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Hypernatremic Dehydration and Hypothermia in Congenital Lamellar Ichthyosis
Authors:Ben-Zion Garty  M.D.    Yeheskel Wiseman  M.D.    Arie Metzker  M.D.    Solomon H. Reisner  M.D.    Menachem Nitzan  M.D.
Affiliation:Department of Pediatrics A, Department of Neonatology and the Pediatric Dermatology Clinic, Beilinson Medical Center, Petah Tiqva and the Tel Aviv University Sackler School of Medicine, Israel
Abstract:
Several events of hypernatremic dehydration occurred in five infants suffering from severe congenital lamellar ichthyosis. Simultaneously, four of them developed hypothermia. Both phenomena were probably caused by excessive water loss through the affected skin. In lamellar ichthyosis the skin loses its function as an effective barrier and therefore its role in water and temperature homeostasis is defective.
Keywords:
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