Document Detail


Erdheim-Chester syndrome, presenting as hypogonadotropic hypogonadism and diabetes insipidus.
MedLine Citation:
PMID:  12240906     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Erdheim-Chester syndrome is a rare multisystem disease in which progressive xanthogranulomatous infiltration of several tissues are seen. Knee and leg pain are the most common symptoms and bilateral symmetric sclerosis of metaphyseal region of long bones of the lower extremity is typical. Histologically, it resembles Langerhans cell histiocytosis (LCH). However, it is still a matter of discussion whether Erdheim-Chester syndrome is a distinct entity or a type of LCH. The present case is a 46-yr-old man, that presented with signs and symptoms of diabetes insipidus and hypogonadotropic hypogonadism simultaneously. X-rays and bone scintigraphy showed typical and pathogonomic findings of Erdheim-Chester syndrome. Bone biopsy and immunohistochemical staining strongly support the diagnosis of non-Langerhans cell histiocytosis.
Authors:
M E Khamseh; S Mollanai; F Hashemi; A Rezaizadeh; F Azizi
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of endocrinological investigation     Volume:  25     ISSN:  0391-4097     ISO Abbreviation:  J. Endocrinol. Invest.     Publication Date:  2002 Sep 
Date Detail:
Created Date:  2002-09-20     Completed Date:  2003-04-15     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7806594     Medline TA:  J Endocrinol Invest     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  727-9     Citation Subset:  IM    
Affiliation:
Endocrine Research Center, Shaheed Beheshti University of Medical Sciences, Tehran, IR Iran.
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MeSH Terms
Descriptor/Qualifier:
Diabetes Insipidus / etiology*
Granuloma / complications*,  radiography,  radionuclide imaging
Humans
Hypogonadism / etiology*
Male
Middle Aged
Syndrome
Xanthomatosis / complications*,  radiography,  radionuclide imaging

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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