| Erdheim-Chester syndrome, presenting as hypogonadotropic hypogonadism and diabetes insipidus. | |
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MedLine Citation:
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PMID: 12240906 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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M E Khamseh; S Mollanai; F Hashemi; A Rezaizadeh; F Azizi |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of endocrinological investigation Volume: 25 ISSN: 0391-4097 ISO Abbreviation: J. Endocrinol. Invest. Publication Date: 2002 Sep |
Date Detail:
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Created Date: 2002-09-20 Completed Date: 2003-04-15 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 7806594 Medline TA: J Endocrinol Invest Country: Italy |
Other Details:
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Languages: eng Pagination: 727-9 Citation Subset: IM |
Affiliation:
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Endocrine Research Center, Shaheed Beheshti University of Medical Sciences, Tehran, IR Iran. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Diabetes Insipidus
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etiology* Granuloma / complications*, radiography, radionuclide imaging Humans Hypogonadism / etiology* Male Middle Aged Syndrome Xanthomatosis / complications*, radiography, radionuclide imaging |
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