Risk factors associated with pediatric acute recurrent and chronic pancreatitis

Lessons from INSPPIRE

Soma Kumar, Chee Y. Ooi, Steven Werlin, Maisam Abu-El-Haija, Bradley Barth, Melena D. Bellin, Peter R. Durie, Douglas S. Fishman, Steven D. Freedman, Cheryl Gariepy, Matthew J. Giefer, Tanja Gonska, Melvin B. Heyman, Ryan Himes, Sohail Z. Husain, Tom K. Lin, Mark E. Lowe, Veronique Morinville, Joseph J. Palermo, John F. Pohl & 5 others Sarah Jane Schwarzenberg, David Troendle, Michael Wilschanski, M. Bridget Zimmerman, Aliye Uc

Research output: Contribution to journalArticle

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Abstract

IMPORTANCE: Pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) are poorly understood. OBJECTIVE: To characterize and identify risk factors associated with ARP and CP in childhood. DESIGN, SETTING, AND PARTICIPANTS: A multinational cross-sectional study of children with ARP or CP at the time of enrollment to the INSPPIRE (International Study Group of Pediatric Pancreatitis: In Search for a Cure) study at participant institutions of the INSPPIRE Consortium. From August 22, 2012, to February 8, 2015,155 children with ARP and 146 with CP (aged <19 years) were enrolled. Their demographic and clinical information was entered into the REDCap (Research Electronic Data Capture) database at the 15 centers. Differences were analyzed using 2-sample t test or Wilcoxon rank sum test for continuous variables and Pearson Χ2 test or Fisher exact test for categorical variables. Disease burden variables (pain variables, hospital/emergency department visits, missed school days) were compared using Wilcoxon rank sum test. MAIN OUTCOMES AND MEASURES: Demographic characteristics, risk factors, abdominal pain, and disease burden. RESULTS: A total of 301 children were enrolled (mean [SD] age, 11.9 [4.5] years; 172 [57%] female); 155 had ARP and 146 had CP. The majority of children with CP (123 of 146 [84%]) reported prior recurrent episodes of acute pancreatitis. Sex distribution was similar between the groups (57% female in both). Hispanic children were less likely to have CP than ARP (17% vs 28%, respectively; odds ratio [OR] = 0.51; 95% CI, 0.29-0.92; P =.02). At least 1 gene mutation in pancreatitis-related genes was found in 48% of patients with ARP vs 73% of patients with CP(P <.001). Children with PRSS1 or SPINK1 mutations were more likely to present with CP compared with ARP (PRSS1: OR = 4.20; 95% CI, 2.14-8.22; P <.001; and SPINK1: OR = 2.30; 95% CI, 1.03-5.13; P =.04). Obstructive risk factors did not differ between children with ARP or CP (33% in both the ARP and CP groups), but toxic/metabolic risk factors were more common in children with ARP (21% overall; 26% in the ARP group and 15% in the CP group; OR = 0.55; 95% CI, 0.31-0.99; P =.046). Pancreatitis-related abdominal pain was a major symptom in 81% of children with ARP or CP within the last year. The disease burden was greater in the CP group compared with the ARP group (more emergency department visits, hospitalizations, and medical, endoscopic, and surgical interventions). CONCLUSIONS AND RELEVANCE: Genetic mutations are common in both ARP and CP. Ethnicity and mutations in PRSS1 or SPINK1 may influence the development of CP. The high disease burden in pediatric CP underscores the importance of identifying predisposing factors for progression of ARP to CP in children.

Original languageEnglish (US)
Pages (from-to)562-569
Number of pages8
JournalJAMA Pediatrics
Volume170
Issue number6
DOIs
StatePublished - Jun 1 2016

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Chronic Pancreatitis
Pancreatitis
Pediatrics
Nonparametric Statistics
Odds Ratio
Mutation
Abdominal Pain
Hospital Emergency Service
Demography
Sex Distribution
Poisons
Hospital Departments

Cite this

Risk factors associated with pediatric acute recurrent and chronic pancreatitis : Lessons from INSPPIRE. / Kumar, Soma; Ooi, Chee Y.; Werlin, Steven; Abu-El-Haija, Maisam; Barth, Bradley; Bellin, Melena D.; Durie, Peter R.; Fishman, Douglas S.; Freedman, Steven D.; Gariepy, Cheryl; Giefer, Matthew J.; Gonska, Tanja; Heyman, Melvin B.; Himes, Ryan; Husain, Sohail Z.; Lin, Tom K.; Lowe, Mark E.; Morinville, Veronique; Palermo, Joseph J.; Pohl, John F.; Schwarzenberg, Sarah Jane; Troendle, David; Wilschanski, Michael; Zimmerman, M. Bridget; Uc, Aliye.

