中华实用儿科临床杂志
中華實用兒科臨床雜誌
중화실용인과림상잡지
Journal of Applied Clinical Pediatrics
2014年
8期
595-598
,共4页
李豫川%巩纯秀%吴迪%谷奕%曹冰燕
李豫川%鞏純秀%吳迪%穀奕%曹冰燕
리예천%공순수%오적%곡혁%조빙연
中枢性特发性性早熟%青春期%多囊卵巢综合征%高胰岛素血症
中樞性特髮性性早熟%青春期%多囊卵巢綜閤徵%高胰島素血癥
중추성특발성성조숙%청춘기%다낭란소종합정%고이도소혈증
Idiopathic central precocious puberty%Adolescence%Polycystic ovary syndrome%Hyperinsulinemia
目的 关注女童同性性早熟相关的青春期多囊卵巢综合征(PCOS),重新认识女童同性性早熟/性早发育对远期健康的影响.方法 报告以同性性早熟起病的女童青春期PCOS的病例并复习文献.结果 接近8岁时,因乳房发育就诊后诊断为特发性中枢性性早熟(ICPP)及肥胖症的女童在予以促性腺激素释放激素类似物(GnRHa)3.75 mg,每28d1次的标准剂量治疗后,性腺发育得到良好控制,生长抑制显著,但体质量持续上升,黄体生成素下降缓慢.GnRHa治疗13个月后加用生长激素(GH)纠正身高.使用GnRHa25个月、GH 12个月后停止治疗.因停药后近20个月无初潮就诊,发现痤疮、黑棘皮、紫纹、多毛、肥胖;高胰岛素血症、糖耐量异常和高睾酮血症,且B超提示卵巢多囊性改变,再进一步行促肾上腺皮质激素兴奋试验和肾上腺B超除外了非典型先天性肾上腺皮质增生症、肾上腺疾病后,诊断为青春期PCOS、肥胖症、高胰岛素血症和糖耐量受损.经减轻体质量和二甲双胍治疗1个月后初潮1次.予以减轻体质量及二甲双胍治疗12个月时,月经共4次,体质量指数下降,高胰岛素血症有明显改善,糖耐量正常,血清睾酮正常,盆腔B超提示卵巢已无多囊改变.在继续减肥及二甲双胍治疗2年左右时电话随访:患儿身高体质量未再增长,近1年月经规律,周期为35 ～ 40 d,每次持续7d左右.文献复习提示性早熟/性早发育并非以往认为的良性过程,其与多囊卵巢综合征可能有着共同的发病基础.除肥胖和高胰岛素血症外,黄体生成素(LH)分泌亢进可能为中枢性性早熟(CPP)和PCOS共同的原因.文献示性早熟患儿青春期后PCOS可多达30％且伴肥胖较多.儿科领域对青春期PCOS的诊断治疗研究少,尚无共识.结论 ICPP女童青春期后有发展为PCOS的可能性.本病例临床特点支持CPP、PCOS存在共同的发病基础,可能机制为下丘脑-垂体-性腺轴功能紊乱,LH分泌亢进;ICPP治疗中LH抑制不良和肥胖现象应该引起足够重视;GnRHa治疗的获益和风险应该充分考虑.应在儿科领域关注青春期PCOS的诊疗研究.
目的 關註女童同性性早熟相關的青春期多囊卵巢綜閤徵(PCOS),重新認識女童同性性早熟/性早髮育對遠期健康的影響.方法 報告以同性性早熟起病的女童青春期PCOS的病例併複習文獻.結果 接近8歲時,因乳房髮育就診後診斷為特髮性中樞性性早熟(ICPP)及肥胖癥的女童在予以促性腺激素釋放激素類似物(GnRHa)3.75 mg,每28d1次的標準劑量治療後,性腺髮育得到良好控製,生長抑製顯著,但體質量持續上升,黃體生成素下降緩慢.GnRHa治療13箇月後加用生長激素(GH)糾正身高.使用GnRHa25箇月、GH 12箇月後停止治療.因停藥後近20箇月無初潮就診,髮現痤瘡、黑棘皮、紫紋、多毛、肥胖;高胰島素血癥、糖耐量異常和高睪酮血癥,且B超提示卵巢多囊性改變,再進一步行促腎上腺皮質激素興奮試驗和腎上腺B超除外瞭非典型先天性腎上腺皮質增生癥、腎上腺疾病後,診斷為青春期PCOS、肥胖癥、高胰島素血癥和糖耐量受損.經減輕體質量和二甲雙胍治療1箇月後初潮1次.予以減輕體質量及二甲雙胍治療12箇月時,月經共4次,體質量指數下降,高胰島素血癥有明顯改善,糖耐量正常,血清睪酮正常,盆腔B超提示卵巢已無多囊改變.在繼續減肥及二甲雙胍治療2年左右時電話隨訪:患兒身高體質量未再增長,近1年月經規律,週期為35 ～ 40 d,每次持續7d左右.文獻複習提示性早熟/性早髮育併非以往認為的良性過程,其與多囊卵巢綜閤徵可能有著共同的髮病基礎.除肥胖和高胰島素血癥外,黃體生成素(LH)分泌亢進可能為中樞性性早熟(CPP)和PCOS共同的原因.文獻示性早熟患兒青春期後PCOS可多達30％且伴肥胖較多.兒科領域對青春期PCOS的診斷治療研究少,尚無共識.結論 ICPP女童青春期後有髮展為PCOS的可能性.本病例臨床特點支持CPP、PCOS存在共同的髮病基礎,可能機製為下丘腦-垂體-性腺軸功能紊亂,LH分泌亢進;ICPP治療中LH抑製不良和肥胖現象應該引起足夠重視;GnRHa治療的穫益和風險應該充分攷慮.應在兒科領域關註青春期PCOS的診療研究.
