Pediatric Gastroenterology, Hepatology & Nutrition

  • 제13권1호
  • /
  • Pages.51-57
  • /
  • 2010
  • /
  • 2234-8646(pISSN)
  • /
  • 2234-8840(eISSN)
대한소아소화기영양학회 (The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition)
권호 표지

뇌하수체저하증 소아에서 발생한 비알코올성 지방간질환

Nonalcoholic Fatty Liver Disease in Children with Hypopituitarism

  • 윤정민 (서울대학교 의과대학 소아과학교실) ;
  • 고재성 (서울대학교 의과대학 소아과학교실) ;
  • 서정기 (서울대학교 의과대학 소아과학교실) ;
  • 신충호 (서울대학교 의과대학 소아과학교실) ;
  • 양세원 (서울대학교 의과대학 소아과학교실) ;
  • 문진수 (인제대학교 일산백병원 소아과학교실) ;
  • 양혜란 (서울대학교 의과대학 소아과학교실) ;
  • 장주영 (서울대학교 의과대학 소아과학교실)
  • Yoon, Jung-Min (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Ko, Jae-Sung (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Seo, Jeong-Kee (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Shin, Choong-Ho (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Yang, Sei-Won (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Moon, Jin-Soo (Department of Pediatrics, Ilsan Paik Hospital, Inje University) ;
  • Yang, Hye-Ran (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Chang, Ju-Young (Department of Pediatrics, Seoul National University College of Medicine)
  • 투고 : 2010.02.01
  • 심사 : 2010.03.03
  • 발행 : 2010.03.30
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

목 적: 뇌하수체저하증 환아들은 비만, 당불내성, 고지질혈증을 포함한 대사증후군의 양상을 보인다. 이 연구의 목적은 뇌하수체저하증과 연관된 소아 지방간질환의 임상 양상과 간조직 소견을 알아보는 것이다. 방 법: 뇌하수체저하증 환자 중에서 비알코올성 지방간질환으로 진단된 소아 11명의 임상자료를 조사하였다. 결 과: 뇌하수체저하증 진단 시 연령은 10.4${\pm}$3.2세였다. 지방간질환 진단 시 연령은 13.1${\pm}$2.7세였다. 두 개인 두종이 뇌하수체저하증의 기저질환 중 가장 흔하였다. 지방간질환 진단 당시 체질량지수 85백분위수 이상이 9명(82%), 금식 시 고혈당이 5명(45%), 고지질혈증이 9명(82%)에서 발견되었다. 신장의 표준편차 점수의 평균은 뇌하수체저하증 진단 시보다 지방간질환 진단시에 유의하게 낮았다. 간생검을 실시한 6명 중에서 1명은 간경변, 2명은 연결 섬유화가 동반된 지방간염, 2명은 경도의 간문맥 섬유화가 동반된 지방간염, 1명은 단순 지방간으로 진단되었다. 결 론: 뇌하수체저하증을 가진 소아는 저신장, 비만, 고지질혈증, 비알코올성 지방간질환이 발생할 위험이 높다. 간조직의 진행된 섬유화가 흔하기 때문에 뇌하수 체저하증 환아에서 지방간질환의 조기 진단이 중요하다.

Purpose: It has been reported that children with hypopituitarism have features of metabolic syndrome, including obesity, impaired glucose tolerance, and dyslipidemia. The aim of this study was to investigate the clinical features and liver histology of pediatric non-alcoholic fatty liver disease (NAFLD) associated with hypopituitarism. Methods: We reviewed the clinical data of 11 children diagnosed with NAFLD among patients with hypopituitarism. Results: The mean age at the time of diagnosis of hypopituitarism was 10.4${\pm}$3.2 years, and the mean age at the time of diagnosis of NAFLD was 13.1${\pm}$2.7 years. A craniopharyngioma was the most common cause of pituitary dysfunction. At the time of diagnosis of NAFLD, 9 patients (82%) had a body mass index greater than the 85th percentile, 5 patients (45%) had elevated fasting blood glucose levels, and 9 patients (82%) had hypertriglyceridemia. The mean height SD score was significantly lower at the time of diagnosis of NAFLD than at the time of diagnosis of hypopituitarism. Of the six patients who were biopsied, one had cirrhosis, two had non-alcoholic steatohepatitis (NASH) with bridging fibrosis, two had NASH with mild portal fibrosis, and one had simple steatosis. Conclusion: Children with hypopituitarism are at risk of short stature, obesity, dyslipidemia, and NAFLD. The early diagnosis of NAFLD is important in children with hypopituitarism because advanced fibrosis is common.

