Andler W
Vestische Kinderklinik, Datteln.
Monatsschr Kinderheilkd. 1991 Apr;139(4):220-3.
Six prepubertal girls presented with precocious pseudopuberty associated with ovarian follicular cysts. Five patients normalized spontaneously after several months, in one patient the cyst was removed by laparotomy. Elevated estrogen serum levels and failure of gonadotropin responses after gonadotropin releasing hormone were the classical findings in these patients during the acute period of the disease. In a later period, however, estrogen and gonadotropin levels may be normalized although breast development is still present. Surgical treatment was performed in one patient although spontaneous remission would have been probable. Three patients showed several episodes of precocious pseudopuberty. In three patients ACTH testing was performed after dexamethason suppression. All three patients showed elevated 17-hydroxyprogesterone responses which were similar to those in heterocygotes for C21-hydroxylase deficiency.
六名青春期前女孩出现与卵巢滤泡囊肿相关的性早熟假性青春期。五名患者在数月后自然恢复正常，一名患者通过剖腹手术切除了囊肿。在疾病急性期，这些患者的典型表现为血清雌激素水平升高以及促性腺激素释放激素刺激后促性腺激素反应异常。然而，在后期，尽管乳房发育仍存在，但雌激素和促性腺激素水平可能恢复正常。尽管可能会自然缓解，但仍对一名患者进行了手术治疗。三名患者出现了多次性早熟假性青春期发作。三名患者在地塞米松抑制后进行了促肾上腺皮质激素检测。所有三名患者的17-羟孕酮反应均升高，与21-羟化酶缺乏杂合子的反应相似。
