지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아소화기영양학회 Pediatric Gastroenterology, Hepatology & Nutrition Pediatric Gastroenterology, Hepatology & Nutrition 제16권 제1호
- 발행연도
- 2013.1
- 수록면
- 28 - 33 (6page)
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초록· 키워드
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Purpose: Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia.
Methods: Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted.
Results: The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems.
Conclusion: This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality. (Pediatr Gastroenterol Hepatol Nutr 2013; 16: 28∼33)
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참고문헌 (23)
참고문헌 신청
Agochukwu NB / 2011 / Analysis of FOXF1 and the FOX gene cluster in patients with VACTERL association / Eur J Med Genet 54 : 323 ~ 328
Aguinaga M / 2010 / Sonic hedgehog mutation analysis in patients with VACTERL association / Am J Med Genet A 152A : 781 ~ 783
Botto LD / 1997 / The spectrum of congenital anomalies of the VATER association: an international study / Am J Med Genet 71 : 8 ~ 15
Casaccia G / 2009 / Congenital gastrointestinal anomalies in anorectal malformations: what relationship and management? / Congenit Anom 49 : 93 ~ 96
Chan BW / 2002 / Maternal diabetes increases the risk of caudal regression caused by retinoic acid / Diabetes 51 : 2811 ~ 2816
Aguinaga M / 2010 / Sonic hedgehog mutation analysis in patients with VACTERL association / Am J Med Genet A 152A : 781 ~ 783
Botto LD / 1997 / The spectrum of congenital anomalies of the VATER association: an international study / Am J Med Genet 71 : 8 ~ 15
Casaccia G / 2009 / Congenital gastrointestinal anomalies in anorectal malformations: what relationship and management? / Congenit Anom 49 : 93 ~ 96
Chan BW / 2002 / Maternal diabetes increases the risk of caudal regression caused by retinoic acid / Diabetes 51 : 2811 ~ 2816
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