| Ten-year course of early-onset Weber-Christian syndrome with recurrent pneumonia: a suggestion for pathogenesis. | |
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MedLine Citation:
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PMID: 3725481 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A surviving 10-year-old boy with infant-onset systemic Weber-Christian syndrome is reported. He has had recurrent episodes of fever, aseptic panniculitis, and pneumonia. Although corticosteroid therapy has succeeded, colchicine and non-steroidal anti-inflammatory drugs have failed to abort or prevent acute episodes. The persistent leukocytosis (even during remission), the recurrent episodes of fever with associated increase in the acute phase reactants, and the failure of the nonsteroidal anti-inflammatory drugs leads us to propose that this form of Weber-Christian syndrome reflects an inborn error in the regulation of the inflammatory response. Systematic investigation of this hypothesis could yield important information on the normal regulation of inflammation and could lead to a rational therapeutic approach to this puzzling and usually devastating illness. |
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Authors:
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R U Sorensen; C R Abramowsky; R C Stern |
Publication Detail:
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Type: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Pediatrics Volume: 78 ISSN: 0031-4005 ISO Abbreviation: Pediatrics Publication Date: 1986 Jul |
Date Detail:
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Created Date: 1986-08-11 Completed Date: 1986-08-11 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0376422 Medline TA: Pediatrics Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 115-20 Citation Subset: AIM; IM |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Child Combined Modality Therapy Humans Leukocytosis / complications Male Panniculitis, Nodular Nonsuppurative / complications*, congenital, therapy Pneumonia / complications*, radiography Skin / pathology |
| Grant Support | |
ID/Acronym/Agency:
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AI 19970/AI/NIAID NIH HHS; RR 00080-21/RR/NCRR NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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