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口服去氨加压素冻干制剂治疗婴儿中枢性尿崩症

Management of central diabetes insipidus with oral desmopressin lyophilisate in infants.

作者信息

Korkmaz Hüseyin Anıl, Demir Korcan, Kılıç Fatma Kaya, Terek Demet, Arslanoğlu Sertaç, Dizdarer Ceyhun, Ozkan Behzat

出版信息

J Pediatr Endocrinol Metab. 2014 Sep;27(9-10):923-7. doi: 10.1515/jpem-2013-0368.

Abstract

AIM

To assess the efficiency of oral desmopressin lyophilisate (ODL) in neonatal central diabetes insipidus (CDI).

METHODS

The characteristics of four newborns with CDI treated with ODL were evaluated.

RESULTS

Four newborns with polyuria and hypernatremia were included [male, 2 (50%); mean postnatal age, 19±17 days]. At the time of hypernatremia, the mean serum and urine osmolality values were 310±16 and 179±48 mOsm/kg, respectively. Antidiuretic hormone levels were undetectable (<0.5 pmol/L) in all cases. Magnetic resonance imaging revealed anatomical malformations in all cases. ODL (60 μg/tablet) dissolved in water (3-5 mL) was initiated with a dose of 5 μg/kg/day in two equal doses, together with limitation of water intake to avoid hyponatremia. Serum sodium levels returned to normal in a mean duration of 58±9.9 h with a mean decline rate of 0.37±0.1 mEq/L/h after desmopressin administration. Rehospitalization was required for one of the infants because of hypernatremia due to non-compliance. No episode of hyponatremia was encountered. Weight gain and growth of the infants were normal during the mean follow-up duration of 8.5±1 months.

CONCLUSIONS

ODL appears to be practical and safe in the treatment of CDI during the first year of life.

摘要

目的

评估口服去氨加压素冻干制剂(ODL)治疗新生儿中枢性尿崩症(CDI)的疗效。

方法

对4例接受ODL治疗的CDI新生儿的特征进行评估。

结果

纳入4例多尿和高钠血症新生儿[男性2例(50%);平均出生后年龄19±17天]。高钠血症时,血清和尿渗透压均值分别为310±16和179±48 mOsm/kg。所有病例抗利尿激素水平均检测不到(<0.5 pmol/L)。磁共振成像显示所有病例均有解剖结构畸形。将ODL(60μg/片)溶于水(3 - 5 mL),开始剂量为5μg/kg/天,分两次等量给药,同时限制水摄入以避免低钠血症。给予去氨加压素后,血清钠水平平均在58±9.9小时恢复正常,平均下降速率为0.37±0.1 mEq/L/小时。1例婴儿因不依从导致高钠血症而需再次住院。未发生低钠血症事件。在平均8.5±1个月的随访期间,婴儿体重增加和生长正常。

结论

ODL在治疗1岁以内CDI方面似乎实用且安全。

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