Complex Saccharide Metabolism in Cystic Fibrosis Fibroblasts
نویسندگان
چکیده
منابع مشابه
Energy metabolism in cystic fibrosis.
Cystic fibrosis (CF) remains a major cause of refractory malnutrition in clinical practice. It is clinically and genetically a heterogeneous disorder, and energy metabolism, as with other manifestations of the disease, is subject to wide variability. Differences in metabolic needs are related to the specific CF genotype, and are the result of a complex interaction of physiological, environmenta...
متن کاملMaintaining Respiratory Health in Cystic Fibrosis Patients
Cystic fibrosis (CF) is an inherited disease that primarily affects the lungs and the digestive system, however, it also affects a number of other organs and systems. More than 90% of mortality of CF patients is due to lung complications. Healthy lungs are important for a long life for people with CF, We will discuss two important topics for maintaining respiratory health. Chronic use of drug...
متن کاملDysregulated Proinflammatory and Fibrogenic Phenotype of Fibroblasts in Cystic Fibrosis
Morbi-mortality in cystic fibrosis (CF) is mainly related to chronic lung infection and inflammation, uncontrolled tissue rearrangements and fibrosis, and yet the underlying mechanisms remain largely unknown. We evaluated inflammatory and fibrosis responses to bleomycin in F508del homozygous and wild-type mice, and phenotype of fibroblasts explanted from mouse lungs and skin. The effect of vard...
متن کاملPrevalence of Cystic Fibrosis Trans-membrane Conductance Regulator Gene common mutations in children with cystic fibrosis in Isfahan, Iran
Background: Cystic fibrosis (CF) is the most common lethal genetic disorder of Cystic Fibrosis Trans-membrane Conductance (CFTR) Regulator gene mutations. We aimed to investigate common mutations in CF patients and to assess its possible relationship with clinical presentations. Materials and Methods: This cross sectional study was conducted on 36 CF patients who were referred to a tertiary ped...
متن کاملBurkholderia cepacia complex and cystic fibrosis
The bacteriae previously known as ‘Burkholderia cepacia’ have been reclassified as nine closely related species, sometimes referred to as ‘genomovars’ (Table 1), (Coenye et al, 2001; Mahenthiralingam et al, 2002). Collectively these are commonly referred to as ‘Burkholderia cepacia complex’ (Bcc). The various genomovars have different pathogenic potential (Keig et al, 1999; Bevivino et al, 2002...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Pediatric Research
سال: 1975
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-197509000-00002