162 Possibilities of early diagnosis of cystic fibrosis related liver disease (CFLD)
نویسندگان
چکیده
منابع مشابه
Cystic Fibrosis Liver Disease
Cystic fibrosis (CF) is the most common fatal autosomal recessive disorder in the white population with a frequency of 1 in 2500 live births. Inherited defects in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in abnormal regulation of salt and water movement across membranes. The overall feature of CF is that secretions are dehydrated due to water deprivation of lum...
متن کاملliver disease in cystic fibrosis: an update
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...
متن کاملLiver disease in cystic fibrosis
Cystic fibrosis-associated liver disease (CFLD) affects ca. 30% of patients. The CFLD is now considered the third cause of death, after lung disease and transplantation complications, in CF patients. Diagnostics, clinical assessment and treatment of CFLD have become a real challenge since a striking increase of life expectancy in CF patients has recently been observed. There is no elaborated "g...
متن کاملCystic fibrosis and liver disease
As the mean age of survival for patients with cystic fibrosis (CF) continues to improve, the diagnosis and treatment of CF liver disease (CFLD) has become a very relevant clinical issue. Cystic fibrosis liver disease is the third most frequent cause of death in CF after respiratory and transplantation complications and accounts for 2.3% of all mortality (Cystic Fibrosis Foundation, 2002). Liver...
متن کاملLiver disease in cystic fibrosis.
The survival of patients with cystic fibrosis (CF) has progressively increased over recent decades, largely attributable to early diagnosis through newborn screening and advances in nutritional and respiratory care. As the life expectancy of patients with CF has improved, non-respiratory complications such as liver disease have become increasingly recognized. Biochemical derangements of liver e...
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ژورنال
عنوان ژورنال: Journal of Cystic Fibrosis
سال: 2016
ISSN: 1569-1993
DOI: 10.1016/s1569-1993(16)30400-3