iraj shahramian
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Introduction
Glycogen is the stored form of glucose and acts as a buffer to regulate glucose levels in all tissues. Various defects in glucose and glycogen metabolism have been discovered, all resulting from mutations in genes responsible for the expression of proteins involved in the synthesis, degradation, or modulation of glycogen. These defects are known as glycogen storage diseases (GSDs). In this study, we investigated the clinical and paraclinical findings in patients with GSD.
MethodsIn this cross-sectional survey, 140 patients with GSD who were referred to the Department of Pediatric Gastroenterology at Shiraz University of Medical Sciences from 2016 to 2022 were studied. The inclusion criteria required a definitive diagnosis of GSD. Data were collected using a researcher-designed questionnaire.
ResultsThe mean age of the participants was 7.8±5.5 years. The male-to-female ratio was 1.12:1. The most common clinical finding among the patients was hepatomegaly, observed in 80.7% of cases. The mean levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), and creatine phosphokinase (CPK) were 263.9±416.8, 193.1±215.5, 652.4±372.9, and 456.6±925.8, respectively. Sonography was performed on 77.1% of the patients, revealing that 87.9% had hepatomegaly or splenomegaly. There was a significant difference between the mean age of patients with normal ultrasound results (6.47 years) and the mean age of those with hepatosplenomegaly (11.54 years) (P=0.036). Among the patients who underwent liver biopsy, only 5.4% (3 out of 55) had normal liver tissue.
ConclusionThe findings of this study indicate that it involves all kinds of diseases. Types of GSD that affect the liver are more common than the types that affect skeletal muscles. Additionally, the serum levels of CPK and also the possibility of hepatosplenomegaly increase with age. In addition, it can be concluded that ultrasound is not a reliable method for diagnosing this disease in children, and it is better to use biopsy as a diagnostic method.
Keywords: Children, Glycogen Storage Disease, Hepatomegaly, Biopsy, Enzymes, Liver -
Govaresh, Volume:29 Issue: 4, Winter 2025, PP 187 -192
Gastrointestinal procedures, ranging from diagnostic to therapeutic, present numerous ethical challenges due to their invasive nature and the complex circumstances in which they are applied. This article explores the ethical principles related to various gastrointestinal procedures, including endoscopy, colonoscopy, endoscopic ultrasound (EUS), capsule endoscopy, enteroscopy, ERCP( Endoscopic Retrograde Cholangiopancreatography), and manometry. Key topics discussed include informed consent, patient autonomy, risk-benefit assessment, and equitable access. With advancements like artificial intelligence and telemedicine, new ethical dilemmas have emerged, necessitating continuous updates to ethical guidelines. This paper aims to provide a comprehensive review of the ethical aspects of these procedures and offer practical recommendations for physicians to ensure patient care.
Keywords: Medical Ethics, Informed Consent, Patient Privacy, Patient Autonomy, Endoscopy -
Govaresh, Volume:29 Issue: 3, Autumn 2024, PP 167 -174Background
In light of the relatively low prevalence of epidermolysis bullosa and the absence of research focused on gastrointestinal complications in Iran, our study sought to address this gap. Given the limited body of knowledge in this particular area, there is a crucial need to explore and identify gastrointestinal complications in patients with epidermolysis bullosa and understand the associated factors.
Materials and MethodsThe present study was conducted cross-sectionally. 67 patients with epidermolysis bullosa were selected by census and included in the study. Demographic information of patients, including age and sex, was recorded. The patients were fully examined by a gastroenterology specialist, and a history was taken for gastrointestinal diseases. The data were statistically analyzed using the SPSS software, version 28.
Results67 individuals with epidermolysis bullosa were evaluated for this investigation. The patients had simplex (23.9%), dystrophic (29.9%), junctional (4.5%), and unknown (41.8%) varieties of epidermolysis bullosa. Halitosis (64.2%), constipation (59.7%), food stuck in the throat (56.7%), mouth ulcers (52.2%), flatulence (50.7%), stomach discomfort and unpleasant taste in the mouth (47.8%), difficult swallowing (44.8%), and reflux (37.3%) were the most frequent gastrointestinal problems among these individuals.
ConclusionThe current study found that gastrointestinal issues are widespread in individuals with epidermolysis bullosa; hence, it is advised that suitable preventative and therapeutic measures be implemented, as well as multispecialty therapy.
