METHODS: All children diagnosed with CD between 1995 and 2019 were reviewed. Response to induction was compared between EEN and standard immunosuppression (IS) using Paediatric Crohn's Disease Activity Index, growth failure, perianal disease and extra-intestinal manifestations. Two study groups were analysed: (i) primary induction and (ii) re-induction for relapses.
RESULTS: Twenty-nine children (mean age (± standard deviation) at diagnosis 9.4 ± 8.5 years old, ileo-colonic 35%, non-stricturing 79%) were studied. At primary induction (group 1; n = 18), no difference was observed in remission rates (9/13 vs. 5/5; P = 0.278), efficacy for improving growth failure (6/8 vs. 0/1; P > 0.999), perianal disease (4/6 vs. 0/2; P > 0.999) and extra-intestinal manifestations (2/2 vs. 0/0; P > 0.999) with EEN or standard IS. Group 2 (n = 38 relapses), no difference was observed in remission rates (16/19 vs. 15/19, P > 0.999), growth failure (0/7 vs. 4/14; P = 0.328), perianal disease (1/10 vs. 7/7; P > 0.999) and extra-intestinal manifestations (0/0 vs. 1/1; P > 0.999) with EEN or standard IS. Both treatment modalities were equally effective as re-induction in relapses in patients previously treated with EEN (P = 0.191).
CONCLUSION: As compared to standard IS, EEN was equally effective in primary induction and re-induction for relapse in Asian children with CD and can be repeatedly used for recurrent relapses.
CLINICAL PICTURE: We report a case of chronic urticaria in an euthyroid patient with evidence of significantly elevated levels of thyroglobulin and microsomal antibodies.
TREATMENT AND OUTCOME: Treatment with thyroxine has brought about clinical remission of the chronic urticaria but no change in the thyroid antibody levels could be demonstrated.
CONCLUSION: Patients with chronic urticaria should be screened for evidence of thyroid autoimmunity. A closely monitored trial of thyroxine therapy for those who have thyroid autoimmunity can be rewarding.
METHODS: A retrospective review of all paediatric systemic lupus erythematosus patients with at least 6 months follow-up at Selayang Hospital from 2004 to 2016. Epidemiological, clinical and outcome data were collected and analysed.
RESULTS: A total of 141 paediatric systemic lupus erythematosus patients, 87.9% females, were followed up for a median 6.3 years (interquartile range 3.6-9.0). The median age at diagnosis was 10.8 years (interquartile range 9.0-12.0 years), positive family history of systemic lupus erythematosus was present in 12.1% and the majority (61.7%) were of Malay ethnicity. Common presentations included fever (87.2%), vasculitic rash (72.3%) and lethargy (69.5%). At diagnosis, leukopenia (51.1%), thrombocytopenia (41.8%) and cutaneous lupus (56%) predominate with significant renal involvement (39.7%). Renal (45.4%), liver (26%) and the central nervous system (17%) were important major organs involved during the course of the disease. At diagnosis, almost all (99.3%) patients had high disease activity (mean Systemic Lupus Erythematosus Disease Activity Index score 20.1 ± 9.6). The majority (62.4%) achieved remission or low disease activity after 6 months, maintained over the next 10 years. Damage occurred early (39.1% at 1 year) and increased with time. Ocular damage was the most common side effect (29%) and was predominantly corticosteroid related (93%). Growth retardation was significant (38.2%) with no gonadal failure or secondary malignancies. End-stage renal disease occurred in 3.1% patients whereas 53.1% had sustained renal remission. Overall mortality was 1.4%.
CONCLUSION: Despite high disease activity at diagnosis, the majority had good sustained response to treatment with low overall mortality. However, there was progressive accrual of organ damage, highlighting the need for further research and refinements into therapies for paediatric systemic lupus erythematosus.