In: JAMA Pediatrics, Vol. 170, No. 6, 01.06.2016, p. 562-569.

Research output: Contribution to journalArticle

Kumar, S, Ooi, CY, Werlin, S, Abu-El-Haija, M, Barth, B, Bellin, MD, Durie, PR, Fishman, DS, Freedman, SD, Gariepy, C, Giefer, MJ, Gonska, T, Heyman, MB, Himes, R, Husain, SZ, Lin, TK, Lowe, ME, Morinville, V, Palermo, JJ, Pohl, JF, Schwarzenberg, SJ, Troendle, D, Wilschanski, M, Zimmerman, MB & Uc, A 2016, 'Risk factors associated with pediatric acute recurrent and chronic pancreatitis: Lessons from INSPPIRE', JAMA Pediatrics, vol. 170, no. 6, pp. 562-569. https://doi.org/10.1001/jamapediatrics.2015.4955
Kumar, Soma ; Ooi, Chee Y. ; Werlin, Steven ; Abu-El-Haija, Maisam ; Barth, Bradley ; Bellin, Melena D. ; Durie, Peter R. ; Fishman, Douglas S. ; Freedman, Steven D. ; Gariepy, Cheryl ; Giefer, Matthew J. ; Gonska, Tanja ; Heyman, Melvin B. ; Himes, Ryan ; Husain, Sohail Z. ; Lin, Tom K. ; Lowe, Mark E. ; Morinville, Veronique ; Palermo, Joseph J. ; Pohl, John F. ; Schwarzenberg, Sarah Jane ; Troendle, David ; Wilschanski, Michael ; Zimmerman, M. Bridget ; Uc, Aliye. / Risk factors associated with pediatric acute recurrent and chronic pancreatitis : Lessons from INSPPIRE. In: JAMA Pediatrics. 2016 ; Vol. 170, No. 6. pp. 562-569.
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abstract = "IMPORTANCE: Pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) are poorly understood. OBJECTIVE: To characterize and identify risk factors associated with ARP and CP in childhood. DESIGN, SETTING, AND PARTICIPANTS: A multinational cross-sectional study of children with ARP or CP at the time of enrollment to the INSPPIRE (International Study Group of Pediatric Pancreatitis: In Search for a Cure) study at participant institutions of the INSPPIRE Consortium. From August 22, 2012, to February 8, 2015,155 children with ARP and 146 with CP (aged <19 years) were enrolled. Their demographic and clinical information was entered into the REDCap (Research Electronic Data Capture) database at the 15 centers. Differences were analyzed using 2-sample t test or Wilcoxon rank sum test for continuous variables and Pearson Χ2 test or Fisher exact test for categorical variables. Disease burden variables (pain variables, hospital/emergency department visits, missed school days) were compared using Wilcoxon rank sum test. MAIN OUTCOMES AND MEASURES: Demographic characteristics, risk factors, abdominal pain, and disease burden. RESULTS: A total of 301 children were enrolled (mean [SD] age, 11.9 [4.5] years; 172 [57{\%}] female); 155 had ARP and 146 had CP. The majority of children with CP (123 of 146 [84{\%}]) reported prior recurrent episodes of acute pancreatitis. Sex distribution was similar between the groups (57{\%} female in both). Hispanic children were less likely to have CP than ARP (17{\%} vs 28{\%}, respectively; odds ratio [OR] = 0.51; 95{\%} CI, 0.29-0.92; P =.02). At least 1 gene mutation in pancreatitis-related genes was found in 48{\%} of patients with ARP vs 73{\%} of patients with CP(P <.001). Children with PRSS1 or SPINK1 mutations were more likely to present with CP compared with ARP (PRSS1: OR = 4.20; 95{\%} CI, 2.14-8.22; P <.001; and SPINK1: OR = 2.30; 95{\%} CI, 1.03-5.13; P =.04). Obstructive risk factors did not differ between children with ARP or CP (33{\%} in both the ARP and CP groups), but toxic/metabolic risk factors were more common in children with ARP (21{\%} overall; 26{\%} in the ARP group and 15{\%} in the CP group; OR = 0.55; 95{\%} CI, 0.31-0.99; P =.046). Pancreatitis-related abdominal pain was a major symptom in 81{\%} of children with ARP or CP within the last year. The disease burden was greater in the CP group compared with the ARP group (more emergency department visits, hospitalizations, and medical, endoscopic, and surgical interventions). CONCLUSIONS AND RELEVANCE: Genetic mutations are common in both ARP and CP. Ethnicity and mutations in PRSS1 or SPINK1 may influence the development of CP. The high disease burden in pediatric CP underscores the importance of identifying predisposing factors for progression of ARP to CP in children.",
author = "Soma Kumar and Ooi, {Chee Y.} and Steven Werlin and Maisam Abu-El-Haija and Bradley Barth and Bellin, {Melena D.} and Durie, {Peter R.} and Fishman, {Douglas S.} and Freedman, {Steven D.} and Cheryl Gariepy and Giefer, {Matthew J.} and Tanja Gonska and Heyman, {Melvin B.} and Ryan Himes and Husain, {Sohail Z.} and Lin, {Tom K.} and Lowe, {Mark E.} and Veronique Morinville and Palermo, {Joseph J.} and Pohl, {John F.} and Schwarzenberg, {Sarah Jane} and David Troendle and Michael Wilschanski and Zimmerman, {M. Bridget} and Aliye Uc",
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TY - JOUR

