Devaraja Janani, Sloan Sarah, Lee Vicki, Dimitri Paul
Sheffield Children's NHS Foundation Trust, Sheffield, UK.
Department of Paediatric Oncology, Sheffield Children's NHS Foundation Trust, Sheffield, UK.
Endocrinol Diabetes Metab Case Rep. 2021 Feb 17;2021. doi: 10.1530/EDM-20-0122.
An 11-year-old girl presented with acute lower limb weakness, dehydration, hypernatraemia and secondary rhabdomyolysis on a background of an 8-month history of polyuria. Radiological investigations revealed a suprasellar tumour which was diagnosed on biopsy as a non-metastatic germinoma. Further endocrinological investigations confirmed panhypopituitarism and she commenced desmopressin, hydrocortisone and thyroxine. Her chemotherapeutic regime consisted of etoposide, carboplatin and ifosfamide, the latter of which required 4 litres of hyperhydration therapy daily. During the first course of ifosfamide, titration of oral desmopressin was trialled but this resulted in erratic sodium control leading to disorientation. Based on limited literature, we then trialled an arginine-vasopressin (AVP) infusion. A sliding scale was developed to adjust the AVP dose, with an aim to achieve a urine output of 3-4 mL/kg/h. During the second course of ifosamide, AVP infusion was commenced at the outset and tighter control of urine output and sodium levels was achieved. In conclusion, we found that an AVP infusion during hyperhydration therapy was required to achieve eunatraemia in a patient with cranial diabetes insipidus. Developing an AVP sliding scale requires individual variation; further reports/case series are required to underpin practice.
Certain chemotherapeutic regimens require large fluid volumes of hyperhydration therapy which can result in significant complications secondary to rapid serum sodium shifts in patients with diabetes insipidus. The use of a continuous AVP infusion and titrating with a sliding scale is more effective than oral desmopressin in regulating plasma sodium and fluid balance during hyperhydration therapy. No adverse effects were found in our patient using a continuous AVP infusion. Adjustment of the AVP infusion rate depends on urine output, fluid balance, plasma sodium levels and sensitivity/response of the child to titrated AVP doses.
一名11岁女孩,有8个月多尿病史,现出现急性下肢无力、脱水、高钠血症及继发性横纹肌溶解。影像学检查发现鞍上肿瘤,活检诊断为非转移性生殖细胞瘤。进一步的内分泌检查证实为全垂体功能减退,她开始使用去氨加压素、氢化可的松和甲状腺素治疗。她的化疗方案包括依托泊苷、卡铂和异环磷酰胺,后者每天需要4升液体水化治疗。在第一个异环磷酰胺疗程中,尝试了口服去氨加压素滴定,但这导致钠控制不稳定,进而引起定向障碍。基于有限的文献,我们随后尝试了精氨酸加压素(AVP)输注。制定了一个滑动量表来调整AVP剂量,目标是使尿量达到3 - 4 mL/kg/h。在第二个异环磷酰胺疗程中,一开始就开始AVP输注,并实现了对尿量和钠水平更严格的控制。总之,我们发现对于颅性尿崩症患者,在液体水化治疗期间需要AVP输注以实现血钠正常。制定AVP滑动量表需要因人而异;需要更多的报告/病例系列来支持实践。
某些化疗方案需要大量的液体水化治疗,这可能会因尿崩症患者血清钠快速变化而导致严重并发症。在液体水化治疗期间,持续输注AVP并使用滑动量表滴定比口服去氨加压素在调节血浆钠和液体平衡方面更有效。我们的患者使用持续AVP输注未发现不良反应。AVP输注速率的调整取决于尿量、液体平衡、血浆钠水平以及儿童对滴定AVP剂量的敏感性/反应。