Very Early-Onset IBD-Associated IL-18opathy Treated with an Anti-IL-18 Antibody.

: The aetiology of inflammatory bowel disease (IBD), particularly if occurring early in childhood, is a diverse and patient-focused treatment that is required when standard therapy is ineffective. A clinical case report is presented of a child with very early-onset IBD (VEOIBD) and evidence of high serum IL-18 responding to anti-IL-18 immunotherapy. Detailed cytokine profiling was performed by ELISA and multiplex assay flow cytometry.

Non-Graft-versus-Host Disease Enterocolitis Following Cord Blood Transplantation is Real, with Poorly Understood Pathophysiology, and Requires Distinct Management, with Eventual Resolution without Immune Suppression.

Enterocolitis is common after cord blood transplantation (CBT) and a specific, non-graft-versus-host disease (GVHD) entity with specific histopathologic features ("cord colitis") has been described in some cases in selected series. Immune suppression is not without risk, and we have used it only when biopsy features are consistent with classical GVHD. In the absence of biopsy features of classical GVHD, our management of intestinal failure has been supportive, and we have withdrawn immune suppression to allow immune reconstitution and better prevent relapse of malignant disease and reduce infectious complications.

Reducing Metabolic Bone Disease Burden in Intestinal Failure Children on Home Parenteral Nutrition.

Objective: To determine the prevalence of secondary hyperparathyroidism in a cohort of pediatric patients receiving home parenteral nutrition.

Methods: For a service review, a population-based cohort of 37 pediatric intestinal failure patients receiving long-term parenteral nutrition that underwent serial biochemical monitoring during a study period of approximately 4 years were examined. Following the production of an algorithm, a follow-up audit was carried out (n = 33) after approximately 6 months.

Efficacy and Safety of Teduglutide in Infants and Children With Short Bowel Syndrome Dependent on Parenteral Support.

Objectives: Our objective was to evaluate the short- and long-term safety and efficacy of teduglutide treatment in infants and children with short bowel syndrome with intestinal failure (SBS-IF).

Methods: Two open-label phase 3 studies and 1 extension study investigated the short- and long-term safety and efficacy of teduglutide (0.05 mg/kg/day) in infants and children with SBS-IF: NCT03571516, 24-week study of infants who were randomized to receive teduglutide or standard of care (SoC); NCT02980666, 24-week study of infants and children who all received teduglutide; and NCT03268811, 24-week extension study of patients who completed NCT02980666 (patients could receive up to 48 weeks of total treatment).

Transition Services for Paediatric Inflammatory Bowel Disease: A Multicentre Study of Practice in the United Kingdom.

Objectives: Patients with paediatric inflammatory bowel disease (IBD) constitute one of the largest cohorts requiring transition from paediatric to adult services. Standardised transition care improves short and long-term patient outcomes. This study aimed to detail the current state of transition services for IBD in the United Kingdom (UK).

Neutrophil dysfunction triggers inflammatory bowel disease in G6PC3 deficiency.

The glucose-6-phosphatase catalytic subunit 3 (G6PC3) encodes a ubiquitously expressed enzyme that regulates cytoplasmic glucose availability. Loss-of-function biallelic G6PC3 mutations cause severe congenital neutropenia and a diverse spectrum of extra-hematological manifestations, among which inflammatory bowel disease (IBD) has been anecdotally reported. Neutrophil function and clinical response to granulocyte colony-stimulating factor (G-CSF) and hematopoietic stem cell transplantation (HSCT) were investigated in 4 children with G6PC3 deficiency-associated IBD.

Refractory very early-onset inflammatory bowel disease associated with cytosolic isoleucyl-tRNA synthetase deficiency: A case report.

Background: Aminoacyl tRNA synthetases/ligases (ARSs) are highly conserved enzymes involved in attaching amino acids to tRNA promoting protein synthesis. Although deficiencies of ARSs localized to the mitochondria classically present with neuropathology, the clinical features of cytosolic ARS deficiencies are more variable. They have previously been associated with neonatal hepatitis, but never with early-onset inflammatory bowel disease.

Use of Infliximab Biosimilar Versus Originator in a Pediatric United Kingdom Inflammatory Bowel Disease Induction Cohort.

Objectives: The aim of the study was to summarize short-term effectiveness, safety, and cost of using infliximab biosimilar (IFX-B) drugs, (Inflectra [Hospira] and Remsima [NAAP]) compared to originator infliximab (IFX-O) (Remicade [MSD]) in biologic naive pediatric inflammatory bowel disease in the United Kingdom.

Methods: Prospective audit of patients starting anti-tumour necrosis factor (TNF) therapy. Disease severity, response to treatment, and remission rate was measured by Pediatric Crohn's Disease Activity Index (PCDAI) and/or Physician Global Assessment.

