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垂体巨人症的管理。溴隐亭和放射治疗的作用。

Management of pituitary gigantism. The role of bromocriptine and radiotherapy.

作者信息

Ritzén E M, Wettrell G, Davies G, Grant D B

出版信息

Acta Paediatr Scand. 1985 Sep;74(5):807-14. doi: 10.1111/j.1651-2227.1985.tb10040.x.

Abstract

True gigantism with overproduction of growth hormone (GH) and prolactin (PRL) was diagnosed in two boys, aged 13 years (case I) and 7 1/2 years (case II). Both had shown increased growth rates since early childhood (from 4 years and 1 1/2 years, respectively), but no skeletal acromegalic features were noted. However, both showed increased sweating and both had advanced pubic hair relative to testis volume. No other pituitary dysfunction was recorded. Case I underwent transsphenoidal surgery with only incomplete and temporary suppression of GH and PRL levels. However, in both patients bromocriptine administration promptly suppressed PRL levels. Following combined irradiation and bromocriptine treatment, GH also gradually normalized over a period of 2 years. Both boys are still on treatment, and both showed an increase in plasma GH concentrations when the dose of bromocriptine was reduced or discontinued, indicating that even 3 1/2-5 years after irradiation therapy (and during continuous treatment with bromocriptine) the disease was controlled but not cured. However, in these two boys bromocriptine has proved effective in controlling the PRL/GH oversecretion.

摘要

两名男孩被诊断为真正的巨人症,伴有生长激素(GH)和催乳素(PRL)分泌过多,年龄分别为13岁(病例I)和7.5岁(病例II)。自幼儿期(分别从4岁和1.5岁起),两人的生长速度均加快,但未发现骨骼肢端肥大症特征。然而,两人均出现出汗增多,且相对于睾丸体积,阴毛提前发育。未记录到其他垂体功能障碍。病例I接受了经蝶窦手术,仅GH和PRL水平得到不完全且暂时的抑制。然而,在两名患者中,服用溴隐亭均迅速抑制了PRL水平。在联合放疗和溴隐亭治疗后,GH也在2年的时间里逐渐恢复正常。两名男孩仍在接受治疗,当溴隐亭剂量减少或停药时,两人的血浆GH浓度均升高,这表明即使在放疗治疗后3.5 - 5年(以及在持续服用溴隐亭期间),疾病得到了控制但未治愈。然而,在这两名男孩中,溴隐亭已被证明对控制PRL/GH分泌过多有效。

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