TITLE

Outcome in Cystic Fibrosis Liver Disease

AUTHOR(S)
Rowland, Marion; Gallagher, Charles G.; Ó'Laoide, Risteard; Canny, Gerard; Broderick, Annemarie; Hayes, Roisin; Greally, Peter; Slattery, Dubhfeassa; Daly, Leslie; Durie, Peter; Bourke, Billy
PUB. DATE
January 2011
SOURCE
American Journal of Gastroenterology;Jan2011, Vol. 106 Issue 1, p104
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
OBJECTIVES:Evidence suggests that cystic fibrosis liver disease (CFLD) does not affect mortality or morbidity in patients with cystic fibrosis (CF). The importance of gender and age in outcome in CF makes selection of an appropriate comparison group central to the interpretation of any differences in mortality and morbidity in patients with CFLD.METHODS:This is a 7-year follow-up of 42 children with CFLD and their age- and sex-matched controls. Participants were reviewed clinically, biochemically, and radiologically at follow-up.RESULTS:Overall, 85% (72 of 84) of the original cohort were included, 36 CFLD participants and 36 CF controls. There was no significant difference in the number of deaths/transplants between groups (7 of 36 (19.4%) CFLD participants, 3 of 36 (8.3%) CF controls). There was a tendency for participants with CFLD to die younger than their respective CF controls. There was no difference in height, weight, body mass index, or pulmonary function between the groups. Nutritional parameters (sum skinfold thickness 31.6 vs. 42.3, P=0.03; mean upper arm fat area 15.08 vs. 10.59, P=0.001; Shwachman score 43.7 vs. 32.1, P=0.001) were worse among CFLD participants than among CF controls. Cystic fibrosis-related diabetes was more common in CFLD participants (11 of 27 (40.7%) vs. 5 of 33 (15.2%), P=0.02). Eight children (22.2%) with evidence of CFLD at baseline had no clinical evidence of liver disease as adults.CONCLUSIONS:Patients with CFLD have a more severe CF phenotype than do CF patients without liver disease. However, a subgroup of children with CFLD will not manifest clinically significant liver disease as adults.
ACCESSION #
57161919

 

Related Articles

  • EARLY DIAGNOSIS OF CYSTIC FIBROSIS MAY PROTECT AGAINST DEVELOPMENT OF CYSTIC FIBROSIS-ASSOCIATED LIVER DISEASE.  // Archives of Disease in Childhood;Apr2003 Supplement, Vol. 88, pA66 

    Presents an abstract of the study 'Early Diagnosis of Cystic Fibrosis May Protect Against Develoment of Cystic Fibrosis-Associated Liver Disease: Population-Based Case Control Study,' by S. Kelleher, K. Corbett, M. Rowland et al, presented at the 7th Spring Meeting of the Royal College of...

  • Cystic Fibrosis-Related Diabetes: Current Trends in Prevalence, Incidence, and Mortality. Moran, Antoinette; Dunitz, Jordan; Nathan, Brandon; Saeed, Asad; Holme, Bonnie; Thomas, William // Diabetes Care;Sep2009, Vol. 32 Issue 9, p1626 

    OBJECTIVE -- Cystic fibrosis (CF)-related diabetes (CFRD) diagnosis and management have considerably changed since diabetes was first shown to be associated with a poor prognosis in subjects with CF. Current trends in CFRD prevalence, incidence, and mortality were determined from a comprehensive...

  • Hyperglycemia and death in cystic fibrosis-related diabetes.  // Current Medical Literature: Diabetes;2011, Vol. 28 Issue 4, p146 

    The article reports on a study which shows the importance of cystic fibrosis-related diabetes (CFRD) as an indicator of mortality risk and which identifies three significant risk factors such as body mass index (BMI), forced expiratory volume (FEV) and glycated hemoglobin.

  • Determinants of Mortality from Cystic Fibrosis in Canada, 1970–1989. Corey, Mary; Farewell, Vemon // American Journal of Epidemiology;1996, Vol. 143 Issue 10, p1007 

    The frequency, prevalence, and mortality patterns of cystic fibrosis were analyzed in 3,795 patients documented in the Canadian Patient Data Registry in 1970–1989. Cystic fibrosis frequency in the 1970–1979 birth cohort was virtually identical to the commonly quoted 1 in 2,500. In...

  • Cystic Fibrosis Presenting as Massive Hepatomegaly. Arumugam, Ramalingam; Reid, Barbara // Clinical Pediatrics;Dec1999, Vol. 38 Issue 12, p731 

    Presents a case study that illustrates the importance of the consideration of cystic fibrosis in the differential diagnosis of children with liver disease. Details on the patient's physical examination; Result of the patient's laboratory evaluation; Result of the patient's CT scan; Discussion.

  • Love your liver. Birch, Kate // Practice Nursing;Mar2008, Vol. 19 Issue 3, p110 

    The article discusses the prevalence of liver disease in Great Britain. According to the author, liver disease is one of the top five causes of mortality in the country which is expected to increase because of alcohol abuse, viral hepatitis and fatty liver. In addition, the author notes that...

  • CYSTIC FIBROSIS DEATHS IN USA AND UK: COMPARISONS OF REGISTRY AND ROUTINE DATA. Chowdhury, F.; Wildman, M. J.; Gunn, E.; Bilton, D. // Thorax;Dec2011 Supplement, pA163 

    Background MDT care in dedicated centres has been a contributor to improved Cystic Fibrosis (CF) survival and in UK and USA outcomes for patients in centres, and is collected by national CF registries. Routine national death data using ICD-10 codes will identify all CF deaths giving an...

  • Intl Liver Congress 2012: Chronic hepatic diseases generate high costs to Europe.  // Biomedical Market Newsletter;4/21/2012, Vol. 21, p1 

    The article offers information on two studies presented at the International Liver Congress 2012 which show the impact of liver disease across Europe.

  • Declining prevalence of cystic fibrosis since the introduction of newborn screening. Massie, John; Curnow, Lisette; Gaffney, Lydia; Carlin, John; Francis, Ivan // Archives of Disease in Childhood -- Fetal & Neonatal Edition;Jul2010, Vol. 95 Issue 7, p10 

    Objectives Newborn screening for cystic fibrosis (CF) facilitates early diagnosis and genetic counselling for parents of affected infants. Many parents elect to use prenatal testing for subsequent pregnancies, and this may affect the prevalence of CF. The aim of this study was to assess the...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics