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

Identifieur interne : 000A78 ( Pmc/Checkpoint ); précédent : 000A77; suivant : 000A79

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

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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PMC:4380827

Le document en format XML

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<name sortKey="Ooi, Chee Y" sort="Ooi, Chee Y" uniqKey="Ooi C" first="Chee Y." last="Ooi">Chee Y. Ooi</name>
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<name sortKey="Werlin, Steven L" sort="Werlin, Steven L" uniqKey="Werlin S" first="Steven L." last="Werlin">Steven L. Werlin</name>
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<name sortKey="Yen, Elizabeth" sort="Yen, Elizabeth" uniqKey="Yen E" first="Elizabeth" last="Yen">Elizabeth Yen</name>
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<name sortKey="Uc, Aliye" sort="Uc, Aliye" uniqKey="Uc A" first="Aliye" last="Uc">Aliye Uc</name>
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<name sortKey="Davis, Heather" sort="Davis, Heather" uniqKey="Davis H" first="Heather" last="Davis">Heather Davis</name>
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<name sortKey="Durie, Peter R" sort="Durie, Peter R" uniqKey="Durie P" first="Peter R." last="Durie">Peter R. Durie</name>
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<name sortKey="Fishman, Douglas S" sort="Fishman, Douglas S" uniqKey="Fishman D" first="Douglas S." last="Fishman">Douglas S. Fishman</name>
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<name sortKey="Freedman, Steven D" sort="Freedman, Steven D" uniqKey="Freedman S" first="Steven D." last="Freedman">Steven D. Freedman</name>
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<nlm:aff id="A7">Harvard Medical School, Boston, MA, USA</nlm:aff>
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<region type="state">Massachusetts</region>
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<name sortKey="Gariepy, Cheryl E" sort="Gariepy, Cheryl E" uniqKey="Gariepy C" first="Cheryl E." last="Gariepy">Cheryl E. Gariepy</name>
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<nlm:aff id="A8">Nationwide Children’s Hospital, Columbus, OH, USA</nlm:aff>
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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>
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<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>
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<region type="state">Washington (État)</region>
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<name sortKey="Gonska, Tanja" sort="Gonska, Tanja" uniqKey="Gonska T" first="Tanja" last="Gonska">Tanja Gonska</name>
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<nlm:aff id="A5">Hospital for Sick Children, Toronto, ON, Canada</nlm:aff>
<country xml:lang="fr">Canada</country>
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<wicri:noRegion>ON</wicri:noRegion>
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<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></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>
<pmc article-type="research-article">
<pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<pmc-dir>properties manuscript</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-journal-id">0375410</journal-id>
<journal-id journal-id-type="pubmed-jr-id">5127</journal-id>
<journal-id journal-id-type="nlm-ta">J Pediatr</journal-id>
<journal-id journal-id-type="iso-abbrev">J. Pediatr.</journal-id>
<journal-title-group>
<journal-title>The Journal of pediatrics</journal-title>
</journal-title-group>
<issn pub-type="ppub">0022-3476</issn>
<issn pub-type="epub">1097-6833</issn>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">25556020</article-id>
<article-id pub-id-type="pmc">4380827</article-id>
<article-id pub-id-type="doi">10.1016/j.jpeds.2014.11.019</article-id>
<article-id pub-id-type="manuscript">NIHMS642580</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Article</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>PEDIATRIC CHRONIC PANCREATITIS IS ASSOCIATED WITH GENETIC RISK FACTORS AND SUBSTANTIAL DISEASE BURDEN</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author" equal-contrib="yes">
<name>
<surname>Schwarzenberg</surname>
<given-names>Sarah Jane</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author" equal-contrib="yes">
<name>
<surname>Bellin</surname>
<given-names>Melena</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author" equal-contrib="yes">
<name>
<surname>Husain</surname>
<given-names>Sohail Z.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ahuja</surname>
<given-names>Monika</given-names>
</name>
<xref ref-type="aff" rid="A3">3</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Barth</surname>
<given-names>Bradley</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Davis</surname>
<given-names>Heather</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A3">3</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Durie</surname>
<given-names>Peter R.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A5">5</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Fishman</surname>
