBA-1 to determine the number of activated microglial cells in different brain regions. Results: At this point the analysis of the toxicity study is still ongoing.

In the hypoxic-ischemic injury study, no significant differences in microglial cell activation were detected between the different treatment groups. There were significant differences in the number of activated microglial cells between male and female mice in three different brain regions of the right hemisphere, namely the thalamus ( PBS, p = 0.048), hippocampal formation ( PBS/ DMSO, p = 0.02) and at white matter ( DHEA 10 µg/g bodyweight, p = 0.007). Discussion/conclusion: The study did not reveal an effect of DHEA or DHEAS on microglial cell activation in the hypoxic ischemic injured mouse brain. There are some limitations, such as the small number of cases or the manual microscopic analysis, which might affect the number of detected activated cells. The relevance of the observed sex difference is uncertain, as only some regions without a distinct pattern were affected and the number of cases is low, but further investigation is ongoing. Introduction: At our PMU "Research Institute for Rehabilitation, Transition and Palliation" we care for a large number of patients with rare pediatric onset epilepsies. 15 years ago our chair, Prof. Gerhard Kluger, initiated the international non-funded and non-sponsored network NETRE (NEtwork Therapy Rare Epilepsies). In this group about 350 clinicians are sharing their treatment experience in rare pediatric onset epilepsies. Patients and methods: With our project PATRE we investigate these diseases and their therapies based on information directly from the patients and their parents using the example of one etiology, SYNGAP1. The parents are nowadays extremely well networked worldwide through the social media. Therefore we use a server with REDCap for secure state of the art data collection. The aim of the project is therefore to develop a standardised procedure for researching the phenotype and therapies of rare epilepsy by means of electronic parent questionnaires. SYNGAP1 is a gene that encodes the cytosolic protein SYNGAP1 (SYNaptic GTPase Activating Protein). Mutations in SYNGAP1 result in a neurodevelopmental disorder termed Mental RetarDation-type 5 (MRD5, OMIM #612621) with a phenotype consisting of intellectual disability, motor impairments, and epilepsy. In a first survey, we asked parents' subjective assessment of the effect of medication in SYNGAP1. For this purpose, the parents indicated their subjective assessment of the effect on seizures, behavior, development and sleep on a scale from -50 to +50. Results: We received 28 complete data sets, representing more than half of the patients in the support groups. Of these, we received data on 18 medications. 

Graz; 2 D&F Institut für Humangenetik/Medizinische Universität Graz; 3 Pädiatrie/Univ. Klinik für Kinder-und Jugendheilkunde/Medizinische Universität Graz; 4 Abteilung für Kinder-und Jugendheilkunde/ LKH Hochsteiermark

Die extra-und intramurale Versorgung von Kindern und Jugendlichen mit lebenslimitierenden Erkrankungen: Erfahrungen des Mobilen Kinderteams ( MKT) am

Klinikum Graz in den Jahren

Klinische Abteilung für pädiatrische Hämato-Onkologie

The Viennese EDDY Study as a Role Model for Obesity: Prevention by Means of Nutritional and Lifestyle Interventions

Dramatic Increase of Body Weight during Covid-19 Breakdown: Data from the Austrian Prevention Project Eddy Kids 2021/19 and Eddy Kids 2019/20