Keywords: Epidermolysis Bullosa, Gastrointestinal Complications, EB, Dermatology -
Background
The occurrence of specific mutations within the Hepatitis B virus (HBV) genome is associated with the progression of chronic hepatitis B infection towards more severe outcomes.
ObjectivesThis study aimed to investigate mutational patterns in the X -gene and their influence on the outcome of chronic HBV infection (CHB) across three generations in a family.
MethodsNinety CHB patients, meeting the inclusion criteria, were recruited from cases referred to the Center of Hepatology at Golestan University of Medical Sciences between September 2020 and January 2021. The HBx gene was amplified using semi-nested PCR from serum samples and then subjected to sequencing.
ResultsA comparison of the sequences from CHB patients indicated that children and mothers in the two-generation group exhibited the highest similarity (79.3%) in the X -gene, with the lowest mutation rate (20.7%). The N-terminal region of the X -gene showed the highest mutation frequency in the three-generation group, including C1491G (25%), G1613T (23.9%), C1500T (43.4%), and G1658T (33.4%). The mutation rate was notably higher in HBeAg -negative patients across the three groups compared to HBe- Ag-positive CHB patients, with a statistically significant difference (P = 0.03). A1762T/G1764A mutations were observed in 15.6% of patients, and their presence showed a significant difference (P = 0.03). Additionally, in the three-generation group, a silent mutation (A1727G, 10%) and a missense mutation (A1727T, 30%) were detected.
ConclusionsSpecific mutational patterns in the HBx gene may be valuable in predicting clinical outcomes in CHB patients and could serve as warning indicators for increased susceptibility to hepatocellular carcinoma (HCC).
Keywords: Hepatitis B-Virus, X Protein, Mutation, Three Generations Group -
BackgroundThis study aimed to determine the electroencephalogram (EEG) findings in children with hepatic cirrhosis, which occurred without clinical encephalopathy.MethodsThe present study was conducted in an observational-analytical way at Amir-al-momenin Zabol Hospital, Zabol University of Medical Sciences, Iran. In this study, 50 children with hepatic cirrhosis without encephalopathy symptoms and 50 healthy children were evaluated and studied regarding abnormal findings in EEG. Finally, the data were analyzed using SPSS V22 software.ResultsThe mean and standard deviation of the age of the studied population was 57.6 ± 76.17 months. Out of a total of 50 children with hepatic cirrhosis, 21 children (42%) had abnormal findings in EEG, while none of the children in the healthy group had abnormal findings in EEG. There was a significant relationship between abnormal EEG findings and older age (P=0.001), underlying autoimmune hepatitis disease (P=0.011), and abnormal (increased) serum levels of Alanineamino Transferase (ALT) (P=0.030) and aspartate amino transferase (AST) (P=0.010) enzymes. Children with cirrhosis who had abnormal EEG findings had a higher average Pediatric End-Stage Liver Disease (PELD) score (18.1 ± 4.1) than patients with normal EEG findings (17.2 ± 3.7), but these findings were not statistically significant and noticeable (P=0.073). The sensitivity of EEG for predicting the severity of cirrhosis was estimated to be 70% and its specificity was 65%.ConclusionThe results of the present study indicated that the higher sensitivity of EEG compared to the specificity in predicting the severity of cirrhosis indicates that EEG is more useful to rule out severe cirrhosis or to screen cirrhosis patients at risk of deterioration than to confirm its diagnosis.Keywords: Child, Electroencephalography, Hepatic Cirrhosis
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Govaresh, Volume:29 Issue: 2, Summer 2024, PP 120 -123
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a liver disease. This is a chronic autoimmune disease, which can worsen over time and recur periodically. If left untreated, biliary cirrhosis may lead to liver failure. Considering that PBC is a rare disease in all age groups, especially in children, the purpose of this report was to describe a girl with hepatitis A virus (HAV) who was then diagnosed with PBC. This child is the first case reported in Iran. The patient is a 7-year-old girl with a pale complexion and eyes with a yellowish cross who was referred to the doctor. Considering that this patient had hepatitis, a blood test was requested to check the level of cholesterol and liver enzymes. The absence of cholestatic liver enzymes was observed in tests. Then, an anti-mitochondrial (AMA) test was requested for the patient, the result of which was negative. Finally, with imaging and biopsy, the diagnosis of PBC was confirmed for the patient. After the definite diagnosis of the disease, the child was treated with ursodeoxycholic acid (UDCA). The child in question is suffering from two medical and immunological diseases, HAV and PBC. Since this child was first infected with HAV, it is possible that the cause of PBC was HAV.
