Stier B, Ranke M B
Universitäts-Kinderklinik Tübingen.
Klin Padiatr. 1987 Sep-Oct;199(5):376-81. doi: 10.1055/s-2008-1026824.
A patient is presented with the syndrome of polyostatic fibrous dysplasia and precocious puberty (PP). The endocrinopathy in McCune-Albright-syndrome (MAS) has formerly been ascribed to a central (hypothalamic) origin. In this patient the PP was caused by a luteinized follicular cyst, suggesting autonomous hyperfunction of this gland. High serum estradiol levels returned to normal after cystectomie. The review of the literature suggests the peripheral origin of PP to be more frequent in younger age groups (under 6 years). It appears possible that peripheral hypersecretion of sexual steroids may cause a rise of gonadotropins secondarily followed by true PP in older children provided such longstanding hypersecretion leads to a generalized maturation of the body including skeletal maturation. Treatment in pseudoprecocious puberty seems to be not effective with LH-RH-analogues. Cyproteroneacetate alone or in combination with a LH-RH analogue gave the impression of being more successful. Cystectomy can lead to a cure but a recurrence of ovarian cysts is possible. A combination of surgical and drug therapy may be beneficial under these circumstances. Until now there is no sufficient treatment for polyostotic fibrous dysplasia.
一名患有多骨型纤维发育不良和性早熟（PP）综合征的患者。McCune-Albright综合征（MAS）中的内分泌病变以前被归因于中枢（下丘脑）起源。在该患者中，性早熟是由黄素化卵泡囊肿引起的，提示该腺体自主功能亢进。囊肿切除术后，血清雌二醇水平恢复正常。文献回顾表明，性早熟的外周起源在较年轻年龄组（6岁以下）更为常见。在年龄较大的儿童中，如果长期的性激素外周分泌过多导致包括骨骼成熟在内的身体全面成熟，那么性激素外周分泌过多可能继发促性腺激素升高，进而导致真性性早熟。假性性早熟的治疗使用促性腺激素释放激素（LH-RH）类似物似乎无效。单独使用醋酸环丙孕酮或与LH-RH类似物联合使用似乎更有效。囊肿切除术可治愈，但卵巢囊肿可能复发。在这种情况下，手术和药物联合治疗可能有益。到目前为止，对于多骨型纤维发育不良尚无足够的治疗方法。