T1 - Risk factors associated with pediatric acute recurrent and chronic pancreatitis

T2 - Lessons from INSPPIRE

AU - Kumar, Soma

AU - Ooi, Chee Y.

AU - Werlin, Steven

AU - Abu-El-Haija, Maisam

AU - Barth, Bradley

AU - Bellin, Melena D.

AU - Durie, Peter R.

AU - Fishman, Douglas S.

AU - Freedman, Steven D.

AU - Gariepy, Cheryl

AU - Giefer, Matthew J.

AU - Gonska, Tanja

AU - Heyman, Melvin B.

AU - Himes, Ryan

AU - Husain, Sohail Z.

AU - Lin, Tom K.

AU - Lowe, Mark E.

AU - Morinville, Veronique

AU - Palermo, Joseph J.

AU - Pohl, John F.

AU - Schwarzenberg, Sarah Jane

AU - Troendle, David

AU - Wilschanski, Michael

AU - Zimmerman, M. Bridget

AU - Uc, Aliye

PY - 2016/6/1

Y1 - 2016/6/1

N2 - IMPORTANCE: Pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) are poorly understood. OBJECTIVE: To characterize and identify risk factors associated with ARP and CP in childhood. DESIGN, SETTING, AND PARTICIPANTS: A multinational cross-sectional study of children with ARP or CP at the time of enrollment to the INSPPIRE (International Study Group of Pediatric Pancreatitis: In Search for a Cure) study at participant institutions of the INSPPIRE Consortium. From August 22, 2012, to February 8, 2015,155 children with ARP and 146 with CP (aged <19 years) were enrolled. Their demographic and clinical information was entered into the REDCap (Research Electronic Data Capture) database at the 15 centers. Differences were analyzed using 2-sample t test or Wilcoxon rank sum test for continuous variables and Pearson Χ2 test or Fisher exact test for categorical variables. Disease burden variables (pain variables, hospital/emergency department visits, missed school days) were compared using Wilcoxon rank sum test. MAIN OUTCOMES AND MEASURES: Demographic characteristics, risk factors, abdominal pain, and disease burden. RESULTS: A total of 301 children were enrolled (mean [SD] age, 11.9 [4.5] years; 172 [57%] female); 155 had ARP and 146 had CP. The majority of children with CP (123 of 146 [84%]) reported prior recurrent episodes of acute pancreatitis. Sex distribution was similar between the groups (57% female in both). Hispanic children were less likely to have CP than ARP (17% vs 28%, respectively; odds ratio [OR] = 0.51; 95% CI, 0.29-0.92; P =.02). At least 1 gene mutation in pancreatitis-related genes was found in 48% of patients with ARP vs 73% of patients with CP(P <.001). Children with PRSS1 or SPINK1 mutations were more likely to present with CP compared with ARP (PRSS1: OR = 4.20; 95% CI, 2.14-8.22; P <.001; and SPINK1: OR = 2.30; 95% CI, 1.03-5.13; P =.04). Obstructive risk factors did not differ between children with ARP or CP (33% in both the ARP and CP groups), but toxic/metabolic risk factors were more common in children with ARP (21% overall; 26% in the ARP group and 15% in the CP group; OR = 0.55; 95% CI, 0.31-0.99; P =.046). Pancreatitis-related abdominal pain was a major symptom in 81% of children with ARP or CP within the last year. The disease burden was greater in the CP group compared with the ARP group (more emergency department visits, hospitalizations, and medical, endoscopic, and surgical interventions). CONCLUSIONS AND RELEVANCE: Genetic mutations are common in both ARP and CP. Ethnicity and mutations in PRSS1 or SPINK1 may influence the development of CP. The high disease burden in pediatric CP underscores the importance of identifying predisposing factors for progression of ARP to CP in children.

