Gastroenterology and Hepatology From Bed to Bench最新文献

Aim: This review sought to evaluate the significance of a functional assessment for liver transplant candidates, i.e., frailty, in the pre-transplant setting and its association with mortality and morbidities.

Background: Liver transplantation (LT) remains the treatment of choice for patients with end-stage liver disease. Due to the shortage of organs for LT, a careful selection of suitable recipients is essential. Frailty, a measure of physiologic reserve and increased vulnerability to stressors, was initially used in geriatrics and then introduced to the field of transplantation for better patient selection.

Methods: PubMed, Scopus, and Web of Science databases were reviewed up until January 2023. The search terms included: "frail*", "liver", and "transplant*". A Meta-analysis was conducted for the hazard ratios (HRs) obtained from the COX regression models. Fifty-five studies were included in this review; ten were included in the meta-analysis.

Results: The prevalence of frailty varied from 2.82% to 70.09% in the studies. Meta-analysis showed that overall frailty had a significant association with mortality (pooled adjusted HR [95%CI]: 2.66 [1.96-3.63]). Subgroup analyses revealed that both the Liver Frailty Index and Fried Frailty Index were significantly associated with mortality. Furthermore, these studies have demonstrated that this population's frailty is associated with ascites, hepatic encephalopathy, and esophageal varices.

Conclusion: According to emerging evidence, frailty is associated with increased morbidity and mortality of the patients on the LT waiting list. Further randomized trials are required to determine the efficacy and safety of variable interventions in the frail population.

Primary enteropathies of infancy comprise of epithelial defects including microvillus inclusion disease, tufting enteropathy, and enteroendocrine cell dysgenesis and autoimmune enteropathies. The diseases in this group cause severe chronic (>2-3 weeks) diarrhoea starting in the first weeks of life and resulting in failure to thrive in the infant. Duodenal biopsies show moderate villous shortening together with crypt hyperplasia which are the main features causing resemblance to coeliac disease. We, hereby, report a term-born male infant of consanguineous parents. His two siblings died during infancy. He developed watery, urine-like diarrhea on the 3rd day of his life. On the postnatal 6th day he weighed 2750 grams, became dehydrated and had metabolic acidosis. Upper GI endoscopy performed on the postnatal 20th day appeared normal. Light microscopic examination of the duodenal biopsy showed moderate villous blunting, with mildly increased inflammatory cells in the lamina propria or and intraepithelial lymphocytosis. Enterocytes at the villous tips showed an irregular vacuolated appearance in the apical cytoplasm with patchy absence of the brush border demonstared by PAS and CD10. Electron microscopy revealed intracytoplasmic inclusions that were lined by intact microvilli in the apical cytoplasm of enterocytes. As he was dependent on TPN and aggressive intravenous fluid replacement he was hospitalized throughout his life. He died when he was 3 years and 4 months old. Paediatric coeliac disease is in the differential diagnosis of primary enteropathies of childhood. The differentiation lies on duodenal biopsy interpretation together with genetic analysis to detect the underlying genetic defect in childhood enteropathies.

Aim: We evaluated the Persian version of the pediatric constipation score-parent report (PCS) validity and reliability.

Background: Functional constipation in children results in physical and psychological problems. Therefore, it is necessary to utilize a questionnaire to assess the health-related quality of life in children with chronic constipation.

Methods: First, our team translated the English version of the questionnaire into the Persian language. Second, the psychometric properties of the Persian version were collected in 149 children with functional constipation referred to a pediatrics hospital by an expert team. We assessed content validity (CV) through the CV index (CVI) and CV ratio (CVR). The construct validity was evaluated by exploratory factor analysis, and reproducibility was tested based on test-retest reliability using the intra-class correlation coefficient (ICC). Internal consistency was calculated using Cronbach's α. we also evaluated the ceiling or floor.

Results: Results showed acceptable CVI in relevancy, clarity, and simplicity, acceptable CVR for all items, moderate internal consistency (Cronbach's alpha=0.548), and almost perfect reproducibility (ICC=0.93). No ceiling or floor effect was seen.

