Floret D, Chapard A, Scherrer M, Philippe N, Chappuis J P, Bouvier R, Claustrat B, Forest M, Monnet P
Arch Fr Pediatr. 1981 Feb;38(2):129-32.
A 8 and a half year-old boy presented with precocious puberty related to a malignant thoracic teratoma. He was also shown to have a Klinefelter syndrome. Precocious puberty related mainly to the liver, intracranial or thoracic tumors is rare. It seems to be exclusively observed in boys. The slight testicular enlargement is the main clinical sign. The contrast between high LH and low FSH levels is the most striking biological data. The diagnosis is proved by plasma HCG, beta-HCG and alpha-foetoprotein determination. Our patient is the third one with Klinefelter syndrome; this this association is certainly not fortuitous.
一名8岁半男孩因恶性胸段畸胎瘤出现性早熟。他还被诊断患有克兰费尔特综合征。主要与肝脏、颅内或胸部肿瘤相关的性早熟较为罕见。似乎仅在男孩中观察到。轻度睾丸肿大是主要临床体征。促黄体生成素(LH)水平高而促卵泡生成素(FSH)水平低之间的差异是最显著的生物学数据。通过测定血浆人绒毛膜促性腺激素(HCG)、β-HCG和甲胎蛋白可证实诊断。我们的患者是第三例患有克兰费尔特综合征的;这种关联肯定并非偶然。