Severe hypoglycemia as a presenting symptom of carbohydrate-deficient glycoprotein syndrome.

Babovic-Vuksanovic, D; Patterson, M C; Schwenk, W F; O'Brien, J F; Vockley, J; Freeze, H H; Mehta, D P; Michels, V V · J Pediatr · 1999

case_report · Level V

Where this comes from

Abstract

We describe clinical, biochemical, and molecular findings in a 2(1/2)-year-old girl with a phosphomannose isomerase deficiency who presented with severe and persistent hypoglycemia and subsequently developed protein-losing enteropathy, liver disease, and coagulopathy. Six months of therapy with mannose supplementation resulted in clinical improvement and partial correction of biochemical abnormalities.

Medical subject headings