Background:Cystic Fibrosis (CF) is a systemic disease affecting extra pulmonary dysfunction as a result of CF-related lung disease. Because of lack of enough studies in this field and utilization of TDE in this field, we aimed to evaluate t...

Cystic fibrosis (CF) is a common progressive genetic disorder among children which involves lungs, kidneys, intestine and liver. Apart from the significance of genetic factors, various environmental factors particularly bone mineral density are directly associated with CF. Remarkably, bone disease is appeared as a routine and commo...

background and objectives: this study was carried out with the objective of determining the genomic variability of p. aeruginosa strains isolated from patients suffering from cystic fibrosis or from environmental cultures collected from different locations in the unit they admitted. materials and methods: a total of 57 clinical and environmental p. aeruginosa isolates were genotyped by enteroba...

BACKGROUND Adherence to treatment is often reported to be low in children with cystic fibrosis. Adherence in cystic fibrosis is an important research area and more research is needed to better understand family barriers to adherence in order for clinicians to provide appropriate intervention. The aim of this study was to evaluate adherence to enzyme supplements, vitamins and chest physiotherapy...

OBJECTIVE To determine whether oral protein energy supplements, used long term in children with cystic fibrosis who are moderately malnourished, improve nutritional and other outcomes. DESIGN Multicentre randomised controlled trial. SETTING Seven specialist paediatric cystic fibrosis centres and their associated shared care clinics and seven smaller paediatric cystic fibrosis clinics. PAR...

Stool energy losses and the sources of energy within the stool were determined in 20 healthy controls and 20 patients with cystic fibrosis while on their habitual pancreatic enzyme replacement treatment. Stool energy losses were equivalent to 3.5% of gross energy intake in healthy children (range 1.3-5.8%). Despite a comparable gross energy intake, stool energy losses were three times greater i...

BackgroundBronchiectasis is defined by permanent and abnormal widening of the bronchi. Although this process occurs in the context of chronic airway infection and inflammation, since there is no accurate estimation of the etiology of the disease. This study aimed to determine the most important cause of bronchiectasis in Tehran, Iran.Materials and MethodsIn this retrospective cohort study we us...

Background: Appendicovesical fistula in patients suffering from cystic fibrosis is a rare condition. Although this situation is so rare it should be considered in the differential diagnosis, dealing with a patient with cystic fibrosis and chronic abdominal pain with no response to primary management. To the best of our knowledge, this is the third case of appendicovesical fistula in a patient w...

Three children with cystic fibrosis developed steatorrhoea unresponsive to changes in pancreatic supplements. The final diagnoses were chronic giardiasis, stagnant loop syndrome, and Crohn's disease. Refractory intestinal symptoms in cystic fibrosis merit further investigation.