히르슈슈프룽병환자에서미성숙신경절세포의빈도및그의의 본연구는서울대학교병원의임상연구비지원에의하여이루어진것임 접수일 게재승인일 교신저자 김현영 서울특별시종로구연건동대학로 서울대학교어린이병원소아외과

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1 히르슈슈프룽병환자에서미성숙신경절세포의빈도및그의의 본연구는서울대학교병원의임상연구비지원에의하여이루어진것임 접수일 게재승인일 교신저자 김현영 서울특별시종로구연건동대학로 서울대학교어린이병원소아외과

2 Fig. 1. H&E and Immunohistochemical Staining of Ganglion Cell. A: Ganglion Cell in H&E, B: Mature Ganglion Cell in MAP-2, C: Immature Ganglion Cell in bcl-2

3 Table 1. Comparisons according to Immunohistochemistry bcl-2(+) (N=55) bcl-2(-) (N=2) p-value Gestational age (w) 39 (32-43) 40 (39-41) Birth weight (kg) 3.20 ( ) 3.41 ( ) Age at operation (d) 143 ( ) 680 ( ) Body weight at operation (kg) 7.89 ( ) ( ) <0.001 Postoperative day (d) 9 (6-15) 12.5 (10-15) Constipation 6 m y Soiling 6 m y Perianal excoriation 6 m y Medication use 6 m y

6 1. Lyford G, Foxx-Orenstein A: Chronic intestinal pseudoobstruction. Curr Treat Options Gastroenterol 7:317-25, Toyosaka A, Okamoto E, Okasora T, Nose K, Tomimoto Y: Clinical, laboratory and prognostic features of congenital large intestinal motor dysfunction (pseudo-hirschsprung's disease). Clin Auton Res 3:243-8, Langer JC: Hirschsprung disease. Curr Opin Pediatr 25:368-74, Miyahara K, Kato Y, Seki T, Arakawa A, Lane GJ, Yamataka A: Neuronal immaturity in normoganglionic colon from cases of Hirschsprung disease, anorectal malformation, and idiopathic constipation. J Pediatr Surg 44:2364-8, Burtelow MA, Longacre TA: Utility of microtubule associated protein-2 (MAP-2) immunohistochemistry for identification of ganglion cells in paraffin-embedded rectal suction biopsies. Am J Surg Pathol 33: , Park SH, Min H, Chi JG, Park KW, Yang HR, Seo JK: Immunohistochemical studies of pediatric intestinal pseudoobstruction: bcl2, a valuable biomarker to detect immature enteric ganglion cells. Am J Surg Pathol 29: , Kim DY, Lee SC, Park KW, Kim WK: Cathepsin D Expression in Intestinal Ganglion Cells of Neonate. J Korean Assoc Pediatr Surg 5:39-44, Feichter S, Meier-Ruge WA, Bruder E: The histopathology of gastrointestinal motility disorders in children. Semin Pediatr Surg 18:206-11, Ieiri S, Nakatsuji T, Akiyoshi J, Higashi M, Hashizume M, Suita S, Taguchi T: Long-term outcomes and the quality of life of Hirschsprung disease in adolescents who have reached 18 years or older--a 47-year single-institute experience. J Pediatr Surg 45: , Menezes M, Pini Prato A, Jasonni V, Puri P: Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review. J Pediatr Surg 43:1696-9, Park KM, Choe YM, Kim JY, Choi SK, Heo YS, Lee KY, Kim SJ, Cho YU, Ahn SI, Hong KC, Shin SH, Kim KR, Seo JM: Clinical experience with Hirschsprung s disease. J Korean Assoc Pediatr Surg 16: , Stensrud KJ, Emblem R, Bjørnland K: Functional outcome after operation for Hirschsprung disease--transanal vs transabdominal approach. J Pediatr Surg 45:1640-4, Sun X, Wang R, Zhang L, Li D, Li Y: Efficacy of pelvic floor muscle training for the treatment of fecal incontinence after Soave procedure for Hirschsprung disease. Eur J Pediatr Surg 22:300-4, Levitt MA, Dickie B, Pena A: The Hirschsprungs patient who is soiling after what was considered a successful pull-through. Semin Pediatr Surg 21: , Friedmacher F, Puri P: Residual aganglionosis after pull-through operation

7 for Hirschsprung's disease: a systematic review and meta-analysis. Pediatr Surg Int 27:1053-7, Bettolli M, De Carli C, Jolin-Dahel K, Bailey K, Khan HF, Sweeney B, Krantis A, Staines WA, Rubin S: Colonic dysmotility in postsurgical patients with Hirschsprung's disease. Potential significance of abnormalities in the interstitial cells of Cajal and the enteric nervous system. J Pediatr Surg 43:1433-8, Kapur RP: Practical pathology and genetics of Hirschsprung's disease. Semin Pediatr Surg 18:212-23, Lawal TA, Chatoorgoon K, Collins MH, Coe A, Peña A, Levitt MA: Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel. J Pediatr Surg 46:342-7, Venugopal S, Mancer K, Shandling B: The validity of rectal biopsy in relation to morphology and distribution of ganglion cells. J Pediatr Surg 16:433-7, Smith B: Pre- and postnatal development of the ganglion cells of the rectum and its surgical implications. J Pediatr Surg 3:386-91, Song Y, Li JC, Li MJ: Bcl-2 expression in enteric neurons of Hirschsprung's disease and its significance. Shi Yan Sheng Wu Xue Bao 35:155-8, Wester T, Olsson Y, Olsen L: Expression of bcl-2 in enteric neurons in normal human bowel and Hirschsprung disease. Arch Pathol Lab Med 123:1264-8, Puri P: Variant Hirschsprung's disease. J Pediatr Surg 32:149-57, Ure BM, Holschneider AM, Schulten D, Meier-Ruge W: Clinical impact of intestinal neuronal malformations: a prospective study in 141 patients. Pediatr Surg Int. 12:377-82, 1997

히르슈슈프룽병의일차성복강경보조 Endorectal Pull-Through 술식의임상적고찰 접수일 게재승인일 교신저자 설지영 대전시중구문화로 충남대학교병원외과

히르슈슈프룽병의일차성복강경보조 Endorectal Pull-Through 술식의임상적고찰 접수일 게재승인일 교신저자 설지영 대전시중구문화로 충남대학교병원외과 Table 1. General Patients and Surgical Characteristics Male : female 32 : 10 Mean age at operation (range) 1 month