Clinical features of very early-onset inflammatory bowel disease in Japan: a retrospective single-center study

Abstract

Background/Aims: Very early-onset inflammatory bowel disease (VEO-IBD), defined as IBD diagnosed in patients younger than 6 years, is a challenge for pediatric gastroenterologists. Although there have been reports regarding VEO-IBD in Western countries, those in Asia are still lacking. This study aimed to investigate the clinical features of Japanese VEO-IBD patients.Methods: Patients with VEO-IBD diagnosed between 2006 and 2019 were evaluated retrospectively. The disease phenotypes were classified into ulcerative colitis type (UC-type) and Crohn’s disease type (CD-type), and the clinical features and courses were compared between the phenotypes.Results: Overall, 54 VEO-IBD patients (19 patients with UC-type and 35 patients with CD-type) were evaluated. The median age at onset was 18 months. One patient had severe combined immunodeficiency (SCID), and 9 patients had monogenic IBD. Monogenic IBD was more prevalent in the CD-type patients with perianal disease (CD-type (PD)). The age at onset was significantly lower in the CD-type group (P<0.05). The most common initial symptom was bloody stools (70%), followed by diarrhea (63%), weight loss (24%), fever (20%), and perianal disease (20%). Excluding patients with SCID and monogenic IBD, 23 out of 44 patients (52%) required biologics. The biologics were switched in 11 out of 44 patients (25%), and the majority of these patients (82%) were in the CD-type group. Overall, 9 patients (20%) required intestinal resection or ostomy placement.Conclusions: CD-type tends to occur at an earlier age, and monogenic IBD occurs significantly more frequently in CD-type (PD). Disease severity and treatment should be individualized, owing to the disease heterogeneity.

Neither hepatic steatosis nor fibrosis is associated with clinical outcomes in patients with intestinal Behçet’s disease

“…Another reason is that patients with IBD are lean and have greater nutritional deficits, which may result in less fibrosis. As patients with intestinal BD are older at diagnosis and tend to have fewer nutritional deficits, they may have a higher incidence of NAFLD than patients with IBD [36,37].…”

Surgical outcomes of very-early-onset ulcerative colitis: retrospective comparative study with older pediatric patients

“…This study has some limitations. Firstly, VEO-IBD is a complex and heterogeneous disease, and the diagnosis of VEO-UC may change as the patient ages [25]. Rialon et al reported that 24% of patients originally diagnosed with UC or IBD-unclassi ed were later reclassi ed as having Crohn's disease [26].…”

“…However, high-output ileostomy and long duration of CVC placement may pose management challenges.anthropometric measurements in pediatric UC patients; therefore, to our knowledge, this is the rst study to show the effect of surgery on postoperative growth using Z-scores in pediatric UC patients.This study has some limitations. Firstly, VEO-IBD is a complex and heterogeneous disease, and the diagnosis of VEO-UC may change as the patient ages [25]. Rialon et al reported that 24% of patients originally diagnosed with UC or IBD-unclassi ed were later reclassi ed as having Crohn's disease [26].…”

Impact of age at diagnosis on long‐term prognosis in patients with intestinal Behçet's disease

“…7,16,17 CD diagnosed in pediatric patients has an aggressive disease course and often exhibits poor response to conventional treatments, thus requiring immunosuppressants or biologics immediately after diagnosis. [18][19][20] However, little is known about its effect on the long-term prognosis of intestinal BD.…”

“…Many studies have consistently reported that age at disease onset is associated with the prognosis for CD 7,15 ; however, the results are inconsistent for ulcerative colitis 7,16,17 . CD diagnosed in pediatric patients has an aggressive disease course and often exhibits poor response to conventional treatments, thus requiring immunosuppressants or biologics immediately after diagnosis 18–20 . However, little is known about its effect on the long‐term prognosis of intestinal BD.…”