Yablokova E A, Gorelovl A V, Sichinava I V, Borisova E V, Polotnyanko E Y, Grammatopulo M I, Kanshina A A
Eksp Klin Gastroenterol. 2016(8):62-66.
Inflammatory bowel disease (IBD) are systemic diseases with different intestinal and extra-intestinal manifestations (ElM). This fact can determin an examination plan and the disease course and prognosis. Up to 8.4% of children with IBD have ELM like the first symptom of the disease. The range of ElM of iBD in children is differ from that one of adult patients: rarer arthropathy and arthritis, skin and liver diseases, an extremely rare eye disease. The physical development disorder is a typical children's manifestation of the IBD, the "failure of the weight curve" is an early symptom of the IBD debut (82% of examined children), especially ulcerative colitis. The article discusses the clinical features and therapy of the most frequent extraintestinal manifestations of IBD in children: skin, mucous, eye diseases, axial and peripheral arthropathies, primary scierosing cholangitis, physical, sexual development disorders, decreased bone mineral density. Most often, we observed patients with arthritis and primary scierosing cholangitis - up to 12.5% of patients with active disease. A growth failure was observed in 20% of children with IBD debut, and remained in 9.7% children despite adequate anti-inflammatory therapy. Decreased bone mineral density in children with IBD observed in 30% cases (ISCD-recommendations). Extraintestinal manifestations are very important to make the early diagnosis of IBD without any intestinal symptoms. The pediatrician can suspect a systemic diseases course. ElMs adversely impact upon patientsquality of life and some can be life-threatening.
炎症性肠病(IBD)是具有不同肠道和肠外表现(EIM)的全身性疾病。这一事实可决定检查计划以及疾病进程和预后。高达8.4%的IBD患儿以EIM作为疾病的首发症状。儿童IBD的EIM范围与成年患者不同:关节病和关节炎、皮肤和肝脏疾病较为少见,眼部疾病极为罕见。身体发育障碍是IBD典型的儿童表现,“体重曲线下降”是IBD初发的早期症状(82%的受检儿童),尤其是溃疡性结肠炎。本文讨论了儿童IBD最常见的肠外表现的临床特征和治疗方法:皮肤、黏膜、眼部疾病、轴性和周围关节病、原发性硬化性胆管炎、身体和性发育障碍、骨密度降低。我们最常观察到患有关节炎和原发性硬化性胆管炎的患者——活动性疾病患者中高达12.5%。20%的IBD初发儿童出现生长发育迟缓,尽管进行了充分的抗炎治疗,仍有9.7%的儿童存在生长发育迟缓。30%的IBD患儿出现骨密度降低(符合国际临床骨密度学会的建议)。肠外表现在无任何肠道症状时对IBD的早期诊断非常重要。儿科医生可怀疑为全身性疾病病程。EIM会对患者的生活质量产生不利影响,有些甚至可能危及生命。