Question 8: How should distal intestinal obstruction syndrome [DIOS] be managed?

The underlying pathophysiology of Cystic Fibrosis (CF) is related to the abnormal function of chloride and bicarbonate transporters, secondary to mutations in the CF transmembrane conductance regulator (CFTR) gene. While much of the associated morbidity and mortality is due to pulmonary complications, improved life expectancy and late abnormal CF presentations in adulthood have given rise to an increasing awareness of patients suffering from gastrointestinal manifestations of CF [1,2]. Delayed intestinal obstruction syndrome (DIOS), formerly known as ‘meconium ileus equivalent’, describes a range of clinical conditions from incomplete to complete bowel obstruction in the post-neonatal period [3].
Source: Paediatric Respiratory Reviews - Category: Respiratory Medicine Authors: Tags: Cystic Fibrosis: Frequently Asked Questions Source Type: research