Evaluating the Association between Anemia and the Severity of Liver Disease in Children with Cirrhosis: A Cross-Sectional Study from 2015 to 2020.

Purpose: Liver cirrhosis is a major cause of hospital admission and mortality among children. Understanding the factors that influence disease severity is essential for preventing and reducing mortality. This study explored the association between hemoglobin levels and liver disease severity in children with cirrhosis.

Mutation spectrum of Tyrosinemia type I in Iran, A retrospective cohort study.

Hereditary Tyrosinemia Type 1 (HT1) is a genetic disorder characterized by an autosomal recessive inheritance pattern, caused by mutations in the fumarylacetoacetate hydrolase (FAH) gene, which results in a deficiency of fumarylacetoacetase. In our study, we identified a total of 15 mutations, including 12 newly discovered and 3 previously reported pathogenic mutations, in a cohort of 19 Iranian patients with the acute form of HT1. Out of the 12 novel variants identified, 11 were missense variants: p.

Comparing body composition measures in children with end stage liver disease using noninvasive bioimpedance analysis.

Background: Chronic liver disease (CLD) in children, often leads to cirrhosis and end-stage liver disease (ESLD). CLD poses significant challenges in management and prognosis. Assessing body composition, including sarcopenia, is increasingly recognized as important in understanding outcomes in this population.

Impact of sarcopenia on clinical outcomes in pediatric chronic liver disease post-liver transplantation: prevalence and implications.

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.

Clinical Findings, Bacterial Agents, and Antibiotic Resistance in Children with Spontaneous Peritonitis in Southern Iran: An Academic Tertiary Referral Center's Experience.

Background: Spontaneous bacterial peritonitis (SBP) is a fatal complication of ascites fluid infection. The causes of SBP in children differ from those in adults, and these bacteria are frequently resistant to antibiotics. Therefore, this study investigated the clinical findings, bacterial etiology, and antimicrobial resistance in children with SBP.

Comparison of Mir122 expression in children with biliary atresia and healthy group.

Biliary atresia (BA) is the primary cause of neonatal jaundice with various pathological mechanisms. Many BA patients may experience progressive liver dysfunction and eventually need a liver transplant. Therefore, identifying potential non-invasive biomarkers for BA is crucial.

A Comparison of the Effect of Kegel Exercises and Conventional Therapy versus Conventional Therapy Alone in the Treatment of Functional Constipation in Children: A Randomized Clinical Trial.

Background: There have been few studies on the effect of Kegel exercises on the treatment of functional constipation in children. Hence, the present study investigated the add-on role of Kegel exercises in children with functional constipation.

Methods: This clinical trial was conducted on children with functional constipation, according to Rome IV, who were referred to the pediatric department of Imam Reza Clinic (Shiraz, Iran) in 2022.

Cohort Profile: Shiraz Pediatric Liver Cirrhosis Cohort (SPLCCS).

Liver diseases in children and adolescents are a significant and arising public health issue and should be surveyed from different dimensions (clinical and para-clinical, psychological, socio-economic) and in diverse populations. Shiraz Liver Transplant Center, Shiraz, Iran is the only center for pediatric liver transplantation and its pre-operative evaluations. This provides a unique and valuable situation for studying this vulnerable population.

Relapse Rate of Clinical Symptoms After Stopping Treatment in Children with Cyclic Vomiting Syndrome.

Cyclic vomiting syndrome (CVS) is a chronic functional gastrointestinal disorder. It is characterized by recurrent episodes of vomiting typically separated by periods of symptom-free or baseline health. The present study aimed at evaluating the effectiveness of propranolol and the relapse rate of clinical symptoms after stopping treatment in children suffering from CVS.

Large Volume Paracentesis in Patients with Liver Cirrhosis Temporarily Diminishes Blood Cell Count.

Background: Large-volume paracentesis is the preferred treatment for patients with severe and refractory ascites. Several complications were reported during therapeutical paracentesis. However, there are very few published studies on the change in blood cell count after paracentesis.

Evaluation of Outcomes and Complications of Large Volume Paracentesis without Albumin and Coagulopathy Therapy in Pediatrics with Severe Ascites.

Background: Large-volume paracentesis has become the first treatment choice for patients with severe and refractory ascites. The studies have reported several complications after therapeutic paracentesis. But there are few published data on the complications with or without Albumin therapy.

Chylous ascites as a rare complication of abdominal trauma in a 7-year-old girl: A case report.

