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Pathol Res Pract. 2010 Jan 15;206(1):14-8. doi: 10.1016/j.prp.2009.06.012. Epub 2009 Jul 18.

Non-specific histopathological changes in kidney with renal tubular dysgenesis.

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Department of Pathology, Ziv Medical Center, Zefat, Israel.


Absence of proximal convoluted tubules (APCT) is a specific pathomorphological change found in kidney with renal tubular dysgenesis (RTD). Non-specific structural abnormalities in the kidney with this disorder have rarely been reported. The aim of this study was to detect non-specific histopathological changes (NSHC) in RTD kidney, to evaluate their incidence, and to establish a possible relationship to various etiological-pathogenic variants of RTD. Kidneys of 12 patients with RTD diagnosed in 9 Israeli hospitals were studied. Paraffin sections were examined using histological, histochemical, and immunohistochemical stains as well as morphometry. APCT was found to be associated with microcalcifications (MC) (66.6%), medullary ray nodules (MRN) (16.6%), extramedullary hematopoiesis (EH) (16.6%), and multinuclear giant cell (MGC) reaction (8.3%). MC contained calcium oxalate (33.3%) and calcium phosphate (carbonate) (33.3%), and were predominantly formed prenatally. MRN were also formed before birth. Definitive differences regarding NSHC character and frequency were found in diverse etiological-pathogenic variants of RTD. APCT in kidney with RTD is not infrequently associated with histopathological changes, which have extremely rarely been reported in prenatal and early postnatal kidney. MRN and EH in an autosomal recessive (AR) variant of RTD are more frequent than in twin-twin transfusion (TTT) syndrome.

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