<given-names>Douglas S.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A6">6</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Freedman</surname>
<given-names>Steven D.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A7">7</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Gariepy</surname>
<given-names>Cheryl E.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A8">8</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Giefer</surname>
<given-names>Matthew J.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A9">9</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Gonska</surname>
<given-names>Tanja</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A5">5</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Heyman</surname>
<given-names>Melvin B.</given-names>
</name>
<degrees>MD, MPH</degrees>
<xref ref-type="aff" rid="A10">10</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Himes</surname>
<given-names>Ryan</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A6">6</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kumar</surname>
<given-names>Soma</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A8">8</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Morinville</surname>
<given-names>Veronique D.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A11">11</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Lowe</surname>
<given-names>Mark E.</given-names>
</name>
<degrees>MD, PhD</degrees>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Nuehring</surname>
<given-names>Neil E.</given-names>
</name>
<xref ref-type="aff" rid="A3">3</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Ooi</surname>
<given-names>Chee Y.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A12">12</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Pohl</surname>
<given-names>John F.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A13">13</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Troendle</surname>
<given-names>David</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Werlin</surname>
<given-names>Steven L.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A14">14</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Wilschanski</surname>
<given-names>Michael</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A15">15</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Yen</surname>
<given-names>Elizabeth</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A10">10</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Uc</surname>
<given-names>Aliye</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A3">3</xref>
</contrib>
</contrib-group>
<aff id="A1">
<label>1</label>
University of Minnesota Children’s Hospital, Minneapolis, Minnesota, USA</aff>
<aff id="A2">
<label>2</label>
Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA</aff>
<aff id="A3">
<label>3</label>
University of Iowa Carver College of Medicine, Iowa City, IA, USA</aff>
<aff id="A4">
<label>4</label>
University of Texas Southwestern Medical School, Dallas, TX, USA</aff>
<aff id="A5">
<label>5</label>
Hospital for Sick Children, Toronto, ON, Canada</aff>
<aff id="A6">
<label>6</label>
Baylor College of Medicine, Houston, TX, USA</aff>
<aff id="A7">
<label>7</label>
Harvard Medical School, Boston, MA, USA</aff>
<aff id="A8">
<label>8</label>
Nationwide Children’s Hospital, Columbus, OH, USA</aff>
<aff id="A9">
<label>9</label>
Seattle Children’s Hospital, Seattle, WA, USA</aff>
<aff id="A10">
<label>10</label>
University of California at San Francisco, San Francisco, CA, USA</aff>
<aff id="A11">
<label>11</label>
Montreal Children’s Hospital, McGill University, Montreal, QC, Canada</aff>
<aff id="A12">
<label>12</label>
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</aff>
<aff id="A13">
<label>13</label>
University of Utah, Salt Lake City, UT, USA</aff>
<aff id="A14">
<label>14</label>
Medical College of Wisconsin, Milwaukee, WI, USA</aff>
<aff id="A15">
<label>15</label>
Hadassah Hebrew University Hospital, Jerusalem, Israel</aff>
<author-notes>
<corresp id="CR1">
<bold>Corresponding author:</bold>
Aliye Uc, M.D., 2865 JPP Stead Family Department of Pediatrics, The University of Iowa Carver College of Medicine, 200 Hawkins Drive, Iowa City, IA 52242; Tel: (319) 384-6032; Fax:(319) 353-8967;
<email>aliye-uc@uiowa.edu</email>
</corresp>
</author-notes>
<pub-date pub-type="nihms-submitted">
<day>18</day>
<month>11</month>
<year>2014</year>
</pub-date>
<pub-date pub-type="epub">
<day>30</day>
<month>12</month>
<year>2014</year>
</pub-date>
<pub-date pub-type="ppub">
<month>4</month>
<year>2015</year>
</pub-date>
<pub-date pub-type="pmc-release">
<day>01</day>
<month>4</month>
<year>2016</year>
</pub-date>
<volume>166</volume>
<issue>4</issue>
<fpage>890</fpage>
<lpage>896.e1</lpage>
<pmc-comment>elocation-id from pubmed: 10.1016/j.jpeds.2014.11.019</pmc-comment>
<permissions>
<copyright-statement>© 2014 Elsevier Inc. All rights reserved.</copyright-statement>
<copyright-year>2014</copyright-year>
</permissions>
<abstract>
<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>
</abstract>
<kwd-group>
<kwd>abdominal pain</kwd>
<kwd>quality of life</kwd>
<kwd>pediatric gastroenterology</kwd>
<kwd>genetics</kwd>
</kwd-group>
</article-meta>
</front>
</pmc>
<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>
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