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Background Celiac disease (CD) is an autoimmune enteropathy in which HLA-DQ haplotypes define susceptibility. Our aim was to evaluate if belonging to a certain HLA-DQ class risk could be associated to the clinical, serological and histological presentation of CD. Methods We performed a retrospective observational monocentric study including all 300 patients diagnosed with CD, who underwent HLA typing. Clinical, serological and histological data was collected from clinical records and their association with HLA-DQ class risk was verified through statistical tests. Results In our sample mean age at onset was 6.7 ± 4.2 years, with a prevalence of females (n = 183; 61%), typical symptoms (n = 242; 80.6%) and anti-tTG IgA ≥ 100 U/mL (n = 194; 64.7%). Family history was present only in 19% (n = 57) of patients, and it was not significantly associated with any of the clinical and demographical data analyzed or the belonging to a certain HLA-DQ class risk. We found in the male population more frequently a coexistence of CD and atopic syndrome (males: n = 47; 40.2%; females: n = 50; 27.3%; p = 0.020). Early age of onset, instead, was associated with typical symptoms (m = 6.4 ± 4; p = 0.045) and elevated liver enzymes (m = 5 ± 3.8; p < 0.001), while later age of onset was associated with presence of other autoimmune diseases (m = 8.2 ± 4; p = 0.01). We observed statistically significant influences of HLA class risk on antibodies and liver enzymes levels: G1, G4 and G2 classes showed more frequently anti-tTG IgA ≥ 100 U/mL (n = 44; 80%, n = 16; 69.6%, n = 48; 67.6% respectively; p-value = 0.037), and in patients from G2 class we found enhanced liver enzymes (n = 28; 39.4%; p-value = 0.005). HLA class risk was still significantly associated with anti-tTG ≥ 100 (p = 0.044) and with hypertransaminasemia (p = 0.010) after a multiple logistic regression adjusted for the effect of gender, age at onset and family history. Conclusions We failed to prove an association between HLA-DQ genotypes and the clinical features in our CD pediatric patients. Although, our results suggest an effect of the DQB1–02 allele not only on the level of antibodies to tTG, but possibly also on liver involvement.

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Objectives: Polyethylene glycol (PEG) split-dose regimen is recommended as the option of choice for colon preparation before colonoscopy in children and adults. Sodium picosulfate plus magnesium citrate (SPMC) is equally effective but better tolerated than PEG for bowel preparation before colonoscopy in children. The aim of this study was to assess the superiority of SPMC split-dose regimen compared with SPMC day-before regimen for bowel cleansing before colonoscopy in children. Methods:This was a multicenter, randomized, single-blind study. Pediatric inpatients undergoing colonoscopy received SPMC either in the day-before dosing or in split dosing. Overall bowel cleansing was assessed using the Boston Bowel Preparation Scale (BBPS) and was rated as successful when BBPS was ≥6. Patient tolerability, acceptability, and compliance were recorded. Results:The rate of successful cleansing level was significantly higher in the split-dose group than in the day-before group (P < 0.001). The BBPS scores were significantly higher in the split-dose group than in the day-before group for the whole colon (P < 0.001), the right colon (P < 0.001) and transverse colon (P < 0.001). Patient acceptability was better in the splitdose group (P = 0.0003; P = 0.005). The percentage of children needing nasogastric tube placement was better in the split-dose group (P = 0.007). Conclusions:The split-dose regimen of SPMC was superior to the day-before regimen in terms of successful colon cleansing and acceptability.

Tissue transglutaminase (tTG or TG2; E.C. 2.3.2.13) belongs to the transglutaminase family, a group of closely related enzymes that share the ability to catalyze the cross-linking of a glutaminyl residue of a protein/peptide substrate to a lysyl residue of a protein/peptide co-substrate. tTG is a multifunctional enzyme since it is also capable of catalyzing other biochemical reactions. The distribution and physiological roles of tTG have been widely studied in numerous cell types and tissues, but only recently its role in human diseases has started to be clarified. For example, transglutaminase activity has been hypothesized to be involved in the pathogenetic mechanisms responsible for several human diseases, including neurodegenerative diseases, such as polyglutamine diseases hitherto identified. Among human diseases, a large and recent series of studies have clearly shown that the activity of the tTG is critical for a very diffuse human pathology known as Celiac Disease. This disease is due to intolerance to a food component, gliadin, and is characterized by a very complex clinical syndrome, including gastrointestinal pathological manifestations, often associated with extra-intestinal manifestations. Interestingly, a subset of celiac patients also develops certain neurological disorders. In this review we describe the roles played by tTG in the molecular mechanisms responsible for pathophysiology of Celiac Disease.

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