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AIM: To gather the current evidence and to offer recommendations for follow-up and management.METHODS: The Special Interest Group on Coeliac Diseases of the European Society of Paediatric Gastroenterology Hepatology and Nutrition formulated ten questions considered to be essential for follow-up care. A literature search (January 2010 - March 2020) was performed in PubMed or Medline. Relevant publications were identified and potentially eligible studies were assessed. Statements and recommendations were developed and discussed by all co-authors. Recommendations were voted upon: joint agreement was set as at least 85%.RESULTS: Publications (n=2775) were identified and 164 were included. Using evidence and/or expert opinion, 37 recommendations were formulated on: The need to perform follow-up, its frequency and what should be assessed, how to assess adherence to the gluten-free diet, when to expect catch-up growth, how to treat anaemia, how to approach persistent high serum levels of antibodies against tissue-transglutaminase, the indication to perform biopsies, assessment of quality of life, management of children with unclear diagnosis for which a gluten challenge is indicated, children with associated type 1 diabetes or IgA deficiency, cases of potential coeliac disease, which professionals should perform follow-up, how to improve the communication to patients and their parents/caregivers and transition from paediatric to adult health-care.CONCLUSIONS: We offer recommendations to improve follow-up of children and adolescents with coeliac disease and highlight gaps that should be investigated to further improve management.

Portal hypertension (PH) is a common complication in children with both cirrhotic and non-cirrhotic chronic liver diseases. The most important pathogenetic factors for the development of PH are the increased portal vascular resistance and an increased portal blood flow. In such a scenario, the hyperdynamic syndrome is a main feature contributing to PH, characterized by hemodynamic changes including high cardiac output, increased heart rate and total blood volume, and reduced total systemic vascular resistance. Its most threatening manifestation, variceal bleeding, represents a major cause of morbidity in this setting. Although often self-limiting at pediatric age, gastrointestinal bleeding has high morbidity, and is regarded as a frightening event by patients and carers, giving the impression of impending death. PH and its complications are also among the commonest indications to liver transplantation, therefore having relevance in the management of pediatric chronic liver disease. For these reasons, it is important to raise the awareness on the natural history of PH in children, the utility of tools that help preventing and managing acute bleeding, and the signs predicting a poor outcome, thus indicating surgery. Unlike in adults, in the pediatric setting, there is lack of data on pathophysiology, clinical features, and effective management altering the outcome of PH, and therefore children are often managed according to guidelines developed in adults. In this chapter, we discuss the advances made in the management of PH in children, compare it with the larger adult experience, and propose diagnostic and therapeutic algorithms for acute and chronic complications.

Contains fulltext : 215586.pdf (publisher's version ) (Open Access), Tissue-resident lymphocytes play a key role in immune surveillance, but it remains unclear how these inherently stable cell populations respond to chronic inflammation. In the setting of celiac disease (CeD), where exposure to dietary antigen can be controlled, gluten-induced inflammation triggered a profound depletion of naturally occurring Vgamma4(+)/Vdelta1(+) intraepithelial lymphocytes (IELs) with innate cytolytic properties and specificity for the butyrophilin-like (BTNL) molecules BTNL3/BTNL8. Creation of a new niche with reduced expression of BTNL8 and loss of Vgamma4(+)/Vdelta1(+) IELs was accompanied by the expansion of gluten-sensitive, interferon-gamma-producing Vdelta1(+) IELs bearing T cell receptors (TCRs) with a shared non-germline-encoded motif that failed to recognize BTNL3/BTNL8. Exclusion of dietary gluten restored BTNL8 expression but was insufficient to reconstitute the physiological Vgamma4(+)/Vdelta1(+) subset among TCRgammadelta(+) IELs. Collectively, these data show that chronic inflammation permanently reconfigures the tissue-resident TCRgammadelta(+) IEL compartment in CeD. VIDEO ABSTRACT.

Viral infections have been proposed to elicit pathological processes leading to the initiation of T helper 1 (TH1) immunity against dietary gluten and celiac disease (CeD). To test this hypothesis and gain insights into mechanisms underlying virus-induced loss of tolerance to dietary antigens, we developed a viral infection model that makes use of two reovirus strains that infect the intestine but differ in their immunopathological outcomes. Reovirus is an avirulent pathogen that elicits protective immunity, but we discovered that it can nonetheless disrupt intestinal immune homeostasis at inductive and effector sites of oral tolerance by suppressing peripheral regulatory T cell (pTreg) conversion and promoting TH1 immunity to dietary antigen. Initiation of TH1 immunity to dietary antigen was dependent on interferon regulatory factor 1 and dissociated from suppression of pTreg conversion, which was mediated by type-1 interferon. Last, our study in humans supports a role for infection with reovirus, a seemingly innocuous virus, in triggering the development of CeD.

Portal hypertension (PH) is a common complication in children with chronic liver disease, and its major manifestation, variceal bleeding, represents a major cause of morbidity in this setting. Although often self-limiting, gastrointestinal bleeding is regarded as a frightening event by patients and carers, giving the impression of impending death. PH and its complications are also among the commonest indications to liver transplantation, therefore having relevance in the management of pediatric chronic liver disease. For these reasons, it is important to raise the awareness on the natural history of PH in children, the utility of tools that help preventing and managing acute bleeding, and the signs predicting a poor outcome, thus indicating surgery. Unlike in adults, in the pediatric setting, there is lack of data on pathophysiology, clinical features, and effective management altering the outcome of PH, and therefore children are often managed according to guidelines developed in adults. In this chapter, we discuss the advances made in the management of PH in children, compare it with the larger adult experience, and propose diagnostic and therapeutic algorithms for acute and chronic complications.

Objective: To systematically evaluate and update evidence on the efficacy and safety of Bifidobacterium animalis subsp lactis CNCM I-3446 supplementation in preterm infants. Materials and Methods: The Cochrane Library and MEDLINE databases and major pediatric conference proceedings were searched in December 2008 for randomized controlled trials (RCTs). The company that manufactures B lactis was contacted for unpublished data. The review was restricted to RCTs performed in preterm infants<37weeks of gestation and/orwith a birthweight <2500 g. Results: Four RCTs involving 324 infants met the inclusion criteria. Compared with controls, B lactis supplementation has the potential to increase fecal bifidobacteria counts and to reduce Enterobacteriaceae and Clostridium spp counts. It also can reduce stool pH and fecal calprotectin concentrations, increase fecal immunoglobulin A and short-chain fatty acid concentrations, and decrease intestinal permeability. Compared with controls, B lactis supplementation had no effect on the risk of necrotizing enterocolitis stage 2 (3 RCTs, n¼293, risk ratio [RR] 0.53, 95% CI 0.16–1.83), risk of sepsis (2RCTs, 397 cultures, RR 0.6, 95%CI 0.07–5.2), and use of antibiotics (2 RCTs, n¼255, RR 0.67, 95% CI 0.28–1.62). The power of these studies, however, does not allow for a definitive statement regarding a reduced risk of necrotizing enterocolitis. B lactis supplementation did have some effects on anthropometric parameters. No adverse events associated with B lactis supplementation were reported. Conclusions: Evidence regarding the potential beneficial effects of B lactis supplementation in preterm infants is encouraging. Further studies to assess clinically relevant outcomes are needed.