Growth in Prader Willi Syndrome

被引:0
|
作者
Medeiros, P. F. V. [1 ]
Medeiros, I. A. C. M. [1 ]
Rocha, A. M. R. [1 ]
Santos, R. R. R. [1 ]
Filho, J. A. B. A. [1 ]
机构
[1] Univ Fed Campina Grande, Campina Grande Pb, Brazil
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中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Growth failure is a recognized feature of Prader Willi syndrome (PWS); however. there is controversy surrounding the etiology. Some studies have suggested that it is the result of inadequate growth hormone (GH) secretion resulting from hypothalamic dysfunction. The diagnosis of three studied girls, aged 6, 8 and 16 years, was based on clinical standard criteria and confirmed by genetic methods. GH deficiency on provocative test, after the evaluation of the thyroid function, was found in the patients. They all have obesity, two have growth failure but one has tall stature. Parent-child resemblance in growth or variation in size of deleted genes can explain the variation in PW patients growth. Normal or tall stature in PWS patients should not be a delay factor in the diagnosis of the syndrome.
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页码:391 / 395
页数:5
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