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PEDIATRIC CHRONIC PANCREATITIS IS ASSOCIATED WITH GENETIC RISK FACTORS AND SUBSTANTIAL DISEASE BURDEN

Identifieur interne : 001C85 ( Main/Exploration ); précédent : 001C84; suivant : 001C86

PEDIATRIC CHRONIC PANCREATITIS IS ASSOCIATED WITH GENETIC RISK FACTORS AND SUBSTANTIAL DISEASE BURDEN

Auteurs : Sarah Jane Schwarzenberg [États-Unis] ; Melena Bellin [États-Unis] ; Sohail Z. Husain [États-Unis] ; Monika Ahuja [États-Unis] ; Bradley Barth [États-Unis] ; Heather Davis [États-Unis] ; Peter R. Durie [Canada] ; Douglas S. Fishman [États-Unis] ; Steven D. Freedman [États-Unis] ; Cheryl E. Gariepy [États-Unis] ; Matthew J. Giefer [États-Unis] ; Tanja Gonska [Canada] ; Melvin B. Heyman [États-Unis] ; Ryan Himes [États-Unis] ; Soma Kumar [États-Unis] ; Veronique D. Morinville [Canada] ; Mark E. Lowe [États-Unis] ; Neil E. Nuehring [États-Unis] ; Chee Y. Ooi [Australie] ; John F. Pohl [États-Unis] ; David Troendle [États-Unis] ; Steven L. Werlin [États-Unis] ; Michael Wilschanski [Israël] ; Elizabeth Yen [États-Unis] ; Aliye Uc [États-Unis]

Source :

RBID : PMC:4380827

Descripteurs français

English descriptors

Abstract

Objective

To determine the clinical presentation, diagnostic variables, risk factors and disease burden in children with chronic pancreatitis.

Study design

We performed a cross-sectional study of data from INSPPIRE (International Study Group of Pediatric Pancreatitis: In search for a cuRE), a registry of children with acute recurrent pancreatitis and chronic pancreatitis. Between-group differences were compared using Wilcoxon rank-sum test.

Results

Among 170 subjects in the registry, 76 (45%) had chronic pancreatitis; 57% were female, 80% were Caucasian, median age at diagnosis was 9.9 years. Pancreatitis-predisposing genetic mutations were identified in 51 (67%) and obstructive risk factors in 25 (33%). Toxic/metabolic and autoimmune factors were uncommon. Imaging demonstrated ductal abnormalities and pancreatic atrophy more commonly than calcifications. Fifty-nine (77%) reported abdominal pain within the past year; pain was reported as constant and receiving narcotics in 28%. Children with chronic pancreatitis reported a median of 3 emergency room visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed one day of school in the past month due to chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (p=0.002), visit emergency room (p=0.01) and experience hospitalizations (p=0.03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic ERCP; one or more pancreatic surgeries were performed in 30 (39%).

Conclusions

Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.


Url:
DOI: 10.1016/j.jpeds.2014.11.019
PubMed: 25556020
PubMed Central: 4380827


Affiliations:


