Prevalence and significance of autoantibodies in children with metabolic dysfunction-associated steatotic liver disease: A systematic review and meta-analysis.

Significance of autoantibodies in pediatric metabolic dysfunction-associated steatotic liver disease (MASLD) is unknown. Our aim was to determine the prevalence and significance of autoantibodies in MASLD. PubMed and Scopus were searched and six articles (689 [487 males] MASLD patients) were identified. Antinuclear antibodies (ANA) was positive in 28% (95% confidence interval [CI]: 17%-39%, n = 6 studies), Antismooth muscle antibodies (ASMA) in 28% (95% CI: 8%-50%, n = 5 studies), Actin-positive in 15% (95% CI: 10%-20%, n = 2 studies) and elevated immunoglobulin G in 17% (95% CI: 1%-39%, n = 4 studies). Anti-liver-kidney-microsomal antibody was not present in any patient. There was no significant association of ANA positivity with degree of liver steatosis, liver fibrosis or nonalcoholic fatty liver disease activity score (NAS) but patients with ASMA positivity had advanced fibrosis (pooled risk ratio [RR] 1.77; 95% CI 1.16-2.71) and higher risk of NAS ≥5 (pooled RR 1.21; 95% CI: 1.01-1.44, n = 2 studies, 243 patients). To conclude, non-organ specific autoantibodies are present in over one-fourth of children with MASLD and the presence of ASMA maybe associated with increased disease severity.

Oral Tacrolimus in Steroid Refractory and Dependent Pediatric Ulcerative Colitis-A Systematic Review and Meta-Analysis.

BACKGROUND: There are limited treatment options for children with steroid-refractory or dependent ulcerative colitis (UC). A few observational studies suggest efficacy of oral tacrolimus. We performed a systematic review and meta-analysis to assess the efficacy of tacrolimus in pediatric UC. METHODS: PubMed and Scopus were searched for publications related to the use of oral tacrolimus in pediatric UC. Data regarding the clinical response and colectomy-free survival were extracted from studies that met the selection criteria. RESULTS: The search strategy yielded 492 articles of which 7 studies were included in the final review. They included 166 children (111 steroid-refractory, 52 steroid-dependent, 3 no steroids). Majority of cases (150/166 [90%]) were naïve to biologics. An initial response to tacrolimus therapy was seen in 84% (95% CI: 73%-93%) (n = 7 studies). No difference was observed between children with high (>10 ng/mL) or low tacrolimus levels (127/150 [85%] vs 12/16 [75%], P = 0.3). No difference in initial response between the children who were steroid refractory or dependent (92/111 [83%] vs 46/52 [88%], P = 0.36). The response in the biologic-exposed group (n = 10) was 70%. At 1-year follow-up, 15.2% (95% CI: 7%-21%) (n = 2 studies, 85 patients) had a sustained response on only tacrolimus. The pooled frequency of 1-year colectomy-free survival in children treated with initial oral tacrolimus was 64% (95% CI: 53%-75%). Twelve (7.2%) patients required cessation of therapy because of side effects. CONCLUSION: Tacrolimus has a high initial response in biologic naïve UC children. It can be effectively used as a bridge to other therapies with a 1-year colectomy-free survival of 64%.

Magnetic resonance imaging brain yield in developmental delay: A developing country perspective.

BACKGROUND: Developmental delay (DD) is an important neuro-morbidity in children affecting the quality of life. MRI plays a crucial role by delineating the underlying structural, metabolic, and genetic abnormalities. AIM: To determine the yield of MRI brain in delineating the various underlying abnormalities and etiological factors in children with DD and to correlate these findings with the clinical presentation. METHODS: This cross-sectional study enrolled 50 children with the developmental delay between 6 months to 6 years of age. OUTCOMES AND RESULTS: The mean age was 31.32 ± 20.56 months. The sensitivity of MRI was 72%. 81.3% of the children with microcephaly had abnormal MRI. The most common underlying etiology was hypoxic-ischemic encephalopathy (42%), followed by congenital/developmental defects and metabolic diseases (10% each). The most commonly involved region of the cerebral cortex was the occipital lobe (44%) because of the high occurrence of coexisting hypoglycemic brain injury, which is extremely common in developing countries and rare in developed countries, with 80% of them having visual abnormalities. Frontal lobe involvement was significantly more in children with abnormal motor findings and behavioral changes. Cortical grey matter abnormalities were significantly more in children with seizures. CONCLUSION AND IMPLICATIONS: It is to be emphasized that children with developmental delays should be evaluated with MRI whenever possible. Apart from hypoxic-ischemic encephalopathy, other etiologies should also be looked for.

A Prospective Observational Study of Hepatic Dysfunction in Children on Antitubercular Drugs.

The frequency, risk factors, and prognosis of antitubercular drug-induced liver injury (TB-DILI) was assessed in this prospective observational study. All consecutive children < 18 y put on antitubercular therapy (ATT) for pulmonary or extrapulmonary tuberculosis between July 2019 and December 2020 were included. Liver function tests (LFTs) were done at baseline and at 2, 4, 6 wk, and then 2 monthly after initiation of therapy till completion of ATT regimen. A total of 81 children [14.27 ± 3.38 y, 34 (42%) males] were included. Out of the patients enrolled, 10 (12.3%) developed TB-DILI at a median of 8.5 (3-18) d of starting ATT. All patients were symptomatic with the most common symptoms being anorexia and nausea (80%). A higher baseline ALT was independently associated with DILI with adjusted OR 2.1 (95% CI 1.3-3.4), p = 0.01. Eight patients tolerated reintroduction of ATT in a sequential manner, 9-24 d after discontinuation.

Comparison of Endoscopic and Histological Findings between Typical and Atypical Celiac Disease in Children.

PURPOSE: Celiac disease is a common non-communicable disease with varied presentations. Purpose of this study was to find the duodeno-endoscopic features in celiac disease and to compare duodeno-endoscopic and histological findings between typical and atypical celiac disease in children. METHODS: Hospital based observational study was conducted at Sir Padampat Mother and Child Health Institute, Jaipur from June 2015 to May 2016. Patients were selected and divided in two groups- typical and atypical celiac disease based upon the presenting symptoms. Upper gastrointestinal endoscopy and duodenal biopsy was performed for serology positive patients. Results were analysed using appropriate statistical test of significance. RESULTS: Out of 101 enrolled patients, 47.5% were male. Age ranged from 1 to 18 years. Study showed that 54.5% were typical and 45.5% were atypical. Patients presenting with atypical symptoms were predominantly of older age group. On endoscopy, scalloping, mosaic pattern, reduced fold height and absent fold height; and in histology, advanced Marsh stage were significantly higher in the typical group. CONCLUSION: Awareness of atypical presentations as well as duodeno-endoscopic features may have considerable practical importance for the diagnosis of celiac disease in children. Scalloping, mosaic pattern, reduced fold height and nodularity are main endoscopic markers of celiac disease in children. Endoscopic markers of duodenal mucosa may be important in early diagnosis of celiac disease, in children subjected to endoscopy for atypical presentations or indication other than suspected celiac disease.