REVIEW OF CELIAC DISEASE PRESENTATION IN A PEDIATRIC TERTIARY CENTRE.

Background: Celiac disease is an immune-mediated disorder with a multiform presentation and therefore a challenging diagnosis.

Objective: Our purpose is to identify the epidemiological, clinical, laboratory and histologic characteristics of children with celiac disease at diagnosis and on follow-up.

Methods: Children with previously established or newly diagnosed celiac disease, admitted in a tertiary centre in a two-year period (2014-2016) were recruited.

'Holiday sickness'-reported exploratory outcome of over 500 United Kingdom holidaymakers with travellers' diarrhoea.

Aims: To ascertain any predictors of potential food poisoning pathogens and development of post-infective irritable bowel syndrome (IBS) in UK travellers. An analysis was undertaken on prospectively collected data on 527 patients reporting symptoms of suspected food poisoning between June 2012 and June 2015.

Main Outcome Measures: Positive stool sample indicative of food poisoning pathogens and diagnosis of post-infective IBS.

Telephone Consultation as a Substitute for Routine Out-patient Face-to-face Consultation for Children With Inflammatory Bowel Disease: Randomised Controlled Trial and Economic Evaluation.

Background: Evidence for the use of telephone consultation in childhood inflammatory bowel disease (IBD) is lacking. We aimed to assess the effectiveness and cost consequences of telephone consultation compared with the usual out-patient face-to-face consultation for young people with IBD.

Methods: We conducted a randomised-controlled trial in Manchester, UK, between July 12, 2010 and June 30, 2013.

Enteric neural disruption in necrotizing enterocolitis occurs in association with myenteric glial cell CCL20 expression.

Objective: The aetiology of necrotising enterocolitis (NEC) is unknown, but luminal factors and epithelial leakiness appear critical triggers of an inflammatory cascade. A separate finding has been suggested in mouse models, in which disruption of glial cells in the myenteric plexus induced a severe NEC-like lesion. We have thus looked for evidence of neuroglial abnormality in NEC.

Acute disseminated encephalomyelitis in conjunction with inflammatory bowel disease.

Neurological complications in paediatric patients with inflammatory bowel disease (IBD) are rare. Most previous reports involve cerebral venous thrombosis, central nervous system vasculitis, or peripheral nerve inflammation. We report a child with active inflammatory bowel disease complicated by acute disseminated encephalomyelitis (ADEM).

Bezafibrate to prevent relapsing pancreatitis in WAGR syndrome.

Recurrent episodes of acute, severe pancreatitis are a feature of the rare WAGR congenital anomaly syndrome. Although pancreatitis is well recognised as a life-threatening association of WAGR syndrome, treatment to prevent pancreatitis is not recommended. We report the successful prevention of hypertriglyceridaemia-associated relapsing pancreatitis in a child with WAGR syndrome with Bezafibrate.

Probiotics for maintenance of remission in ulcerative colitis.

Background: Ulcerative colitis is a chronic relapsing disease characterised by diffuse mucosal inflammation limited to the colon. Current maintenance treatments have multiple adverse events and an effective treatment with minimal adverse events is desired. Several studies have demonstrated the importance of intestinal flora in the pathogenesis of ulcerative colitis.

Reduced production of sulfated glycosaminoglycans occurs in Zambian children with kwashiorkor but not marasmus.

Background: Kwashiorkor, a form of severe malnutrition with high mortality, is characterized by edema and systemic abnormalities. Although extremely common, its pathophysiology remains poorly understood, and its characteristic physical signs are unexplained.

Objective: Because kwashiorkor can develop in protein-losing enteropathy, which is caused by a loss of enterocyte heparan sulfate proteoglycan (HSPG), and previous observations suggest abnormal sulfated glycosaminoglycan (GAG) metabolism, we examined whether intestinal GAG and HSPG are abnormal in children with kwashiorkor.

Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion.

There is increasing recognition that Henoch-Schonlein purpura may present in an atypical form in which gastrointestinal symptoms may predominate, and classic cutaneous changes may be delayed or absent. This may lead to significant diagnostic delay. We report the case of a 9-year-old girl who presented acutely with life-threatening gastrointestinal haemorrhage from multiple intestinal sites, with no skin rash and only mild evidence of renal involvement.

Constipation with acquired megarectum in children with autism.

Objective: Recent evidence suggests that autistic children may have significant gastrointestinal symptoms. Although constipation occurs in 2% to 5% of healthy children, its clinical diagnosis is often difficult in children with behavioral disorders. We thus aimed to assess the prevalence of fecal loading in autistic children with gastrointestinal symptoms and to identify possible predictors of constipation.