카할세포 결핍과 연관된 미숙아 가성 장폐쇄 1례

Neonatal Intestinal Pseudo-obstruction Associated with Deficiency of the Interstitial Cells of Cajal in a Premature Infant

저자들은 Hirschsprung 병을 의심한 환아에서 Cajal세포 감소에 의한 미숙아 가성 장 폐쇄를 경험하였기에 보고하는 바이다. 심한 복부 팽만과 장 폐쇄 증상으로 결장 절개술이 필요한 미숙아에서 ganglion cell의 존재 유무 뿐만 아니라 Cajal 세포의 결핍도 확인할 필요가 있을 것으로 생각된다.

The interstitial cells of Cajal are the pacemakers in the gastrointestinal tract that modulate gastrointestinal motility. A case of a neonate with intestinal pseudo-obstruction caused by a decreased number of the interstitial cells of Cajal is presented. A premature male infant born at 32 weeks of gestation showed progressive abdominal distention beginning 3 days after initiation of enteral feeding at 15 days of life. No etiologic factors were identified on radiologic studies, a gastrographin enema, and an intestinal biopsy other than a markedly decreased number of the intestinal cells of Cajal. An ileostomy, followed by repair of the ileostomy was done, which resulted in but a limited improvement of the abdominal gas pattern. Respiratory distress, pancytopenia, and abdominal distention persisted, and the infant expired on 142 days of life.