key: cord-0768498-kr5cr19r
authors: Saad‐Naguib, Michael; Barbouth, Deborah; Thorson, Willa; Hacker, Stephanie; Tekin, Mustafa
title: COVID‐19 in a child with severe propionic acidemia
date: 2021-05-18
journal: Pediatr Int
DOI: 10.1111/ped.14633
sha: 9aaaa71945b1fd36727a30fa38ad659f3c258fee
doc_id: 768498
cord_uid: kr5cr19r

nan

the risk of developing a metabolic crisis secondary to a SARS-CoV2 infection, metabolic disease centers have been cautious handling these cases.

We present a 7-year-old female of Hispanic and Caucasian ancestry with propionic acidemia who was recently diagnosed with, and recovered from, COVID-19. Propionic acidemia is a well characterized inborn error of metabolism in which patients may experience encephalopathy, seizures, or a coma resulting in death. 3 Despite early intervention, she has global developmental delay, seizures, chronic constipation, pancytopenia, and recurrent pancreatitis. An echocardiogram in the spring of 2020 demonstrated normal cardiac function. She is G-Tube dependent with limited oral intake. Her diet meets energy recommendations but exceeds protein recommendations. 4 Despite increased protein intake, she has low levels of essential amino acids. Prior to 2020, she was hospitalized 3-4 times a year. This year, she has been admitted monthly with various indications. During admissions, her arterial blood gas pH has ranged from 7.01 to 7.45 with an anion gap between 4 and 27. The patient's white blood cell count has been between 1-2 (10) 3 /uL, and she has required platelet transfusions. Ammonia levels were 17-105 micromol/L, which is within her normal range.

Earlier this year, we received a call from her mother stating that she had been lethargic for 2 days; however, she was tolerating her feeds, afebrile, and having normal bowel movements. At her local emergency room, critical labs, including ammonia level, were consistent with the patient's baseline. Due to concerns from previous admissions, the patient was transferred to our hospital. She then experienced recurrent emesis and was made nil per os (NPO). Total parenteral nutrition was started thereafter. On hospital day 2, the patient's routine SARS-CoV2 test resulted positive. She did not exhibit any respiratory symptoms, and her breathing was stable on room air. She experienced a one-time fever to 38.1°C, which resolved spontaneously. The virus was treated with supportive care, and the use of novel treatments, such as remdesivir, was not employed. The patient was empirically started on the antibiotics vancomycin and cefepime due to her high risk of bacterial infection. Regular feeds without protein restriction were restarted on hospital day 3, and she tolerated them well. Her lethargy also began to improve. She was monitored closely for the next 2 days with continued improvement and was discharged home on hospital day 6. We have since followed up with her, and she was back at her baseline and does not appear to have any lingering symptoms of COVID-19. A repeat polymerase chain reaction (PCR) test for SARS-CoV2 25 days after the initial test was positive during another admission to her local hospital for emesis; however, a repeat test 33 days after her initial positive test was negative. She did not present with any symptoms of COVID-19 on the second admission. We do not believe that this was a new SARS-CoV2 infection, but rather this test had remained positive from her first admission.

Prior to the pandemic, the patient attended a prescribed pediatric extended center (PPEC), but she had not gone since spring 2020. A second-degree relative of the patient had symptoms of fever and myalgia prior to the early July admission and subsequently tested positive for SARS-CoV2. Her family believes this was likely the exposure that led to the patient's positive test as they had quarantined for several months. After her admissions, the rest of her family tested negative.

It is well documented that children with COVID-19 do not present with severe complications, and fatalities have been seen in only 0.05% of pediatric patients. 2 Some children do present with fever, pneumonia, or a rare multisystem inflammatory syndrome. 2 A case report by Caciotti et al. described a 1 year old with propionic acidemia and COVID-19 who exhibited fever up to 38.5°C, emesis, skin pallor, and dyspnea. A chest X-ray demonstrated small subpleural thickenings. Anabolic support and supportive care were provided, and he improved within 7 days of admission and was discharged home. 5 Our observations indicate that having a severe form of a metabolic disorder is not necessarily associated with a worse clinical outcome. Perhaps the anabolic support and reversal of catabolism provided by both our team and the team in Italy prevented the patients' decompensation. It is important to note that this is a small sample size. It is possible that children with metabolic disease who contract COVID-19 may be treated similarly to other patients with decompensating metabolic diseases; however, several more cases need to be observed and treated to confirm this. The prevention of metabolic decompensation in patients with inborn errors of metabolism is likely a crucial component in the outcome of COVID-19 cases. 5 Disclosure

The impact of adenovirus infection on the immunocompromised host

Adenovirus infection among pediatric patients with cancer and in pediatric recipients of hematopoietic stem cell: a multicenter nationwide study

Appropriate dose reduction in induction therapy is essential for the treatment of infants with acute myeloid leukemia: a report from the Japanese Pediatric Leukemia/Lymphoma Study Group

Etiology of viral gastroenteritis in children <5 years of age in the United States

Adenovirus infections in immunocompetent and immunocompromised patients

Epidemiology of COVID-19 among children in China

Propionic acidemia

Nutrition management guideline for propionic acidemia: An evidence-and consensusbased approach

SARS-CoV-2 infection in a patient with propionic acidemia

This work was partially supported by Grant-in-Aid for Young Scientists (B) from the Japan Society for the Promotion of Science (17K16239). We would like to thank Mr Thomas Mayers from the Medical English Communications Center of the University of Tsukuba for scientific writing assistance.

Informed consent from the patient's parents and approval from the institutional review board of the University of Tsukuba Hospital were obtained (H27-167). The authors declare no conflict of interest.

A.Y.S. and H.F. treated the patient. A.Y.S. wrote the article with support from H.F. R.S., Y.Y., and H.T. critically reviewed the manuscript. All authors read and approved the final manuscript.The authors declare no conflicts of interest.

M.S.N. and M.T. and S.H. conceptualized and designed the study, collected data, drafted the initial manuscript, and reviewed and revised the manuscript. D.B. and W.T. carried out the initial analyses, and critically reviewed and revised the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work. Written consent was obtained from the patient.