| Diabetes insipidus is a clinical syndrome characterized by excretion of large volumes of dilute urine due to deficiency and reduction of AVP secretion (central diabetes insipidus, CDI) or renal insensitive to AVP (nephrogenic diabetes insipidus, NDI). The main clinical manifestations are persistent polyuria, thirst and polydipsia due to the consequence of dehydration, which can results in some severe complications such as shock, mental retardation, growth retardation and pyelectasis. The CDI patients might be life-threatened if accompanied with tumors in sella region and hypo-function of anterior pituitary. The objective of this study is to analyze the various causes and clinical features of diabetes insipidus, the relationship between prognosis and different treatments and the mutations of AVP related genes (AVP-NPII and AVPR2) detection in patients with diabetes insipidus.We retrospectively analyzed the clinical data of 408 patients with central diabetes insipidus (accounting for 96% of all diabetes insipidus,n=425) which were diagnosed in the department of endocrinology in Peking Union Medical Collage Hospital from 1956 to 2000. In group of children and adolescents (n=211), most of the onset age was between 8 to 12 years (average age 9.5 ±4.8yrs); in adult group (n=197), most of onset age was between 25 to 35 years (average age 35.7 ±10.3yrs). In adult group, idiopathic CDI accounted for 52%; tumor in sella region caused CDI accounted for 22% and Trauma-induced CDI accounted for 11%. In child and adolescent group, tumor in sella region caused CDI accounted for about 33% , among which germinoma made up about 65%, Trauma-induced CDI accounted for 5% and 8 histiocytosis X were all children, accounting for 3%. A two-year follow-up is helpful for patients whose diagnosis were undetermined at first visit (n=35), because 86% of them had got their final diagnosis during this period. Radiotherapy is the most effective method for gerninoma in sella region. The long-term (3... |
PDF Full Text Request