Revista de Ciencias Médicas de Pinar del Río
versión On-line ISSN 1561-3194
PILONA RUIZ, Sergio et al. Meconium peritonitis and cystic fibrosis. Rev Ciencias Médicas [online]. 2008, vol.12, n.1, pp. 22-28. ISSN 1561-3194.
Cystic fibrosis (CF) is a genetic autosomal recesive disease caused by more than 600 known mutatins of the gene that codifies for the protein of the membrane: Regulator of the CF transmembrane (CFTR). It is a disease of the neonatalperiod that can be manifested by respiratory distress, intrahepatic cholestasis and meconium ileus. A case of amale newborn (throug a cesarean section delivery), suspecting the diagnosis of Cystic Fibrosis is reported. In prenatal echographies signs of meconium peritonitis due to meconium ileus was observed, the patient underwent a surgery where a great quantity of fibrin covering all intestinalloops with adherences showing a perforation inthe ileum jejune join was observed. The segment was filled with a hard andcompact meconium. An ileostomy was performed, then the patient presented a clinical and hemodynamic worsening with cholestatic jaundice and respiratory distress deteriorating his condition up to showing signs of septic shock and multiple-organ failure, dying at 6 days old. The post-mortem pathologic study matched with a cystic fibrosis.
Palabras llave : Cystic fibrosis; meconium ileus; bowel obstruction; meconium peritonitis.