키워드

참고문헌

  1. Patton HM, Sirlin C, Behling C, Middleton M, Schwimmer JB, Lavine JE. Pediatric nonalcoholic fatty liver disease: a critical appraisal of current data and implications for future research. J Pediatr Gastroenterol Nutr 2006;43:413-27. https://doi.org/10.1097/01.mpg.0000239995.58388.56
  2. Hubscher SG. Histological assessment of non-alcoholic fatty liver disease. Histopathology 2006;49:450-65. https://doi.org/10.1111/j.1365-2559.2006.02416.x
  3. Manco M, Marcellini M, Devito R, Comparcola D, Sartorelli MR, Nobili V. Metabolic syndrome and liver histology in paediatric non-alcoholic steatohepatitis. Int J Obes (Lond) 2008;32:381-7. https://doi.org/10.1038/sj.ijo.0803711
  4. Srinivasan S, Ogle GD, Garnett SP, Briody JN, Lee JW, Cowell CT. Features of the metabolic syndrome after childhood craniopharyngioma. J Clin Endocrinol Metab 2004;89:81-6. https://doi.org/10.1210/jc.2003-030442
  5. Adams LA, Feldstein A, Lindor KD, Angulo P. Nonalcoholic fatty liver disease among patients with hypothalamic and pituitary dysfunction. Hepatology 2004;39:909-14. https://doi.org/10.1002/hep.20140
  6. Lee HH, Byun YH, Shin WY, Kim SH, Song MH, Jo YJ, et al. A case of cryptogenic liver cirrhosis with hypopituitarism. Korean J Intern Med 2007;72:116-20.
  7. Altuntas B, Ozcakar B, Bideci A, Cinaz P. Cirrhotic outcome in patients with craniopharyngioma. J Pediatr Endocrinol Metab 2002;15:1057-8.
  8. Basenau D, Stehphani U, Fischer G. Development of complete liver cirrhosis in hyperphagia-induced fatty liver. Klin Padiatr 1994;206:62-4. https://doi.org/10.1055/s-2008-1046584
  9. Takano S, Kanzaki S, Sato M, Kubo T, Seino Y. Effect of growth hormone on fatty liver in panhypopituitarism. Arch Dis Child 1997;76:537-8. https://doi.org/10.1136/adc.76.6.537
  10. Nakajima K, Hashimoto E, Kaneda H, Tokushige K, Shiratori K, Hizuka N, et al. Pediatric nonalcoholic steatohepatitis associated with hypopituitarism. J Gastroenterol 2005;40:312-5. https://doi.org/10.1007/s00535-004-1541-4
  11. Park JY, Ko JS, Seo JK, Lee R, Shin CH, Kang GH, et al. Nonalcoholic fatty liver disease progressing to cirrhosis in an obese boy with hypopituitarism. Koean J Gastroenterol Nutr 2008;11:204-9.
  12. Brunt EM, Janney CG, Di Bisceglie AM, Neuschwander-Tetri BA, Bacon BR. Nonalcoholic steatohepatitis: a proposal for grading and staging the histological lesions. Am J Gastroenterol 1999;94:2467-74. https://doi.org/10.1111/j.1572-0241.1999.01377.x
  13. Lustig RH, Post SR, Srivannaboon K, Rose SR, Danish RK, Burghen GA, et al. Risk factors for the development of obesity in children surviving brain tumors. J Clin Endocrinol Metab 2003;88:611-6. https://doi.org/10.1210/jc.2002-021180
  14. Muller HL, Bueb K, Bartels U, Roth C, Harz K, Graf N, et al. Obesity after childhood craniopharyngioma--German multicenter study on pre-operative risk factors and quality of life. Klin Padiatr 2001;213:244-9. https://doi.org/10.1055/s-2001-16855
  15. Johansson JO, Fowelin J, Landin K, Lager I, Bengtsson BA. Growth hormone-deficient adults are insulin-resistant. Metabolism 1995;44:1126-9. https://doi.org/10.1016/0026-0495(95)90004-7
  16. Hwu CM, Kwok CF, Lai TY, Shih KC, Lee TS, Hsiao LC, et al. Growth hormone (GH) replacement reduces total body fat and normalizes insulin sensitivity in GHdeficient adults: a report of one-year clinical experience. J Clin Endocrinol Metab 1997;82:3285-92. https://doi.org/10.1210/jc.82.10.3285
  17. Kaji H, Sakurai T, Iguchi G, Murata M, Kishimoto M, Yoshioka S, et al. Adult growth hormone deficiency in Japan: results of investigation by questionnaire. Endocr J 2002;49:597-604. https://doi.org/10.1507/endocrj.49.597
  18. Ko JS, Yoon JM, Yang HR, Myung JK, Kim HR, Kang GH, et al. Clinical and histological features of nonalcoholic fatty liver disease in children. Dig Dis Sci 2009;54:2225-30. https://doi.org/10.1007/s10620-009-0949-3
  19. Loria P, Carulli L, Bertolotti M, Lonardo A. Endocrine and liver interaction: the role of endocrine pathways in NASH. Nat Rev Gastroenterol Hepatol 2009;6:236-47. https://doi.org/10.1038/nrgastro.2009.33
  20. Lonardo A, Loria P, Leonardi F, Ganazzi D, Carulli N. Growth hormone plasma levels in nonalcoholic fatty liver disease. Am J Gastroenterol 2002;97:1071-2. https://doi.org/10.1111/j.1572-0241.2002.05641.x
  21. Ichikawa T, Hamasaki K, Ishikawa H, Ejima E, Eguchi K, Nakao K. Non-alcoholic steatohepatitis and hepatic steatosis in patients with adult onset growth hormone deficiency. Gut 2003;52:914. https://doi.org/10.1136/gut.52.6.914
  22. Fukuda I, Hizuka N, Yasumoto K, Morita J, Kurimoto M, Takano K. Metabolic co-morbidities revealed in patients with childhood-onset adult GH deficiency after cessation of GH replacement therapy for short stature. Endocr J 2008;55:977-84. https://doi.org/10.1507/endocrj.K08E-079
  23. Takahashi Y, Iida K, Takahashi K, Yoshioka S, Fukuoka H, Takeno R, et al. Growth hormone reverses nonalcoholic steatohepatitis in a patient with adult growth hormone deficiency. Gastroenterology 2007;132:938-43. https://doi.org/10.1053/j.gastro.2006.12.024
  24. Evans LM, Davies JS, Anderson RA, Ellis GR, Jackson SK, Lewis MJ, et al. The effect of GH replacement therapy on endothelial function and oxidative stress in adult growth hormone deficiency. Eur J Endocrinol 2000;142:254-62. https://doi.org/10.1530/eje.0.1420254
  25. Liangpunsakul S, Chalasani N. Is hypothyroidism a risk factor for non-alcoholic steatohepatitis? J Clin Gastroenterol 2003;37:340-3. https://doi.org/10.1097/00004836-200310000-00014