Key Clinical Message: Abdominal trauma can be one of the causes of chylous ascites in pediatric cases, along with tuberculosis and malignancy. However, a definitive diagnosis is more reasonable to be done by excluding other causes.

Abstract: Chylous ascites (CA) is a rare type of ascites.

Expression of miR-let7b and miR-19b in progressive familial intrahepatic cholestasis (PFIC) children.

Background: MicroRNAs (miRNAs) are a group of small non-coding RNAs that bind to the target mRNA and regulate gene expression. Recently circulating microRNAs were investigated as markers of diseases and therapeutic targets. Although various studies analyze the miRNA expression in liver disease, these studies on PFIC are few.

Identification of a novel mutation in the gene in hereditary fructose intolerance.

Objectives: Hereditary fructose intolerance (HFI) is caused by aldolase B enzyme deficiency. There has been no report about HFI from Iran and the type of mutations has not been reported in the Iranian population so far.

Case Presentation: Herein we report a 2 year old girl presented with failure to thrive, hepatomegaly, and liver dysfunction.

Extra-intestinal manifestations of Celiac disease in children: their prevalence and association with human leukocyte antigens and pathological and laboratory evaluations.

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.

Aplastic anemia: a new complication in the recent mysterious hepatitis outbreak among children worldwide: two case reports.

Background: Recently, an unknown hepatitis outbreak among children has concerned many individuals worldwide. These cases are frequently reported, mainly from Europe and other countries. In this study, we present two similar patients, who, to the best of our knowledge, are the first cases reported in the Middle East (Shiraz, Fars Province, Iran).

Rosa damascena together with brown sugar mitigate functional constipation in children over 12 months old: A double-blind randomized controlled trial.

Ethnopharmacological Relevance: Rosa × damascena Herrm., known as damask rose, is a bushy shrub that is found abundantly in Fars province, Iran. This species has been used in Iranian traditional practices for the treatment of abdominal pain and constipation, as gastrointestinal diseases.

An evaluation of ascitic calprotectin for diagnosis of ascitic fluid infection in children with cirrhosis.

Background: The most common infection in children with the hepatic disease with or without cirrhotic ascites is spontaneous bacterial peritonitis (SBP), which occurs in the absence of an evident intra-abdominal source of infection. The present study aims to assess the value of calprotectin in ascitic fluid in the diagnosis of ascitic fluid infection in children with liver cirrhosis.

Materials And Methods: In this cross-section study, 80 children with underlying liver disease who attended the Hepatology and Emergency Department in Shiraz University Hospitals were studied.

Comparison of lymphocyte-to-monocyte ratio with Child-Pugh and PELD/MELD scores to predict the outcome of children with cirrhosis.

Aim Of The Study: Prognostic scores are highly needed to properly manage children with cirrhosis and improve their clinical outcomes. The relationship between lymphocyte-to-monocyte ratio (LMR) at the time of admission to hospital and outcome of cirrhosis has been studied in adults, but to the best of our knowledge, there is no study regarding its utility as a prognostic marker of poor outcome in children with cirrhosis. Thus, this study aimed to investigate the potential prognostic value of LMR in such patients.

Effect of COVID-19 on Kawasaki Disease: Decrease Age of Onset and Increase Skin Manifestation.

Background: Kawasaki Disease (KD) is the most common childhood vasculitis and cause of acquired heart disease for no apparent reason. There is some evidence indicating infectious agents as possible triggers for KD. During the COVID-19 pandemic, vasculitis has been a presentation of COVID-19 in children.

Different Clinical Features of Celiac Disease in Children, Adolescents, and Adults; a Cross-sectional Study.

BACKGROUND Celiac disease is a common disorder but there are few studies comparing the clinical features of the disease in adults, adolescents and children. METHODS Demographic and clinical characteristics of all patients with celiac disease referred to the Celiac Clinic were evaluated and compared in different age groups. RESULTS Of 3416 participants, 473 patients were included.

Determination of allograft fibrosis by measurement of liver stiffness using transient elastography in children after liver transplantation at Shiraz Organ Transplant Center.

Aim: The aim of this study was to determine allograft fibrosis by measuring LS using TE in children after liver transplantation at Shiraz Organ Transplant Center.

Background: Liver stiffness (LS) assessment using fibro-scanning (transient elastography-TE) is a non-invasive method for evaluating liver fibrosis.

Methods: All children undergoing liver transplant from 2012 to 2016 were included in the study.