Clinical and biochemical characterization of a patient with congenital disorder of glycosylation (CDG) IIx.

Miura, Yoshiaki; Tay, Stacey K H; Aw, Marion M; Eklund, Erik A; Freeze, Hudson H · J Pediatr · 2005

case_report · Level V

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Abstract

We describe a case of congenital disorder of glycosylation with chronic diarrhea, progressive liver cirrhosis, and recurrent infections. Transferrin analysis showed only hyposialylation, but analysis of total serum N-glycans indicated loss of additional sugars, arguing that the latter generates a more informative picture to search for the primary defect.

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