Goyal Alka, Hyams Jeffery S, Lerer Trudy, Leleiko Neal S, Otley Anthony R, Griffiths Anne M, Rosh Joel R, Cabrera Jose M, Oliva-Hemker Maria M, Mack David R, Rick James N, Pfefferkorn Marian D, Carvalho Ryan, Grossman Andrew B, Hitch Meredith C, Sudel Boris, Kappelman Michael D, Saeed Shehzad A, Faubion William A, Schaefer Marc E, Markowitz James F, Keljo David J
*Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA †Connecticut Children's Medical Center, Hartford ‡Hasbro Children's Hospital, Providence, RI §I.W.K. Health Center, Halifax, Nova Scotia, Canada ||Hospital for Sick Children, Toronto, Ontario, Canada ¶Goryeb Children's Hospital, Morristown, NJ #Children's Hospital of Wisconsin, Milwaukee **Johns Hopkins Hospital, Baltimore, MD ††Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada ‡‡Children's Medical Center, Dayton, OH §§Riley Hospital for Children, Indianapolis, IN ||||Nationwide Children's Hospital, Columbus, OH ¶¶Children's Hospital of Philadelphia, Philadelphia, PA ##Russell Children's Hospital, Birmingham, AB ***University of Minnesota, Minneapolis †††University of North Carolina, Chapel Hill ‡‡‡Cincinnati Children's Medical Center, Cincinnati, OH §§§Mayo Clinic, Rochester, MN ||||||Penn State Hershey Children's Hospital, Hershey, PA ¶¶¶Cohen Children's Medical Center, New Hyde Park, NY.
J Pediatr Gastroenterol Nutr. 2014 Sep;59(3):321-3. doi: 10.1097/MPG.0000000000000409.
Inflammatory bowel disease-associated liver diseases (IBD-LDs) include autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and an overlap syndrome. Prospective unbiased multicenter data regarding the frequency of IBD-LD in patients with pediatric inflammatory bowel disease (IBD) are lacking. We examined early alanine aminotransferase (ALT) and γ-glutamyl transpeptidase (GGT) elevations in children diagnosed as having IBD and assessed the likelihood of IBD-LD.
Data collected from the prospective observational Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry enrolling children of age <16 years within 30 days of diagnosis. AIH, PSC, and overlap syndrome were diagnosed using local institutional criteria.
A total of 1569 subjects had liver enzymes available. Of the total, 757 had both ALT and GGT, 800 had ALT only (no GGT), and 12 had GGT only (no ALT). Overall, 29 of 1569 patients (1.8%) had IBD-LD. IBD-LD was diagnosed in 1 of 661 (0.15%) of patients with both ALT and GGT ≤ 50 IU/L compared with 21 of 42 (50%) of patients with both ALT and GGT > 50 (odds ratio 660, P < 0.0001). Of the 29 patients with IBD-LD, 21 had PSC, 2 had AIH, and 6 had overlap syndrome. IBD-LD was more common in patients with ulcerative colitis and IBD-unclassified (indeterminate colitis) than in those with Crohn disease (4% vs 0.8%, respectively, P < 0.001).
Elevation of both ALT and GGT within 90 days after the diagnosis of IBD is associated with a markedly increased likelihood of IBD-LD. Both ALT and GGT levels should be measured in all of the pediatric patients newly diagnosed as having IBD.
炎症性肠病相关肝病（IBD-LD）包括自身免疫性肝炎（AIH）、原发性硬化性胆管炎（PSC）以及重叠综合征。目前缺乏关于儿童炎症性肠病（IBD）患者中IBD-LD发病率的前瞻性、无偏倚多中心数据。我们研究了诊断为IBD的儿童早期丙氨酸氨基转移酶（ALT）和γ-谷氨酰转肽酶（GGT）升高情况，并评估了IBD-LD的可能性。
数据来自前瞻性观察性儿科炎症性肠病协作研究组登记处，登记的是诊断后30天内年龄<16岁的儿童。AIH、PSC和重叠综合征根据当地机构标准进行诊断。
共有1569名受试者有可用的肝酶数据。其中，757名同时有ALT和GGT数据，800名仅有ALT数据（无GGT数据），12名仅有GGT数据（无ALT数据）。总体而言，1569名患者中有29名（1.8%）患有IBD-LD。在ALT和GGT均≤50 IU/L的661名患者中有1名（0.15%）被诊断为IBD-LD，而在ALT和GGT均>50的42名患者中有21名（50%）被诊断为IBD-LD（比值比660，P<0.0001）。在29名患有IBD-LD的患者中，21名患有PSC，2名患有AIH，6名患有重叠综合征。IBD-LD在溃疡性结肠炎和未分类IBD（不确定性结肠炎）患者中比在克罗恩病患者中更常见（分别为4%和0.8%，P<0.001）。
IBD诊断后90天内ALT和GGT均升高与IBD-LD的可能性显著增加相关。所有新诊断为IBD的儿科患者均应检测ALT和GGT水平。
