background: cystic fibrosis (cf) is a common autosomal recessive disorder that affects many body systems and is produced by mutations in the cystic fibrosis transmembrane conductance regulator (cftr) gene. cf is also the most frequently inherited disorder in the west. the aim of this study was to detect the mutations in the cftr gene in two iranian families with cf. methods: after dna extractio...

â  â  b ackground cystic fibrosis (cf) is an autosomal recessive disease caused by a cf trans-membrane regulator (cftr) defect. its prevalence is 1:2500 in caucasians, 1:15300 among african americans and is rare in southâ­east asia. the present study aims to review demographic data, clinical manifestations and laboratory findings of iranian children diagnosed with cf who referred to a childrenâ...

context cystic fibrosis (cf) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the caucasian population. as the median survival has increased related to early multidisciplinary intervention, other manifestations of cf have emergedespecially for the broad spectrum of hepatobiliary involvement. the present study reviews the existing literature on liv...

cystic fibrosis is an inheritant autosomal recessive disease. it is associated with mutations in cystic fibrosis trans regulator gene (cftr) and has different presentations.we report two 2 month old female patients, products of a twin delivery presented with anemia, edema, hypoalbuminemia and pneumonia.after some work ups, diagnosis of cystic fibrosis was confirmed. this is an uncommon and inte...

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: 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...

background and objectives: cystic fibrosis (cf) is an autosomal recessive genetic disease.  infections in these patients gene.are  mostly  caused  by  three  bacteria:  staphylococcus  aureus ,  haemophilus  influenza and  particularly  pseudomonas aeruginosa .  carbapenems including antibiotics are used to combat infections with pseudomonas aeruginosa . in recent years, carbapenems resistant s...