Hypertension among obese children and youth age 8-12: project EDDY-Kids

Auswirkung von "picky eating" bei einem Jugendlichen mit ADHS und ASS Aguiriano

The use of mobile health apps plays an increasingly important role in patients' lives and in the medical field, making mHealth a sta-ple in the future of asthma (self-)treatment plans. Data on apps intended for children are insufficient, especially in non-English-speaking countries. This study aims to identify functional asthma apps for children, both in German and English. The Google Play and Apple App Store are systematically searched to preselect the most efficient apps, which are then compared according to a criteria catalog. Patients and methods: Both app stores were screened for the term asthma. Following a standardized preselection process ( PRISMA), the apps that met the inclusion criteria (available free of charge, German or English language, suitable for children) were rated by 3 independent persons following a criteria catalog consisting of 9 categories, some conceived for this purpose (availability, child-friendly, learning factor, and range of functions), and some adopted from existing validated catalogs (functionality and design, ease of use, potential for improving asthma self-management, fun factor and incentives, and information management and medical accuracy). The highest rated apps in German and English were compared. Results: A total of 403 apps were identified. Finally, 24 apps that met the inclusion criteria were analyzed. The top German language apps were compared with the highest rated English language apps. The English language apps scored significantly higher in the following categories: potential for improving self-management, child-friendly, fun factor, learning factor, and range of function. English language apps (mean total points 34.164, SD 1.09) performed significantly better than German language asthma apps (mean total points 22.91, SD 2.898; P = 0.003). The best rated English language app was Kiss my asthma (36/42 points), whereas the best rated German language app Kata achieved only 27.33 points. Discussion/conclusion: Although validated recommendations on rating mHealth apps have been published, it remains a challenging task for physicians and patients to choose a suitable app for each case. The recommended English language apps are Kiss my asthma, AsthmaXcel, AsthmaAustralia, and Ask Me, AsthMe!, whereas the only recommended German language app is Kata. Introduction: Lung function deterioration in CF patients may be caused by allergic bronchopulmonary aspergillosis ( ABPA) or mycosis ( ABPM). The lack of SPT extracts or specific IgE tests against most fungi possibly leads to underestimation of ABPM. We aimed to investigate if CF patients are sensitised against fungal species colonising their airways, if a sensitisation is detectable via SPTs using individually produced extracts, and if a sensitisation is associated with lung function deterioration. Patients and methods: This prospective study included CF patients whose sputum cultures grew fungi. Fungal extracts individually produced from colonising fungi, and Aspergillus fumigatus and Candida albicans commercial extracts were used to perform SPTs. The patients' clinical state, lung function, eosinophil count, and total and specific IgE against A. fumigatus and C. albicans were analysed. Results: Out of 111 CF patients, 44 (39.6 %) were colonised with fungi in relevant amounts and were tested with a total of 67 individually produced fungal extracts of colonising Aspergillus species (10 patients), Candida species (39), Exophiala dermatitidis (8), Scedosporium species (6), and Cryptococcus cyanovorans, Geotrichium capitatum, Penicillium variotii and Rasamsonia argillacea (1 each).

