Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden

Sarah Jane Schwarzenberg, Melena Bellin, Sohail Z. Husain, Monika Ahuja, Bradley Barth, Heather Davis, Peter R. Durie, Douglas S. Fishman, Steven D. Freedman, Cheryl E. Gariepy, Matthew J. Giefer, Tanja Gonska, Melvin B. Heyman, Ryan Himes, Soma Kumar, Veronique D. Morinville, Mark E. Lowe, Neil E. Nuehring, Chee Y. Ooi, John F. Pohl & 5 others David Troendle, Steven L. Werlin, Michael Wilschanski, Elizabeth Yen, Aliye Uc

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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 the 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 white; 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 department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; 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.

Original languageEnglish (US)
Pages (from-to)890-896.e1
JournalJournal of Pediatrics
Volume166
Issue number4
DOIs
StatePublished - Jan 1 2015

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Chronic Pancreatitis
Pediatrics
Pancreatitis
Nonparametric Statistics
Pain
Registries
Hospital Emergency Service
Hospitalization
Poisons
Narcotics
Abdominal Pain
Atrophy
Cross-Sectional Studies
Mutation

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Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden. / Schwarzenberg, Sarah Jane; Bellin, Melena; Husain, Sohail Z.; Ahuja, Monika; Barth, Bradley; Davis, Heather; Durie, Peter R.; Fishman, Douglas S.; Freedman, Steven D.; Gariepy, Cheryl E.; Giefer, Matthew J.; Gonska, Tanja; Heyman, Melvin B.; Himes, Ryan; Kumar, Soma; Morinville, Veronique D.; Lowe, Mark E.; Nuehring, Neil E.; Ooi, Chee Y.; Pohl, John F.; Troendle, David; Werlin, Steven L.; Wilschanski, Michael; Yen, Elizabeth; Uc, Aliye.

In: Journal of Pediatrics, Vol. 166, No. 4, 01.01.2015, p. 890-896.e1.

Research output: Contribution to journalArticle

Schwarzenberg, SJ, Bellin, M, Husain, SZ, Ahuja, M, Barth, B, Davis, H, Durie, PR, Fishman, DS, Freedman, SD, Gariepy, CE, Giefer, MJ, Gonska, T, Heyman, MB, Himes, R, Kumar, S, Morinville, VD, Lowe, ME, Nuehring, NE, Ooi, CY, Pohl, JF, Troendle, D, Werlin, SL, Wilschanski, M, Yen, E & Uc, A 2015, 'Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden', Journal of Pediatrics, vol. 166, no. 4, pp. 890-896.e1. https://doi.org/10.1016/j.jpeds.2014.11.019
Schwarzenberg, Sarah Jane ; Bellin, Melena ; Husain, Sohail Z. ; Ahuja, Monika ; Barth, Bradley ; Davis, Heather ; Durie, Peter R. ; Fishman, Douglas S. ; Freedman, Steven D. ; Gariepy, Cheryl E. ; Giefer, Matthew J. ; Gonska, Tanja ; Heyman, Melvin B. ; Himes, Ryan ; Kumar, Soma ; Morinville, Veronique D. ; Lowe, Mark E. ; Nuehring, Neil E. ; Ooi, Chee Y. ; Pohl, John F. ; Troendle, David ; Werlin, Steven L. ; Wilschanski, Michael ; Yen, Elizabeth ; Uc, Aliye. / Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden. In: Journal of Pediatrics. 2015 ; Vol. 166, No. 4. pp. 890-896.e1.
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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 the 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 white; 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 department visits and 2 hospitalizations in the last year. Forty-seven subjects (70{\%}) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34{\%}) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43{\%}) underwent therapeutic endoscopic retrograde pancreatography; 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.",
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T1 - Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden

AU - Schwarzenberg, Sarah Jane

AU - Bellin, Melena

AU - Husain, Sohail Z.

AU - Ahuja, Monika

AU - Barth, Bradley

AU - Davis, Heather

AU - Durie, Peter R.

AU - Fishman, Douglas S.

AU - Freedman, Steven D.

AU - Gariepy, Cheryl E.

AU - Giefer, Matthew J.

AU - Gonska, Tanja

AU - Heyman, Melvin B.

AU - Himes, Ryan

AU - Kumar, Soma

AU - Morinville, Veronique D.

AU - Lowe, Mark E.

AU - Nuehring, Neil E.

AU - Ooi, Chee Y.

AU - Pohl, John F.

AU - Troendle, David

AU - Werlin, Steven L.

AU - Wilschanski, Michael

AU - Yen, Elizabeth

AU - Uc, Aliye

PY - 2015/1/1

Y1 - 2015/1/1

N2 - 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 the 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 white; 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 department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; 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.

AB - 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 the 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 white; 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 department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; 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.

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