Cystic fibrosis is an autosomal-recessive disorder. In 1989 the gene mutation that causes cystic fibrosis was localized on the long arm of chromosome 7. Cystic fibrosis occurs in 1/2000 children and the majority now reach adulthood. In view of numerous clinical manifestations of cystic fibrosis, these patients frequently require surgery. Cystic fibrosis is therefore of increasing interest to an...

BACKGROUND The respiratory tract in patients with cystic fibrosis is frequently colonised with Staphylococcus aureus. There is great diversity of clinical practice in this area of cystic fibrosis. A systematic review was conducted to study the evidence relating antistaphylococcal therapy to clinical outcome in patients with cystic fibrosis. METHODS A search strategy already evaluated for the ...

introduction: patients with cystic fibrosis have increased risk of malnutrition. early detection of nutritional deterioration enables prompt intervention and correction. the aims of this project were to: - define the nutritional status of cf patients in iran and new zealand -    compare and contrast the macdonald nutritional screening tool  with the australasian guidelines for nutrition in cyst...

AIM To determine how early diagnosis of cystic fibrosis, using neonatal screening, affects long term clinical outcome. METHODS Fifty seven children with cystic fibrosis born before neonatal screening was introduced (1978 to mid 1981) and a further 60 children born during the first three years of the programme (mid 1981 to 1984), were followed up to the age of 10. The cohorts were compared on ...

A higher frequency (25%) of gastrooesophageal reflux (GOR) has been previously reported in patients over 5 years old with cystic fibrosis compared with controls without cystic fibrosis. It was believed that GOR was caused by the complications of cystic fibrosis. We looked for GOR in all 26 children younger than 60 months who had cystic fibrosis diagnosed. They had a classical genetic profile an...

OBJECTIVES To evaluate fecal elastase 1 (FE) levels in young children with cystic fibrosis and pancreatic insufficiency and to explore the relationship between FE and growth, nutrition, pulmonary status and fat absorption over a 24-month period. METHODS FE, indicating pancreatic lipase activity, was assessed in children (6.0 to 8.9 years of age) with cystic fibrosis and pancreatic insufficien...

BACKGROUND Children with cystic fibrosis may have a deficiency of micronutrients, including zinc, which may affect their susceptibility to infections. There is a paucity of data on zinc supplementation among children with cystic fibrosis. We hypothesized that a pharmacologic dose of zinc administered daily for 12 months would reduce the need for antibiotics by 50%. METHODS This double-blind r...

The mode of presentation, clinical course, and outcome of 12 infants with cystic fibrosis and liver disease referred over an 18 year period were investigated retrospectively. Median age at presentation was 6.5 weeks (range, 5-12). Two thirds were boys. Conjugated hyperbilirubinaemia was the presenting symptom in 11 patients, and hypoalbuminaemia in one. Jaundice was cleared over a median period...

Cystic fibrosis is an autosomal recessive disease caused by a wide spectrum of mutations in the gene encoding for the cystic fibrosis transmembrane conductance regulator protein. These mutations that correlate with different phenotypes, vary in their frequency and distribution in different populations. In this study missense mutation R117H that associated with the different clinical symptoms wa...

In order to investigate whether circulating immune complexes containing Pseudomonas aeruginosa antigens mediate pulmonary damage in cystic fibrosis, we studied lung function, serum immune complex levels, and immunoglobulin concentrations in relationship to chronic pseudomonas colonisation in 69 affected children. Sixteen of the children with cystic fibrosis had increased levels of immune comple...