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

Autor: Schwarzenberg SJ; University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA., Bellin M; University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA., Husain SZ; Children's Hospital of Pittsburgh, Pittsburgh, PA, USA., Ahuja M; University of Iowa Carver College of Medicine, Iowa City, IA, USA., Barth B; University of Texas Southwestern Medical School, Dallas, TX, USA., Davis H; University of Iowa Carver College of Medicine, Iowa City, IA, USA., Durie PR; Hospital for Sick Children, Toronto, ON, Canada., Fishman DS; Baylor College of Medicine, Houston, TX, USA., Freedman SD; Harvard Medical School, Boston, MA, USA., Gariepy CE; Nationwide Children's Hospital, Columbus, OH, USA., Giefer MJ; Seattle Children's Hospital, Seattle, WA, USA., Gonska T; Hospital for Sick Children, Toronto, ON, Canada., Heyman MB; University of California at San Francisco, San Francisco, CA, USA., Himes R; Baylor College of Medicine, Houston, TX, USA., Kumar S; Nationwide Children's Hospital, Columbus, OH, USA., Morinville VD; Montreal Children's Hospital, McGill University, Montreal, QC, Canada., Lowe ME; Children's Hospital of Pittsburgh, Pittsburgh, PA, USA., Nuehring NE; University of Iowa Carver College of Medicine, Iowa City, IA, USA., Ooi CY; 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., Pohl JF; University of Utah, Salt Lake City, UT, USA., Troendle D; University of Texas Southwestern Medical School, Dallas, TX, USA., Werlin SL; Medical College of Wisconsin, Milwaukee, WI, USA., Wilschanski M; Hadassah Hebrew University Hospital, Jerusalem, Israel., Yen E; University of California at San Francisco, San Francisco, CA, USA., Uc A; University of Iowa Carver College of Medicine, Iowa City, IA, USA.
Jazyk: angličtina
Zdroj: The Journal of pediatrics [J Pediatr] 2015 Apr; Vol. 166 (4), pp. 890-896.e1. Date of Electronic Publication: 2014 Dec 30.
DOI: 10.1016/j.jpeds.2014.11.019
Abstrakt: 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.
(Copyright © 2015 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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