McCune Albright syndrome in association with excessive GH secretion: case report.
McCune-Albright Syndrome is a rare syndrome characterized with excessive function of peripheral endocrine organs and activating mutations of the stimulatory G protein alpha subunit are involved in the pathogenesis. The three main findings of the disease include hyperpigmented café au lait spots, fibrous dysplasia and increased endocrine functions and excessive secretion of growth hormone is observed in 21% of the patients. Clinical signs may be missed in these patients because of precocious puberty and craniofacial fibrous dysplasia. Since radiotherapy causes to sarcomatous changes and transsphenoidal surgery may cause to severe thickening in the cranial bones, they are not appropriate treatment options and medical treatment is recommended. Bromocriptine, cabergoline and octreotide or different combinations of these drugs are used in treatment and pegvisomant has also been used in recent years. Here, we present a male patient aged 12 years and 7 months to show gigantism as a rare clinical reflection of McCune-Albright Syndrome with an excessive height (197 cm), café au lait spots, growht hormone levels which could not be supressed with oral glucose tolerance test and increased prolactin levels.
Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.
Elif
E
Özsu
Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.
Gül Yeşiltepe
GY
Mutlu
Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.
Filiz Mine
FM
Çizmecioğlu
Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.
Şükrü
Ş
Hatun
Excess growth hormone; McCune Albright syndrome; cabergoline; somatostatin