Abstract

Background. The severe complications caused by cystic fibrosis require comprehensive study. Consideration of rare clinical variants of the course of the disease is an important task for understanding the polymorphism of cystic fibrosis clinical manifestations. The aim of the work. To present a clinical case of congenital meconium peritonitis, small intestine volvulus and necrosis of meconium pseudocyst of the abdominal cavity associated with cystic fibrosis, for further treatment improvement and prevention of complications. Results. The article presents a clinical case of cystic fibrosis caused by a combination of pathogenic mutations in the CFTR gene (mutation delta F508; p.Phe508 and CFTRdele2,3). Against this background, meconium pseudo-cyst, meconium peritonitis and small intestine volvulus developed. Conclusion. Doctors are required to be wary of hereditary pathology if patients have concomitant congenital intestinal malformations.

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