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Aim: The purpose of this retrospective single-center study was to determine the frequency of sarcopenia and its association with mortality and other morbidities in children with chronic liver disease who had undergone liver transplantation. Background: Sarcopenia, a muscle-wasting syndrome, is common in patients with advanced liver disease and is associated with increased morbidity and mortality. While sarcopenia in adults has been extensively studied, there is little information in this regard about children and adolescents with chronic liver diseases. Methods: The study included 108 children and adolescents who had undergone liver transplantation. Sarcopenia was measured using skeletal muscle index at the third lumbar vertebral level and assessed using abdominal computed tomography imaging. Results: The frequency of sarcopenia in the studied population was found to be 45.7%. Patients with sarcopenia were more likely to be male (P<0.0001), older (P<0.0001), and had lower height-for-age z-scores (P=0.012). Genetic/metabolic diseases were the most common underlying cause of sarcopenia in children. Except for a higher rate of transplant rejection in the sarcopenia group (P=0.035), there was no significant difference in mortality rates (P=0.688) or post-LT complications between the two groups. One year after LT, computed tomography-derived body composition parameters revealed no significant differences between children who survived and those who did not. Conclusion: Our findings indicated a high frequency of sarcopenia in children with chronic liver disease, implying that more research is needed to better understand its impact on clinical outcomes in this population. [ABSTRACT FROM AUTHOR]

Background: Metabolic syndrome (MetS) is a set of conditions that occur together and increase the risk of cardiovascular disease. Previous studies have linked a gluten-free diet (GFD) to obesity and MetS in some populations. However, others have suggested that weight gain is usually regulated only in underweight individuals with celiac disease (CD). Owing to the lack of sufficient data and the importance of GFD in controlling cardiovascular disease, we surveyed the prevalence of MetS and its components before and after a year of GFD in patients referred to the main celiac clinic in southern Iran. Methods: This was a repeated cross-sectional study conducted on 69 patients with a definite diagnosis of cardiovascular disease who were on follow-up and registered at the Shiraz Celiac Clinic. Demographic, anthropometric, and laboratory measurements at the time of diagnosis and one year after the GFD were extracted from their medical records. Results: The participants' mean age was 35.53, and 68.1% were women. The prevalence of MetS increased from 5.8% to 11.6% after a year of the GFD; however, this increase was not statistically significant. Waist circumference (WC) and serum triglyceride levels were significantly elevated during the study period. Conclusion: A GFD may contribute to the development of MetS in patients with cardiovascular disease; however, the rate of MetS is still lower than that in the general population. It is critical to educate patients about these potential risks and encourage them to have a healthy lifestyle that includes a balanced diet and physical activity. [ABSTRACT FROM AUTHOR]

Background: Celiac disease (CD) is an autoimmune disease caused by gluten intake. Traditionally CD was believed to be a disease of the gut, although a wide range of extra-intestinal manifestations (EIM) was recognized. The exact prevalence of EIM and the associated risk factors have not been well studied. Aim: We aimed to assess the prevalence of EIM in children with CD and their association with human leukocyte antigen (HLA) typing, and pathological and laboratory indices. Method: We conducted a cross-sectional study on children and adolescents with a definite diagnosis of CD. They were followed in the main Celiac Clinic of Southern Iran. Results: We included 204 children who were visited between 2012 and 2017. Nearly 85% of them were positive for HLA-DQ2 and 40.6% for HLA-DQ8. The most prevalent intestinal complaints reported were abdominal pain (42.6%) and chronic constipation (19.1%). Failure-to-thrive (32.7%), iron deficiency anemia (25%), short stature (20.5%), and eczema (18.6%) were the most common EIMs. However, failure-to-thrive and short stature were presented at significantly younger ages, whereas those patients with concomitant type 1 diabetes mellitus (DM) were significantly older. We also found significant relationships between autoimmune thyroid disease and HLA-DQ5, and the presence of headaches with HLA-DQ7. The prevalence of HLA types of DQ2, DQ8, DQ6, and DQ7 significantly varied among different Marsh groups. Patients who were positive for HLA-DQ8, were significantly older, taller, and weightier. No significant association was found between HLA types and any of the gastrointestinal symptoms, anti-tTG and compliance to gluten free diet. Moreover, there were no statistically significant differences detected between the presence of each individual EIM, the level of IgA anti-tTG, sex, and Marsh typing. Conclusion: This study highlights the presence of EIM in CD and their associated factors. We show the potential role of HLA typing in some EIMs, which may shed light for future studies. [ABSTRACT FROM AUTHOR]

