Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Prader-Willi 증후군 소아에서 성장호르몬 치료의 효과와 부작용

Effects and adverse-effects of growth hormone therapy in children with Prader-Willi syndrome: A two year study

  • 김수진 (성균관대학교 의과대학 소아과학교실) ;
  • 조중범 (성균관대학교 의과대학 소아과학교실) ;
  • 곽민정 (성균관대학교 의과대학 소아과학교실) ;
  • 백경훈 (성균관대학교 의과대학 소아과학교실) ;
  • 권은경 (성균관대학교 의과대학 소아과학교실) ;
  • 진동규 (성균관대학교 의과대학 소아과학교실)
  • Kim, Su Jin (Department of Pediatrics, Sungkyunkwan University School of Medicine) ;
  • Cho, Joong Bum (Department of Pediatrics, Sungkyunkwan University School of Medicine) ;
  • Kwak, Min Jung (Department of Pediatrics, Sungkyunkwan University School of Medicine) ;
  • Paik, Kyung Hoon (Department of Pediatrics, Sungkyunkwan University School of Medicine) ;
  • Kwon, Eun Kyung (Department of Pediatrics, Sungkyunkwan University School of Medicine) ;
  • Jin, Dong-Kyu (Department of Pediatrics, Sungkyunkwan University School of Medicine)
  • 투고 : 2008.01.08
  • 심사 : 2008.05.09
  • 발행 : 2008.07.15
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초록

목 적 : 본 연구에서는 Prader-Willi 증후군 환아에서 성장호르몬 치료의 효과와 부작용을 살펴보고자 하였다. 방 법 : 2004년 3월부터 2007년 8월까지 삼성서울병원에서 2년 이상 성장호르몬(지노트로핀$^{{R)}$) 치료를 받은 41명을 대상으로 하였다. 이들의 신장과 체중, DXA를 이용한 체지방비율을 측정하고 혈액검사를 시행하여 치료 전후를 비교하였다. 결 과 : 41명의 환자 중 남자가 24명, 여자는 17명이었으며, 치료시작 시의 평균 연령은 $7.3{\pm}3.3$세이고 평균 치료기간은 $34.4{\pm}4.8$개월이였다. 치료시작시 Ht-SDS는 $-1.19{\pm}1.37$이였고, Bwt-SDS는 $1.02{\pm}2.42$이었으며, 체지방비율은 $44.6{\pm}9.9%$였다. 치료 2년 후 Ht-SDS는 $-0.02{\pm}1.45$로 증가하였고(P<0.001), Bwt-SDS는 $1.63{\pm}2.22$로 증가하였으며(P=0.002), 체지방비율은 $38.1{\pm}10.5%$로 감소하였다(P<0.001). 치료 전후의 갑상선 기능 검사와 수시혈당은 변화가 없었고, 혈청 콜레스테롤은 감소하였으며, IGF-I은 증가하였다. 당화 혈색소는 $5.2{\pm}0.5%$에서 $5.5{\pm}0.5%$로 증가하였으나 정상범위였다. 성장호르몬을 사용한 적이 있는 환자 77명 중 7명이 부작용으로 인해 치료를 중단하였으며, 부작용은 측만증 등 정형외과적 문제가 가장 많았고, 1명의 환아가 치료 후 아데노이드 비대가 진행하여 아데노이드 절제술을 시행하였다. 결 론 : Prader-Willi 증후군 환아에게서 저신장을 호전시키고, 체지방비율을 감소시키기 위해 성장호르몬 치료는 필요하며, 치료 중 부작용을 모니터링하기 위해 정기적인 경구 당부하 검사를 통한 당대사 이상의 조기 발견과 측만증과 아데노이드 비대의 진행 여부에 대한 관찰이 중요하다.

Purpose : The objective of this study was to evaluate the effects and adverse side-effects of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). Methods : Forty-one patients who had been treated with GH for more than two years (24 boys and 17 girls, mean age $7.3{\pm}3.3$ years during treatment initiation) were enrolled for this study. Results : After 2 years of GH therapy, the height and weight standard deviation scores (SDS) increased significantly ($-1.19{\pm}1.37$ vs. $-0.02{\pm}1.45$, and $1.02{\pm}2.42$ vs. $1.63{\pm}2.22$, P<0.002); however the percentage body fat decreased ($44.6{\pm}9.9%$ vs. $38.1{\pm}10.5%$, P<0.001). Further, no change was observed in the thyroid and serum glucose levels, but the total cholesterol level decreased. GH therapy did not impact glucose control in the patients with diabetes. The most common adverse effects of GH therapy were the progression of scoliosis and adenoid hypertrophy. Conclusion : GH therapy improved the height SDS and body composition in patients with PWS. However, GH should be used with caution in patients with scoliosis and adenoid hypertrophy.

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참고문헌

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피인용 문헌

  1. Quality of Life in Prader-Willi Syndrome Patients vol.1, pp.2, 2015, https://doi.org/10.19125/jmrd.2015.1.2.60
  2. Beneficial Effects of Growth Hormone Treatment in Prader-Willi Syndrome vol.3, pp.2, 2008, https://doi.org/10.19125/jmrd.2017.3.2.41