목적 관주녀동동성성조숙상관적청춘기다낭란소종합정(PCOS),중신인식녀동동성성조숙/성조발육대원기건강적영향.방법 보고이동성성조숙기병적녀동청춘기PCOS적병례병복습문헌.결과 접근8세시,인유방발육취진후진단위특발성중추성성조숙(ICPP)급비반증적녀동재여이촉성선격소석방격소유사물(GnRHa)3.75 mg,매28d1차적표준제량치료후,성선발육득도량호공제,생장억제현저,단체질량지속상승,황체생성소하강완만.GnRHa치료13개월후가용생장격소(GH)규정신고.사용GnRHa25개월、GH 12개월후정지치료.인정약후근20개월무초조취진,발현좌창、흑극피、자문、다모、비반;고이도소혈증、당내량이상화고고동혈증,차B초제시란소다낭성개변,재진일보행촉신상선피질격소흥강시험화신상선B초제외료비전형선천성신상선피질증생증、신상선질병후,진단위청춘기PCOS、비반증、고이도소혈증화당내량수손.경감경체질량화이갑쌍고치료1개월후초조1차.여이감경체질량급이갑쌍고치료12개월시,월경공4차,체질량지수하강,고이도소혈증유명현개선,당내량정상,혈청고동정상,분강B초제시란소이무다낭개변.재계속감비급이갑쌍고치료2년좌우시전화수방:환인신고체질량미재증장,근1년월경규률,주기위35 ～ 40 d,매차지속7d좌우.문헌복습제시성조숙/성조발육병비이왕인위적량성과정,기여다낭란소종합정가능유착공동적발병기출.제비반화고이도소혈증외,황체생성소(LH)분비항진가능위중추성성조숙(CPP)화PCOS공동적원인.문헌시성조숙환인청춘기후PCOS가다체30％차반비반교다.인과영역대청춘기PCOS적진단치료연구소,상무공식.결론 ICPP녀동청춘기후유발전위PCOS적가능성.본병례림상특점지지CPP、PCOS존재공동적발병기출,가능궤제위하구뇌-수체-성선축공능문란,LH분비항진;ICPP치료중LH억제불량화비반현상응해인기족구중시;GnRHa치료적획익화풍험응해충분고필.응재인과영역관주청춘기PCOS적진료연구.
Objective The report was focused on polycystic ovary syndrome(PCOS) in female adolescents related to homosexual precocious puberty,and the new awareness of its influence on long-term health of homosexual precocious girls.Methods A girl with idiopathic central precocious puberty (ICPP) but diagnosed as PCOS in adolescence was reported and the data were reviewed.Results A girl was diagnosed as ICPP when she was near 8 years old with obesity,but without hyperinsulinemia,then she received the treatment of a 3.75 mg dose gonadotropin hormone analogues (GnRHa) every 28 days.Her gonads development was under control while her growth was arrested.Growth hormone(GH) injection started at 13 months.She stopped all medications when finished GH and GnRHa treatment for 12 months and 25 months.She went to see doctors again because of no menarche after discontinuing medication for nearly 2 years.She appeared obese and acne,hirsutism,athanens negricans and purple purple striae on the skin.Hyperinsulinemia and hypertestosterone were demonstrated.Pelvic B ultrasonography showed polycystic ovary,and she was diagnosed as PCOS.She was ordered to lose weight and to take metformin.And adrenocorticotropic hormone stimulating test was done,and B ultrasonography again ruled out atypical congenital adrenal hyperplasia and tumor of adrenal gland.She got her menarche 1 month later.Twelve months after the PCOS diagnosis and treatment,she had 4 menses,her insulin level decreased,glucose tolerance and her serum testosterone level turned normal.At the same time,the form of ovarian and follicular was significantly reduced.After taking mefformin for 20 months,her height and weight did not change,her menstrual was regular every 35-40 days,each time lasting about 7 days.She was followed up.We also reviewed literatures and learnt that precocious puberty might not be a benign situation and it might have an intrinsic relation to obesity,precocious puberty and PCOS.It might be the cofactors for causing PP and PCOS that accessed luteinizing hormone(LH) secretion and disorder of hypothalamus pituitary gonad(HPG) axis except obesity with hyperinsulinemia.Reported pubertas praecox in childhood developed to PCOS at 30％ with high prevalence of obesity.GnRHa suppressive therapy might relate to PCOS and had disputation for improving final height.There were fewer investigations on adolescent PCOS,and no consensus guideline on it in China.Conclusions Girls with ICPP may develop to PCOS some time later.The clinical features of the reported girl and the knowledge from literatures support the hypothesis that inner relationship between the CPP and the PCOS.The LH high secretion and disorder of HPG axis may be the causes of them; LH treatment on suppressing obesity should be cautious during treatment.The benefit and risk from GnRHa treatment should be evaluated thoroughly.Further clinical research should be conducted on adolescent PCOS.
