Clinical, histopathological features and efficacy of elimination diet and proton-pump inhibitor therapy in achieving histological remission in Asian children with eosinophilic gastritis.

AIM: Paediatric eosinophilic gastritis (EG) is a rare disorder and existing literature on diagnostic criteria and management remains lacking. We aim to describe the clinical spectrum and assess the efficacy of dietary elimination and proton-pump inhibitor (PPI) therapy, with particular emphasis on histologic remission in children with primary EG. METHODS: We performed a retrospective study of patients aged 0-18 years diagnosed with EG at a single centre in Singapore from 2013 to 2021. EG was diagnosed based on histological criteria of infiltration of >30 eosinophils per high-power film (HPF) in >5 separate HPFs from gastric biopsies, in the absence of other causes. First-line treatment consisted of PPI therapy and empiric 1-6 food elimination diet (FED). Outcomes measured were clinical, endoscopic and histological remission (defined as eosinophil count <20/HPF in gastric biopsies). RESULTS: Twenty-one (66.7% females) patients were included with median age at diagnosis of 15 months (range:3-192). Majority presented with vomiting (76.2%) and gastrointestinal bleeding (71.4%). Twenty patients were initiated on FED+PPI and 16 had post-treatment biopsies. Clinical, endoscopic and histologic remissions were achieved in 94.7%, 81.3% and 68.8% respectively following FED+PPI. Histologic remission was significantly associated with younger age (9 vs. 132 months; P = 0.026). Four patients who did not respond to FED+PPI were started on oral viscous budesonide, of whom one achieved histological remission and two had clinical improvement. CONCLUSIONS: FED+PPI is effective as first-line treatment in achieving histological remission in paediatric EG particularly in younger patients. Topical corticosteroids can be considered for those who have failed FED+PPI therapy.

Non-Invasive Predictors for the First Variceal Hemorrhage in Children With Biliary Atresia After Kasai Portoenterostomy.

BACKGROUND/AIM: : Portal hypertension and variceal hemorrhage (VH) are significant complications in biliary atresia (BA). The study aims to evaluate risk factors and noninvasive markers that predict actual VH for the first time in children with BA without prior endoscopic surveillance or treatment. METHODS: Retrospective review was performed of patients diagnosed with BA from 1989 to 2016 at a single center. Primary outcome was the first episode of VH. Patients were stratified into VH and non-VH groups according to the development of VH, and laboratory and ultrasonographic data were analyzed at 2 time points: pre-VH and the last follow-up. Existing indices, varices prediction rule (VPR), and aspartate aminotransferase (AST)-platelet ratio index (APRI) were also applied retrospectively to evaluate their performance in prediction of VH in our cohort. RESULTS: Seventy-two patients were included; 16 patients developed the first VH at median age of 5.5 years. On univariate analysis, serum albumin (P = 0.034), AST (P = 0.017), hemoglobin (P = 0.019), platelet count (P = <0.001), spleen size Z-score (P = <0.001), and rate of splenic enlargement (P = 0.006) were associated with VH. On multivariable regression analysis, only platelet count was independently predictive (P = 0.041). The optimal cutoff values for prediction of the first VH were platelet count ≤100 × 109/L (sensitivity 75.0%, specificity 80.4%, positive predictive value [PPV] 52.2%, negative predictive value [NPV] 91.8%), VPR ≤3.0 (sensitivity 81.3%, specificity 85.7%, PPV 61.9%, NPV 94.1%), and APRI ≥3.0 (sensitivity 81.3%, specificity 76.8%, PPV 50.0%, NPV 93.5%). CONCLUSIONS: Platelet count <100 × 109/L and VPR <3.0 are simple, reproducible and effective noninvasive markers in predicting the first episode of acute VH in children with BA and may be used in pediatrics for the selection of patients to undergo primary prophylactic endoscopic therapy.

Characteristics and outcome of primary sclerosing cholangitis associated with inflammatory bowel disease in Asian children.

BACKGROUND: Current knowledge on the clinical features and natural history of childhood primary sclerosing cholangitis - inflammatory bowel disease in Asia is limited. We described the presenting features and natural history of primary sclerosing cholangitis-inflammatory bowel disease seen in a cohort of Southeast Asian children. METHODS: We conducted a retrospective review of childhood primary sclerosing cholangitis-inflammatory bowel disease from three tertiary centers in Singapore and Malaysia. RESULTS: Of 24 patients (boys, 58%; median age at diagnosis: 6.3 years) with primary sclerosing cholangitis-inflammatory bowel disease (ulcerative colitis, n = 21; Crohn's disease, n = 1; undifferentiated, n = 2), 63% (n = 15) were diagnosed during follow-up for colitis, and 21% (n = 5) presented with acute or chronic hepatitis, 17% (n = 4) presented simultaneously. Disease phenotype of liver involvement showed 79% had sclerosing cholangitis-autoimmune hepatitis overlap, 54% large duct disease, and 46% small duct disease. All patients received immunosuppression therapy. At final review after a median [±S.D.] duration follow-up of 4.7 [±3.8] years, 12.5% patients had normal liver enzymes, 75% persistent disease, and 12.5% liver failure. The proportion of patients with liver cirrhosis increased from 13% at diagnosis to 29%; 21% had portal hypertension, and 17% had liver dysfunction. One patient required liver transplant. Transplant-free survival was 95%. For colitis, 95% had pancolitis, 27% rectal sparing, and 11% backwash ileitis at initial presentation. At final review, 67% patients had quiescent bowel disease with immunosuppression. One patient who had UC with pancolitis which was diagnosed at 3 years old developed colorectal cancer at 22 years of age. All patients survived. CONCLUSIONS: Liver disease in primary sclerosing cholangitis-inflammatory bowel disease in Asian children has variable severity. With immunosuppression, two-thirds of patients have quiescent bowel disease but the majority have persistent cholangitis and progressive liver disease.