Bart´s syndrome associated with epidermolysis bullosa junctionalis and with pyloric atresia. An autopsy case report

Česká verze

Authors: Katarína Adamicová ¹; Tomáš Balhárek ¹; Lucia Lúčanová ²; Oľga Nyitrayová ³; Želmíra Fetisovová ⁴
Authors place of work: Ústav patologickej anatómie JLF UK a UN Martin ¹; Neonatologická klinika JLF UK a UN Martin ²; Cytopathos, spol. s r. o., Bratislava ³; Dermatovenerologická klinika JLF UK a UN Martin ⁴
Published in the journal: Čes.-slov. Patol., 50, 2014, No. 4, p. 155-158
Category: Původní práce

Summary

Bart‘s syndrome, in literature also known under the name CLAS (Congenital Localised Absence of Skin), first described by Bart in 1966 as congenital localized absence of skin, epidermolysis bullosa congenita and nail abnormalities. The authors present a macroscopic and histological findings of a newborn with Bart‘s syndrome, with epidermolysis bullosa junctionalis and atresia pylori, who died 17 days after birth and 13 days after surgery for pyloric stenosis.

Key words:
Bart´s syndrome – atresia pylori – epidermolysis bullosa junctionalis – CLAS

Zdroje

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