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Russell-Silver 증후군 환아에서 성장 호르몬의 치료효과

Growth Hormone Therapy in Russell-Silver Syndrome

최병규|박상우|방인국|김흥식
대한소아내분비학회 (Korean Society Of Pediatric Endocrinology)|2007.06|Annals of Pediatric Endocrinology & Metabolism|제12권 제1호|pp. 77-81

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참고문헌

주제분류소아과학

초록

자궁내 성장 부전의 가장 심한 형태인 Russell-Silver 증후군에서 가장 문제가 될 수 있는 것이 저신장증이다. 저자들은 Russell-Silver 증후군으로 진단받은 세 명의 환아에게 성장 호르몬 치료 효과를 경험을 하였기에 보고하는 바이다.

Abstract

Russell-Silver syndrome is characterized by low birth weight, growth retardation, delayed bone age, asymmetry, abnormal sexual development, cranio-facial disproportion, short little finger and clinodactyly. Short stature is the major concern, so there have been interests treating Russell-Silver syndrome with human growth hormone. However, there are no large-scale controlled studies to confirm the effectiveness of growth hormone therapy until now because of the rarity of this disorder. In Korea, we cannot find reports dealing with growth hormone treatment in Russell-Silver syndrome. We present three children with Russell-Silver syndrome who showed accelerated growth with growth hormone treatment.

참고문헌 (19)

  1. Price S.M, "The spectrum of Silver-Russell syndrome: a clinical and molecular genetic study and new diagnostic criteria.", J Med Genet, 36, pp.837~842 (1999)
  2. 양세원, "소아기 왜소증의 원인으로서의 Russell-Silver 증후군.", 소아과, 29, pp.715~721 (1986)
  3. Rakover Y, "Growth hormone therapy in Silver Russell syndrome: 5 years experience of the Australian and New Zealand Growth database (OZGROW).", Eur J Pediatr, 155, pp.851~857 (1996)
  4. Rizzo V, "Growth hormone treatment does not alter lower limb asymmetry in children with Russell-Silver syndrome.", Horm Res, 56, pp.114~116 (2001)
  5. Stanhope R, "Growth hormone treatment of Russell-Silver syndrome.", Horm Res, 49, pp.37~40 (1998)
  6. Silver H.K, "Syndrome of congenital hemihypertrophy, shortness of stature, and elevated urinary gonadotropins.", Pediatrics, 12, pp.368~376 (1953)
  7. Russell A, "A syndrome of intra-uterine dwarfism recognizable at birth with cranio-facial dysostosis, disproportionately short arms, and other anomalies (5 examples).", Proc R Soc Med, 47, pp.1040~1044 (1954)
  8. Abraham E, "Musculoskeletal manifestations of Russell-Silver syndrome.", J Pediatr Orthop, 24, pp.552~564 (2004)
  9. Hitchins M.P, "Silver-Russell syndrome: a dissection of the genetic aetiology and candidate chromosomal regions.", J Med Genet, 38, pp.810~819 (2001)
  10. Hannula K, "Genetic screening for maternal uniparental disomy of chromosome 7 in prenatal and postnatal growth retardation of unknown cause.", Pediatrics, 109, pp.441~448 (2002)
  11. Arico M, "The 47,XXY karyotype and unrelated malformative patterns: an unusual association.", Helv Paediatr Acta, 42, pp.457~461 (1987)
  12. Azcona C, "Stanhope R. Hypoglycaemia and Russell- Silver syndrome.", J Pediatr Endocrinol Metab, 18, pp.663~670 (2005)
  13. Lai K.Y, "Cognitive abilities associated with the Silver-Russell syndrome.", Arch Dis Child, 71, pp.490~496 (1994)
  14. Noeker M, "Cognitive development in Silver-Russell syndrome: a sibling-controlled study.", Dev Med Child Neurol, 46, pp.340~346 (2004)
  15. Tanner J.M, "Low birth weight dwarfism with asymmetry (Silver's syndrome): treatment with human growth hormone.", Arch Dis Child, 44, pp.231~243 (1969)
  16. Azcona C, "Growth hormone treatment in growth hormone-sufficient and -insufficient children with intrauterine growth retardation", Russell-Silver syndrome., 1998, pp.50, 22~27
  17. Di Pietro A, "Low levels of somatomedin C and short stature in the Silver-Russell syndrome.", Pediatr Med Chir, 16, pp.153~154 (1994)
  18. Fukushima K, "IGF-related proteins at birth in a case of antenatally diagnosed Silver- Russell syndrome.", Pediatr Res, 51, pp.323~327 (2002)
  19. Azcona C, "Absence of catch-down growth in Russell-Silver syndrome after short-term growth hormone treatment.", Horm Res, 51, pp.47~49 (1999)

관련 논문AI추천

저혈당성 경련으로 내원한 환자에서 진단된 Silver-Russell 증후군 1례김영아, 김자형, 김준성, 박상규, 오기원, 이경연, 이정용, 정진영대한소아신경학회 · 대한소아신경학회지2010 · 제18권 제1호저신장 소아를 위한 성장호르몬 치료박용훈영남대학교 의과대학 · Journal of Yeungnam Medical Science2005 · 제22권 제1호정상 신장 아동의 성장호르몬 치료에 대한 체계적 문헌고찰김정연, 안도희, 윤지은, 정예련한국보건의료기술평가학회 · 보건의료기술평가2025 · 제13권 제1호Effect of growth hormone treatment on children with idiopathic short stature and idiopathic growth hormone deficiency김용대, 임민지, 한헌석대한소아내분비학회 · Annals of Pediatric Endocrinology & Metabolism2017 · 제22권 제2호Noonan 증후군과 성장호르몬결핍증 환아에서 성장호르몬 치료 효과이슬, 권아름, 김호성, 채현욱대한소아내분비학회 · Annals of Pediatric Endocrinology & Metabolism2011 · 제16권 제2호The Effect of Combined Growth Hormone and a Gonadotropin-Releasing Hormone Agonist Therapy on Height in Korean 3-M Syndrome Siblings김유미, 이인경, 임한혁연세대학교의과대학 · Yonsei Medical Journal2020 · 제61권 제11호

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