| Diagnosis of congenital nephrotic syndrome: a clinical and a pathologic challenge. | |
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MedLine Citation:
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PMID: 12556291 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The diagnosis of congenital nephrotic syndrome (NS) is a challenge both for clinicians and for pathologists. We observed three cases in a series of 50 children with NS nonresponsive to therapy, corresponding to one case each of minimal change disease, Finnish-type glomerulopathy, and diffuse mesangial sclerosis--two histopathologic studies were performed in each case. The age at presentation did not predict the diagnosis nor the prognosis: The NS presented at 7 months of age in the patient with diffuse mesangial sclerosis, but it was present at birth in the patient with minimal change disease. In these 2 patients the final diagnosis was made with the first renal biopsy. Conversely, in the patient with Finnish-type glomerulopathy, the diagnosis was only possible in the repeat biopsy, as the early pathologic changes were nonspecific. This study shows the essential role of the renal biopsy in determining the etiologic diagnosis and prognosis in patients with congenital nephrotic syndrome. congenital nephrotic syndrome diffuse mesangial sclerosis Finnish-type glomerulopathy |
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Authors:
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Ada Rivera; Suzanne Meleg-Smith; Igor Yosipiv; Samir El-Dahr; Frank Boineau |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Pediatric pathology & molecular medicine Volume: 22 ISSN: 1522-7952 ISO Abbreviation: Pediatr Pathol Mol Med Publication Date: 2003 Mar-Apr |
Date Detail:
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Created Date: 2003-01-30 Completed Date: 2003-09-11 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 100885435 Medline TA: Pediatr Pathol Mol Med Country: United States |
Other Details:
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Languages: eng Pagination: 105-16 Citation Subset: IM |
Affiliation:
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Department of Pathology and Laboratory Medicine, Tulane University Health Sciences Center, New Orleans, Louisiana 70112-2699, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Biopsy Child, Preschool Glomerular Mesangium / pathology Humans Infant Infant, Newborn Kidney Diseases / diagnosis, pathology Kidney Glomerulus / pathology Male Nephrotic Syndrome / congenital, diagnosis*, pathology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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