Miyauchi Wataru, Matsunaga Tomoyuki, Sakano Yu, Makinoya Masahiro, Shimizu Shota, Miyatani Kozo, Shishido Yuji, Sakamoto Teruhisa, Fujiwara Yoshiyuki
Division of Gastrointestinal and Pediatric Surgery, Department of Surgery, School of Medicine, Faculty of Medicine, Tottori University, Yonago 683-8504, Japan.
Yonago Acta Med. 2023 May 11;66(2):311-316. doi: 10.33160/yam.2023.05.015. eCollection 2023 May.
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.
先天性肾性尿崩症(CNDI)是一种罕见疾病,由于肾脏集合管对抗利尿激素的反应性降低而导致多尿。如果不通过大量饮水进行代偿,脱水和高钠血症会迅速发展。我们报告一例最初诊断为CNDI的患者,因粘连性肠梗阻需要手术并禁食。该患者为一名46岁男性,最初诊断为CNDI。他曾被处方服用三氯噻嗪,但在此过程中自行停药。他的正常尿量约为每天7000 - 8000毫升。他因膀胱癌接受了机器人辅助根治性膀胱切除术和输尿管皮肤造口术。两年后,他因粘连性肠梗阻住院。输注了5%葡萄糖溶液,并根据尿量和电解质调整剂量。由于短期内反复出现肠梗阻,进行了粘连松解术。围手术期以5%葡萄糖溶液作为主要输注液。术后一旦恢复饮水,尿量和电解质很容易得到控制。总之,对于CNDI患者,应以5%葡萄糖溶液作为主要输注液,并通过监测每日尿量、电解质和血糖水平来调整输注量。如果尽早开始口服摄入,输液管理会更容易。