Cystic fibrosis: an update for clinicians. Part 2: hepatobiliary and pancreatic manifestations.
نویسندگان
چکیده
This paper, the second in the series, will build on the first and explore the importance of liver and pancreatic manifestations of cystic fibrosis (CF) and the effect on morbidity and mortality of this multifaceted genetic condition. It will also further develop the critical role of the gastroenterologist as part of the multidisciplinary group of clinicians and allied health staff in the effective management of patients with CF.
منابع مشابه
Gastrointestinal Manifestations of Cystic Fibrosis.
Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobili...
متن کاملPrevention of chronic Pseudomonas aeruginosa infection in people with cystic fibrosis.
Cystic fibrosis is the most common genetically inherited disease in the Caucasian population, with approximately 30,000 patients in the USA and more than 50,000 patients worldwide. The primary defect in the cystic fibrosis transmembrane regulator gene affects the production and/or function of the cystic fibrosis transmembrane regulator protein. Depending on the severity of the genetic defect, p...
متن کاملAnimal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology.
Multiple organ systems, including the gastrointestinal tract, pancreas, and hepatobiliary systems, are affected by cystic fibrosis (CF). Many of these changes begin early in life and are difficult to study in young CF patients. Recent development of novel CF animal models has expanded opportunities in the field to better understand CF pathogenesis and evaluate traditional and innovative therape...
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 emerged especially for the broad spectrum of hepatobiliary involvement. The present study reviews the existing literature on li...
متن کاملAn update on cystic fibrosis and implications for anesthesia.
Cystic fibrosis is a recessive inherited disorder that affects about 30,000 Americans. The majority of infants born with cystic fibrosis now reach adulthood, making surgical procedures performed in this patient group more common. The disease is a heterogeneous disorder caused by widespread dysfunction of exocrine glands and involving salivary, sweat, digestive, and pulmonary secretions. The bro...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Journal of gastroenterology and hepatology
دوره 29 12 شماره
صفحات -
تاریخ انتشار 2014