| Nail-patella syndrome and IgA nephropathy in a Chinese woman. | |
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MedLine Citation:
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PMID: 10643004 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Nail-patella syndrome (NPS), also known as hereditary onycho-osteodysplasia, is an autosomal dominant pleiotropic disorder characterized by nailbed dysplasia or hypoplasia, absent or hypoplastic patellae, iliac horns and deformation or luxation of the radial head. Nephropathy is a known serious complication associated with NPS. In this report, we describe an adult Chinese woman with the clinical and radiological features of NPS who presented with the nephrotic syndrome. Renal biopsy disclosed focal segmental glomerulosclerosis on light microscopy, while immunofluorescence revealed predominant staining for IgA in the glomerular mesangium and along some capillary walls. Ultrastructural study confirmed the presence of paramesangial deposits as well as subendothelial collagen fibrils in the glomeruli. The histological findings were those of combined NPS and IgA disease, an association which has rarely been described. |
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Authors:
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K L Chuah; P H Tan; H L Choong; D Lai; G Chiang |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Pathology Volume: 31 ISSN: 0031-3025 ISO Abbreviation: Pathology Publication Date: 1999 Nov |
Date Detail:
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Created Date: 2000-02-04 Completed Date: 2000-02-04 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0175411 Medline TA: Pathology Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 345-9 Citation Subset: IM |
Affiliation:
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Department of Pathology, Singapore General Hospital, Singapore. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult China / ethnology Female Glomerular Mesangium / chemistry, ultrastructure Glomerulonephritis, IGA / complications, diagnosis*, ethnology Glomerulosclerosis, Focal Segmental / pathology Humans Immunoglobulin A / analysis Knee Joint / radiography Microscopy, Fluorescence Nail-Patella Syndrome / complications, diagnosis*, ethnology Nephrotic Syndrome / ethnology, etiology Patella / radiography |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulin A |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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