Topics Discussed: cystic fibrosis; gastroenterology and hepatology; hepatic disease; liver disease due to cystic fibrosis; systemic disease.
Sections: Heart Disease, Sickle Cell Disease, Hematopoietic Stem Cell Transplantation, Endocrine Disorders, Human Immunodeficiency Virus Infection, Other Immunologic Disorders, Other Systemic Inflammatory Disorders, References.
Excerpt:"Cystic fibrosis (CF) is a genetic multisystem disorder that is
discussed in greater detail in Chapter 512. Cystic fibrosisassociated liver disease is increasingly
recognized as mortality from lung disease decreases. In many cases
the course is benign and does not contribute significantly to morbidity
or mortality. However, in a minority of cystic fibrosis (CF) patients,
liver disease may directly affect survival. The incidence of CF
liver disease appears to peak during adolescence and it is rare
for liver disease to have its onset after 20 years of age. Symptomatic
liver disease is observed in 20% to 50% of patients,
and it can be the presenting or dominant feature of CF. Pathologic
evidence of liver disease is found at autopsy in more than 75% of
patients. Cirrhosis complicates CF in 1.4% of patients,
with a peak frequency of 2.7% in those 16 to 20 years of
age. Cirrhosis now accounts for virtually all nonpulmonary causes
of death in patients with CF.1..."
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