Triple-x syndrome accompanied by congenital adrenal hyperplasia: case report

The 47,XXX karyotype is a rare sex chromosome anomaly. This karyotype is usually not associated with a characteristic physical phenotype. In the presented case, a triple-X girl patient associated with 11beta-hydroxylase deficiency is identified. The case was referred to the Endocrinology Unit...

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Bibliographic Details
Main Authors: Selim Kurtoğlu, M Emre Atabek, Mustafa Akçakuş, Yusuf Ozkul, Cetin Saatçi
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 2004-10-01
Series:The Turkish Journal of Pediatrics
Online Access:https://turkjpediatr.org/article/view/2840
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Summary:The 47,XXX karyotype is a rare sex chromosome anomaly. This karyotype is usually not associated with a characteristic physical phenotype. In the presented case, a triple-X girl patient associated with 11beta-hydroxylase deficiency is identified. The case was referred to the Endocrinology Unit at six days of age because of ambiguous genitalia. The karyotype in this case was 47,XXX, an unexpected finding. Diagnosis of 47,XXX individuals remains difficult because specific clinical criteria used to identify this condition are not available. Congenital adrenal hyperplasia has not been previously reported in patients with triple-X syndrome.
ISSN:0041-4301
2791-6421