소아에서 특발성 뇌하수체저하증의 임상적 특성
The Clinical Characteristics of Idiopathic Hypopituitarism in Children and Adolescents
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목 적: 특발성 뇌하수체저하증은 호르몬 분비 장애에 의해 높은 이환율과 사망률을 보이는 질환임에도 불구하고 국내에서 그 원인과 특성에 대해 기술된 보고가 없었다. 이에 저자들은 뇌의 기질적 병변이 없는 뇌하수체저하증 환자를 대상으로 임상적 특징 및 내분비학적 특성을 알아보고자 하였다. 방 법: 2008년 5월까지 서울대학교 병원에서 특발성 뇌하수체저하증으로 진단 받은 뒤 적절한 호르몬 대체 요법을 시행 받고 있으면서 최종 성인키에 도달한 환자를 대상으로 하였다. 결 과: 관찰 대상은 총 33명으로 이 중 남자는 28명, 여자는 5명이었다. 둔위 분만이 26명으로 79%였고, 첫째 아이로 출생한 경우는 18명으로 55%였다. 내원 당시 평균 연령은 10.1±4.6세였고, 역연령에 비해 골 연령은 평균 4.9±3.0세가 지연되었으며 진단 시 키 표준편차는 -3.6±2.9였다. 성장호르몬 결핍은 모두에서 관찰되었고 갑상선자극호르몬 결핍은 97%, 성선자극호르몬 결핍은 81.8%, 부신피질자극호르몬 결핍은 45.5%, 항이뇨호르몬 결핍은 18.2%에서 나타났다. 결핍된 뇌하수체 호르몬의 종류가 2가지인 경우가 6명(18%), 3가지 결핍은 11명(33%), 4가지 결핍은 14명(43%)이었으며 5가지 호르몬 모두가 결핍된 경우는 2명(6%)이었다. 자발적으로 사춘기가 발현된 경우는 6명으로 (21.4%) 모두 남자였으며, 자발적으로 사춘기가 시작된 군과 사춘기 발현이 없어 호르몬 대체 요법을 시행 받은 군 사이에서 성선자극호르몬 결핍을 제외한 다른 호르몬의 분비 장애 정도에는 유의한 차이가 없었다. 결 론: 저신장을 주소로 내원한 환아들 중 둔위 분만으로 출생한 경우에는 특발성 뇌하수체저하증의 가능성을 염두에 두어 적절한 시기에 자극 검사를 시행하여 호르몬 결핍 여부를 확인하는 것이 필요하다.
Abstract
Purpose: The purpose of this study was to evaluate the clinical evolution and endocrine features of idiopathic hypopituitarism. Methods: We reviewed the medical records of 33 patients with idiopathic hypopituitarism who had been followed up from 1982 to 2008, attained their adult final height, and received appropriate hormonal replacement therapy. Results: 28 of these patients (85%) were male, 26 patients (79%) were delivered by breech presentation, and 18 (55%) were firstborns. The mean age at diagnosis was 10.1±4.6 years, and bone age was delayed a mean of 4.9±3.0 years. The mean standard deviation score (SDS) for height at diagnosis was -3.6±2.9. Deficiencies of pituitary hormones were observed in the following order of frequency: growth hormone (100%), thyroid- stimulating hormone (97.0%), luteinizing hormone/follicle-stimulating hormone (81.8%), adrenocorticotropic hormone (45.5%), and antidiuretic hormone (12.1%). Deficiencies of 4 hormones were observed in 14 (42.4%); 3 hormones, in 11 (33.3%); 2 hormones, in 5 (18.2%); and 5 hormones, in 2 (6.1%). Spontaneous onset of puberty occurred in 6 boys (21.4%), and there were no significant differences in the clinical presentations of pituitary hormone deficiency between these boys and those with gonadotropin-releasing hormone deficiency. Conclusion: Idiopathic hypopituitarism should be considered in patients of short stature who are delivered by breech presentation, and their hormone levels should be monitored periodically.
참고문헌 (22)
- Bates AS, "The effect of hypopituitarism on life expectancy", J Clin Endocrinol Metab, 81, pp.1169~1172 (1996)
- Tomlinson JW, "Association between premature mortality and hypopituitarism. West Midlands Prospective Hypopituitary Study Group", Lancet, 357, pp.425~431 (2001)
- Craft WH, "High incidence of perinatal insult in children with idiopathic hypopituitarism", J Pediatr, 96, pp.397~402 (1980)
- Vance ML, "Hypopituitarism", N Engl J Med, 330, pp.1651~1662 (1994)
- Littley MD, "Hypopituitarism following external radiotherapy for pituitary tumours in adults", Q J Med, 70, pp.145~160 (1989)
- Forest MG, "Adrenal function tests. In:Diagnostics of endocrine function in children and adolescents. 3rd ed", pp.372~426 (2003), Karger
- Blizzard RM, "Hypopituitarism, hypoadrenalism and hypogonadism in the newborn infant", J Pediatr, 48, pp.782~792 (1956)
- Maghnie M, "Hypopituitarism and stalk agenesis: a congenital syndrome worsened by breech delivery?", Horm Res, 35, pp.104~108 (1991)
- Braun FH, "Breech presentation as an indicator of fetal abnormality", J Pediatr, 86, pp.419~421 (1975)
- Brenner WE, "The characteristics and perils of breech presentation", Am J Obstet Gynecol, 118, pp.700~712 (1974)
- Rona RJ, "Aetiology of idiopathic growth hormone deficiency in England and Wales", Arch Dis Child, 52, pp.197~208 (1977)
- Ranke MB, "A note on adults with growth hormone deficiency", Acta Paediatr Scand Suppl, 331, pp.80~82 (1987)
- Distiller LA, "The role of releasing hormones in the diagnosis of hypopituitarism. an analysis of 9 patients", S Afr Med J, 48, pp.1612~1614 (1974)
- Arrigo T, "Catch-up growth and height prognosis in early treated children with congenital hypopituitarism", Horm Res, 44(Suppl 3), pp.26~31 (1995)
- Hayes FJ, "Hypogonadotropic hypogonadism", Endocrinol Metab Clin North Am, 27, pp.739~763 (1998)
- Burns EC, "Final height and pubertal development in 55 children with idiopathic growth hormone deficiency, treated for between 2 and 15 years with human growth hormone", Eur J Pediatr, 137, pp.155~164 (1981)
- Bourguignon JP, "Pubertal growth and final height in hypopituitary boys: a minor role of bone age at onset of puberty", J Clin Endocrinol Metab, 63, pp.376~382 (1986)
- Hibi I, "The influence of gonadal function and the effect of gonadal suppression treatment on final height in growth hormone (GH)-treated GH-deficient children", J Clin Endocrinol Metab, 69, pp.221~226 (1989)
- Frisch H, "Final height and pubertal development in children with growth hormone deficiency after longterm treatment", Horm Res, 43, pp.132~134 (1995)
- Uriarte MM, "The effect of pubertal delay on adult height in men with isolated hypogonadotropic hypogonadism", J Clin Endocrinol Metab, 74, pp.436~440 (1992)
- Arrigo T, "Relationships between neuroradiological and clinical features in apparently idiopathic hypopituitarism", Eur J Endocrinol, 139, pp.84~88 (1998)
- Prabhakar VK, "Aetiology, diagnosis, and management of hypopituitarism in adult life", Postgrad Med J, 82, pp.259~266 (2006)