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Using heparin therapy to reverse protein-losing enteropathy in a patient with CDG-Ib
Ylian S Liem1, Lars Bode, Hudson H Freeze
1Department of Internal Medicine, Erasmus University Medical Center, Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands.
Background:
A 22-year-old female presented with edema, diarrhea, hypoalbuminemia and pancytopenia. She had previously been diagnosed with congenital disorder of glycosylation type Ib, and had a history of congenital hepatic fibrosis, portal hypertension and esophageal varices. In the past she had refused mannose therapy because of associated diarrhea and abdominal pain.
Investigations:
Laboratory examinations, abdominal ultrasonography, bacterial and viral cultures of blood, urine and stools, double-balloon enteroscopy and fecal excretion test using 51Cr-labeled albumin.
Diagnosis:
Protein-losing enteropathy.
Management:
Infusion of albumin followed by intravenous and subcutaneous therapy with unfractionated heparin.
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