[Clinical and pathological features and the misdiagnosis of childhood Alport syndrome: a retrospective analysis of 91 cases].

Objective: To explore the clinical and pathological features and the diagnosis of childhood Alport syndrome (AS).

Methods: A retrospective analysis was performed on clinical data of 91 children with AS.

Results: Hematuria was observed in all 91 patients, of whom 86 were accompanied with proteinuria.

[Clinical and pathological features of Denys-Drash syndrome: report of 3 cases].

Objective: To study the clinical and pathological features of Denys-Drash syndrome (DDS).

Method: Three DDS cases who were treated in our department from December 2009 to June 2011 were subjected to this study by reviewing of literature.

Result: Both case 1 and case 2 were female, with karyotype 46, XX.

Expression and significance of TGF-beta1/Smad signaling pathway in children with IgA nephropathy.

Background: IgA nephropathy (IgAN) exhibits an indolent but slowly progressive course, and about 30% of children with IgAN are found to deteriorate to end-stage renal failure characterized by overaccumulation of extracellular matrix, diffuse glomerular sclerosis, and tubulointerstitial fibrosis. The TGF-beta/Smad signaling pathway plays an important role in glomerulosclerosis and tubulointerstitial fibrosis. The present study aimed to elucidate the significance of expressions of TGF-beta1, phosphorylated Smad3 (p-Smad3), Smad7 and fibronectin (FN) in the renal tissue of children with IgAN.