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<name sortKey="Giefer, Matthew J" sort="Giefer, Matthew J" uniqKey="Giefer M" first="Matthew J." last="Giefer">Matthew J. Giefer</name>
<affiliation wicri:level="2">
<nlm:aff id="A9">Seattle Children’s Hospital, Seattle, WA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Seattle Children’s Hospital, Seattle, WA</wicri:regionArea>
<placeName>
<region type="state">Washington (État)</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Gonska, Tanja" sort="Gonska, Tanja" uniqKey="Gonska T" first="Tanja" last="Gonska">Tanja Gonska</name>
<affiliation wicri:level="1">
<nlm:aff id="A5">Hospital for Sick Children, Toronto, ON, Canada</nlm:aff>
<country xml:lang="fr">Canada</country>
<wicri:regionArea>Hospital for Sick Children, Toronto, ON</wicri:regionArea>
<wicri:noRegion>ON</wicri:noRegion>
</affiliation>
</author>
<author>
<name sortKey="Heyman, Melvin B" sort="Heyman, Melvin B" uniqKey="Heyman M" first="Melvin B." last="Heyman">Melvin B. Heyman</name>
<affiliation wicri:level="2">
<nlm:aff id="A10">University of California at San Francisco, San Francisco, CA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of California at San Francisco, San Francisco, CA</wicri:regionArea>
<placeName>
<region type="state">Californie</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Himes, Ryan" sort="Himes, Ryan" uniqKey="Himes R" first="Ryan" last="Himes">Ryan Himes</name>
<affiliation wicri:level="2">
<nlm:aff id="A6">Baylor College of Medicine, Houston, TX, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Baylor College of Medicine, Houston, TX</wicri:regionArea>
<placeName>
<region type="state">Texas</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Kumar, Soma" sort="Kumar, Soma" uniqKey="Kumar S" first="Soma" last="Kumar">Soma Kumar</name>
<affiliation wicri:level="2">
<nlm:aff id="A8">Nationwide Children’s Hospital, Columbus, OH, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Nationwide Children’s Hospital, Columbus, OH</wicri:regionArea>
<placeName>
<region type="state">Ohio</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Morinville, Veronique D" sort="Morinville, Veronique D" uniqKey="Morinville V" first="Veronique D." last="Morinville">Veronique D. Morinville</name>
<affiliation wicri:level="4">
<nlm:aff id="A11">Montreal Children’s Hospital, McGill University, Montreal, QC, Canada</nlm:aff>
<country xml:lang="fr">Canada</country>
<wicri:regionArea>Montreal Children’s Hospital, McGill University, Montreal, QC</wicri:regionArea>
<orgName type="university">Université McGill</orgName>
<placeName>
<settlement type="city">Montréal</settlement>
<region type="state">Québec</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Lowe, Mark E" sort="Lowe, Mark E" uniqKey="Lowe M" first="Mark E." last="Lowe">Mark E. Lowe</name>
<affiliation wicri:level="2">
<nlm:aff id="A2">Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Children’s Hospital of Pittsburgh, Pittsburgh, PA</wicri:regionArea>
<placeName>
<region type="state">Pennsylvanie</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Nuehring, Neil E" sort="Nuehring, Neil E" uniqKey="Nuehring N" first="Neil E." last="Nuehring">Neil E. Nuehring</name>
<affiliation wicri:level="2">
<nlm:aff id="A3">University of Iowa Carver College of Medicine, Iowa City, IA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Iowa Carver College of Medicine, Iowa City, IA</wicri:regionArea>
<placeName>
<region type="state">Iowa</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Ooi, Chee Y" sort="Ooi, Chee Y" uniqKey="Ooi C" first="Chee Y." last="Ooi">Chee Y. Ooi</name>
<affiliation wicri:level="1">
<nlm:aff id="A12">Discipline of Paediatrics, School of Women’s and Children’s Health, Medicine, University of New South Wales and Sydney Children’s Hospital Randwick Sydney, Australia</nlm:aff>
<country xml:lang="fr">Australie</country>
<wicri:regionArea>Discipline of Paediatrics, School of Women’s and Children’s Health, Medicine, University of New South Wales and Sydney Children’s Hospital Randwick Sydney</wicri:regionArea>
