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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have studied the characteristics of 202 cystic fibrosis adult patients, all with chronic respiratory symptoms, with a median age of 27 yrs (18 to 55 yrs) and a male predominance (56%). At genetic analysis, delta F508 homozygotes were 41%, delta F508 heterozygotes 42% and 17% had no delta F508. The respiratory disease was more severe and complications were more frequent in adults: hemoptysis in 14%, pneumothorax in 15%, lung transplantation in 25 patients. Chronic bronchial colonisation with Pseudomonas aeruginosa, in 76% of patients, contributed to making treatments more severe because of antibiotic i.v. courses and nebulised antibiotics. Respiratory function showed a mean FVC of 62 +/- 22% and a mean FEVI of 48 +/- 94%. External pancreatic insufficiency was found in 83%, diabetes in 14%. Intestinal occlusion syndromes were observed in 11% of patients and hepatic cirrhosis in 8%. In spite of the severity of the respiratory disease, theses patients succeeded in social and occupational insertion; 62% were independent, 18% had children and 77% were working or studying. Analysis of the patients according to age at diagnosis showed that, in 38 patients diagnosed after the age of 18 yrs, the respiratory disease was less severe, pancreatic insufficiency and non-respiratory complications were less frequent (34% had pancreatic insufficiency, 5% had diabetes and none had cirrhosis). This may partly be due to the presence of milder CFTR mutations. In conclusion, cystic fibrosis in adulthood frequently looks like an evolutive form of cystic fibrosis in childhood. Nevertheless, some late diagnosed forms in adults, with better prognosis, have been recently identified.
Rev Mal Respir 2000 Aug
PMID:[Characteristics and specificities of cystic fibrosis in adults: evolutive disease of childhood or recently diagnosed disease?]. 1107 85

Digestive disturbances are frequent in adults with cystic fibrosis. They can lead to malnutrition which in turn is deleterious to the prognosis. We summarise the information on epidemiology, pathogenicity, signs, diagnostic criteria and treatments of exocrine pancreatic insufficiency, gastro-oesophageal reflux and denutrition (which are all frequent) but also of constipation, rectal prolapse, distal intestinal obstruction syndrome, and liver diseases. The main recommendations are the following: 1--know how to treat pancreatic insufficiency with enzyme replacement and vitamins; 2--know how to treat aggressively any gastro-esophageal reflux; 3--diagnose and treat denutrition as early as possible; 4--know the distal intestinal obstruction syndrome to avoid abusive and dangerous surgery; 5--know that the most severe hepatic diseases can be treated by liver transplantation.
Rev Mal Respir 2000 Aug
PMID:[Digestive and nutritional management of adults with cystic fibrosis]. 1107 88

The natural history of cystic fibrosis (CF) may be associated both with acute respiratory complications (respiratory exacerbations, haemoptysis, pneumothorax) and with non-respiratory complications (distal intestinal obstruction syndrome, dehydration) that may result in hospitalizations. The aim of this article is to describe the main therapeutic approaches that are adopted in the management of acute complications occurring in CF adults, and to discuss indications for admission of these patients to intensive care units. Adult CF patients admitted to intensive care unit often benefit from antibiotic courses adapted to their chronic bronchial infection, especially when the hospitalization is related to respiratory disease (including haemoptysis and pneumothorax). Nutritional support, including hypercaloric diet, control of hyperglycemia and pancreatic enzyme supplementation is warranted. The recommended therapy for major haemoptysis is bronchial artery embolization. Patient with significant pneumothorax should have a chest tube inserted, while the treatment of distal intestinal obstruction syndrome will most often be medical. In case of respiratory failure, non-invasive ventilation is the preferred mode of ventilatory support because invasive ventilation is associated with poor outcomes. Therapeutic options should always have been discussed between the patient, family members and the CF medical team to allow for informed decision making.
Rev Mal Respir 2011 Apr
PMID:[Management of acute and severe complications in adults with cystic fibrosis]. 2154 5