A 46-year-old man with polyneuropathy, hepatosplenomegaly, endocrinopathy,monoclonal gammopathy, skin changes, sclerotic bone lesions and refractoryascites

Citation
Eg. Siegel et Ur. Folsch, A 46-year-old man with polyneuropathy, hepatosplenomegaly, endocrinopathy,monoclonal gammopathy, skin changes, sclerotic bone lesions and refractoryascites, Z GASTROENT, 37(4), 1999, pp. 283-286
Citations number
17
Categorie Soggetti
Gastroenerology and Hepatology
Journal title
ZEITSCHRIFT FUR GASTROENTEROLOGIE
ISSN journal
00442771 → ACNP
Volume
37
Issue
4
Year of publication
1999
Pages
283 - 286
Database
ISI
SICI code
0044-2771(199904)37:4<283:A4MWPH>2.0.ZU;2-6
Abstract
The POEMS syndrome is a rare multisystemic disorder with polyneurppathy. or ganomegaly. endocrinopathy of various forms, production of monoclonal (M) c omponent, and skin changes. We describe a 46-year-old man who developed asc ites one year after the onset of peripheral neuropathy with accompanying mu scle atrophies and increasing weakness. Extensive evaluation revealed that the patient had no underlying liver disease, malignancy. infection, or card iac or renal disease. The ascites initially responded to high-dose corticos teroid therapy. The patient had many clinical features of the described POE MS syndrome: Including sclerotic bone lesions. a persistent lambda-paraprot ein and refractory ascites. In this case ascites was a main presenting feat ure. Thus, the POEMS syndrome must be added to the list of rare causes of r efractory ascites.