Bramsenkronborg4134

Age ≥13 years old represents a risk factor for lack of compliance at V1 (p = 0,02) and V2 (p = 0,04), as well as foreign nationality at V2 (p = 0,001). CONCLUSIONS The BS, serology, and a clinical interview, integrated, are reliable tools for assessing pediatric adherence to GFD. We argue that referring patients to the GP after remission of CD is important, but the process must be improved and recommendations are required.OBJECTIVES To assess differences in the diagnosis and management of Eosinophilic Esophagitis (EoE) by European pediatric (PG) and adult gastroenterologists (AG), and their self-reported adherence to guidelines. METHODS A multiple-choice questionnaire gauged the diagnostic and management strategies of gastroenterologists treating children or adults in 14 European countries and the United Arab Emirates (UAE). RESULTS Questionnaires were completed by 465 PG and 743 AG. PG were significantly more likely to take biopsies in patients with symptoms of esophageal dysfunction (86.2% PG vs. 75.4% AG, p  less then  0.001) and to perform endoscopic follow-up (86.3% PG vs. selleck chemicals 80.6% AG, p  less then  0.001). After failure of proton-pump inhibitors, topical steroids were the preferred second line therapy, however PG opted more frequently for elimination diets (47.5% PG vs. 13.7% AG, p  less then  0.001). More PG than AG indicated having read recent guidelines (89.4% PG vs. 58.2% AG, p  less then  0.001). Geographic differences in practice were reported, with respondents from the United Kingdom, Portugal and Spain more often adhering to recommended biopsy protocols. Physicians in the UAE, France, Lithuania and Poland tended to opt for steroid therapy or elimination diets as first line therapy, in contrast to most other countries. CONCLUSIONS Significant differences in general practice between PG and AG were demonstrated with notable divergence from consensus guidelines. International practice variations are also apparent. Among other strategies, educational activities to highlight current recommendations may help harmonize and optimize clinical practice.OBJECTIVES Current pediatric guidelines allow non-invasive diagnosis of celiac disease in selected children. We investigated in a large cohort study whether the severity of villous atrophy at diagnosis is associated with clinical characteristics or long-term health outcomes, thus having a prognostic significance. METHODS Comprehensive medical data on 906 children with celiac disease were analyzed. Long-term health outcomes of 503 adult patients diagnosed in childhood were moreover assessed with a specific study questionnaire and validated Gastrointestinal Symptom Rating Scale (GSRS) and Psychological General Well-Being (PGWB) questionnaires. Patients were classified into three groups according to severity of villous atrophy at diagnosis and all variables were compared. RESULTS Altogether 34% of the patients had partial, 40% subtotal, and 26% total villous atrophy. Children with milder lesions were diagnosed more recently (median year 2007 vs. 2006 vs. 2001 respectively, p  less then  0.001), more often by screening (30% vs. 25% vs. 17%, p  less then  0.001) and they suffered less often from anemia (16% vs. 21% vs. 32%, p  less then  0.001) and growth disturbances (22% vs. 36% vs. 54%, p  less then  0.001) and had lower transglutaminase-2 antibody levels (median 64 U/l vs. 120 U/l vs. 120 U/l, p  less then  0.001). There was no difference in other disease features.Altogether 212 adults diagnosed in childhood completed the questionnaires. Severity of villous atrophy at childhood diagnosis did not predict presence of complications or comorbidities, persistent symptoms, and self-perceived health, quality of life or adherence to a gluten-free diet in adulthood. CONCLUSIONS Presence of advanced villous atrophy at diagnosis is associated with more severe clinical characteristics but not with poorer long-term health and treatment outcomes.Pathogenic sequence variants in the nuclear bile acid receptor FXR, encoded by NR1H4, have been reported in a small number of children with low-GGT cholestasis progressing to liver failure. We describe three additional children from two unrelated families with cholestasis and liver failure due to pathologic variants in NR1H4. One patient underwent liver transplantation and has had good clinical outcomes in six years of follow-up. While that patient has biochemical evidence of increased bile acid synthetic activity, he has not experienced post-transplant diarrhea or allograft steatosis, as has been reported among other transplanted patients.Non-thermal methods are more efficient at preserving various biological properties of human milk, as compared to holder pasteurisation (HoP), which is the most common preservation method. This study was performed to assess the effects of non-thermal processing on bactericidal activity against Escherichia coli in human milk. Milk samples obtained from the Regional Human Milk Bank in Warsaw at Holy Family Hospitalwere processed by HoP, irradiated with UV-C for 5, 10, and 15 min (6,720 J/L each minute), subjected to two variations of high-pressure processing (HPP) 1) 450 MPa for 15 min and 2) 200 MPa for 10 min + 400 MPa for 10 min, with a 10-min break. The samples were then evaluated by a bactericidal assay (raw untreated human milk was used as a control). The bactericidal capacity after HoP was preserved in 12.1% of samples, showing a significant reduction in bactericidal properties compared to in raw milk (p  0.05). Non-thermal methods of human milk treatment better preserve the bactericidal capacity compared to holder pasteurisation. Those alternative technologies to HoP can be proposed after further investigation for milk processing for Human Milk Banks facilities.OBJECTIVES To assess the effect of long-term (104 weeks) treatment with recombinant sebelipase alpha (rhSA) on serum lipid and hepatic transaminase levels, and liver histopathology in four siblings diagnosed with lysosomal acid lipase deficiency (LAL-D). METHODS Four male siblings from the same non-consanguineous parents were diagnosed with the late-onset phenotype of LAL-D in 2015. Liver specimens were obtained by biopsy at baseline and after 104 weeks of enzyme replacement with rhSA (1 mg/kg, IV, every 2 weeks). Hepatic transaminase, lipid and lipoprotein levels were assessed at baseline and sequentially every 16 weeks for 104 weeks. Hepatic steatosis was evaluated from hematoxylin and eosin stained specimens, and fibrosis was evaluated (Metavir scoring system) from trichrome-stained specimens obtained at baseline and following 104 weeks of treatment with rhSA. RESULTS All four siblings had improvement in their serum lipid and hepatic transaminase levels after treatment with rhSA. Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels decreased from baseline by an average of 47% and 56%, respectively.