AB - IMPORTANCE: Pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) are poorly understood. OBJECTIVE: To characterize and identify risk factors associated with ARP and CP in childhood. DESIGN, SETTING, AND PARTICIPANTS: A multinational cross-sectional study of children with ARP or CP at the time of enrollment to the INSPPIRE (International Study Group of Pediatric Pancreatitis: In Search for a Cure) study at participant institutions of the INSPPIRE Consortium. From August 22, 2012, to February 8, 2015,155 children with ARP and 146 with CP (aged <19 years) were enrolled. Their demographic and clinical information was entered into the REDCap (Research Electronic Data Capture) database at the 15 centers. Differences were analyzed using 2-sample t test or Wilcoxon rank sum test for continuous variables and Pearson Χ2 test or Fisher exact test for categorical variables. Disease burden variables (pain variables, hospital/emergency department visits, missed school days) were compared using Wilcoxon rank sum test. MAIN OUTCOMES AND MEASURES: Demographic characteristics, risk factors, abdominal pain, and disease burden. RESULTS: A total of 301 children were enrolled (mean [SD] age, 11.9 [4.5] years; 172 [57%] female); 155 had ARP and 146 had CP. The majority of children with CP (123 of 146 [84%]) reported prior recurrent episodes of acute pancreatitis. Sex distribution was similar between the groups (57% female in both). Hispanic children were less likely to have CP than ARP (17% vs 28%, respectively; odds ratio [OR] = 0.51; 95% CI, 0.29-0.92; P =.02). At least 1 gene mutation in pancreatitis-related genes was found in 48% of patients with ARP vs 73% of patients with CP(P <.001). Children with PRSS1 or SPINK1 mutations were more likely to present with CP compared with ARP (PRSS1: OR = 4.20; 95% CI, 2.14-8.22; P <.001; and SPINK1: OR = 2.30; 95% CI, 1.03-5.13; P =.04). Obstructive risk factors did not differ between children with ARP or CP (33% in both the ARP and CP groups), but toxic/metabolic risk factors were more common in children with ARP (21% overall; 26% in the ARP group and 15% in the CP group; OR = 0.55; 95% CI, 0.31-0.99; P =.046). Pancreatitis-related abdominal pain was a major symptom in 81% of children with ARP or CP within the last year. The disease burden was greater in the CP group compared with the ARP group (more emergency department visits, hospitalizations, and medical, endoscopic, and surgical interventions). CONCLUSIONS AND RELEVANCE: Genetic mutations are common in both ARP and CP. Ethnicity and mutations in PRSS1 or SPINK1 may influence the development of CP. The high disease burden in pediatric CP underscores the importance of identifying predisposing factors for progression of ARP to CP in children.

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