Keywords: Primary Biliary Cholangitis, Hepatitis, Biliary Cirrhosis -
Background
There are ongoing concerns about the safety of endoscopic retrograde cholangiopancreatography (ERCP) in children, as recorded cases have shown complications.
ObjectivesThe objective of this study is to investigate the indications, complications, and outcomes of ERCP in pediatric patients.
MethodsThis cross-sectional study was conducted at Namazi and Abu Ali Sina Hospitals between September 2022 and December 2023. The study focused on pediatric patients aged 1 month to 18 years undergoing ERCP. The demographics, indications for ERCP, pre-procedural imaging findings, and complications within 72 hours and up to 6 months after ERCP were extracted from medical records.
ResultsDuring the study period, a total of 100 ERCP procedures were performed on 56 pediatric patients. The mean age of the patients was 10.22 ± 4.80 years, with females accounting for 53.6% of the group. The main indications for performing ERCP were the presence of a stricture after orthotopic liver transplantation (22%) and the presence of a common bile duct stone (19%). The incidence of complications was notable for acute pancreatitis (3%) and bleeding (2%), while the failure rate for ERCP operations was determined to be 4%.
ConclusionsThe infrequent occurrence of failures and complications highlights the substantial benefit of ERCP in treating biliary and pancreatic illnesses in children.
Keywords: ERCP, MRCP, Pediatrics, Pancreatitis, Biliary Stone -
Background
Liver transplantation is a critical treatment option for end-stage liver disease, albeit associated with potential complications. Among the most common post-transplant complications are biliary issues, including leaks, strictures, and obstructions. Endoscopic retrograde cholangiopancreatography (ERCP) is frequently employed to manage these complications despite its inherent risks. While the complications of ERCP in the general population are well-documented, specific risks and protective factors for liver transplant recipients have not been extensively studied.
Materials and MethodsWe conducted a systematic review and meta-analysis in accordance with PRISMA guidelines. In order to find relevant studies published between 1/1/2011 and 1/3/2023, two independent researchers conducted searches (MT, I SH). A literature search of English language publications was conducted using MEDLINE through PubMed, EMBASETM through Ovid, the Cochrane Library, and Trip. In addition to Magiran and SID, we searched KoreaMed and LILACS for literature published in other languages. Endoscopic retrograde cholangiopancreatography OR ERCP, OR liver Transplantation, OR Complication are terms used in the search strategy. The Newcastle-Ottawa Scale (NOS) was used to assess the quality of the studies, and data were independently extracted by two researchers.
ResultsA total of 274 studies were selected. After analyzing their correspondence with the required criteria, a final review of eleven studies was conducted. Our meta-analysis identified several risk and protective factors for complications following ERCP in liver transplant patients. Protective factors included male sex and intraoperative stenting, which were associated with reduced complications. Conversely, high serum bilirubin and creatinine levels and a history of hepatitis B were found to increase the likelihood of complications or failure after ERCP. Factors such as age, history of diabetes, serum Alanine Aminotransferase (ALT) level, steroid administration, Mammalian Target of Rapamycin (mTOR) inhibitor use, and sphincterotomy did not significantly impact ERCP outcomes and complications.
ConclusionThis systematic review and meta-analysis provide valuable insights into the risk and protective factors associated with complications following ERCP in liver transplant recipients. These findings can inform clinical decision-making and guide healthcare providers in optimizing the management of post-transplant biliary complications.
Keywords: Liver Transplantation, Endoscopic Retrograde Cholangiopancreatography (ERCP), Complications, Risk Factors, Systematic Review -
With Currently, Endoscopic retrograde cholangiopancreatography (ERCP) has evolved as a therapeutic intervention for treating disorders related to the biliary system and plays an important role in treating benign and malignant strictures of the biliary system.
The placement of biliary stents is a frequent and useful procedure to decompress the biliary system, and endoscopic biliary stenting seems relatively simple. However, in some cases, it can lead to several complications. Distal or proximal migration of endoscopically placed biliary stents is one of the known complications of ERCP and can affect up to 6-10% of patients.
