Winter Dwight A, Karolewska-Bochenek Katarzyna, Lazowska-Przeorek Izabella, Lionetti Paolo, Mearin M Luisa, Chong Sonny K, Roma-Giannikou Eleftheria, Maly Jan, Kolho Kaija-Leena, Shaoul Ron, Staiano Annamaria, Damen Gerard M, de Meij Tim, Hendriks Daniëlle, George Elvira K, Turner Dan, Escher Johanna C
1Pediatric Gastroenterology, Department of Pediatrics, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands; 2Department of Pediatric Gastroenterology and Nutrition, The Medical University of Warsaw, Warsaw, Poland; 3Gastroenterology and Nutrition Unit, Meyer Pediatric Hospital, Florence, Italy; 4Department of Pediatrics, Leiden University Medical Center, Leiden, the Netherlands; 5Department of Pediatric Gastroenterology, Queen Mary's Hospital for Children, Surrey, United Kingdom; 6First Department of Pediatrics, Athens University, Athens, Greece; 7Department of Pediatrics, Charles University in Prague, Hradec Kralove, Czech Republic; 8Department of Pediatric Gastroenterology, Helsinki Children's Hospital, Helsinki, Finland; 9Pediatric Gastroenterology Unit, Meyer Children's Hospital, Haifa, Israel; 10Department of Pediatrics, University of Naples, Naples, Italy; 11Department of Pediatric Gastroenterology, Radboud University Medical Centre, Nijmegen, the Netherlands; 12Department of Pediatric Gastroenterology, VU University Medical Center, Amsterdam, the Netherlands; 13Department of Pediatrics, Juliana Children's Hospital, The Hague, the Netherlands; 14Department of Pediatrics, Medical Center Alkmaar, Alkmaar, the Netherlands; and 15Pediatric Gastroenterology Unit, Shaare Zedek Medical Center, The Hebrew University of Jerusalem, Jerusalem, Israel.
Inflamm Bowel Dis. 2015 Sep;21(9):2145-53. doi: 10.1097/MIB.0000000000000483.
Inflammatory bowel disease-unclassified (IBD-U) is diagnosed in ∼10% of pediatric and adolescent onset IBD patients. The EUROKIDS registry (2004) initiated by the Porto IBD working group of ESPGHAN prospectively monitors diagnostic workup of newly diagnosed pediatric and adolescent onset IBD patients. We aimed to describe diagnostic workup, phenotype, and change of diagnosis over time in pediatric IBD-U patients.
Data were collected on children from 52 centers across 20 European countries and Israel, diagnosed with IBD from May 2005 through November 2013. Full endoscopy plus small bowel radiology was considered complete diagnostic workup. Participating centers reporting IBD-U patients were queried in 2014 for follow-up data.
IBD-U was the provisional first diagnosis in 265 of 3461 children (7.7%) (91/158 [58%] with pancolitis; 140 [53%] male), diagnosed more frequently under the age of 10 (median age 12.3 years, 89 [34%] under 10 years). Half (48%) had undergone complete diagnostic workup. Lack of small bowel radiology was the prevailing reason for incomplete workup. As a result of reinvestigations (endoscopy in 54%, radiology in 38%) during a median follow-up of 5.7 years (interquartile range, 2.5-7.8), a change in diagnosis from IBD-U to Crohn's disease (12%) or ulcerative colitis (20%) was reported.
Only half of patients reported as IBD-U in EUROKIDS had undergone complete diagnostic workup. Follow-up with reinvestigations resulted in a reduction of IBD-U rate to 5.6%. A diagnosis of IBD-U becomes less likely in case of complete diagnostic workup. Implementation of clear diagnostic criteria will further reduce the rate of IBD-U in the future.
炎症性肠病未分类（IBD-U）在10%左右的儿童及青少年起病的炎症性肠病（IBD）患者中被诊断出来。由欧洲儿科胃肠病、肝病和营养学会（ESPGHAN）的波尔图IBD工作组发起的EUROKIDS注册研究（2004年）前瞻性地监测新诊断的儿童及青少年起病的IBD患者的诊断检查情况。我们旨在描述儿童IBD-U患者的诊断检查、表型及诊断随时间的变化情况。
收集了来自20个欧洲国家和以色列的52个中心的儿童数据，这些儿童在2005年5月至2013年11月期间被诊断为IBD。全结肠镜检查加小肠放射学检查被视为完整的诊断检查。2014年对报告有IBD-U患者的参与中心进行随访数据查询。
IBD-U是3461名儿童中265名（7.7%）的初步诊断（91/158[58%]为全结肠炎；140名[53%]为男性），在10岁以下儿童中诊断更为频繁（中位年龄12.3岁，89名[34%]在10岁以下）。一半（48%）的患者接受了完整的诊断检查。小肠放射学检查缺失是检查不完整的主要原因。在中位随访5.7年（四分位间距，2.5 - 7.8年）期间，由于再次检查（54%进行了内镜检查，38%进行了放射学检查），报告有从IBD-U转变为克罗恩病（12%）或溃疡性结肠炎（20%）的诊断变化。
在EUROKIDS中报告为IBD-U的患者中只有一半接受了完整的诊断检查。通过再次检查进行随访使IBD-U的比例降至5.6%。进行完整的诊断检查时，IBD-U的诊断可能性降低。实施明确的诊断标准将在未来进一步降低IBD-U的比例。