Einleitung: Frühgeborene ( FG) <32 Schwangerschaftswochen haben ein erhöhtes Risiko für eine Sprachentwicklungsverzögerung. Um das individuelle Risiko abschätzen zu können, sind prognostische Marker nötig. Es ist bekannt, dass ein Zusammenhang zwischen Gehirngröße und kognitivem sowie motorischem Outcome besteht. In dieser Studie wurde die Beziehung zwischen linearen Messungen in der cerebralen Magnetresonanztomographie ( MRT) und dem sprachlichen Outcome untersucht. Patienten und Methoden: FG <32 Schwangerschaftswochen mit MRT am errechneten Geburtstermin wurden eingeschlossen. In den MRT-Bildern wurden der transcerebellärer Durchmesser ( TCD), die biparietale Weite ( BPW), der extracerebrale Raum ( ECR) rechts und links sowie der Interhemisphärenspalt ( IHS) bestimmt. Für Kinder mit Gehirnläsion (intraventrikuläre Blutung, Kleinhirnblutung, Schädigung der weißen Substanz) wurde eine Subgruppen-Analyse durchgeführt. Sprach-Scores wurden im Alter von 2 Jahren mittels Bayley Scales of Infant and Toddler Development III erhoben. Als Sprachentwicklungsverzögerung galt ein Wert von <85. Ergebnisse: 255 FG mit einem Gestationsalter von 29,0 (28,0; 31,0) SSW und einem Geburtsgewicht von 1250 (985;1500) g wurden inkludiert. 34,1 % der FG hatten eine Sprachentwicklungsverzögerung. FG mit einem TCD<50 mm erreichten niedrigere Sprach-Scores als jene mit einem TCD≥50 mm ( Introduction: Since the foundation of the "International League Against Epilepsy ( ILAE)" in 1909, there has been vivid debate about establishing a classification system of epilepsy that continuously reflects the increasing scientific knowledge. The current classifications were published based on an international agreement in 2017, as well as two other papers for the classification of seizures and instruction for its usage. In the daily life of a clinician, the epilepsy classification has an indispensable role in the evaluation of patients who are experiencing seizures. There is a necessity to examine the usefulness of the new classification system of epilepsy in daily clinical practice. Patients and methods: This cross-sectional study presents a retrospective data analysis for application of the currently used ILAE-classification criteria of 2017 in children and adolescents diagnosed with epilepsy aged from 0-18 years. The data collection includes 120 children and adolescents, all patients under the care of paediatric neurology at the Department of Paediatrics, Kepler University Hospital between 2019 and 2020. Results: The frequency distribution of onset of epilepsy shows one peak in the first year of life (20 %) and one in the eighth year of life (14,2 %). Boys (50,8 %) and girls (49,2 %) were equally affected. Focal seizures were the most common type of seizures (55,8 % of the cases), followed by generalized seizures (37,5 % of the patients). Unclassified seizures were only found in 6,7 % of children. The etiological causes could be described in 32,5 % of children. Structural causes were the most common with 23,3 %, followed by genetic causes with 9,2 %. Among the group of epileptic syndromes, the most common was Rolando-Epilepsy (9,2 %) closely followed by Absence-Epilepsy (6,7 %) and West-Syndrome (5,8 %). At least one comorbidity existed in 39,3 % of patients. The most common was developmental delay (29,2 %) followed by somatic comorbidities (17,5 %) and psychiatric comorbidities (5,8 %). Discussion/conclusion: The ILAE-classification of 2017 enables the clinician to have a more precise description of diagnosis when assessing patients with epilepsy. Among the currently used classification system, twothird of the patients still have an unknown cause. This might decrease with further progress in genetic testing of children. Screening for comorbidities creates a broader awareness of the diverse phenotypic spectrum in patients with epilepsy. 

The study included all children aged up to 18 years visiting the pediatric emergency unit of the Department of Pediatrics and Adolescent Medicine Vienna from January to December 2014. The MTS modification represents an upgrade of one-urgency level for patients with complex syndromic diseases, specific symptoms related to chronic diseases, reduced general condition, profound language difficulties, unknown current history or special needs. As reference standard a four-level outcome severity index was established as classification for the correct MTS urgency level, which included diagnostic and therapeutic interventions, admission to hospital and follow-up strategies. Ergebnisse: In total 19264 patients could be included, of them 940 had a CHD. 266 patients from the CHD group fulfilled the inclusion criteria for the MTS modification. The MTS modification was significantly more often applied in under-triaged (65.9 %) than in correct-triaged or over-triaged (25 %) children with CHD (p-value X 2 test <0.0001, OR 5.767 95 % CI: 3.585-9.465). Discussion/conclusion: A clear reduction of under-triage could be seen in children with CHD by using the MTS modification with one urgency level upgrading. Therefore applying a safety strategy concept to the MTS could avoid under-triage in high-risk patients.

The SPTs of 16/44 (36.4 %) patients resulted positive: 4 were positive to individually produced extracts, 3 of them to individually produced and commercial extracts of A. fumigatus. The fourth patient was positive to individually produced and commercial extracts of C. albicans, and an individually produced extract of A. fumigatus "non-sporulating" subtype, but negative to A. fumigatus "sporulating" subtype and commercial extract. Twelve patients were positive to commercial extracts (10 to A. fumigatus, 2 to C. albicans) but were not colonised and therefore not tested with individually produced extracts. Only in 1/28 cases (3.6 %) a discordant result was observed between commercial and individually produced extracts. No patient had ABPM. Discussion/conclusion: We performed SPTs using individually produced fungal extracts to identify sensitisations against colonising fungi. We found no sensitisation to other fungi than A. fumigatus or C. albicans. This finding might be due to other fungi not inducing sensitisation or to the low number of colonised patients. The method appears to be reliable but other studies are needed for confirmation.Topic: Rheumatologie P12-01 MEFV-und NLRC4-assoziierte Sjögren-Symptomatik: ein Fallbericht Astl J., Artacker G., Rath-Wacenovsky R., Kurz H.

