AustralieFrV1, Main, Curation, bibRecord, 001E95

Features of Severe Liver Disease With Portal Hypertension in Patients with Cystic Fibrosis

Identifieur interne : 001E95 ( Main/Curation ); précédent : 001E94; suivant : 001E96

Features of Severe Liver Disease With Portal Hypertension in Patients with Cystic Fibrosis

Auteurs : Jaclyn R. Stonebraker [États-Unis] ; Chee Y. Ooi [Australie] ; Rhonda G. Pace [États-Unis] ; Harriet Corvol [France] ; Michael R. Knowles [États-Unis] ; Peter R. Durie [Canada] ; Simon C. Ling [Canada]

Source :

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association [ 1542-3565 ] ; 2016.

RBID : PMC:4955685

Descripteurs français

KwdFr :
- Adolescent, Adulte, Adulte d'âge moyen, Enfant, Enfant d'âge préscolaire, Facteurs sexuels, Femelle, Humains, Hypertension portale (anatomopathologie), Hypertension portale (épidémiologie), Jeune adulte, Maladies du foie (), Maladies du foie (anatomopathologie), Maladies du foie (épidémiologie), Mucoviscidose (), Mâle, Études rétrospectives.
MESH :
- anatomopathologie : Hypertension portale, Maladies du foie.
- épidémiologie : Hypertension portale, Maladies du foie.
- Adolescent, Adulte, Adulte d'âge moyen, Enfant, Enfant d'âge préscolaire, Facteurs sexuels, Femelle, Humains, Jeune adulte, Maladies du foie, Mucoviscidose, Mâle, Études rétrospectives.

English descriptors

KwdEn :
- Adolescent, Adult, Child, Child, Preschool, Cystic Fibrosis (complications), Female, Humans, Hypertension, Portal (epidemiology), Hypertension, Portal (pathology), Liver Diseases (complications), Liver Diseases (epidemiology), Liver Diseases (pathology), Male, Middle Aged, Retrospective Studies, Sex Factors, Young Adult.
MESH :
- complications : Cystic Fibrosis, Liver Diseases.
- epidemiology : Hypertension, Portal, Liver Diseases.
- pathology : Hypertension, Portal, Liver Diseases.
- Adolescent, Adult, Child, Child, Preschool, Female, Humans, Male, Middle Aged, Retrospective Studies, Sex Factors, Young Adult.

Abstract

Background & Aims

Liver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been fully characterized.

Methods

We performed a retrospective analysis of data from 561 patients with CF (63% male, 99% with pancreatic insufficiency), liver disease (hepatic parenchymal abnormalities consistent with cirrhosis, confirmed by imaging), and portal hypertension (esophageal varices, portosystemic collaterals, or splenomegaly), with no alternate causes of liver disease. All patients were enrolled in the Genetic Modifier Study of Severe CF Liver Disease at 76 international centers, from January 1999 through July 2013.

Results

Male patients were diagnosed with liver disease at a younger age than female patients (10 vs 11 years; P=.01). Splenomegaly was observed in 99% of patients and varices in 71%. Levels of liver enzymes were near normal in most patients. Thrombocytopenia affected 70% of patients and was more severe in patients with varices (88×10⁹/L vs 145×10⁹/L; P<.0001). Ninety-one patients received liver transplants (16%), at a median age of 13.9 years. Compared to patients who did not receive liver transplants, patients who received liver transplants had lower platelet counts (78×10⁹/L vs 113×10⁰/L; P<.0001), higher international normalized ratios (P<.0001), and lower levels of albumin (P=.0002). The aminotransferase to platelet ratio index (APRi) and fibrosis index based on 4 factor (FIB-4) values were above diagnostic thresholds for CF liver disease in 96% and 90% of patients, respectively. Patients who received liver transplants or who had varices had higher APRi and FIB-4 values than patients who did not.

Conclusions

In patients with CF, severe liver disease develops early in childhood (around 10 years of age) and is more common in boys than girls. Patients with varices and those who receive liver transplants have more abnormal platelet counts and APRi and FIB-4 scores.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4955685

DOI: 10.1016/j.cgh.2016.03.041
PubMed: 27062904
PubMed Central: 4955685

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Le document en format XML

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<term>Adult</term>
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<term>Female</term>
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<term>Hypertension, Portal (epidemiology)</term>
<term>Hypertension, Portal (pathology)</term>
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<term>Liver Diseases (epidemiology)</term>
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<term>Hypertension portale (épidémiologie)</term>
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<term>Maladies du foie (épidémiologie)</term>
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<term>Liver Diseases</term>
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<keywords scheme="MESH" qualifier="epidemiology" xml:lang="en"><term>Hypertension, Portal</term>
<term>Liver Diseases</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Hypertension, Portal</term>
<term>Liver Diseases</term>
</keywords>
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<term>Maladies du foie</term>
</keywords>
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<term>Adult</term>
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<term>Adulte</term>
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<term>Jeune adulte</term>
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</teiHeader>
<front><div type="abstract" xml:lang="en"><sec id="S1"><title>Background & Aims</title>
<p id="P2">Liver disease is the third leading cause of death in patients with cystic fibrosis (CF), but features of patients with CF, severe liver disease, and portal hypertension have not been fully characterized.</p>
</sec>
<sec id="S2"><title>Methods</title>
<p id="P3">We performed a retrospective analysis of data from 561 patients with CF (63% male, 99% with pancreatic insufficiency), liver disease (hepatic parenchymal abnormalities consistent with cirrhosis, confirmed by imaging), and portal hypertension (esophageal varices, portosystemic collaterals, or splenomegaly), with no alternate causes of liver disease. All patients were enrolled in the Genetic Modifier Study of Severe CF Liver Disease at 76 international centers, from January 1999 through July 2013.</p>
</sec>
<sec id="S3"><title>Results</title>
<p id="P4">Male patients were diagnosed with liver disease at a younger age than female patients (10 vs 11 years; <italic>P</italic>
=.01). Splenomegaly was observed in 99% of patients and varices in 71%. Levels of liver enzymes were near normal in most patients. Thrombocytopenia affected 70% of patients and was more severe in patients with varices (88×10<sup>9</sup>
/L vs 145×10<sup>9</sup>
/L; <italic>P</italic>
<.0001). Ninety-one patients received liver transplants (16%), at a median age of 13.9 years. Compared to patients who did not receive liver transplants, patients who received liver transplants had lower platelet counts (78×10<sup>9</sup>
/L vs 113×10<sup>0</sup>
/L; <italic>P</italic>
<.0001), higher international normalized ratios (<italic>P</italic>
<.0001), and lower levels of albumin (<italic>P</italic>
=.0002). The aminotransferase to platelet ratio index (APRi) and fibrosis index based on 4 factor (FIB-4) values were above diagnostic thresholds for CF liver disease in 96% and 90% of patients, respectively. Patients who received liver transplants or who had varices had higher APRi and FIB-4 values than patients who did not.</p>
</sec>
<sec id="S4"><title>Conclusions</title>
<p id="P5">In patients with CF, severe liver disease develops early in childhood (around 10 years of age) and is more common in boys than girls. Patients with varices and those who receive liver transplants have more abnormal platelet counts and APRi and FIB-4 scores.</p>
</sec>
</div>
</front>
</TEI>
</record>

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Features of Severe Liver Disease With Portal Hypertension in Patients with Cystic Fibrosis

Features of Severe Liver Disease With Portal Hypertension in Patients with Cystic Fibrosis

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