Clinical and pathological features of Alport syndrome in children / 中国当代儿科杂志
Chinese Journal of Contemporary Pediatrics
; (12): 188-191, 2010.
Article
en Zh
| WPRIM
| ID: wpr-270392
Biblioteca responsable:
WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To study the clinical and pathological features of Alport syndrome in children.</p><p><b>METHODS</b>The clinical and histopathological data of 10 hospitalized children with Alport syndrome from February 2007 to February 2009 were retrospectively reviewed.</p><p><b>RESULTS</b>There were 7 males and 3 females, with the age ranging from 2 years to 6 years and 7 months (mean 3 years and 2 months). Five of 10 cases had positive family history. X-linked dominant inheritance Alport syndrome was diagnosed in 8 cases, and autosomal recessive inheritance Alport syndrome in 2 cases. Recurrent gross hematuria was found in 5 cases, hematuria and proteinuria in 3 cases, massive proteinuria in 1 case, and nephritic syndrome in 1 case. Under the light microscope, 8 cases presented with mesangial proliferation glomerulonephritis, and 2 cases with focal segmental glomerulosclerosis. Immunofluorescence assay showed that all cases had IgM deposition in glomerulus. Only 1 case showed typical glomerular basement membrane (GBM) pathological changes. All cases showed abnormal alpha-chain distribution in renal collagen IV.</p><p><b>CONCLUSIONS</b>The children with Alport syndrome have diverse clinical manifestations. Characteristic histopathological presentations could not be found under a light microscope, mesangial proliferation glomerulonephritis is the dominant pathological change, and IgM deposition in glomerulus is common. The GBM pathological change in children is not common. Immunofluorescence assay of alpha-chain in collagen IV is needed for the diagnosis of Alport syndrome.</p>
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Índice:
WPRIM
Asunto principal:
Patología
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Colágeno Tipo IV
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Diagnóstico
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Genética
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Riñón
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Nefritis Hereditaria
Tipo de estudio:
Diagnostic_studies
Límite:
Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
Zh
Revista:
Chinese Journal of Contemporary Pediatrics
Año:
2010
Tipo del documento:
Article