<wicri:noRegion>University of New South Wales and Sydney Children’s Hospital Randwick Sydney</wicri:noRegion>
</affiliation>
</author>
<author>
<name sortKey="Pohl, John F" sort="Pohl, John F" uniqKey="Pohl J" first="John F." last="Pohl">John F. Pohl</name>
<affiliation wicri:level="2">
<nlm:aff id="A13">University of Utah, Salt Lake City, UT, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Utah, Salt Lake City, UT</wicri:regionArea>
<placeName>
<region type="state">Utah</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Troendle, David" sort="Troendle, David" uniqKey="Troendle D" first="David" last="Troendle">David Troendle</name>
<affiliation wicri:level="2">
<nlm:aff id="A4">University of Texas Southwestern Medical School, Dallas, TX, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Texas Southwestern Medical School, Dallas, TX</wicri:regionArea>
<placeName>
<region type="state">Texas</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Werlin, Steven L" sort="Werlin, Steven L" uniqKey="Werlin S" first="Steven L." last="Werlin">Steven L. Werlin</name>
<affiliation wicri:level="2">
<nlm:aff id="A14">Medical College of Wisconsin, Milwaukee, WI, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Medical College of Wisconsin, Milwaukee, WI</wicri:regionArea>
<placeName>
<region type="state">Wisconsin</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Wilschanski, Michael" sort="Wilschanski, Michael" uniqKey="Wilschanski M" first="Michael" last="Wilschanski">Michael Wilschanski</name>
<affiliation wicri:level="1">
<nlm:aff id="A15">Hadassah Hebrew University Hospital, Jerusalem, Israel</nlm:aff>
<country xml:lang="fr">Israël</country>
<wicri:regionArea>Hadassah Hebrew University Hospital, Jerusalem</wicri:regionArea>
<wicri:noRegion>Jerusalem</wicri:noRegion>
</affiliation>
</author>
<author>
<name sortKey="Yen, Elizabeth" sort="Yen, Elizabeth" uniqKey="Yen E" first="Elizabeth" last="Yen">Elizabeth Yen</name>
<affiliation wicri:level="2">
<nlm:aff id="A10">University of California at San Francisco, San Francisco, CA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of California at San Francisco, San Francisco, CA</wicri:regionArea>
<placeName>
<region type="state">Californie</region>
</placeName>
</affiliation>
</author>
<author>
<name sortKey="Uc, Aliye" sort="Uc, Aliye" uniqKey="Uc A" first="Aliye" last="Uc">Aliye Uc</name>
<affiliation wicri:level="2">
<nlm:aff id="A3">University of Iowa Carver College of Medicine, Iowa City, IA, USA</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Iowa Carver College of Medicine, Iowa City, IA</wicri:regionArea>
<placeName>
<region type="state">Iowa</region>
</placeName>
</affiliation>
</author>
</analytic>
<series>
<title level="j">The Journal of pediatrics</title>
<idno type="ISSN">0022-3476</idno>
<idno type="eISSN">1097-6833</idno>
<imprint>
<date when="2014">2014</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc>
<textClass>
<keywords scheme="KwdEn" xml:lang="en">
<term>Child</term>
<term>Cholangiopancreatography, Endoscopic Retrograde</term>
<term>Cross-Sectional Studies</term>
<term>Cystic Fibrosis Transmembrane Conductance Regulator (genetics)</term>
<term>DNA (genetics)</term>
<term>DNA Mutational Analysis</term>
<term>Female</term>
<term>Genetic Predisposition to Disease</term>
<term>Humans</term>
<term>Incidence</term>
<term>Male</term>
<term>Mutation</term>
<term>Pancreatitis, Chronic (diagnosis)</term>
<term>Pancreatitis, Chronic (epidemiology)</term>
<term>Pancreatitis, Chronic (genetics)</term>
<term>Prevalence</term>
<term>Risk Factors</term>
<term>United States (epidemiology)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr">
<term>ADN (génétique)</term>
<term>Analyse de mutations d'ADN</term>
<term>Cholangiopancréatographie rétrograde endoscopique</term>
<term>Enfant</term>
<term>Facteurs de risque</term>
<term>Femelle</term>
<term>Humains</term>
<term>Incidence</term>
<term>Mutation</term>
<term>Mâle</term>
<term>Pancréatite chronique (diagnostic)</term>
