This article considers the case of a 22-year-old female with congenital disorder of glycosylation type Ib and a history of congenital hepatic fibrosis, portal hypertension and esophageal varices, who presented with edema, diarrhea, hypoalbuminemia and pancytopenia. Protein-losing enteropathy was diagnosed. As previous mannose therapy was associated with diarrhea and abdominal pain, albumin was infused followed by intravenous and subcutaneous therapy with unfractionated heparin.
- Ylian S Liem
- Lars Bode
- JH Paul Wilson
