Korean J Gastrointest Endosc.  2006 Nov;33(5):307-312.

A Case of Protein Losing Enteropathy Caused by Primary Intestinal Lymphangiectasia

• Lee SY
• Yeo JC
• Youn YD
• Kim SR
• Kwon YL
• Ryu HU
• Kim JC
• Lee MK
• Park CK
• Lee SM

Abstract

Primary intestinal lymphangiectasia is a rare congenital cause of protein losing enteropathy that is characterized by chronic diarrhea, generalized edema, ascites, hypoproteinemia, hypoalbuminemia, and lymphopenia. We encountered an 18-year-old woman who suffered from longstanding diarrhea and progressive leg edema. The laboratory findings showed the typical features of this disorder. The presence of enteric protein loss was documented with the 24 hour fecal clearance of alpha(1)-antitrypsin and (99m)Tc human serum albumin scintigraphy. A duodenoscopy and biopsy showed scattered white spots and markedly dilated lymphatics in the tips of the villi, respectively. The patient's clinical symptoms improved after placing her on a high protein and low fat diet with medium chain triglyceride supplements.

Keyword

Protein losing enteropathy; Primary intestinal lymphangiectasia; (99m)Tc human serum albumin scintigraphy; alpha(1)-antitrypsin clearance

MeSH Terms

Adolescent
Ascites
Biopsy
Dental Caries
Diarrhea
Diet
Duodenoscopy
Edema
Female
Humans
Hypoalbuminemia
Hypoproteinemia
Leg
Lymphopenia
Protein-Losing Enteropathies*
Radionuclide Imaging
Serum Albumin
Triglycerides
Serum Albumin