Conclusion: The Persian version of PCS showed good validity and reliability in children with functional constipation in Iran. Therefore, we can use it in clinical and research domains in Persian-speaking countries.

The celiac disease (CD) diagnosis sometimes is challenging and diagnostic process cannot always follow a simple algorithm but it requires a close collaboration between histo-pathologists, clinicians, laboratory and genetic experts. The genetic predisposition for CD is related to HLA-DQ2 and/or DQ8 but other HLA haplotypes and non-HLA genes may be involved in genetic predisposition. In particular DQ7 may represent an additive and independent CD risk associated haplotype. We describe an unusual case of a female 42 year old with a previous diagnosis of Hodgkin lymphoma, who has a clinical presentation suggestive for CD with negativity for anti-transglutaminase and anti-endomysium antibodies and HLA-DQ7 positivity.

The diagnosis of celiac disease relies on the assessment of serological data and the presence of histological alterations in the duodenal mucosa. The duodenal biopsy is pivotal in adults, and in some circumstances in children, to confirm the clinical suspicion of celiac disease. The correct interpretation of duodenal biopsies is influenced by numerous variables. The aim of this overview is to describe the correct methodological approach including the procedures of biopsy sampling, orientation, processing, staining and histopathological classification in order to avoid or minimize the errors and the variability in duodenal biopsy interpretation. Multiple biopsies taken from different sites of the duodenum during endoscopy maximize the diagnostic yield of duodenal histological sampling. Proper orientation of the biopsy samples is of the utmost importance to assess histological features of pathological duodenal mucosa and to avoid artifacts that may lead even an experienced pathologist to a wrong histological interpretation with subsequent misdiagnosis of celiac disease. An immunohistochemical stain for CD3 can be invaluable to aid the pathologist in obtaining a more accurate intra-epithelial T lymphocytes count. A simplified histological classification facilitates the clinician's work and improves the communication between pathologist and clinician. An integrated clinical and pathological approach is required for a correct diagnosis of celiac disease since a relatively large number of conditions may cause duodenal damage with a histological appearance similar to that of celiac disease.

Serology has significantly revolutionized the knowledge of celiac disease (CD), leading to the identification of unsuspected patients in at-risk CD groups, thereby increasing the number of CD diagnoses compared to the pre-screening era. Several markers for CD with a progressive diagnostic accuracy have been identified over the years, but only three of them, i.e. anti-tissue transglutaminase (anti-tTG), anti-endomysial (EmA) and anti-deamidated gliadin antibodies (DGP) are currently assessed in the daily clinical practice. A thorough review of the literature identified 44 original studies published between 1998 to 2022 for a total of 5098 pediatric and adult CD patients (without selective IgA deficiency) and 11930 disease controls. The results highlighted that anti-tTG IgA exhibited a higher sensitivity for CD (93.4%) than EmA IgA (92.8%), DGP IgG (81.8%) and DGP IgA (83.8%). The specificity of EmA IgA (99%) resulted to be higher than those of anti-tTG IgA (95.8%), DGP IgG (96.4%) and DGP IgA (92.1%). In patients with selective IgA deficiency, a condition closely related to CD, serological screening should include one of the three antibodies of IgG class, since anti-tTG, DGP and EmA have a very similar diagnostic accuracy in this clinical setting. According to age, there are two main diagnostic strategies for CD detection. In children, the revised ESPGHAN 2020 guidelines established that CD could be diagnosed in both symptomatic and asymptomatic children by high anti-tTG IgA titers (>10 times the cut-off) and EmA positivity with no need to obtain duodenal biopsy and HLA typing. In adult patients, although high tTG IgA titers (confirmed by EmA IgA positivity) correlate with villous atrophy, an intestinal biopsy is still considered mandatory for confirming CD diagnosis. Currently, a case finding approach in at-risk groups is preferred to mass screening for CD detection.