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首页> 外文期刊>Hormones: International Journal of Endocrinology and Metabolism >Adult height following a combined treatment of ketoconazole - cyproterone acetate - leuprolide depot in a boy with atypical McCune-Albright syndrome
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Adult height following a combined treatment of ketoconazole - cyproterone acetate - leuprolide depot in a boy with atypical McCune-Albright syndrome

机译：酮康唑-醋酸环丙孕酮-亮丙瑞德组合疗法治疗的非典型McCune-Albright综合征男孩的成人身高

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BACKGROUND: This study was carried out because of the rarity of peripheral precocious puberty (PPP) in boys with McCune-Albright syndrome (MAS) and the lack of data on adult height of treated MAS males, treatment for this disorder being not as yet standardized. AIMS: To report the adult height of a MAS boy with PPP who was treated with ketoconazole – cyproterone acetate – leuprolide depot and to describe some atypical aspects of MAS presentation and course in this boy. CASE HISTORY: The case concerns a boy presenting with unilateral macroorchidism, no signs of PPP or initially isolated Sertoli cell activation and MAS mutation that subsequently also activated Leydig cells, thus inducing a change in phenotypic expression. CONCLUSIONS: a) In a MAS boy presenting with unilateral macroorchidism and no other signs of PPP, a consecutive involvement of Leydig cells may follow the initially isolated activation of Sertoli cells; b) prolonged treatment with ketoconazole – cyproterone acetate – leuprolide depot may be well tolerated and effective, as demonstrated by the very good adult height outcome recorded in our patient.
机译：背景：进行这项研究的原因是，患有McCune-Albright综合征（MAS）的男孩的外周性早熟（PPP）稀少，并且缺乏治疗过的MAS男性成年身高的数据，该疾病的治疗方法尚未标准化。目的：报告一名接受酮康唑-醋酸环丙孕酮-亮丙立德治疗的患有PPP的MAS男孩的成人身高，并描述该男孩MAS表现和病程的一些非典型方面。案例历史：该案例涉及一个男孩，表现为单侧大兰花症，没有PPP征兆或最初分离的Sertoli细胞激活和MAS突变，随后也激活了Leydig细胞，从而诱导了表型表达的改变。结论：a）在单侧大兰花症且没有其他PPP征象的MAS男孩中，Leydig细胞的连续受累可能与最初分离出的Sertoli细胞活化有关。 b）长期接受酮康唑–醋酸环丙孕酮–亮丙瑞德治疗可能是很好的耐受性和有效性，这在我们的患者中记录的非常好的成人身高结局证明了这一点。

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