COVID-19 in children at Strasbourg University Hospital: A retrospective study of the first 2months of the epidemic.

CONTEXT: The emergence and rapid spread of coronavirus disease 2019 (COVID-19) have shaken the planet, both in terms of health and economical aspects, constituting a real challenge for the scientific community. PROBLEM: At the time of the arrival of the epidemic in France, there were limited data regarding how COVID-19 could affect children. A lesser severity compared with adults was described, but knowledge concerning clinical forms and screening strategies was missing. METHODOLOGY: In this retrospective and non-interventional epidemiological study, we aimed to describe the epidemiology and the clinical features of COVID-19 pediatric disease in the first university hospital affected by the epidemic in France. We included all underage patients who tested positive for SARS-CoV-2 by polymerase chain reaction (PCR) assays on nasopharyngeal smears performed between February 25, 2020 and April 30, 2020. RESULTS: The presence of fever and respiratory signs was frequent (>50%), as was the presence of general or digestive signs, but patients were also frequently asymptomatic, making the discovery of a positive smear fortuitous. There were no deaths in our cohort. CONCLUSION: No patient with a serious form of COVID-19 was treated in the pediatrics departments of Strasbourg University Hospital during the first 2 months of the epidemic. Diagnostic strategies have evolved over the course of the epidemic, ranging from exploring relevant symptoms to systematic screening.

Severe hypernatremic dehydration in an infant with Netherton syndrome.

Netherthon syndrome is a rare autosomal recessive disease characterized by ichthyosis, the characteristic hair abnormality trichorrhexis invaginata and atopic manifestations. We report a female child with the severe hypernatremic dehydration form of the Netherton syndrome born as the first child of consanguineous parents. Ichthyosis was present at birth. She was admitted to the intensive care unit at the age of 4 days with important loss of weight and dehydration. Severe hypernatremia and convulsions occurred. Despite intensive care the baby died at the age of 11 days. The diagnosis of Netherton syndrome was confirmed by the finding of the pathognomonic hair shaft anomaly trichorrhexis invaginata (bamboo hair) and premature lamellar body secretion and foci of electron-dense material in the intercellular spaces of stratum corneum as relatively specific markers for Netherton syndrome. Netherton syndrome is characterized by a large variability in phenotypic expression. The major neonatal complication is the hypernatremic dehydration, which can be fatal as in this patient or complicated by neurologic signs (intracranial hemorrhage) and secondary sequellae. Molecular studies revealed a mutation in SPINK 5, encoding a serine protease inhibitor. Prenatal diagnosis was performed in the second pregnancy and showed that the fetus was equally affected.