Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden.
Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden.
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DOI：
10.1016/j.jpeds.2014.11.019
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发表时间：
2015-04
影响因子：
5.1
通讯作者：
Uc, Aliye
中科院分区：
文献类型：
--
作者：
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
探讨儿童慢性胰腺炎的临床表现、诊断变量、危险因素和疾病负担。我们对来自INSPPIRE（小儿胰腺炎国际研究组：寻找治愈方法）的数据进行了横断面研究，INSPPIRE是一项急性复发性胰腺炎和慢性胰腺炎儿童登记研究。使用Wilcoxon秩和检验比较组间差异。在登记研究的170例受试者中，76例（45%）患有慢性胰腺炎; 57%为女性，80%为白人，诊断时的中位年龄为9.9岁。在51例（67%）中发现了胰腺炎易感基因突变，在25例（33%）中发现了梗阻性风险因素。毒性/代谢和自身免疫因素并不常见。影像学显示导管异常和胰腺萎缩比钙化更常见。59例（77%）患者在过去一年内报告腹痛; 28%的患者报告持续疼痛并接受麻醉剂。患有慢性胰腺炎的儿童在过去一年中报告了3次急诊和2次住院。47名受试者（70%）在过去一个月内因慢性胰腺炎缺课一天; 26名受试者（34%）缺课3天或3天以上。与发作性疼痛的儿童相比，报告持续疼痛的儿童更可能缺课（p=0.002），去急诊室（p=0.01）和住院（p=0.03）。33名儿童（43%）接受了治疗性ERCP; 30名（39%）进行了一次或多次胰腺手术。慢性胰腺炎发生于年轻人，具有独特的临床特征。遗传和梗阻性风险因素很常见，疾病负担很大。
To determine the clinical presentation, diagnostic variables, risk factors and disease burden in children with chronic pancreatitis. 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. 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%). 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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影响因子：
30.8
作者：
Witt, Heiko;Beer, Sebastian;Rosendahl, Jonas;Chen, Jian-Min;Chandak, Giriraj Ratan;Masamune, Atsushi;Bence, Melinda;Szmola, Richard;Oracz, Grzegorz;Macek, Milan, Jr.;Bhatia, Eesh;Steigenberger, Sandra;Lasher, Denise;Buehler, Florence;Delaporte, Catherine;Tebbing, Johanna;Ludwig, Maren;Pilsak, Claudia;Saum, Karolin;Bugert, Peter;Masson, Emmanuelle;Paliwal, Sumit;Bhaskar, Seema;Sobczynska-Tomaszewska, Agnieszka;Bak, Daniel;Balascak, Ivan;Choudhuri, Gourdas;Reddy, D. Nageshwar;Rao, G. Venkat;Thomas, Varghese;Kume, Kiyoshi;Nakano, Eriko;Kakuta, Yoichi;Shimosegawa, Tooru;Durko, Lukasz;Szabo, Andras;Schnur, Andrea;Hegyi, Peter;Rakonczay, Zoltan, Jr.;Pfuetzer, Roland;Schneider, Alexander;Groneberg, David Alexander;Braun, Markus;Schmidt, Hartmut;Witt, Ulrike;Friess, Helmut;Alguel, Hana;Landt, Olfert;Schuelke, Markus;Krueger, Renate;Wiedenmann, Bertram;Schmidt, Frank;Zimmer, Klaus-Peter;Kovacs, Peter;Stumvoll, Michael;Blueher, Matthias;Mueller, Thomas;Janecke, Andreas;Teich, Niels;Gruetzmann, Robert;Schulz, Hans-Ulrich;Moessner, Joachim;Keim, Volker;Loehr, Matthias;Ferec, Claude;Sahin-Toth, Miklos
通讯作者：
Sahin-Toth, Miklos
影响因子：
2.5
作者：
LaRusch J;Whitcomb DC
通讯作者：
Whitcomb DC
影响因子：
12.6
作者：
Bellin, Melena D.;Freeman, Martin L.;Sutherland, David E. R.
通讯作者：
Sutherland, David E. R.
影响因子：
9.8
作者：
Gelrud, A;Sheth, S;Freedman, SD
通讯作者：
Freedman, SD
影响因子：
12.6
作者：
Cote, Gregory A.;Yadav, Dhiraj;Slivka, Adam;Hawes, Robert H.;Anderson, Michelle A.;Burton, Frank R.;Brand, Randall E.;Banks, Peter A.;Lewis, Michele D.;Disario, James A.;Gardner, Timothy B.;Gelrud, Andres;Amann, Stephen T.;Baillie, John;Money, Mary E.;O'Connell, Michael;Whitcomb, David C.;Sherman, Stuart
通讯作者：
Sherman, Stuart