Mechanism of cystogenesis in nephrotic kidneys: a histopathological study

BMC Nephrology

Mirna Saraga-Babić²  Danica Galešić Ljubanović⁵  Bernd Dworniczak⁶  Stefanie Weber³  Merica Glavina Durdov⁸  Ivana Bočina¹  Zvonimir Puretić⁴  Vjekoslav Krželj⁷  Katarina Vukojević²  Marijan Saraga⁷ 

[1]Department of Biology, University of Split, Split, Croatia

[2]Department of Anatomy, Histology and Embryology, School of Medicine, University of Split, Split, Croatia

[3]Division of Pediatric Nephrology, University Children’s Hospital Essen, Essen, Germany

[4]Department of Dialysis, University Hospital Centre Zagreb, Zagreb, Croatia

[5]Department of Pathology, University Hospital in Zagreb, Zagreb, Croatia

[6]Department of Human Genetics, University Münster, Münster, Germany

[7]Department of Paediatrics, University Hospital in Split, Split, Croatia

[8]Department of Pathology, University Hospital in Split, Split, Croatia

关键词: Apoptosis;    Primary cilia;    Cell proliferation;    FSGS;    CNF;    Cystogenesis;    Nephrotic kidney;   

Others  :  1082755
DOI  :  10.1186/1471-2369-15-3

 received in 2013-06-27, accepted in 2014-01-02,  发布年份 2014

【 摘 要 】

Background

Nephrotic syndrome (NS) is pathological condition characterized by heavy proteinuria. Our study investigates hypothesis that change in cell proliferation of proximal tubules influences primary cilia structure and function and promotes cystogenesis in congenital nephrotic syndrome of the Finnish type (CNF) and focal segmental glomerulosclerosis (FSGS).

Methods

CNF kidneys were analyzed genetically. Proliferation (Ki-67), apoptosis (caspase-3), and primary cilia (α-tubulin) length and structure were analyzed immunohistochemically and ultrastructurally in healthy, CNF and FSGS kidneys. Cyst diameters were measured and correlated with proliferation index.

Results

Proximal tubules cells of healthy kidneys did not proliferate. In nephrotic kidneys, tubules with apparently normal diameter covered by cuboidal/columnar epithelium (PTNC) contained 81.54% of proliferating cells in CNF and 36.18% in FSGS, while cysts covered with columnar epithelium (CC) contained 37.52% of proliferating cells in CNF and 45.23% in FSGS. The largest cysts, covered with squamous epithelium (CS) had 11.54% of proliferating cells in CNF and 13.76% in FSGS. Increase in cysts diameter correlated with changes in proliferation index, tubular cells shape, primary cilia formation and appearance of apoptotic cells.

Conclusions

We present a novel histopathological data on the structure and possible changes in function of tubular cell in NS kidneys during cystogenesis. We suggest existence of common principles of cystogenesis in CNF and FSGS kidneys, including serious disturbances of tubular cells proliferation and apoptosis, and faulty primary cilia signaling leading to deterioration of proteinuria in NS kidneys.

【 授权许可】

   
2014 Saraga et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

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