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Introduction: In Venezuela has been described a new form of nephronophthisis, called adolescent nephronophthisis, with clinical and histological findings very similar to others varieties described. However, pathogenesis in not well known. The aim of this study was to determine the expression of human epidermal growth factor receptor (EGFR) in tubular epithelial cells of patients with adolescent nephronophthisis., Methods: Renal biopsies of 8 patients with adolescent nephronophthisis were studied by immunohistochemistry to determine renal expression of EGFR., Results: In all patients, there was no expression of epidermal growth factor receptor., Conclusion: These findings indicate a deficiency of growth factor receptor in undifferentiated epithelial cells, which could be one factor in the development of cysts in nephronophthisis.

Objective: Renal autosomal recessive policystic disease is responsible for a great number of deaths among affected individuals (mainly prenatal and neonatal forms)., Methods: We report the necropsy studies of two successive newborns from the same mother with such disease., Results: We analyze the macroscopic and microscopic characteristics of this cystic disease., Conclusions: Those children that make it through the breast-feeding period will surely suffer from the associated liver pathology.