Background

Experience with nasogastric administration of oral DDAVP [desamino-d-arginine-8-vasopressin] lyophilisate (ODL) for central diabetes insipidus (CDI) in disabled children with swallowing coordination difficulties is limited.

Objective

We aimed to assess the safety and efficacy of nasogastric use of ODL in disabled children with CDI. Time to serum sodium normalisation was compared with that of children with normal intellect and CDI treated with sublingual DDAVP.

Methods

Clinical, laboratory and neuroimaging characteristics were evaluated for 12 disabled children with CDI treated with ODL through nasogastric tube at Dr Behcet Uz Children's Hospital, Turkey, between 2012 and 2022.

Results

Six boys and six girls with a mean (±SD) age of 43 (± 40) months were evaluated. These children (mean [±SD] weight standard deviation score [SDS] − 1.2 ± 1.7; mean [±SD] height SDS − 1.3 ± 1.4) presented with failure to thrive, irritability, prolonged fever, polyuria and hypernatraemia (mean serum sodium 162 [±3.6] mEq/L). At diagnosis, mean serum and urine osmolality were 321 (± 14) mOsm/kg and 105 (± 7.8) mOsm/kg, respectively. Arginine vasopressin (AVP) levels were undetectable (< 0.5 pmol/L) at diagnosis in all patients. Nasogastric tube administration of DDAVP lyophilisate (120 µg/tablet) dissolved in water (10 mL) was commenced at a dose of 1–5 µg/kg/day in two divided doses together with controlled water intake to avoid hyponatraemia. The frequency and dose of DDAVP were titrated based on urine output and serum sodium concentration. Serum sodium declined at a rate of 0.11 ± 0.03 mEq/L/h and reached normal range in a mean duration of 174 ± 46.5 h. Serum sodium declined faster in children with normal intellect and CDI treated with sublingual DDAVP (1.28 ± 0.39 mEq/L/h; p = 0.0003). Three disabled children needed rehospitalisation because of hypernatraemia due to unintentional DDAVP omission by caregivers. No episode of hyponatraemia was observed. Weight gain and growth were normal during the median (± interquartile range) follow-up duration of 32 ± 67 months.

Conclusions

Nasogastric administration of oral DDAVP lyophilised formulation was safe and effective in the treatment of CDI in disabled children in this small retrospective series.

中文翻译：

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通过鼻胃管给予稀释口服去氨加压素冻干制剂治疗残疾儿童中枢性尿崩症：回顾性病例系列

背景

对于患有吞咽协调困难的残疾儿童，经鼻胃给予口服 DDAVP [脱氨基-d-精氨酸-8-加压素]冻干物 (ODL) 治疗中枢性尿崩症 (CDI) 的经验有限。

客观的

我们的目的是评估患有 CDI 的残疾儿童经鼻胃管使用 ODL 的安全性和有效性。将血清钠恢复正常的时间与接受舌下 DDAVP 治疗的智力正常和 CDI 的儿童进行比较。

方法

研究人员对 2012 年至 2022 年间在土耳其 Behcet Uz 儿童医院通过鼻胃管接受 ODL 治疗的 12 名患有 CDI 的残疾儿童的临床、实验室和神经影像学特征进行了评估。

结果

对平均 (±SD) 年龄为 43 (± 40) 个月的 6 名男孩和 6 名女孩进行了评估。这些儿童（平均[±SD]体重标准差评分[SDS]−1.2±1.7；平均[±SD]身高SDS−1.3±1.4）表现出生长迟缓、烦躁、长时间发烧、多尿和高钠血症（平均血清钠） 162 [±3.6] 毫当量/升）。诊断时，平均血清和尿液渗透压分别为 321 (± 14) mOsm/kg 和 105 (± 7.8) mOsm/kg。所有患者诊断时精氨酸加压素 (AVP) 水平均检测不到 (< 0.5 pmol/L)。鼻饲管将 DDAVP 冻干物（120 µg/片）溶解在水（10 mL）中，剂量为 1-5 µg/kg/天，分两次给药，同时控制饮水量以避免低钠血症。根据尿量和血清钠浓度滴定 DDAVP 的频率和剂量。血清钠以 0.11 ± 0.03 mEq/L/h 的速度下降，并在平均 174 ± 46.5 小时内达到正常范围。智力正常且患有 CDI 的儿童接受舌下含服 DDAVP 治疗后，血清钠下降较快 (1.28 ± 0.39 mEq/L/h；p  = 0.0003)。三名残疾儿童因护理人员无意漏服 DDAVP 而导致高钠血症，需要再次住院。没有观察到低钠血症的发生。在 32 ± 67 个月的中位随访期间（±四分位距），体重增加和生长正常。

结论

在这个小型回顾性系列研究中，经鼻胃给药口服 DDAVP 冻干制剂治疗残疾儿童 CDI 是安全有效的。