Central precocious puberty in a girl with Prader-Willi syndrome.

Abstract

Prader-Willi syndrome (PWS) is characterized by infantile lethargy and hypotonia causing poor feeding and failure to thrive, childhood obesity, short stature, and hypogonadism. The complex phenotype is most probably caused by a hypothalamic dysfunction that is responsible for the hormonal dysfunction. The resulting hypogonadism in PWS causes incomplete, delayed, and sometimes disordered pubertal development. Precocious puberty is very rare in PWS. We report the case of a girl with PWS who was diagnosed with idiopathic precocious puberty and was treated with gonadotropin-releasing hormone analog.