先天性肾性尿崩症临床罕见。传统的观点认为本病常由男性发病,而由表型正常的女性携带和传递病理基因。近发现一个特殊家系,表现为男女性同等发病,且可通过表型不正常的男性将病理基因传递其子女。 Congenital renal diabetes insipidus is rare. The traditional view that the disease is often caused by men, while the normal phenotype of women carry and pass the pathological genes. A special pedigree was found that showed the same incidence of both men and women and passed the pathology gene to their children through men with abnormal phenotypes.