| C1q nephropathy in two young sisters. | |
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MedLine Citation:
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PMID: 17952693 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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C1q nephropathy (C1qNP) is a controversial and uncommon form of glomerulonephritis, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. Clinically, it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microhematuria, hypertension, or renal insufficiency. We describe two sisters with C1qNP, who presented with steroid-resistant nephrotic syndrome. Both sisters presented before the age of 2 years, and they showed a poor response to other immunosuppressive therapy. Both girls had normal serum complement levels, negative antinuclear antibodies (ANAs) and negative hepatitis B antigen. Renal biopsy in both patients showed histological features of mesangioproliferative glomerulonephritis, with diffuse "full-house" positive immunofluorescence reaction in the mesangial area. The immunofluorescence reaction for C1q was most intense and co-dominant with IgG in both patients. Correspondingly, electron microscopy demonstrated dense deposits mainly in the mesangial areas too. We report on two young sisters with the characteristic features of C1qNP presented in early childhood. To the best of our knowledge, this is the first report of C1qNP in siblings. |
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Authors:
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Jameela A Kari; Sawsan M Jalalah |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2007-10-21 |
Journal Detail:
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Title: Pediatric nephrology (Berlin, Germany) Volume: 23 ISSN: 0931-041X ISO Abbreviation: Pediatr. Nephrol. Publication Date: 2008 Mar |
Date Detail:
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Created Date: 2008-01-28 Completed Date: 2008-07-15 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8708728 Medline TA: Pediatr Nephrol Country: Germany |
Other Details:
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Languages: eng Pagination: 487-90 Citation Subset: IM |
Affiliation:
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Pediatrics Department, King AbdulAziz University Hospital, P.O. Box 80215, Jeddah 21589, Saudi Arabia. jkari@doctors.org.uk |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Complement C1q*
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metabolism Female Glomerulonephritis / metabolism, pathology* Humans Infant |
| Chemical | |
Reg. No./Substance:
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80295-33-6/Complement C1q |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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