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先天性肾原性尿崩症少见,国内仅见一个家系报道[王维力,等。中华内科杂志1984;23(4):206.],现将我院所见父女2例报道如下: 例1,男60岁,住院号102414,1986年5月9日入院。患者曾于3岁时因烦渴而偷饮大量淘米水引起家人注意。13岁出现多食。18岁入伍后,常因水源紧缺而饮用稻田或水沟中之泥浆水解渴。24岁时曾住院一次,记录到每日尿量达18升。28岁起体重激增,手足增粗、增大,在至38岁的十年中,鞋码由原先的39增至43。现每日尿量仍在10升以上,饭量1000克/日。无智力障碍,惟时感胸闷、乏力、心悸、思睡。无头颅外伤和脑炎史。30岁结婚,生育二男一女,其女有类似症状,二子正常。
Congenital nephrogenic diabetes insipidus rare, only one pedigree reported in China [Wang Weili, et al. Chinese Journal of Internal Medicine 1984; 23 (4): 206.], Now my hospital see 2 cases of father and daughter reported as follows: Example 1, male 60 years old, hospital number 102414, May 9, 1986 admission. Patient was 3 years old because of polydipsia and steal a large number of Taomi Shui cause family attention. 13-year-old appear to eat more. After joining the army at age 18, they often drink thirsty muddy water from paddy fields or ditches due to lack of water. At the age of 24, she was hospitalized and recorded a daily urine output of 18 liters. 28-year-old body weight surge, hand and foot thickening, increased in the 10-year-old to 38 years, from the original 39 to 43 shoes. Daily urine output is still above 10 liters, appetite 1000 grams / day. No mental retardation, but the time feeling chest tightness, fatigue, palpitations, thinking sleep. No history of cranial trauma and encephalitis. 30-year-old married, giving birth to two men and a woman, their women have similar symptoms, two normal.