Tóth Miklós, Töke Judit, Kiss Edit, Bernád Irén, Miheller Pál, Szücs Nikolette, Rácz Károly
AOK II. Belgyógyászati Klinika, Semmelweis Egyetem, Budapest.
Orv Hetil. 2002 May 12;143(19 Suppl):1070-3.
McCune-Albright syndrome is characterized by polyostotic fibrous dysplasia, cafe au lait pigmentation of the skin, and multiple endocrinopathies. The authors report a history of a 30-years-old man who had a pathologic humerus fracture at the age of 14 years. The diagnosis of polyostotic fibrous dysplasia was established by radiologic examinations and bone biopsy. Fourteen years thereafter, active acromegaly due to a pituitary microadenoma was diagnosed using hormone measurements and pituitary magnetic resonance imaging. Pituitary surgery was refused because of an extensive skull involvement caused by the fibrous dysplasia. After an unsuccessful therapy with bromocriptine lasting three months, long-acting octreotide (Sandostatin LAR, Novartis) treatment was started. After a 12-months course of treatment, serum growth hormone levels markedly decreased, clinical symptoms improved, but serum insulin-like growth factor I levels remained unchanged. These observations that serum insulin-like growth factor I levels failed to reflect the decrease of serum growth hormone concentrations after long-acting octreotide treatment suggest that the increased production of insulin-like growth factor I in patients with acromegaly due to McCune-Albright syndrome may involve mechanism(s) other than increased growth hormone levels.
McCune-Albright综合征的特征为多骨型纤维性发育不良、皮肤牛奶咖啡斑色素沉着以及多种内分泌病。作者报告了一名30岁男性的病史，该患者14岁时发生病理性肱骨骨折。通过放射学检查和骨活检确诊为多骨型纤维性发育不良。此后14年，通过激素测定和垂体磁共振成像诊断出因垂体微腺瘤导致的活动性肢端肥大症。由于纤维性发育不良导致广泛的颅骨受累，患者拒绝接受垂体手术。在使用溴隐亭治疗三个月失败后，开始使用长效奥曲肽（善龙，诺华公司）治疗。经过12个月的治疗疗程，血清生长激素水平显著下降，临床症状改善，但血清胰岛素样生长因子I水平保持不变。这些观察结果表明，长效奥曲肽治疗后血清胰岛素样生长因子I水平未能反映血清生长激素浓度的降低，提示McCune-Albright综合征所致肢端肥大症患者中胰岛素样生长因子I产生增加可能涉及生长激素水平升高以外的机制。