<term>Pancréatite chronique (génétique)</term>
<term>Pancréatite chronique (épidémiologie)</term>
<term>Protéine CFTR (génétique)</term>
<term>Prédisposition génétique à une maladie</term>
<term>Prévalence</term>
<term>États-Unis d'Amérique (épidémiologie)</term>
<term>Études transversales</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en">
<term>Cystic Fibrosis Transmembrane Conductance Regulator</term>
<term>DNA</term>
</keywords>
<keywords scheme="MESH" type="geographic" qualifier="epidemiology" xml:lang="en">
<term>United States</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en">
<term>Pancreatitis, Chronic</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr">
<term>Pancréatite chronique</term>
</keywords>
<keywords scheme="MESH" qualifier="epidemiology" xml:lang="en">
<term>Pancreatitis, Chronic</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en">
<term>Pancreatitis, Chronic</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr">
<term>ADN</term>
<term>Pancréatite chronique</term>
<term>Protéine CFTR</term>
</keywords>
<keywords scheme="MESH" qualifier="épidémiologie" xml:lang="fr">
<term>Pancréatite chronique</term>
<term>États-Unis d'Amérique</term>
</keywords>
<keywords scheme="MESH" xml:lang="en">
<term>Child</term>
<term>Cholangiopancreatography, Endoscopic Retrograde</term>
<term>Cross-Sectional Studies</term>
<term>DNA Mutational Analysis</term>
<term>Female</term>
<term>Genetic Predisposition to Disease</term>
<term>Humans</term>
<term>Incidence</term>
<term>Male</term>
<term>Mutation</term>
<term>Prevalence</term>
<term>Risk Factors</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr">
<term>Analyse de mutations d'ADN</term>
<term>Cholangiopancréatographie rétrograde endoscopique</term>
<term>Enfant</term>
<term>Facteurs de risque</term>
<term>Femelle</term>
<term>Humains</term>
<term>Incidence</term>
<term>Mutation</term>
<term>Mâle</term>
<term>Prédisposition génétique à une maladie</term>
<term>Prévalence</term>
<term>Études transversales</term>
</keywords>
<keywords scheme="Wicri" type="geographic" xml:lang="fr">
<term>États-Unis</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front>
<div type="abstract" xml:lang="en">
<sec id="S1">
<title>Objective</title>
<p id="P1">To determine the clinical presentation, diagnostic variables, risk factors and disease burden in children with chronic pancreatitis.</p>
</sec>
<sec id="S2">
<title>Study design</title>
<p id="P2">We performed a cross-sectional study of data from INSPPIRE (
<bold>
<underline>In</underline>
</bold>
ternational
<bold>
<underline>S</underline>
</bold>
tudy Group of
<bold>
<underline>P</underline>
</bold>
ediatric
<bold>
<underline>P</underline>
</bold>
ancreatitis:
<bold>
<underline>I</underline>
</bold>
n search for a cu
<bold>
<underline>RE</underline>
</bold>
), a registry of children with acute recurrent pancreatitis and chronic pancreatitis. Between-group differences were compared using Wilcoxon rank-sum test.</p>
</sec>
<sec id="S3">
<title>Results</title>
<p id="P3">Among 170 subjects in the registry, 76 (45%) had chronic pancreatitis; 57% were female, 80% were Caucasian, median age at diagnosis was 9.9 years. Pancreatitis-predisposing genetic mutations were identified in 51 (67%) and obstructive risk factors in 25 (33%). Toxic/metabolic and autoimmune factors were uncommon. Imaging demonstrated ductal abnormalities and pancreatic atrophy more commonly than calcifications. Fifty-nine (77%) reported abdominal pain within the past year; pain was reported as constant and receiving narcotics in 28%. Children with chronic pancreatitis reported a median of 3 emergency room visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed one day of school in the past month due to chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (p=0.002), visit emergency room (p=0.01) and experience hospitalizations (p=0.03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic ERCP; one or more pancreatic surgeries were performed in 30 (39%).</p>
</sec>
<sec id="S4">
<title>Conclusions</title>
