Using heparin therapy to reverse protein-losing enteropathy in a patient with CDG-Ib.
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.


METHODS
Laboratory examinations, abdominal ultrasonography, bacterial and viral cultures of blood, urine and stools, double-balloon enteroscopy and fecal excretion test using 51Cr-labeled albumin.


METHODS
Protein-losing enteropathy.


RESULTS
Infusion of albumin followed by intravenous and subcutaneous therapy with unfractionated heparin.
Liem
Ylian S
YS
Department of Internal Medicine, Erasmus University Medical Center, Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands.
Bode
Lars
L
Freeze
Hudson H
HH
Leebeek
Frank Wg
FW
Zandbergen
Adrienne Am
AA
Paul Wilson
Jh
J
D000328:Adult; D000418:Albumins / Q000008:administration & dosage*; D018981:Congenital Disorders of Glycosylation / Q000145:classification / Q000150:complications; D005260:Female; D006493:Heparin / Q000008:administration & dosage*; D006801:Humans; D011504:Protein-Losing Enteropathies / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D016896:Treatment Outcome
D000418:Albumins; D006493:Heparin