The challenging management of a series of 43 infants with Netherton syndrome: unexpected complications and novel mutations*
British Journal of Dermatology2020Vol. 184(3), pp. 532–537
Citations Over TimeTop 18% of 2020 papers
N. Bellon, S. Hadj‐Rabia, Florence Moulin, Cécile Lambe, Guillaume Lezmi, Fabienne Charbit‐Henrion, Caroline Alby, L. Le Saché‐de Peufeilhoux, Stéphanie Leclerc‐Mercier, Alice Hadchouel, Julie Steffann, Alain Hovnanian, Alexandre Lapillonne, Christine Bodemer
Abstract
Our data emphasize that neonatal NS is a severe and sometimes lethal multisystem disorder. Patients have a high risk of variable metabolic anomalies (i.e. lethal hypernatraemia) and therefore have major nutritional needs. Cases of NS associated with c.153delT are particularly severe. Unexpected clinical manifestations broadened the phenotypic spectrum of NS. We provide recommendations on the management of the life-threatening manifestations of NS in neonates based on our multidisciplinary experience.
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