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성조숙증 아동의 임상 및 내분비 검사의 특징

Clinical and Endocrinologic Characteristics of Children Referred for Precocious Puberty

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주제분류소아과학

초록

목 적 : 성조숙증은 여아에서 8세 미만, 남아에서 9세 미만에 2차 성징이 나타나는 경우로 정의하며, 진성 및 가성 성조숙증으로 구분한다. 성조숙증의 발생빈도는 최근 급격히 증가하고 있어 사회적 문제로 대두되고 있다. 본 연구에서는 성조숙증 증상을 주소로 내원한 환아 들을 대상으로 진단별 분포, 진단명에 따른 임상적 특징을 살펴보고자 하였다. 방 법 : 2003년 1월부터 2007년 6월까지 연세의료원, 상계백병원, 건국의대병원, 분당 차병원에 성조숙증 증상을 주소로 내원한 환아를 진성 성조숙증, 가성 성조숙증, 유방 조기발육증, 조기 사춘기로 분류하였고, 진성 성조숙증은 특발성 성조숙증과 중추신경성 성조숙증으로 분류하였다. 진단 당시의 증상, 가족력, 과거력, 성별, 신장, 체중, 역연령, 골연령, 체질량지수 등을 조사하였다. 기저 및 성선자극호르몬방출호르몬(GnRH) 자극 후 성호르몬, 황체화호르몬(LH), 난포자극호르몬(FSH) 농도를 측정하였다. 원인질환을 확인하기위해 진성 성조숙증으로 진단된 경우에는 터키안 자기공명영상 검사, 가성 성조숙증으로 진단된 경우에는 복부 및 골반 초음파 검사를 시행하였다. 임상적 특징 및 내분비 검사 소견은 각 진단명별로 비교분석하였다. 결 과 : 대상자는 총 948명이었고, 여자는 915명(96.5%), 남자는 33명(3.5%)이었다. 진단별 분포는 조기 사춘기(39 %), 유방 조기발육증(31%), 진성 성조숙증(27%), 가성 성조숙증(1%)의 순서를 보였다. 연성장속도와 골연령의 증가는 진성 성조숙증에서 가장 뚜렷하였다(P<0.05). 진성 성조숙증과 유방 조기발육증군에서 신장 표준편차점수, 체중 표준편차점수가 성별, 나이별 평균보다 유의한 수준으로 증가 되어 있었으며(P<0.05), 모든 군에서 체질량지수는 60 백분위수 이상이었다. 내분비 검사 상 진성 성조숙증과 조기 사춘기의 LH 및 estradiol 기저치와 GnRH 자극 후 LH 최고치, LH 최고치/LH 기저치의 비는 각각 유방 조기발육증보다 높았다(P<0.05). 진성 성조숙증에서 중추신경성 조숙증이 차지하는 비율은 남자에서 42.8%, 여자에서 10.7%로 남자에서 월등히 높았다. 특발성 성조숙증과 중추신경성 성조숙증간에 성호르몬 및 LH, FSH 기저치와 GnRH 자극 후 최고치는 차이가 없었다. 결 론 : 성조숙증 증상을 주소로 내원한 환아 중 진성 성조숙증이 차지하는 비율이 높으므로 보다 자세한 진찰과 검사가 필요하며, 추후 진성 성조숙증의 발생 빈도와 원인에 대한 보다 광범위한 연구가 필요하리라 사료된다.

Abstract

Purpose : Precocious puberty is defined as the onset of secondary sexual characteristics before 8 year of age in girls and 9 year in boys. The purpose of this study is to analyze the spectrum of diagnoses made in a consecutive group of children referred for signs of precocious puberty and evaluate the clinical and endocrinologic characteristics of patients with precocious puberty. Methods : The charts of all 948 children referred for evaluation of signs of precocious puberty between January 2003 and June 2007 in several referral centers were reviewed. Clinical features including age of onset, presenting symptoms, yearly growth rate, bone age advancement, weight, height, and body mass index were analysed and endocrine investigations included basal and gonadotropin releasing hormone (GnRH)-stimulated levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) as well as sex hormones. Results : Of the 948 children referred for signs of precocious puberty, 915 (96.5%) were female and 33 (3.5%) were male. The final diagnoses made were early puberty (39%), premature thelarche (31%), true precocious puberty (27%) and precocious pseudopuberty (1%). The increases in yearly growth rate and bone age advancement were significant in true precious puberty group (P<0.05). The height and weight standard deviation score were significantly increased in true precious puberty and premature thelarche group compared to the average level according to gender and age (P<0.05). Endocrinologic studies showed that the level of basal LH, basal estradiol and GnRH-stimulated peak LH, peak FSH, peak LH/basal LH, peak FSH/basal FSH, peak LH/peak FSH ratio was all significantly higher in true precicous puberty group and early puberty group when compared to premature thelarche group (P<0.05). Neurogenic true precocious puberty among true precocious puberty was more common in boys (3 out of 7, 42.8%) than in girls (27 out of 253, 10.7%). Endocrinologic studies did not show any difference between idiopathic precocious puberty and neurogenic precocious puberty. Conclusion : The result of this study showed the proportion of true precocious puberty among the children referred for early pubertal signs was rather high. Collectively assessing all available data including detailed history, growth records, physical findings, laboratory studies and radiological imaging is important in the evaluation of a child with concerns of early pubertal maturation. Foregoing extensive studies regarding incidence and causes of true precocious puberty should be needed.

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