[Growth failure in a boy with Klinefelter syndrome and IUGR].

Alicja Korpysz, Mieczysław Szalecki
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Abstract

Background: Children born with a history of Intra-Uterine Growth Retardation (IUGR) may be susceptible for reduced growth velocity and growth deficiency. The causes of GH/IGF-1 resistance are unknown. Klinefelter syndrome is characterized by excessive growth, resulting from hypogonadism, open bone age and a prolonged growth.

Objectives: The case of a 6-year-old boy with Klinefelter syndrome born with IUGR and deep growth deficiency was studied.

A case report: A 6-year-old boy with birth weight 2300 g born of term in 38 Hbd is presented. The cariotype was 47XXY. The phenotype was typical for a child with IUGR. GH in the clonidin test was 37.4 microIU/ml, IGF-1 -- 111 ng/ml. Increased IGF-1 level from 178.1 ng/ml to 360.9 ng/ml was founded in the IGF-1 generation test.

Conclusions: The cause of growth deficiency in this case could be GH/IGF-1 resistance.

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[Klinefelter综合征和IUGR男孩的生长衰竭]。
背景:出生时有子宫内生长迟缓(IUGR)病史的儿童可能容易出现生长速度减慢和生长缺陷。GH/IGF-1抵抗的原因尚不清楚。Klinefelter综合征的特征是性腺功能减退、骨龄开放和生长延长导致的过度生长。目的:对1例6岁Klinefelter综合征男童先天性IUGR合并深度生长缺陷进行研究。病例报告:一个6岁的男孩出生体重2300克出生在38 Hbd足月。基因型为47XXY。该表型是IUGR患儿的典型表型。clonidin试验GH为37.4 microIU/ml, IGF-1—111 ng/ml。IGF-1生成试验发现IGF-1水平从178.1 ng/ml增加到360.9 ng/ml。结论:本病例中生长缺陷的原因可能是GH/IGF-1抵抗。
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[Evaluation of final height in patients with pituitary growth hormone deficiency who were treated with growth hormone replacement]. [Current views on the etiopathogenesis of goiter in children]. [Guidelines concerning insulin dosage in children and adolescents with type 1 diabetes on continuous subcutaneous insulin infusion]. [Familial precocious puberty -- a variant of norm or pathology?]. [Growth failure in a boy with Klinefelter syndrome and IUGR].
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