Although PIBD is uncommon in Singapore, its incidence has risen dramatically over recent decades. A younger age of disease onset and higher proportions of perianal and stricturing/penetrating diseases suggest more aggressive disease than in Western data.

BackgroundExclusive enteral nutrition (EEN) is as effective as corticosteroids in inducing remission in children with Crohn’s disease (CD). However, over 50% of these children relapse by 12 months of diagnosis. Thiopurines are commonly prescribed as maintenance therapy for CD, but evidence for its efficacy is controversial. Data on the effectiveness of EEN in Southeast Asian (SEA) children with CD is scarce. This study aims to evaluate the efficacy of EEN induction therapy in a cohort of SEA children with newly diagnosed CD. The secondary aim was to evaluate concomitant early azathioprine (EAZ) use in determining remission rate at 6 and 12 months.MethodsCase records of all children with newly diagnosed CD from 2011 to 2014 were reviewed and relevant demographic as well as clinical data were extracted. The primary outcome measure was the number of patients who completed EEN induction therapy and achieved remission (Paediatric Crohn’s Disease Activity Index; PCDAI≤10). Factors influencing duration of remission were evaluated in particular early azathioprine (EAZ) defined as starting azathioprine within one month of diagnosis versus late azathioprine (LAZ) use.ResultsForty children with newly diagnosed CD were identified. Thirty-three children: 67% boys, median age 13y (range 3–17) completed 8 weeks of EEN induction therapy and 91% achieved remission. Significant improvements were seen in PCDAI scores (32.7 ± 9.2 to 4.2 ± 5.1; p < 0.001), mean BMI z-score (− 1.38 ± 1.57 to − 0.82 ± 1.27; p = 0.004) and baseline inflammatory markers: Erythrocyte Sedimentation Rate (51.6 ± 30.1 mm/h to 13.3 ± 7.1 mm/h; p < 0.0001) C-Reactive Protein (44.6 ± 51.0 mg/L to 5.2 ± 7.6 mg/L; p = 0.001), Albumin (30.7 ± 7.5 g/L to 38.7 ± 3.9 g/L; p < 0.0001), Platelets (464 ± 161 × 109 to 370 ± 111 × 109; p < 0.0001),. Early azathioprine initiation was associated with a remission rate of 80 and 73% at 6 and 12 months respectively. Remission was also maintained for longer duration in EAZ vs LAZ groups (p = 0.048).ConclusionEEN effectively induces remission in this cohort of SEA children with newly diagnosed CD. Early initiation of thiopurine with EEN induction therapy is effective in maintaining steroid-free remission for at least one year.

Cyclical vomiting syndrome (CVS) is a functional, debilitating disorder of childhood frequently leading to hospitalization. Affected children usually experience a stereotypical pattern of vomiting though it may vary between different individuals. The vomiting is intense often bilious, and accompanied by disabling nausea. Identifiable precipitating factors for CVS include psychosocial stressors, infections, lack of sleep and occasionally even food triggers. Often, it may be difficult to distinguish episodes of CVS from other causes of acute abdomen and altered consciousness. Thus, the diagnosis of CVS remains largely one of exclusion. Investigations routinely done during the work-up of a child with suspected CVS include both blood and imaging modalities. Plasma lactate, ammonia, amino acid and acylcarnitine profiles as well as urine organic acid profile are indicated to exclude inborn errors of metabolism. The treatment remains challenging and targeted at prevention or shortening of the attacks and can be considered as abortive, supportive and prophylactic. Use of non-pharmacological therapy is also part of the management of CVS. The prognosis of CVS is variable. More insight into the pathogenesis of this disorder as well as role of non-pharmacological therapy is needed.

BackgroundParenteral nutrition (PN) is the mainstay of treatment in children with chronic intestinal failure. We review our experience in home parenteral nutrition and describe outcomes of long term home PN therapy.MethodsA retrospective cohort study was conducted by retrieving clinical records of patients with primary digestive diseases who were on home parenteral nutrition between July 2014 to July 2016.Results13 patients (4 boys, 9 girls) received PN for more than 2 months. Out of these, 8 patients required PN therapy for more than 6 months. Main indications for long-term PN included chronic intestinal pseudo-obstruction syndrome (CIPOS, 38%), short bowel syndrome (SBS, 54%) and inflammatory bowel disease (IBD, 8%). Median age for initiation of PN was 1.7 years. Median duration of PN therapy was 1.4 years. 6 patients (46%), all with SBS, were successfully weaned off PN.One of the major complications of long term PN was central line-associated bloodstream infection (CLABSI). Incidence of CLABSI was 5.3 per 1000 catheter days. After implementation of use of Taurolidine-citrate solution (TCS), a catheter-lock solution, incidence of CLABSI improved with a pre and post-TCS CLABSI rate ratio of 0.17 (95% CI 0.04–0.52, p<0.001). There was no biochemical evidence of intestinal failure associated liver disease (IFALD) in all patients. This may be, in part, related to our practice of using SMOF lipid for patients requiring more than 2 weeks of PN. Only 2 patients exhibited lower final BMI Z-scores of < −2. There was a 100% survival in this study group.ConclusionsPN is a safe and crucial modality of treatment in children with chronic intestinal failure. SBS forms the largest subgroup in our paediatric intestinal failure population. However, patients in this subgroup were also more likely to be weaned off PN. Incidence of CLABSI can be effectively controlled with introduction of TCS. Incidence of IFALD can also be reduced with SMOF and other new lipids.

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