Abstract: Abstract: Congenital nephrotic diabetes insipidus is a group of genetically heterogeneous single gene diseases,accounting for about 10% of all nephrotic diabetes insipidus. The main reason is that the kidney loses its response to arginine vasopressin secreted by the posterior pituitary gland, which results in dysfunction of urine concentration. Patients are characterized by excretion of large amounts of low specific gravity urine, dehydration, thirst and secondary polydipsia. Xlinked recessive inheritance accounts for about 90% of patients. About 10% of patients are autosomal dominant or recessive inheritance. In recent years, its genetic and molecular mechanisms have been gradually elucidated, and multiple target therapies and potential therapeutic strategies for the etiology have also emerged. This article reviews the research status of congenital nephrotic diabetes insipidus.

Key words: diabetes insipidus,, nephrogenic, vasopressins, aquaporin 2, review