Einleitung: Genetische Mutationen in MEFV und NLRC4 sind phänotypisch vergesellschaftet mit einem "Familiären Mittelmeerfieber" ( FMF) und einem "Familial-cold-autoinflammatory-syndrome-4" (FCAS4). Jedoch können die klinischen Präsentationen mannigfaltig sein und die genannten genetischen Veränderungen eine Vielzahl an autoinflammatorischen Prozessen auslösen. Wir präsentieren einen Patienten, welcher beide Gen-Mutationen vorweist und eine entsprechende autoinflammatorische Symptomatik aufweist. Patienten und Methoden: Ein 16-jähriger Knabe fiel durch eine abdominelle Symptomatik und Fieber, seit wenigen Tagen, auf. Aufgrund eines inkonklusiven Abdomenultraschalls und persistierender Symptomatik wurde eine laparoskopische Appendektomie durchgeführt. Postoperativ stiegen die Entzündungsparameter nicht-erwartungsgemäß hoch an ( CRP max. 463 mg/L), sowie Lipase und alpha-Amylase. Aufgrund dessen etablierten wir eine parenterale antibiotische Therapie (Cefotaxim, Ampicillin/Sulbactam). Ebenso zeigten sich im weiteren Verlauf vergrößerte cervikale Lymphknoten, sowie Schwellungen sämtlicher Speicheldrüsen und der Tränendrüsen (im Abdomen-Ultraschall zeigte sich eine diffuse Schwellung des Pankreas mit Splenomegalie). Bei gleichzeitig bestehender Keratokonjunktivits sicca stellten wir den initialen Verdacht eines Sjögren-Syndroms, differentialdiagnostisch einer "IgG4-related-disease". Laborchemisch zeigten sich die ANA subsets ( SS-A( RO) und SS-B(La)) sowie die IgG4-subsets im Normbereich. Weitere Befunde waren unauffällig: Komplementfaktoren, Rheumafaktor, ANA, dsDNA, Immunglobuline, Caeruloplasmin, Quantiferon/Tuberkulin-Test, virologische sowie bakterielle Erregerdiagnostik, Knochenmarkspunktion. Eine Feinnadelbiopsie einer Glandula parotis zeigte auch nur unspezifische diffus-entzündliche Veränderungen. Auffällig waren ein erhöhtes Serum-Amyloid-A, Leberfunktionsparameter sowie initial erhöhte Triglyzeride. Wir etablierten eine anti-inflammatorische Steroidtherapie mit Methylprednislon 2 mg/kg/Tag, worunter es rasch zu einer Besserung der Gesamtsymptomatik kam und der Patient schließlich entlassen werden konnte -unter ausschleichender Steroidtherapie. Post-stationär erhielten wir die Ergebnisse der genetischen Untersuchung (Autoinflammations-Panel), welche folgende 2 Mutationen zeigte: MEFV (M694V, heterozygot), ursächlich für ein "Familiäres Mittelmeerfieber" ( FMF), sowie NLRC4 (G174V, heterozygot), ursächlich für ein "familial-coldautoinflammatory-syndrome-4" (FCAS4). Ergebnisse/Schlussfolgerung/Diskussion: In Zusammenschau der Befunde lässt sich hier auf ein autoinflammatorisches Geschehen schließen. Der Klinik zufolge handelt es sich um eine Sjögren-ähnliche Symptomatik, mit Anzeichen einer IgG4-related Disease (insbesondere aufgrund der Dakryocystitis und Pankreatitis). Die laborchemischen Befunde hinsichtlich dessen waren unauffällig, schließen diese beiden Diagnosen jedoch nicht aus. In Zusammenhang mit einem FMF und einem FCAS4 scheinen diese Symptome -aufgrund des autoinflammatorischen Zusammenhangs -nicht unwahrscheinlich bzw. doch plausibel. Differentialdiagnostisch sollte an ein hämophagozystische Lymphohistiozytose -primärer Genese aufgrund der NLRC4 Mutation -aber aufgrund der laborchemischen Konstellation unwahrscheinlich. 

