Esen Ihsan, Demirel Fatma
Department of Pediatric Endocrinology, Ankara Children's Diseases and Hematology Oncology Training Hospital, Ankara, Turkey.
Turk J Pediatr. 2011 Mar-Apr;53(2):210-2.
In children with untreated hypothyroidism, the onset of puberty is usually delayed, but gonadotropin-independent precocious puberty may occur in children with severe hypothyroidism of long duration. The association of hypothyroidism, delayed bone age and gonadotropin-independent precocious puberty is defined as Van Wyk Grumbach syndrome (VWGS). VWGS has been described mostly in girls, and only seldom in boys. The manifestation of VWGS in boys is only testicular enlargement without substantial Leydig cell stimulation or testosterone secretion. We report a case of testicular enlargement due to obvious hypothyroidism secondary to autoimmune thyroiditis in a boy who presented with obesity. With this case report, we would like to emphasize that VWGS is not a real gonadotropin- independent precocious puberty in boys as it is in girls. Additionally, we would like to emphasize that delayed bone age is a special discriminating feature for differentiation of VWGS from the other causes of precocious puberty.
在未经治疗的甲状腺功能减退症患儿中，青春期通常延迟，但在长期患有严重甲状腺功能减退症的患儿中可能会出现非促性腺激素依赖性性早熟。甲状腺功能减退症、骨龄延迟和非促性腺激素依赖性性早熟的关联被定义为范怀克-格伦巴赫综合征（VWGS）。VWGS大多在女孩中被描述，在男孩中很少见。VWGS在男孩中的表现仅为睾丸增大，而没有实质性的睾丸间质细胞刺激或睾酮分泌。我们报告一例肥胖男孩因自身免疫性甲状腺炎继发明显甲状腺功能减退症导致睾丸增大的病例。通过本病例报告，我们想强调，VWGS在男孩中并非真正的非促性腺激素依赖性性早熟，与女孩的情况不同。此外，我们想强调骨龄延迟是将VWGS与其他性早熟原因区分开来的一个特殊鉴别特征。
