Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis
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چکیده
منابع مشابه
Meconium ileus equivalent in older patients with cystic fibrosis.
Meconium ileus equivalent is one of the lesser known manifestations of cystic fibrosis, and occurs most often in older patients. With the improved overall survival of patients with cystic fibrosis, one would expect to see this condition more often in the future. Of 53 patients attending our cystic fibrosis clinic for adolescents and adults, eight had experienced a total of 25 episodes of meconi...
متن کاملIntestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs.
Cystic fibrosis (CF) pigs develop disease with features remarkably similar to those in people with CF, including exocrine pancreatic destruction, focal biliary cirrhosis, micro-gallbladder, vas deferens loss, airway disease, and meconium ileus. Whereas meconium ileus occurs in 15% of babies with CF, the penetrance is 100% in newborn CF pigs. We hypothesized that transgenic expression of porcine...
متن کاملMeconium ileus in the absence of cystic fibrosis.
Although meconium ileus in the absence of cystic fibrosis is considered a rare event, it was found that eight of 37 (21.6%) newborn infants with meconium ileus had no laboratory or clinical evidence of cystic fibrosis.
متن کاملDoes presenting with meconium ileus affect the prognosis of children with cystic fibrosis?
It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS).We collected annual lung function data between the ages of 8-15 years in terms of perce...
متن کاملEarly manifestations of cystic fibrosis in a premature patient with complex meconium ileus at birth
OBJECTIVE To report a case of a preterm infant with complex meconium ileus at birth and cystic fibrosis. CASE DESCRIPTION A male infant was born by vaginal delivery at 33 weeks and 5 days of gestational age with respiratory distress and severe abdominal distension. The exploratory laparotomy in the first day of life identified meconium ileus and secondary peritonitis. Ileal resection and ileo...
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ژورنال
عنوان ژورنال: Nature Genetics
سال: 2012
ISSN: 1061-4036,1546-1718
DOI: 10.1038/ng.2221