ABSTRACT
OBJECTIVES: High rates of inflammatory bowel disease (IBD) are reported in children of South Asian (SA) descent in some western countries. This population-based study describes the incidence and clinical course of IBD in SA children compared to non-South Asian (NSA) children in New Zealand (NZ). METHODS: Children (≤15 years) with new-onset IBD presenting to a centralized tertiary referral center in Auckland, NZ from 2010 to 2020 were identified. Disease phenotype, clinical characteristics, response to exclusive enteral nutrition, clinical remission rates at 3 and 12 months, biologic use, corticosteroid exposure, and disease complications were compared by ethnicity; IBD incidence was calculated. RESULTS: There were 127 (26 SA; 101 NSA) children with Crohn disease, 41 (10 SA; 31 NSA) with ulcerative colitis, and 10 (3 SA; 7 NSA) with IBD-unclassified. IBD incidence in SA and NSA children was 14.1 per 100,000 and 4.3 per 100,000 respectively ( P < 0.001). IBD incidence increased by 5.6% per year ( P = 0.022), due to a greater rise in incidence in SA (SA 16.8% per year, P = 0.015; NSA 4.5% per year, P = 0.317). At presentation, SA children had worse biochemical parameters, severe colitis, and vitamin D deficiency. SA children had lower rates of remission following exclusive enteral nutrition (28.5% vs 65.0%, P < 0.001) or biologic induction (35.7% vs 70.8%, P = 0.020), at 3-month (35.3% vs 69.8%, P < 0.001) and 12-month follow-up (29.4% vs 55.0%, P = 0.005). No significant differences were found in disease location or corticosteroid burden. CONCLUSIONS: Increasing incidence of IBD was disproportionately represented by SA children with more severe disease and lower remission rates following exclusive enteral nutrition or biologic therapy.
Subject(s)
Biological Products , Colitis, Ulcerative , Inflammatory Bowel Diseases , Humans , Retrospective Studies , Incidence , New Zealand/epidemiology , Inflammatory Bowel Diseases/therapy , Inflammatory Bowel Diseases/drug therapy , Colitis, Ulcerative/epidemiology , Colitis, Ulcerative/therapy , Colitis, Ulcerative/complications , Adrenal Cortex Hormones/therapeutic use , Biological Products/therapeutic useABSTRACT
BACKGROUND: Late airway complications, as consequence of immunosuppression following pediatric liver transplantation are uncommonly reported. METHODS: In this retrospective case series, we describe two young children presenting with symptoms of airway obstruction, secondary to differing pathologies in the supraglottic airway, as a result of immunosuppression following liver transplantation. RESULTS: Case 1, a 2-year-old girl who presented with stridor 12-months following liver transplantation, was found to have a proliferative soft tissue mass involving the supraglottic larynx. Biopsies were consistent with infiltrative eosinophilic laryngitis and associated eosinophilic esophagitis. Case 2, a 12-month-old female who presented with stridor 5-months following liver transplantation, was found to have an exophytic soft tissue mass involving the supraglottis and hypopharynx. Biopsies revealed polymorphic Epstein-Barr virus (EBV) driven post-transplant lymphoproliferative disease (PTLD). Case 1 was managed with local resection and high dose oral corticosteroids. Case 2 responded to debulking of the necrotic supraglottic mass, reduction of immunosuppression and rituximab. CONCLUSION: A high index of suspicion needs to be maintained for complications of immunosuppression for appropriate diagnosis of airway presentations following pediatric liver transplantation. Further research is necessary to improve early detection and consolidate management strategies for these airway lesions.
Subject(s)
Epstein-Barr Virus Infections , Liver Transplantation , Lymphoproliferative Disorders , Child , Humans , Female , Child, Preschool , Infant , Epstein-Barr Virus Infections/etiology , Herpesvirus 4, Human , Retrospective Studies , Liver Transplantation/adverse effects , Respiratory Sounds/etiology , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/etiology , Lymphoproliferative Disorders/therapy , Postoperative Complications/diagnosis , Postoperative Complications/etiologySubject(s)
Ichthyosiform Erythroderma, Congenital , Ichthyosis , Lipid Metabolism, Inborn Errors , Muscular Diseases , Humans , Ichthyosiform Erythroderma, Congenital/complications , Ichthyosiform Erythroderma, Congenital/diagnosis , Ichthyosis/complications , Ichthyosis/diagnosis , Lipid Metabolism, Inborn Errors/complications , Lipid Metabolism, Inborn Errors/diagnosis , Liver Cirrhosis/complicationsABSTRACT
We describe a female toddler with rectal bleeding from extensive colonic polyposis, and diagnosed with familial adenomatous polyposis. She has epilepsy from infancy attributed to focal cortical dysplasia. Hepatoblastoma was diagnosed at 13 months of age. Germline testing detected a pathogenic APC (adenomatous polyposis coli gene) variant. We discuss the anecdotal management of this case, including the clinical utility of genetic confirmation. We review the genotype-phenotype correlation of the APC mutational spectrum, and the existing evidence supporting the hypothesis that cortical dysplasia is part of the APC-related spectrum.
