极早期炎症性肠病:儿科胃肠病学家面临的诊断和治疗挑战》(Very Early Onset Infammatory Bowel Disease: Diagnostic and Therapeutic Challenges for Pediatric Gastroenterologists)。

Mymensingh medical journal : MMJ Pub Date : 2024-07-01
K L Nahid, M Rukunuzzaman, K Fathema
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引用次数: 0

摘要

极早发炎症性肠病(VEO-IBD)是指发病年龄低于 6 岁的 IBD 患者。虽然这种病很少见,但在过去十年中,随着发病年龄的降低,这种病的发病率在不断上升。VEO-IBD 与儿童和成人发病的 IBD 相比,在疾病类型、病变位置、疾病行为和遗传易感性等许多方面都有所不同。与年龄较大的儿童和成人相比,患有 VEO-IBD 的儿童通常病情更为严重。VEO-IBD 与单基因缺陷有关。白细胞介素 10(IL-10)受体基因突变导致 IL-10 信号传导受损,首次证实了 VEO-IBD 的单基因病因。单基因 IBD 备受关注,因为它通常表现为传统 IBD 治疗难治或瘘管性克罗恩病,因此可能需要尽早使用生物制剂或造血干细胞移植(HSCT)等替代方法进行治疗。在确定 IBD 之前,我们必须考虑到这一年龄组更常见的疾病。感染和牛奶蛋白过敏(CMPA)是两种常见疾病,可导致严重的结肠炎。通过内窥镜检查确诊慢性肠炎对诊断 IBD 意义重大。进行内窥镜检查不应有年龄限制。严重的疾病应采用生物制剂和手术治疗。鉴定与 IBD 相关的基因有助于更好地了解其发病机制,从而提供更有针对性的干预措施。我们在此讨论这一主题,旨在提高儿科医生的认识,从而使患者受益。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Very Early Onset Inflammatory Bowel Disease: Diagnostic and Therapeutic Challenges for Pediatric Gastroenterologists.

Very early onset inflammatory bowel disease (VEO-IBD) is called when age of onset of IBD occurs below 6 years. Though it is rare, it has been increasing over last decade with decreasing age of onset. VEO-IBD is different compared with pediatric and adult-onset IBD in many aspects, including the disease type, location of the lesion, disease behavior and genetic susceptibility. These children with VEO-IBD are usually present with more severe disease than older children and adults. VEO-IBD is associated with monogenic defect. The thought of a monogenic cause of VEO-IBD was first confirmed by the detection of mutations of interleukin 10 (IL-10) receptor genes that cause impaired IL-10 signaling. Monogenic IBD possesses significant concern because it usually presents with refractory to conventional IBD treatment or fistulous Crohn's disease, so early treatment with biologics or an alternative approach such as hematopoietic stem cell transplantation (HSCT) might be looked-for. Before establishing IBD, we must think of more common diseases of this age group. Infection and Cow's milk protein allergy (CMPA) are two common conditions and it can cause severe colitis. Confirmation of chronic intestinal inflammation by endoscopies is of greatest significance for the diagnosis of IBD. There should be no age limit for performing endoscopies. Severe disease should be treated with biologic agents and surgery. Identification of genes associated with IBD leads to better understanding of its pathogenesis, which could help to provide more targeted interventions. We discuss the topic here to create awareness among Pediatricians so that the patients can be benefited.

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