| A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency. | |
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MedLine Citation:
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PMID: 20221776 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A 14-year-old girl presented with acute glomerulonephritis. Tests revealed hypocomplementemia and elevated Antistreptolysin-O titers, and renal biopsy revealed endocapillary and mesangial proliferative glomerulonephritis with double contours of the glomerular basement membrane (GBM). Despite methylprednisolone pulse therapy and the administration of oral prednisolone, overt proteinuria and hypocomplementemia persisted. A second renal biopsy 6 months later confirmed the initial diagnosis of dense deposit disease (DDD) based on electron-dense deposits in the GBM. C3 nephritic factor (C3NeF) and a deficiency of complement factor H (CFH) were not evident. A nephritis-associated plasmin receptor (NAPlr), nephritogenic group A streptococcal antigen, and the plasmin activity by in situ zymography were been in both the first and second biopsy specimens. The patient received combined immunomodulatory therapy with prednisolone and mizoribine, and the urinary protein decreased to a mild level at 27 months after disease onset. These findings suggest that persistent glomerular NAPlr deposition may be associated with the pathogenesis of DDD in some patients without the involvement of C3NeF or CFH mutation and that DDD patients of this type may respond to immunomodulatory treatment. |
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Authors:
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Kenichi Suga; Shuji Kondo; Sato Matsuura; Yukiko Kinoshita; Etsuko Kitano; Michiyo Hatanaka; Hajime Kitamura; Yoshihiko Hidaka; Takashi Oda; Shoji Kagami |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2010-03-11 |
Journal Detail:
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Title: Pediatric nephrology (Berlin, Germany) Volume: 25 ISSN: 1432-198X ISO Abbreviation: Pediatr. Nephrol. Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-06-18 Completed Date: 2010-10-04 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: 1547-50 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, The Institute of Health Bioscience, The University of Tokushima Graduate School, Tokushima University, Tokushima city, Tokushima, 770-8503, Japan. aksugajp@yahoo.co.jp |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Biopsy / adverse effects Complement C3 Nephritic Factor / metabolism Complement Factor H / diagnostic use, metabolism Female Follow-Up Studies Glomerulonephritis / complications, etiology, pathology Glomerulonephritis, Membranoproliferative / complications, pathology* Humans Immunologic Deficiency Syndromes / complications, metabolism, pathology Immunomodulation Kidney Glomerulus / metabolism, pathology Nephritis / complications, metabolism, pathology Prednisolone / diagnostic use, metabolism Proteinuria / complications, metabolism, pathology Receptors, Peptide Streptococcal Infections / complications, immunology Time Factors |
| Chemical | |
Reg. No./Substance:
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0/Complement C3 Nephritic Factor; 0/Receptors, Peptide; 0/plasmin receptor; 50-24-8/Prednisolone; 80295-65-4/Complement Factor H |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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