Occurrence, outcomes and predictors of portal hypertension in cystic fibrosis: A longitudinal prospective birth cohort study

•Portal hypertension (PH) prevalence of 8.8% in a cohort of 577 children with cystic fibrosis, followed from birth to 18.5 years of age.•Incident rates of 2.81/1000 patient years (3–7 years of age), 3.95/1000 patient years (8–12 years of age) and 2.55/1000 patient years (13–18 years of age).•Higher...

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Veröffentlicht in:Journal of cystic fibrosis Jg. 19; H. 3; S. 455 - 459
Hauptverfasser: Cipolli, Marco, Fethney, Judith, Waters, Donna, Zanolla, Luisa, Meneghelli, Ilaria, Dutt, Shoma, Assael, Baroukh Maurice, Gaskin, Kevin John
Format: Journal Article
Sprache:Englisch
Veröffentlicht: Netherlands Elsevier B.V 01.05.2020
Schlagworte:
Incidence/Prevalence
Liver enzymes
Portal hypertension
Pulmonary/Respiratory
MTx
CF
ALP
GGT
Portal hypertension
Incidence/Prevalence
PH
Liver enzymes
ALT
HR
LFTs
MBC
mortality/transplant
alanine aminotransferase
multilobular biliary cirrhosis
cystic fibrosis
hazard ratio
gamma glutamyl transpeptidase
liver function tests
alkaline phosphatase
ISSN:1569-1993, 1873-5010, 1873-5010
Online-Zugang:Volltext
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Zusammenfassung:•Portal hypertension (PH) prevalence of 8.8% in a cohort of 577 children with cystic fibrosis, followed from birth to 18.5 years of age.•Incident rates of 2.81/1000 patient years (3–7 years of age), 3.95/1000 patient years (8–12 years of age) and 2.55/1000 patient years (13–18 years of age).•Higher mortality/transplant occurrence in those with PH as compared with the non-PH subgroup.•Elevated enzymes ALT, GGT or ALP on 2 or more occasions when ≤6.5 years of age associated with higher risk of PH. The reported prevalence of portal hypertension (PH) in Cystic Fibrosis is variable, incidence rates rarely provided and the utility of liver function tests (LFT's) early in life to predict PH is questionable. The aims were to (1) determine PH prevalence (P) and incidence rate (IR) and combined mortality transplant (MTX) data in PH vs non-PH patients and (2) to assess association of LFTs in early life with liver disease and PH. (1) A double centre longitudinal cohort study of 577 CF patients diagnosed by newborn screening (NBS) with annual examinations for PH up to 18.5 years of age (max) was performed over 28 years for P, IR, and MTX data; (2) Cox proportional hazard models were used to assess the association of elevated LFTs on 2 or more occasions over 0–6.5 years and PH. 51/577(8.8%) developed PH with an average IR of near 3/1000 patient years per 5 year interval representing young, mid and late childhood respectively in patients 3–18 years of age. Combined mortality/liver transplant occurred in 12/51 (23.5%) PH and 25/526 (4.8%) non-PH (p < 0.001). Elevated enzymes particularly GGT (HR:5.71, 95% CI 3.11–10.47); ALT/GGT (HR: 5.56, 95% CI 2.82–10.98); and ALP/GGT (HR: 5.74, 95% CI 2.78–11.86) were associated with the onset of PH. This birth cohort with annual examination for PH provides an accurate assessment of the prevalence, and IR of PH and MTX of PH vs non-PH. Early elevated LFTs are associated with onset of MBC/PH.
Bibliographie:ObjectType-Article-1
SourceType-Scholarly Journals-1
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content type line 23
ISSN:1569-1993
1873-5010
1873-5010
DOI:10.1016/j.jcf.2019.09.016