Meconium ileus.
Meconium ileus is a manifestation of intestinal and pancreatic dysfunction that results in the accumulation of a sticky and inspissated intraluminal meconium, which in most cases results from the autosomal recessive disease cystic fibrosis. Both nonoperative and operative therapies are effective in relieving this small-bowel obstruction; in the past, although less so today, a successful nonoperative treatment was associated with a more favorable outcome. Once the meconium ileus is relieved successfully, and the diagnosis of cystic fibrosis is established, the treatment for the intestinal manifestations of the disease focuses on enzyme replacement to augment patient nutritional status. Simultaneously, the treatment of the life-threatening pulmonary disease focuses on mucous retention and chronic infection in the lungs. Future therapies for patients with cystic fibrosis include lung transplantation, pharmacologic manipulation of the epithelial cell abnormality, and gene transfer therapy into the respiratory epithelium.
Ziegler
M M
MM
Division of Pediatric Surgery, Children's Hospital Medical Center, University of Cincinnati College of Medicine, Ohio.
D003550:Cystic Fibrosis / Q000150:complications / Q000235:genetics; D003937:Diagnosis, Differential; D006801:Humans; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000209:etiology / Q000601:surgery / Q000628:therapy; D008470:Meconium