Severe holocarboxylase synthetase deficiency with incomplete biotin responsiveness resulting in antenatal insult in samoan neonates.

Wilson, Callum J; Myer, Michael; Darlow, Brian A; Stanley, Thorsten; Thomson, Glen; Baumgartner, E Regula; Kirby, Denise M; Thorburn, David R · J Pediatr · 2005

case_report · Level V

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Abstract

We describe 7 Polynesian babies with a unique severe form of holocarboxylase synthetase deficiency characterized by antenatal growth retardation, subependymal cysts, only partial response to biotin, and a poor outcome.

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