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[取决于核型变化的先天性发育缺陷患儿神经内分泌系统的状态]

[State of the neuroendocrine system in children with congenital defects of development dependent on changes in the karyotype].

作者信息

Mutovin G R, Kniazev Iu A, Marchenko L F

出版信息

Pediatriia. 1990(6):41-5.

PMID:2144336
Abstract

Forty children with multiple congenital developmental abnormalities (MCDA) without any visible changes in the karyotype and 36 MCDA children having visible changes in the karyotype were examined for the status of the neuroendocrine system. The children's age ranged from 10 days to 3 years. The children with MCDA manifested dysfunction of the neuroendocrine system, determined by the blood serum content of a number of hormones of the pituitary and the insular apparatus of the pancreas. The neuroendocrine dysfunction appeared to be in a reverse relationship to the intensity of the clinical signs (organic damage to the nervous system, delayed physical development, developmental abnormalities and minor developmental abnormalities). It manifested itself to the greatest degree in patients with numerical and structural chromosomal rearrangements and might be related to morphological or functional failure of the neuroendocrine system on the whole or of one of its components.

摘要

对40名核型无可见变化的多发性先天性发育异常(MCDA)儿童和36名核型有可见变化的MCDA儿童的神经内分泌系统状况进行了检查。儿童年龄为10天至3岁。MCDA儿童表现出神经内分泌系统功能障碍,这由垂体和胰腺岛状器官的多种激素的血清含量决定。神经内分泌功能障碍似乎与临床体征的严重程度呈负相关(神经系统器质性损害、身体发育迟缓、发育异常和轻度发育异常)。在染色体数目和结构重排的患者中表现最为明显,可能与整个神经内分泌系统或其某一组成部分的形态或功能衰竭有关。

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Pediatriia. 1990(6):41-5.
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