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[对伴有黄疸持续时间延长和低血糖的新生儿先天性内分泌疾病的诊断]

[Diagnosis of congenital endocrinological disease in newborns with prolonged jaundice and hypoglycaemia].

作者信息

Braslavsky D, Keselman A, Chiesa A, Bergadá I

机构信息

División de Endocrinología, Centro de Investigaciones Endocrinológicas, Hospital de Niños Dr. Ricardo Gutiérrez, Buenos Aires, Argentina.

出版信息

An Pediatr (Barc). 2012 Mar;76(3):120-6. doi: 10.1016/j.anpedi.2011.08.011. Epub 2011 Oct 20.

DOI:10.1016/j.anpedi.2011.08.011
PMID:22015010
Abstract

INTRODUCTION

The association of prolonged neonatal jaundice and hypoglycaemia may be secondary to an endocrinological disease. Pituitary insufficiency and primary adrenal insufficiency are the most likely endocrine diseases that need to be ruled out.

MATERIAL AND METHODS

We retrospectively analysed the clinical and laboratory characteristics of thirteen patients referred to the Hospital de Niños Ricardo Gutiérrez between years 2003 and 2008 due to prolonged neonatal jaundice and hypoglycaemia secondary to pituitary insufficiency in twelve patients, and in one secondary to primary adrenal insufficiency.

RESULTS

All patients had a history of neonatal hypoglycaemia. Ten patients had conjugated hyperbilirubinaemia and six also had elevated transaminases. Combined pituitary hormone deficiency was observed in the twelve hypopituitarism patients. Hormonal replacement normalised liver function and resolved the prolonged jaundice in all the patients. None of them underwent liver biopsy. Hypoglycaemia also remitted after hormonal therapy.

CONCLUSIONS

Prolonged or cholestatic jaundice associated with neonatal hypoglycaemia is highly likely to be due to pituitary hormone deficiency or primary adrenal insufficiency. Early diagnosis and treatment of these children reverts the prolonged jaundice and prevents morbidity and mortality due to recurrent hypoglycaemia and hormone deficiencies.

摘要

引言

新生儿黄疸延长与低血糖之间的关联可能继发于内分泌疾病。垂体功能不全和原发性肾上腺功能不全是最有可能需要排除的内分泌疾病。

材料与方法

我们回顾性分析了2003年至2008年间转诊至里卡多·古铁雷斯儿童医院的13例患者的临床和实验室特征,其中12例因垂体功能不全导致新生儿黄疸延长和低血糖,1例因原发性肾上腺功能不全导致上述情况。

结果

所有患者均有新生儿低血糖病史。10例患者有结合胆红素血症,6例患者转氨酶也升高。12例垂体功能减退患者中观察到联合垂体激素缺乏。激素替代治疗使所有患者的肝功能恢复正常,并解决了黄疸延长的问题。他们均未接受肝活检。激素治疗后低血糖也得到缓解。

结论

与新生儿低血糖相关的黄疸延长或胆汁淤积性黄疸极有可能是由于垂体激素缺乏或原发性肾上腺功能不全。对这些儿童进行早期诊断和治疗可使黄疸延长情况得到逆转,并预防因反复低血糖和激素缺乏导致的发病和死亡。

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