Experience of low-dose dexamethasone use in the respiratory management of ichthyosis prematurity syndrome.

Davina Henderson, Claire A Murphy, Mary O'Dea, Michael A Boyle

BMJ case reports 2021 Aug 20

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Ichthyosis prematurity syndrome (IPS) is a rare disorder of autosomal recessive inheritance. The cardinal features include prematurity, vernix like hyperkeratosis, eosinophilia and neonatal asphyxiation. This case report discusses the presentation and management of IPS. We aim to characterise the common features, the spectrum of disease within a single family and discuss a potential role for low-dose dexamethasone in the management of ventilator-dependent patients with IPS. © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.

Citation

Davina Henderson, Claire A Murphy, Mary O'Dea, Michael A Boyle. Experience of low-dose dexamethasone use in the respiratory management of ichthyosis prematurity syndrome. BMJ case reports. 2021 Aug 20;14(8)