Clinical and morphological aspects of thin glomerular basement membrane disease. 2009

Jakub Zurawski, and Wiesława Salwa-Zurawska, and Aldona Woźniak, and Elzbieta Bortkiewicz, and Janusz Maciejewski, and Ilona Idasiak-Piechocka, and Bartosz Urbański
Department of Clinical Pathomorphology, Karol Marcinkowski Medical University in Poznań, Poznań.

The report presents the results of investigations carried out in 63 patients (49 children and 14 adults) with thin glomerular basement membrane disease. Of 49 children, 13 came from nine families with family members suffering from kidney diseases (mostly manifested by haematuria). In the group of children, the most frequent initial clinical symptom (noted in 29 cases) was isolated haematuria, more rarely (in eight cases) haematuria and proteinuria, and (in seven cases) nephrotic syndrome. Isolated proteinuria was observed in another three children. In the adults, only in five patients was isolated haematuria the initial symptom of the disease; more frequently (in seven cases), they presented with isolated proteinuria. In two patients, proteinuria and haematuria were noted. In the two groups of patients, in addition to markers of thin glomerular basement membrane disease, the authors also observed markers indicative of other glomerulopathies: in seven children and four adults focal segmental glomerulosclerosis (FSGS) without any other glomerular pathologies (with the exception of thin glomerular basement membrane disease). Also in cases in which in addition to thin glomerular basement membrane disease other glomerular pathologies were present (mesangial hypercellularity, mesangial glomerulonephritis), matrix expansion was detected; this phenomenon might be considered a harbinger of glomerular sclerosis. In general, although not in each and every case, these pathologies were associated with duration of the disease.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007674 Kidney Diseases Pathological processes of the KIDNEY or its component tissues. Disease, Kidney,Diseases, Kidney,Kidney Disease
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011507 Proteinuria The presence of proteins in the urine, an indicator of KIDNEY DISEASES. Proteinurias
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005109 Extracellular Matrix A meshwork-like substance found within the extracellular space and in association with the basement membrane of the cell surface. It promotes cellular proliferation and provides a supporting structure to which cells or cell lysates in culture dishes adhere. Matrix, Extracellular,Extracellular Matrices,Matrices, Extracellular
D005260 Female Females
D006417 Hematuria Presence of blood in the urine. Hematurias

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