A 12-year-old girl presented with a 3-month history of episodes of well-defined bloodish oozing from intact skin (face, forehead, trunk, upper, lower limbs) and mucosal membranes (mouth, tongue, conjunctiva). These attacks occurred at varying sites and preferentially at evening or during sleep. They were occasionally preceded by a subtle burning and tingling sensation and resolved spontaneously after seconds to few minutes. The bloodstain was easily wiped off, revealing normal appearing, non-traumatized skin underneath. Samples of the secretion, appearing clearer and more fluid than blood, contained morphologically inconspicuous erythrocytes. The events became more frequent (to several times a day and during night disturbing her sleep) and widespread with ongoing course posing a great psychological burden. She additionally developed weekly bouts of headaches, hypaesthesia of the left upper extremity, nausea and abdominal pain. Neither psychological, physical (stress), infectious, artificial and medication triggers nor symptoms of arterial hypertension, gastrointestinal, blood, coagulation or psychiatric disorders could be determined. Results: The features of this case are consistent with hematohidrosis, a very rare phenomenon mostly affecting the craniofacial area of female children. The pathomechanism remains unclear. Some authors proposed dermal defects to lead to blood-filled spaces that would exude into follicular canals or directly to the skin surface. Nuclear magnetic resonance ( NMR) analysis in one 6-year-old patient further revealed that fluid samples had a metabolomic profile similar to eccrine sweat, containing constituents derived from both blood and sweat1. The previously reported response to propranolol and the association with stress have been suggested to reflect a pathognomonic adrenergic stimulation and vasoconstriction in sweat glands and small blood vessels leading to blood extravasation. Discussion/conclusion: Although self-limited resolution is commonly described, various therapeutic approaches to address quality of life and issues of stigmatization have been reported in the literature, including psychotherapy, relaxation therapies, anxiolytic drugs, antidepressants and beta blockers. Treatment with the latter (Propranolol 3 × 1.5 mg/kg/day) reduced the frequency of her bloodish oozings to once a month and led to disappearance of all other signs and symptoms. Introduction: Pityriasis rubra pilaris ( PRP) is a rare inflammatory skin disease with no established and specific treatment that occurs in adults of all ages as well as in children. Despite typical clinical features including orange-red plaques, nappes-claires as well as follicular and palmoplantar hyperkeratosis, the phenotype of PRP shows considerable variability which may delay diagnosis and proper treatment. Patients and methods: Here we report on a 5-year-old girl who was initially diagnosed for infection-triggered psoriasis. As the primary therapeutic regimen with topical glucocorticoids, systemic antibiotics as well as adalimumab did not prove beneficial, we re-evaluated the initial diagnosis and initiated additional investigations. Based on the clinical appearance the diagnosis of classical juvenile (type III) pityriasis rubra pilaris ( PRP) was established and a therapeutic attempt with ustekinumab started. Considering excellent tolerability and efficacy, ustekinumab dosage was doubled up and combined with acitretin to target palmoplantar hyperkeratoses and nail involvement. This regimen further improved the phenotype without compromising excellent tolerability. Results/Discussion/conclusion: To our best knowledge, we report the second child receiving an anti-interleukin-12/23 monoclonal antibody therapy off-label for PRP and the first that has been treated in combination with acitretin. As shown in our patient, the diagnosis or PRP may be challenging and impeded by overlapping features with psoriasis. Confirmation of PRP is followed by the challenge to find accurate, effective modalities of care. Due to its rareness, therapeutic approaches lack evidence and rely mainly on single case reports or small case series, mostly involving with type I PRP and CAPE. Ustekinumab was recently approved for the treatment of moderate to severe plaque psoriasis in patients older than 6 years, making it an interesting therapeutic agent for