Raisingani Manish, Palliyil Gopi Resmy, Shah Bina
Department of Pediatrics, Division of Pediatric Endocrinology, New York University School of Medicine, New York, NY, USA.
Case Rep Pediatr. 2017;2017:2407028. doi: 10.1155/2017/2407028. Epub 2017 May 3.
Management of central diabetes insipidus in infancy is challenging. The various forms of desmopressin, oral, subcutaneous, and intranasal, have variability in the duration of action. Infants consume most of their calories as liquids which with desmopressin puts them at risk for hyponatremia and seizures. There are few cases reporting chlorothiazide as a temporizing measure for central diabetes insipidus in infancy. A male infant presented on day of life 30 with holoprosencephaly, cleft lip and palate, and poor weight gain to endocrine clinic. Biochemical tests and urine output were consistent with central diabetes insipidus. The patient required approximately 2.5 times the normal fluid intake to keep up with the urine output. Patient was started on low renal solute load formula and oral chlorothiazide. There were normalization of serum sodium, decrease in fluid intake close to 1.3 times the normal, and improved urine output. There were no episodes of hyponatremia/hypernatremia inpatient. The patient had 2 episodes of hypernatremia in the first year of life resolving with few hours of hydration. Oral chlorothiazide is a potential bridging agent for treatment of central DI along with low renal solute load formula in early infancy. It can help achieve adequate control of DI without wide serum sodium fluctuations.
婴儿期中枢性尿崩症的管理具有挑战性。各种形式的去氨加压素，口服、皮下和鼻内给药，作用持续时间存在差异。婴儿摄入的大部分热量来自液体，使用去氨加压素会使他们面临低钠血症和癫痫发作的风险。很少有病例报告将氢氯噻嗪作为婴儿期中枢性尿崩症的临时治疗措施。一名男婴在出生第30天因全前脑畸形、唇腭裂和体重增加不佳到内分泌门诊就诊。生化检查和尿量与中枢性尿崩症一致。该患者需要大约正常液体摄入量2.5倍的液体来维持尿量。患者开始使用低肾溶质负荷配方奶粉和口服氢氯噻嗪。血清钠恢复正常，液体摄入量降至接近正常的1.3倍，尿量改善。住院期间未发生低钠血症/高钠血症。该患者在生命的第一年有2次高钠血症发作，经数小时补液后缓解。口服氢氯噻嗪是婴儿早期治疗中枢性尿崩症的一种潜在的过渡药物，与低肾溶质负荷配方奶粉联合使用。它有助于在不引起血清钠大幅波动的情况下充分控制尿崩症。