Several factors, including those related to the patient, endoscopy, or stent, are involved in stent migration. When migration occurs, it is necessary to remove or replace endoprosthesis to prevent recurrent biliary complications and more severe consequences such as cholangitis and sepsis.
This review explores the frequency, etiology, risk factors, complications, and management strategies pertaining to biliary stent migration, consolidating diverse management approaches for informed decision-making.Keywords: Biliary Stent, Stent Migration, Bile Ducts, Literature Review, ERCP -
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.
MethodsThis study was conducted on all new pediatric ascites patients, who were admitted to the Department of Pediatric Gastroenterology, Namazi Hospital, affiliated with Shiraz University of Medical Sciences (Shiraz, Iran) from 2021 to 2022. Required data such as demographic information, and clinical information such as complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Gram staining, blood culture by Automated Blood Culture System (BACTEC), and antibiogram of ascites fluids by disc diffusion method were all collected. Finally, the data were statistically analyzed using SPSS Software (version 26). Besides, the t test, Fisher’s exact, Mann-Whitney, and Chi square tests were used for data analysis. In all tests, P≤0.05 was considered statistically significant.
ResultsThe present study examined 62 children with ascites of which 18 (29%) had SBP. The median (IQR) age was 2.5 (8.1) years. Thirty-four (54.8%) of the participants were girls. Abdominal pain was the most common clinical manifestation in patients (54%), and there was a significant association between abdominal pain and SBP (P=0.02). In 12 positive ascites fluid cultures, coagulase-negative staphylococci had the highest frequency (25%), followed by Escherichia coli (16.7%). Third-generation cephalosporins had a 25% sensitivity in the total positive cultures. This sensitivity was 33.3% for Gram-negative cultures and 16.6% for Gram-positive cultures.
ConclusionAlthough third-generation cephalosporins are recommended as the primary antibiotic for the empirical treatment of SBP, the present study found high bacterial resistance. Finally, empirical therapy should be tailored to each region’s bacterial resistance features.
Keywords: Peritonitis, Ascites, Liver Cirrhosis, Drug Resistance -
Govaresh, Volume:28 Issue: 4, Winter 2024, PP 261 -267Background
Celiac disease (CD) is an enteropathy associated with a genetic disorder and several constitutive genes. This study examines the genes and factors affecting CD.
Materials and MethodsThis review was conducted on structured findings up to September 2023 regardless of language published according to the protocol of systematic review articles (PRISMA). To identify relevant studies, online searches were generally conducted in PubMed, Google Scholar, MEDLINE, and SCOPUS databases.
ResultsMany studies have been conducted on CD genes, and researchers have achieved good results. So far, 60 genomic loci related to CD have been discovered, which is the most important genetic loci of CD related to HLA(Human Leukocyte Antigen). Most of the gene loci identified in autoimmune diseases have pleiotropic effects and cause disruption of the immune system, which in turn causes CD.
ConclusionAccording to the linkage studies conducted on genetic regions, the most important genetic positions identified in CD are HLADQ2 and HLA-DQ8. Due to the progress of genetic science and the uncertainty of the genomic position of this disease, they have not been able to use genetic science to prevent this disease. Considering the common genes that this disease has with other gastrointestinal diseases as well as thalassemia, there may be newer and more effective genetic approaches to treat this disease in the future.
Keywords: Celiac disease, HLA, DQ2, DQ8, Genetics -
Background
Hepatitis A is a widespread viral infection with significant public health implications. Assessing glucose 6-phosphate dehydrogenase (G6PD) deficiency in hepatitis A patients is essential for various reasons, including prognosis, disease severity evaluation, encephalopathy risk identification, tailored management, and advancing scientific understanding. This study aimed to investigate the prevalence and clinical implications of G6PD impairment in individuals with fulminant hepatitis A.
MethodsA cross-sectional descriptive analysis was conducted, involving hospitalized patients with fulminant hepatitis A. Demographic data, prevalence rates, and clinical findings were recorded in a database. The diagnosis of hepatitis A infection was confirmed using an anti-HAV IgM antibody test, and G6PD enzyme activity was measured with a fluorescent spot assay.
