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Upper and small bowel Crohn's disease in Brazilian children: Phenotypic characteristic and surgical risk
Jane Oba1, Carlos Walter Sobrado2, Elizete Aparecida Lomazi3
1Departamento de Gastroenterologia e Coloproctologia, Hospital das, Clínicas da Faculdade de Medicina da Universidade de São Paulo, Hospital Israelita Albert Einstein, São Paulo, Brazil.
Insights
Pediatric upper and small bowel Crohn's disease (U-SBCD) is aggressive and challenging to diagnose in low-income countries. Early recognition and improved medical education are crucial to prevent complications and improve outcomes for these patients.
Area of Science:
- Pediatric Gastroenterology
- Inflammatory Bowel Disease (IBD) Research
- Clinical Outcomes in Crohn's Disease
Background:
- Upper and small bowel Crohn's disease (U-SBCD) is an aggressive phenotype with high complication rates.
- Diagnostic challenges and limited awareness in low- and middle-income countries (LMICs) contribute to delays in IBD diagnosis.
- The impact of diagnostic delays on U-SBCD outcomes in LMICs is not well understood.
Purpose of the Study:
- To characterize the clinical features of pediatric U-SBCD in Brazil.
- To analyze management patterns and surgical outcomes for pediatric U-SBCD.
- To establish a baseline for IBD in an LMIC setting for future comparisons.
Main Methods:
- A multicenter retrospective study of 124 pediatric Crohn's disease (CD) patients diagnosed under 18 years old.
- Comparison of U-SBCD patients (n=39) with non-SBCD patients using revised PORTO-ESPGHAN criteria.
- Univariate and multivariable analyses assessed demographics, diagnostic delay, disease behavior, growth, and therapeutic outcomes over 18 months.
Main Results:
- U-SBCD patients were older, with greater height impairment and lower BMI/age z-scores.
- Diagnostic delay significantly increased U-SBCD odds (22% per year), as did severe weight loss (8.3-fold).
- U-SBCD patients required more biologic therapy (97% vs 75%), had higher treatment escalation rates, and were three times more likely to require surgery.
Conclusions:
- Strengthening medical education in LMICs is vital for early U-SBCD recognition and preventing complications.
- The U-SBCD phenotype in this Brazilian cohort resembled Asian rather than Caucasian cohorts.
- Improved diagnostic strategies are needed to mitigate the aggressive nature of U-SBCD in LMIC settings.
Objectives:
Upper and small bowel Crohn's disease (U-SBCD) represents a clinically aggressive phenotype with high complication rates yet remains diagnostically challenging. In low- and middle-income countries (LMICs), limited inflammatory bowel disease (IBD) awareness contributes to diagnostic delays, but their impact on U-SBCD outcomes remains poorly characterized. We aimed to characterize clinical features, management patterns, and surgical outcomes of pediatric U-SBCD in Brazil, establishing IBD baseline for future regional comparisons in LMIC setting.
Methods:
This multicenter retrospective study analyzed 124 pediatric Crohn's disease (CD) patients (diagnosed < 18 years) from six Brazilian centers according to revised PORTO-European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) criteria. Patients with U-SBCD (Paris L4a/L4b, n = 39) were compared to non-SBCD (L1/L2/L3). Univariate and multivariable analyses assessed demographics, diagnostic delay, disease behavior, growth impairment, and therapeutic outcomes over 18 months follow-up. The population comprised predominantly African-Brazilian admixture.
Results:
U-SBCD patients presented at older age (median 14 vs. 12 years; p = 0.001) with greater height impairment (p = 0.010) and lower body mass index (BMI)/age z-scores (-1.14 vs. -0.44; p = 0.029). Severe weight loss (z-score < -2) conferred 8.3-fold increased U-SBCD odds (95% confidence interval [CI]: 2.17-31.66; p = 0.002). Each year of diagnostic delay increased U-SBCD odds by 22% (95% CI: 1.06-1.39; p = 0.004). U-SBCD patients required more biologic therapy (97% vs. 75%; p = 0.003) with higher treatment escalation rates (31.6% vs. 21.2%; p = 0.004). Multivariate analysis confirmed U-SBCD as an independent three-fold predictor of surgery (OR 3.02; 95% CI: 1.02-8.94; p = 0.046). A 9-year-old girl with U-SBCD died perioperatively. Psychiatric symptoms affected 30% of both groups.
Conclusion:
In LMIC settings, strengthening medical education is essential to improve early recognition of U-SBCD and prevent progression to irreversible complications. Notably, the phenotypic pattern observed in our study more closely resembled that reported in Asian pediatric patients than Caucasian cohorts.
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