Yenidoğan Kabızlığında Unutulmaması Gereken Bir Tablo; Hirschprung Hastalığı
Hirschprung Hastalığı enterik sinir sisteminin gelişimsel bir bozukluğu olup distal kolondaki myenterik ve submukozal pleksuslarda ganglion hücrelerinin yokluğu ile karakterizedir. Bu durum etkilenen barsak kısmında peristaltizmin yokluğuna ve fonksiyonel intestinal tıkanıklığa yol açar. Yaklaşık 5000 canlı doğumda bir görülmekte ve genellikle mekonyum çıkışında gecikme, karın distansiyonu, beslenme intoleransı ve safralı kusma ile karşımıza çıkmaktadır. Burada yenidoğan döneminde Hirschprung Hastalığı tanısı konulmuş erkek bebeğin kliniği ve radyolojik tetkikleriyle değerlendirilmesi sunulmuştur.
A Condition Should Be Remembered In Newborn Constipation Hirschprung Disease
Hirschsprung disease is a developmental disorder of the enteric nervous system and is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the distal intestine. This condition results in absence of peristalsis in the affected bowel and the development of a functional intestinal obstruction. It occurs in approximately 1 in 5000 live born infants and usually presented with delay in the meconium passage, distended abdomen, feeding intolerance and bilious vomiting. Here in, clinical and radiological evaluation of a male infant who diagnosed with Hirschsprung's disease in the neonatal period was presented.
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- Koloğlu MB. Yenidoğan Bebeklerde Hirschsprung Hastalığı. Turkiye Klinikleri J Pediatr Surg 2008; 1(2): 52-62
- Martucciello G, Ceccherini I, Lerone M, Jasonni V. Pathogenesis of Hirschsprung's disease. J Pe- diatr Surg. 2000; 35(7): 1017.
- Parisi MA, Kapur RP. Genetics of Hirschsprung disease. Curr Opin Pediatr. 2000; 12(6): 610.
- Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung's disease - a survey of the mem- bers of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg. 1979; 14(5): 588.
- Suita S, Taguchi T, Ieiri S, Nakatsuji T. Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 ye- ars. J Pediatr Surg. 2005; 40(1): 197.
- Clark DA. Times of first void and first stool in 500 newborns. Pediatrics 1977; 60(4): 457-9.
- Ramanath N. Haricharan, MBBS, MPH, Keith E. Georgeson, MD. Hirschsprung disease. Seminars in Pediatr Surg 2008; 17(4): 266-75
- De Lorijn F, Reitsma JB, Voskuijl WP, Aronson DC, Ten Kate FJ, Smets AM, Taminiau JA, Ben- ninga MA. Diagnosis of Hirschsprung's disease: a prospective, comparative accuracy study of common tests. J Pediatr 2005; 146(6): 787
- Garcia R, Arcement C, Hormaza L, Haymon ML, Ward K, Velasco C, Correa H, Congeni JD, Brown R, Tyson P, Udall J, Schmidt-Sommerfeld E. Use of the recto-sigmoid index to diagnose Hirschsprung's disease. Clin Pediatr (Phila) 2007; 46(1): 59
- Osatakul S, Patrapinyokul S, Osatakul N. The diagnostic value of anorectal manometry as a screening test for Hirschsprung's disease. J Med Assoc Thai 1999; 82(11): 1100.
- Lall A, Gupta DK, Bajpai M. Neonatal Hirschsprung's disease. Indian J Pediatr 2000; 67(8): 583.
- Albanese CT, Jennings RW, Smith B, Bratton B, Harrison MR. Perineal one-stage pull-through for Hirschsprung's disease. J Pediatr Surg. 1999; 34(3): 377-80.