pediatric patients. Referring to our observation and in line with other anecdotic reports, the human interleukin-12/23 antagonist proved efficacy and tolerability in the treatment of refractory PRP in our case. Combination with acitretin did not adversely affect the safety profile and may be considered to increase the therapeutic impact especially on hyperkeratotic lesions. Controlled studies yielding higher quality evidence, however, are warranted to corroborate our observation. Introduction: According to the COSI-Report issued by the World Health Organization 30 % of the boys and 22 % of the girls aged between 6 and 9 are overweight or obese in Austria [1] . The COVID-19 pandemic showed a dramatic increase of children's bodyweight during the school closure and therefore stresses the need for action [2] . The Austrian prevention study EDDY "Effect of sports and diet trainings to prevent obesity and secondary diseases and to influence young children 's lifestyle" is an interventional cohort study conducted in Austrian primary schools [3] . In order to adhere to COVID-19 mitigation measures, a new and innovative EDDY online program has been developed. Patients and methods: The aim of the study is to find out if a combination of diet and sports training can change children 's lifestyle, nutritional habits and anthropometric parameters. Weight, body mass index and body composition are assessed as well as blood pressure and waist circumference. Questionnaires are used to analyze the children's eating habits, nutritional knowledge and mental health status. Sports tests are included to measure the physical performance. During a 15-week intervention the contents are transmitted via videos. Results: The results of the EDDY project from the past years showed a significant improvement of the physical performance and an improved nutritional knowledge in the intervention group. Furthermore we showed that overweight children had a higher blood pressure compared to children with normal weight [4] . Discussion/conclusion: The COVID-19 pandemic mitigation measures impact childrens ' lifestyle in a variety of aspects and have shown to increase the risk for unhealthy weight gain. Further research is needed in regard to the long-term effects on children 's health. This project investigates whether a combination of nutrition and sports education with the aim to increase healthy food intake and physical activity is able to prevent the pathological increase of body weight, to positively influence the body composition and the blood pressure and to improve the physical performance. Our goal is to implement the EDDY online program in multiple primary schools within Austria and it could be a first step to facilitate the implementation of health information into the general curriculum. 1,68z) . Methylphenidat wurde bei ADHS während der Schulzeit eingeleitet und führte zu einer Besserung der Beschwerden wie auch zu einer Appetitreduktion. Das G fiel 2014 <1. P (-3,32z); ein elektiver Therapieversuch auf Atomoxetin wurde 2015 unternommen und bei fehlender Appetitsteigerung wieder beendet. Die Wachstumsgeschwindigkeit konnte dank Appetitsteigerung in der schulfreien Zeit bis 2018 gehalten werden, wo der Patient einen Perzentilen-Knick entwickelte. Ein weiterer Alternativversuch mit Lisdexamfetamin führte zu keiner Appetitsteigerung. 2019 wird Methylphenidat auch in der Schulzeit pausiert, was zu erhebliche soziale Benachteiligungen des Patienten führt. Weitere supportive Maßnahmen entsprechend unseres multidisziplinären Therapiekonzeptes sind zu diesem Zeitpunkt ausgeschöpft. 2021 wird bei einem G entsprechend der <1 P. (-8,26z) und einer L. entsprechend der <1 P. (-3,85z) auf eine zusätzliche nasogastrale Sondenernährung zugegriffen; diese wird gut toleriert. Das G stieg mit 10 kg auf die <1 (-2,49z) P innerhalb von 2 Monaten an; die L mit 6,5 cm auf die <1 (-2,88z) P innerhalb von 4 Monaten. Die Sondenernährung wird aktuell zum Erreichen der 1.-5. P angewendet; das Essverhalten des Patienten hat sich dadurch nicht geändert. Schlussfolgerung/Diskussion: Diese Kasuistik verdeutlicht die Komplexität bei ADHS & ASS Komorbidität. ASS verstärkt die appetitreduzierende Nebenwirkung der ADHS-Therapie. Altbewährte Schienen können von einem eklektischen Therapieansatz profitieren, wenn sie bei multimorbiden Krankheitsbildern an ihre Grenzen stoßen.