Background: Celiac disease (CD) is a common autoimmune disorder that presents intestinal and extra-intestinal symptoms. It is also associated with cardiovascular diseases and malignancies, and mortality risk. The only way to control the disease is to follow a strict gluten-free diet (GFD) for the rest of life. Objectives: This survey aimed to investigate GFD non-adherence and causes in the pediatric setting. Methods: In this study, 187 children aged between 2.5 to 14 years old with a confirmed diagnosis of CD at least for a year were studied using a questionnaire-based interview in a census study between 2018 to 2019 in a referral center in southern Iran. Results: About 40% of children adhered to a GFD poorly. This group significantly complained of more symptoms than the group with high adherence. Improper access was the most important cause of non-adherence to a GFD. The mean current weight and at the time of diagnosis as well as the mean current BMI and at the time of diagnosis in the non-adherent group were significantly lower than the adherent group. However, IgA anti-transglutaminase antibodies and histopathologic examination did not change remarkably. Furthermore, no significant relationship was found between following a GFD and age, age at the time of diagnosis, gender, and parental educational status. Conclusions: According to our results, inaccessibility, high costs, and lack of food labeling were the primary reasons for nonadherence to GDF. Therefore, to increase compliance, easy access to GFD with proper food labeling and suitable price should be implemented. [ABSTRACT FROM AUTHOR]

Objectives: The recurrence of underlying diseases remains a major cause of graft failure after liver transplant. This study aimed to identify factors associated with the recurrence of underlying diseases and investigate the incidence of these factors and recurrence at the main liver transplant center in Iran., Materials and Methods: We included adult liver transplant recipients followed at Shiraz Transplant Center between 2011 and 2018 with a confirmed diagnosis of recurrence of underlying disease in our study. We reviewed medical records and extracted data on demographic characteristics, clinical and paraclinical features, medication use, and current status. We used a systematic random sampling method to select a control group of 95 transplant recipients who did not have recurrence. Of 3022 total transplant recipients, 76 recipients experienced a recurrence of their underlying disease., Results: Model for End-Stage Liver Disease score, underlying disease, recipient blood group, donor sex, donor blood group, and rejection frequency were significantly different between study groups with and without recurrence of underlying diseases. Liver transplant recipients with recurrence had lower mean Model for End-Stage Liver Disease score. Recipients with recurrence also had higher rate of drug consumption (eg, prednisolone, tacrolimus, mycophenolate mofetil, sirolimus). Regression analysis showed that donor sex and rejection frequency had an effect on disease recurrence. Death occurred more frequently in liver transplant recipients with recurrence than in the control group (39.5% vs 26.3%), butthe difference was not significant., Conclusions: Donor sex and acute rejection frequency are independent factors predictive of the recurrence of underlying disease. Modifying risk factors can help minimize the recurrence of underlying diseases after liver transplant.

Background: Splenic cysts are quite rare and asymptomatic. They may result from infection by a parasite, especially Echinococcus granulosus (hydatid cyst), or from non-parasitic causes. Since primary splenic cysts are not common, simple cysts can be misdiagnosed with a hydatid cyst in endemic areas., Case Presentation: We reported a 14-year-old Iranian girl initially presented with a vague abdominal pain, which progressed to left shoulder pain, fullness, early satiety, and shortness of breath and remained undiagnosed for 7 months despite seeking medical attention. Finally, imaging revealed a massive splenic cyst measuring 220 mm × 150 mm × 160 mm raising concern for a hydatid cyst due to regional endemicity. Consequently, the patient underwent total splenectomy. However, histopathological examination surprisingly revealed a simple non-parasitic cyst., Conclusions: Detecting rare simple spleen cysts requires early ultrasonography (US) and careful reassessment of diagnoses for non-responsive or worsening symptoms. Distinguishing them from splenic hydatidosis, especially in endemic areas, demands thorough paraclinical evaluations and patient history regarding potential parasitic exposure. While total splenectomy is the primary treatment for these huge cysts, the optimal surgical approach should be tailored case by case. These insights emphasize a comprehensive diagnostic approach to enhance accuracy and optimize patient care for these uncommon cysts., (© 2023. The Author(s).)