<p id="P4">Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.</p>
</sec>
</div>
</front>
</TEI>
<affiliations>
<list>
<country>
<li>Australie</li>
<li>Canada</li>
<li>Israël</li>
<li>États-Unis</li>
</country>
<region>
<li>Californie</li>
<li>Iowa</li>
<li>Massachusetts</li>
<li>Minnesota</li>
<li>Ohio</li>
<li>Pennsylvanie</li>
<li>Québec</li>
<li>Texas</li>
<li>Utah</li>
<li>Washington (État)</li>
<li>Wisconsin</li>
</region>
<settlement>
<li>Montréal</li>
</settlement>
<orgName>
<li>Université McGill</li>
</orgName>
</list>
<tree>
<country name="États-Unis">
<region name="Minnesota">
<name sortKey="Schwarzenberg, Sarah Jane" sort="Schwarzenberg, Sarah Jane" uniqKey="Schwarzenberg S" first="Sarah Jane" last="Schwarzenberg">Sarah Jane Schwarzenberg</name>
</region>
<name sortKey="Ahuja, Monika" sort="Ahuja, Monika" uniqKey="Ahuja M" first="Monika" last="Ahuja">Monika Ahuja</name>
<name sortKey="Barth, Bradley" sort="Barth, Bradley" uniqKey="Barth B" first="Bradley" last="Barth">Bradley Barth</name>
<name sortKey="Bellin, Melena" sort="Bellin, Melena" uniqKey="Bellin M" first="Melena" last="Bellin">Melena Bellin</name>
<name sortKey="Davis, Heather" sort="Davis, Heather" uniqKey="Davis H" first="Heather" last="Davis">Heather Davis</name>
<name sortKey="Fishman, Douglas S" sort="Fishman, Douglas S" uniqKey="Fishman D" first="Douglas S." last="Fishman">Douglas S. Fishman</name>
<name sortKey="Freedman, Steven D" sort="Freedman, Steven D" uniqKey="Freedman S" first="Steven D." last="Freedman">Steven D. Freedman</name>
<name sortKey="Gariepy, Cheryl E" sort="Gariepy, Cheryl E" uniqKey="Gariepy C" first="Cheryl E." last="Gariepy">Cheryl E. Gariepy</name>
<name sortKey="Giefer, Matthew J" sort="Giefer, Matthew J" uniqKey="Giefer M" first="Matthew J." last="Giefer">Matthew J. Giefer</name>
<name sortKey="Heyman, Melvin B" sort="Heyman, Melvin B" uniqKey="Heyman M" first="Melvin B." last="Heyman">Melvin B. Heyman</name>
<name sortKey="Himes, Ryan" sort="Himes, Ryan" uniqKey="Himes R" first="Ryan" last="Himes">Ryan Himes</name>
<name sortKey="Husain, Sohail Z" sort="Husain, Sohail Z" uniqKey="Husain S" first="Sohail Z." last="Husain">Sohail Z. Husain</name>
<name sortKey="Kumar, Soma" sort="Kumar, Soma" uniqKey="Kumar S" first="Soma" last="Kumar">Soma Kumar</name>
<name sortKey="Lowe, Mark E" sort="Lowe, Mark E" uniqKey="Lowe M" first="Mark E." last="Lowe">Mark E. Lowe</name>
<name sortKey="Nuehring, Neil E" sort="Nuehring, Neil E" uniqKey="Nuehring N" first="Neil E." last="Nuehring">Neil E. Nuehring</name>
<name sortKey="Pohl, John F" sort="Pohl, John F" uniqKey="Pohl J" first="John F." last="Pohl">John F. Pohl</name>
<name sortKey="Troendle, David" sort="Troendle, David" uniqKey="Troendle D" first="David" last="Troendle">David Troendle</name>
<name sortKey="Uc, Aliye" sort="Uc, Aliye" uniqKey="Uc A" first="Aliye" last="Uc">Aliye Uc</name>
<name sortKey="Werlin, Steven L" sort="Werlin, Steven L" uniqKey="Werlin S" first="Steven L." last="Werlin">Steven L. Werlin</name>
<name sortKey="Yen, Elizabeth" sort="Yen, Elizabeth" uniqKey="Yen E" first="Elizabeth" last="Yen">Elizabeth Yen</name>
</country>
<country name="Canada">
<noRegion>
<name sortKey="Durie, Peter R" sort="Durie, Peter R" uniqKey="Durie P" first="Peter R." last="Durie">Peter R. Durie</name>
</noRegion>
<name sortKey="Gonska, Tanja" sort="Gonska, Tanja" uniqKey="Gonska T" first="Tanja" last="Gonska">Tanja Gonska</name>
<name sortKey="Morinville, Veronique D" sort="Morinville, Veronique D" uniqKey="Morinville V" first="Veronique D." last="Morinville">Veronique D. Morinville</name>
</country>
<country name="Australie">
<noRegion>
<name sortKey="Ooi, Chee Y" sort="Ooi, Chee Y" uniqKey="Ooi C" first="Chee Y." last="Ooi">Chee Y. Ooi</name>
</noRegion>
</country>
<country name="Israël">
<noRegion>
<name sortKey="Wilschanski, Michael" sort="Wilschanski, Michael" uniqKey="Wilschanski M" first="Michael" last="Wilschanski">Michael Wilschanski</name>
</noRegion>
</country>
</tree>
</affiliations>
</record>

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