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1 483 Vol. 32, pp , 2004 Down Hirschsprung 1 : Hirschsprung Down Down 32 Hirschsprung Down Hirschsprung 269 Hirschsprung Down Hirschsprung intestinal aganglionosis Down 2 Down 3 Down Down g 2 NICU Down X 56 X Caliber change S S 67

2 484 Figure 1. Radiographic findings of the patient taken at the time of admission 2 days after birth. Barium enema shows a distal long narrow segment, a transitional zone arrow, and proximal dilatation of the colon, suggesting a long segment aganglionosis. S long segment aganglionosis Figure 1 38,200 ml, Hb 24.5 g l 63,000 ml AST 54U l, ALT 39U l, Cr 1.0 mg l, BUN 29.4 mg dl, K 5.0 meq l PT- 13, PT-INR 5.21, APTT 120sec Cont, 30.7sec, fibrinogen 194 mg dl, D-dimer 2.1 mg ml Down ; 47,XY, 21 transient abnormal myelopoiesis, TAM 10Fr 24 X 9 Figure 2. Radiographic findings of enterocolitis complicating Hirschspurung[s disease in the present case at the 24 days after birth. Barium enema demonstrates marked mucosal edema with spastic figure of the rectosigmoid and descending colon C S Figure 2 4FMOX CRP 18.0 mg l bacterial translocation AMK Figure

3 Down Hirschsprung 485 Figure 3. Clinical course of the patient. 40 C S 32 6 Duhamel 27 1 Down Hirschsprung 5 6 Carneiro 4 Down Surana 7 Down Down 8 Down Down 9 Down 10 Down T 3 69

4 486 Down 11 Down 2 Down 1 Vanderwinden JM, De Laet MH, Schi#mann SN, Mailleux P, Lowenstein CJ, Snyder SH, Vanderhaeghen JJ. Nitric oxide synthase distribution in the enteric nervous system of Hirschsprung[s disease. Ganstroenterology 1993; 105: Puri P: Hirschsprung[s disease. In: Prem P eds., Newborn Surgery. 2nd ed., Arnold Publishers, London, 2003: Nair MPN, Schwartz SA. Association of decreased T-cell-mediated natural cytotoxicity and interferon production in Down[s syndrome. Clin Immunol Imunopathol 1984; 33: Carneiro PMR, Brereton RJ, Drake DP, Kiely EM, Spitz L, Turnock R. Enterocolitis in Hirschsprung[s disease. Pediatr Surg Int 1992; 7: , 5 Aslam A, Spicer RE, Corfields AP. Children with Hirschsprung[s disease have an abnormal colonic mucus defensive barrier independent of the bowel innervation status. J Pediatr Surg 1997; 32: Lui VCH, Li L, Sham MH, Tam PKH. CDX- 1and CDX-2 are expressed in human colonic mucosa and are down-regulated in patients with Hirschsprung[s disease associated enterocolitis. Biochimica et Biophysica Acta 2001; 1537: , 7 Surana R, Quinn FMJ, Puri P. Evaluation of risk factors in the development of enterocolitis complicating Hirschsprung[s disease. Pediatr Surg Int 1994; 9: , 8 Hirschsprung 1996; 32: Teitelbaum DH, Caniano DA, Qualman SJ. The pathophysiology of Hirschsprung[s-associated enterocolitis: importance of histologic correlates. J Pediatr Surg 1989; 24: Hackman DJ, Reblock K, Barksdale EM, Redlinger R, Lynch J, Gaines BA. The influence of Down[s syndrome on the management and outcome of children with Hirschsprung[s disease. J Pediatr Surg 2003; 38; Down :

5 Down Hirschsprung 487 Abstract ANeonatal Hirschsprung[s Disease Associated with Down[s Syndrome Treatment of the patient[s fulminant enterocolitis Takeshi Aoba, Hirokazu Kawase, Yuriko Satou, Shigeyuki Furuta, Shiho Hamano, Hiroaki Kitagawa, and Kounosuke Nakada We report a neonatal boy with Hirschsprung[s disease su#ering from bowel perforation due to severe enterocolitis. The baby was diagnosed as having Down[s syndrome at birth. He presented an abnormal coagulation screen similar to transient abnormal myelopoiesis TAM. His general condition deteriorated due to severe enterocolitis and he became critically ill. He developed a colonic perforation and underwent emergency laparotomy at 34 days of life. Since there is no reliable prophylactic treatment for fulminant enterocolitis in infants with Hirschsprung[s disease, early diagnosis and an appropriate management of the patient are necessary. This is especially true in cases of Down[s syndrome who may have some immunological insu$ciency. Key words Hirschsprung[s disease, severe colitis, Down[s syndrome Division of Pediatric Surgery, Department of Surgery St. Marianna University School of Medieine Sugao, Miyamae-ku, Kawasaki 71