Treatment with an ileal bile acid transporter inhibitor in patients with TJP2 deficiency.

There are no published data on the use of odevixibat, a selective ileal bile acid transporter (IBAT) inhibitor, in children with tight junction protein 2 (TJP2) deficiency (also named as PFIC-4). We describe a case series of five children treated with odevixibat. After treatment, serum bile acids (sBA) decreased compared to baseline [mean value: 244 (±125), vs 38 (±34) µmol/L; p = 0.

Disease course of ulcerative proctitis in children: A population-based study on behalf of the SIGENP IBD Group.

Background: The natural history of ulcerative proctitis (UP) has been poorly investigated in children.

Aims: We aimed to compare the disease course of children with UP at diagnosis to the other locations and to identify extension predictors.

Methods: This was a multicenter, observational study carried out from data prospectively entered in the SIGENP-IBD-Registry.

Factors associated with quality of life in Italian children and adolescents with IBD.

Improving the quality of life (QoL) is crucial in the management of pediatric inflammatory bowel disease (IBD). We aimed to (1) Validate the IMPACT-III questionnaire in Italian IBD children; (2) explore factors associated to QoL in pediatric IBD. Internal consistency, concurrent validity, discriminant validity and reproducibility of the Italian version of the IMPACT-III questionnaire was measured in IBD children/adolescents in 8 centers.

Phenotype and Natural History of Children With Coexistent Inflammatory Bowel Disease and Celiac Disease.

Background: Adult patients with both inflammatory bowel disease (IBD) and celiac disease (CeD) have peculiar phenotypic features. This study aimed at describing the characteristics and natural history of children with both IBD and CeD.

Methods: This was a case-control study based on a national registry.

Impact of COVID-19 pandemic on the management of paediatric inflammatory bowel disease: An Italian multicentre study on behalf of the SIGENP IBD Group.

Background: IBD management has been significantly affected during the COVID-19 lockdown with potential clinical issues.

Aims: The aim of this study was to analyse the impact of COVID-19 pandemic on the Italian paediatric IBD cohort.

Methods: This was a multicentre, retrospective, cohort investigation including 21 different Italian IBD referral centres.

Management of paediatric IBD after the peak of COVID-19 pandemic in Italy: A position paper on behalf of the SIGENP IBD working group.

Coronavirus Disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2, spreading in Italy during the first months of 2020, abruptly changed the way of practicing medicine in this country. As a consequence of the lockdown, the diagnostic and therapeutic management of paediatric chronic conditions, such as inflammatory bowel disease (IBD) has been affected. During the peak of COVID-19 pandemic, elective visits, endoscopies and infusions have been postponed, with potential clinical and psychological impact on disease course and a high likelihood of increasing waiting lists.

Clinical and Laboratory Features of 184 Italian Pediatric Patients Affected with Selective IgA Deficiency (SIgAD): a Longitudinal Single-Center Study.

Purpose: Selective IgA deficiency (SIgAD) is the most common humoral primary immunodeficiency. Long-term follow-up data in large cohort of pediatric patients are scarce.

Methods: We report on a single-center cohort of 184 pediatric patients affected with selective IgA deficiency and describe the characteristics at diagnosis and during follow-up.

Gastrointestinal Pathologic Abnormalities in Pediatric- and Adult-Onset Common Variable Immunodeficiency.

Background: Common variable immunodeficiency is the most common form of primary symptomatic immunodeficiency. Gastrointestinal manifestations, such as gastritis, diarrhea, gastrointestinal infections, and malabsorption, may complicate the clinical history in almost 50 % of patients.

Aim: To evaluate gastrointestinal histopathological findings in pediatric- and in adult-onset common variable immunodeficiency patients.

Dilated intercellular spaces in eosinophilic esophagitis.

Objectives: Dilated intercellular spaces (DIS) in the esophageal epithelium can be induced by acid and reduced by proton pump inhibitors (PPI), and are thus considered a marker of gastroesophageal reflux disease (GERD). Over the years, however, DIS have also been reported in esophagitis unrelated to GERD. Because DIS have never been formally measured in eosinophilic esophagitis (EoE), we aimed at detecting and measuring DIS in EoE before and after nutritional or pharmacological therapy.

Growth retardation and reduced growth hormone secretion in cystic fibrosis. Clinical observations from three CF centers.

Objective: Growth delay in cystic fibrosis is frequent and is usually the result of several interacting causes. It most often derives from severe respiratory impairment and severe malabsorption. There are however patients whose clinical condition is not severe enough to be held accountable for this phenomenon.

[Acute hepatitis as a side effect of albendazole: a pediatric case].

We describe a case of acute hepatitis with jaundice in a patient of seven years of age in oral treatment with albendazole; a mild increase of liver function tests is a well-known side effect after prolonged administration but acute hepatitis has never been described in childhood.

[Acute hepatitis as a side effect of albendazole: a pediatric case].

We describe a case of acute hepatitis with jaundice in a patient of seven years of age in oral treatment with albendazole; a mild increase of liver function tests is a well-known side effect after prolonged administration but acute hepatitis has never been described in childhood.

Dietary protein-induced proctocolitis in childhood.

Cow's milk protein-induced proctocolitis presents with overt rectal bleeding in otherwise healthy infants and is characterized by an eosinophilic infiltrate of the left colonic mucosa. Although it is the most common cause of proctocolitis in infancy, dietary protein-induced proctocolitis had hardly ever been reported in childhood so far. We hereby report 16 otherwise healthy children aged 2-14 yr, who presented over a 6-yr period with persistent or recurrent rectal bleeding related to a mild form of left-sided colitis characterized by a prominent eosinophilic infiltration, focal lymphoid follicle hyperplasia, and a prompt clinical and histological response to a cow's milk-free diet.