background cystic fibrosis is a complex progressive disease which assessing its progression and severity is essential. for this purpose there are scoring systems available to evaluate the disease severity. objectives the aim of the present study was to determine the clinical status of cf patients using shwachman score system in the pediatric pulmonary ward of masih daneshvari hospital. patients...

objective(s):  more than 1500 registered mutations in cystic fibrosis transmembrane regulator (cftr) gene are responsible for dysfunction of an ion channel protein and a wide spectrum of clinical manifestations in patients with cystic fibrosis (cf). this study was performed to investigate the frequency of a number of well-known cftr mutations in north eastern iranian cf patients. material and m...

cystic fibrosis (cf) is the most common severe autosomal recessive disorder caused by a wide spectrum of mutations in the gene encoding for the cystic fibrosis transmembrane conductance regulator (cftr) protein. the frequencies, types and distributions of mutations vary widely between different populations and ethnic groups. the aim of this study was to perform a comprehensive analysis of the c...

introduction: bronchiectasis is a common problem in children especially under 5 years. early diagnosis of disease and its causes could be useful in early treatment and preventing probable complications. this study aimed at evaluating the cystic fibrosis (cf) in patients with  bronchiectasis. methods: in a cross-sectional study, 374 children with bronchiectasis were studied. the diagnosis was ma...

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...

background cystic fibrosis (cf) is a genetic disease with an autosomal recessive pattern of inheritance. cf caused by a mutation in the cystic fibrosis transmembrane conductance regulator (cftr) gene and characterized by impaired transport of chloride ions across the cell membrane. staphylococcus aureus, pseudomonas aeruginosa, and burkholderia cepacia have been identified in the cultures of re...

A qualitative diagnosis of infertility requires attention to female and male physical abnormalities, endocrine anomalies and genetic conditions that interfere with reproduction. Many genes are likely to be involved in the complex process of reproduction. Cystic fibrosis (CF) incidence varies in different White people populations (a higher incidence of CF is observed in northern–western European...

cystic fibrosis (cf) is an autosomal recessive disease, which affects many organs as it impairs chloride channel. this study was performed to evaluate growth status and its relationship with some laboratory indices such as cholesterol (chol), triglyceride (tg), albumin and total protein in children with cf referred to pediatrics center. this study was designed as a cross-sectional study in one ...

background: cystic fibrosis (cf) is a common genetic disorder chiefly characterized by respiratory and gastrointestinal symptoms. the purpose of this study was to evaluate the clinical presentation and growth status in cf patients at the time of diagnosis and following conventional treatment. materials and methods: a cross-sectional study was conducted on 67 cf patients who enrolled for at leas...