5岁男孩,因多饮多尿、间断肉眼血尿、发热年余,吐泻一月入院。每日饮水量4000ml,尿量3000ml,多次按“肾炎”治疗无好转,且进行性消瘦,乏力。体检:体重13公斤,发育差,神萎,慢性消耗面容,皮肤干燥,皮下脂肪消失,心肺无异常,肝右肋下1cm,肾区无叩痛,腱反射减弱,Hb103g/L,WDC27×10~9/L,尿红细胞0～6/HP,白细胞++,尿糖阴性,尿pH8.0,血浆总蛋 5-year-old boy, due to drink more urine, intermittent gross hematuria, fever more than a year, vomiting and diarrhea January admission. Daily water volume 4000ml, urine output 3000ml, repeatedly by “nephritis” treatment without improvement, and progressive weight loss, fatigue. Physical examination: weight 13 kg, poor development, atrophy, chronic depletion of the face, dry skin, subcutaneous fat disappeared, no abnormal heart and lung, liver right rib 1cm, no percussion pain in the kidney area, tendon reflexes, Hb103g / L, WDC27 × 10 ~ 9 / L, urinary red blood cells 0 ~ 6 / HP, white blood cells ++, urine negative urine pH8.0, the total plasma egg