PDF(Pubmed)
Abstract：
Congenital nephrogenic diabetes insipidus (CNDI) is a rare disease that results in polyuria due to decreased responsiveness to the antidiuretic hormone in the collecting ducts of the kidney. Without compensation by drinking large amounts of water, dehydration and hypernatremia can rapidly develop. We present a case of a patient originally diagnosed with CNDI who required surgery and a fasting period due to adhesive bowel obstruction. The patient was a 46-year-old man who was originally diagnosed with CNDI. He was prescribed trichlormethiazide but self-discontinued treatment in the process. His normal urine output was about 7,000-8,000 mL/day. He underwent robot-assisted radical cystectomy and uretero-cutaneostomy for bladder cancer. Two years later, he was hospitalized due to adhesive bowel obstruction. A 5% glucose solution was infused, and the dose was adjusted according to the urine volume and electrolytes. An adhesiotomy was performed due to recurrent bowel obstruction in a short period of time. A 5% glucose solution was used as the main infusion during the perioperative period. Once drinking water was resumed after surgery, urinary output and electrolytes were easily controlled. In conclusion, patients with CNDI should be given a 5% glucose solution as the primary infusion, and the infusion volume should be adjusted by monitoring daily urine output, electrolytes, and blood glucose levels. Infusion management is easier if oral intake is initiated as early as possible.
摘要：
先天性肾性尿崩症（CNDI）是一种罕见的疾病，由于对肾脏集合管中的抗利尿激素的反应性降低，导致多尿。喝大量的水没有补偿，脱水和高钠血症可以迅速发展。我们介绍了一例最初诊断为CNDI的患者，该患者因粘连性肠梗阻而需要手术和禁食期。患者是一名46岁的男子，最初被诊断患有CNDI。他被开了三氯噻嗪，但在此过程中自行停止治疗。他的正常尿量约为7,000-8,000mL/天。他接受了机器人辅助的根治性膀胱切除术和输尿管皮肤造口术治疗膀胱癌。两年后,他因粘连性肠梗阻住院。注入了5%的葡萄糖溶液，并根据尿量和电解质调整剂量。由于短时间内复发性肠梗阻，进行了粘连切开术。围手术期使用5%葡萄糖溶液作为主要输液。一旦手术后恢复饮用水，尿量和电解质易于控制。总之,CNDI患者应给予5%葡萄糖溶液作为主要输注，并应通过监测每日尿量来调整输液量，电解质，和血糖水平。如果尽早开始口服摄入，则输注管理更容易。