ResultsOut of 81 patients with hepatitis A, 57 (70.4%) were males, and 24 (29.5%) were females, with an average age of 24.6 years. Dark yellow urine and anorexia were the most common clinical symptoms. Notably, 30 (37%) patients lacked G6PD. The group with G6PD deficiency showed significantly higher rates of encephalopathy and mortality (P<0.01), along with elevated bilirubin (P=0.00), abnormal coagulation parameters, and low hemoglobin levels (P=0.00).
ConclusionIn light of these findings, the present study proposes the implementation of routine G6PD level assessments and the evaluation of other relevant markers in regions where hepatitis A is endemic. Furthermore, the study underscores the need for vigilant monitoring of hemolysis and encephalopathy in affected patients to optimize clinical management and reduce morbidity and mortality associated with this condition.
Keywords: G6PD Deficiency, Hepatitis A, Fulminant Hepatitis -
BackgroundThere 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.MethodsThis 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. The sample consisted of 64 children who were randomly assigned to either the intervention or the control groups. In the control group, a pediatrician administered conventional therapy, including diet training, defecation training, and polyethylene glycol (PEG) syrup (0.7 g/Kg daily). In the treatment group, in addition to conventional therapy, a pediatrician taught Kegel exercises to the child both verbally and in writing in the presence of their parents. To investigate the effectiveness of the intervention, frequency of defecation, defecation time, assistance used for defecation, incomplete emptying, unsuccessful defecation, abdominal pain, and painful defecation were selected as the outcomes. Independent sample t test was used for continuous variables. Categorical variables were reported as frequency and percentages. To examine the difference in categorical outcome variables, Wilcoxon (pre and post), Chi square, and Fisher exact tests were used. Data were analyzed using SPSS software version 21. P<0.05 were considered statistically significant.ResultsTwenty-seven (88.4%) patients in the Kegel exercise group reported a defecation time of less than 5 min, while only 12 (37.5%) patients in the control group reached this time, and this difference was statistically significant (P=0.001). Moreover, patients in the treatment group showed significant improvements in terms of incomplete emptying of stool, unsuccessful defecation, abdominal pain, and painless defecation (P=0.001, P=0.001, P=0.001, P=0.037, respectively). After intervention, the use of laxatives, digits, or enemas to assist defecation was not significantly different between the groups (P=0.659).ConclusionKegel exercise was an effective adjunctive treatment for pediatric functional constipation.Trial registration number: IRCT20230424057984N1.Keywords: Exercise Therapy, Kegel Exercises, Constipation, Child
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Background
It has been shown that hyperlipidemia occurs in 71% of patients following liver transplantation. Multiple risk factors, including obesity, diabetes mellitus, and diet, as well as the immunosuppressive medications used, influence the lipid profiles that are seen in these individuals, contributing to the multifactorial etiology of lipid problems.
ObjectivesThe aim of the present study is to compare the lipid profile in liver transplant recipients from living-related (LR) and deceased donors (DDs).
MethodsThis is a retrospective cross-sectional study performed at Shiraz University of Medical Science between 2005 and 2018. Patients under 18 years old who received liver transplants were included in the study and divided into 2 groups who received from LR and DDs, and lipid profiles were compared between the 2 groups.
ResultsA total of 397 patients were included in the study; in the first group, 234 received a liver from a DD, and in the second group, 161 from an LR donor. The mean body mass index (BMI) was 17.51 ± 5.49 in the first group and 16.25 ± 3.29 in the second group. The most common underlying diseases were biliary atresia (22%) and autoimmune hepatitis (15%). The mean triglyceride (TG) and high-density lipoprotein (HDL) levels were 133 and 46 mg/dL in the first group and 118 and 54 mg/dL in the second group, while the differences were statistically significant. As age increased, there was a significant difference in the mean values of fasting blood sugar (FBS) and HDL, with FBS increasing and HDL decreasing. There was no significant difference in the use of immunosuppressant drugs between the 2 groups.
ConclusionsPatients who received a liver from an LR donor have a significantly lower TG, higher HDL, and a lower cardiovascular risk.
Keywords: Hyperlipidemia, Immune System, Lipid Disorders -
Background
Inflammatory bowel diseases (IBDs) are complex and multifaceted disorders characterized by recurrent and persistent intestinal inflammation. The incidence of inflammatory bowel disease (IBD) is on the rise among both children and adults worldwide. In this review, we provide an update on genomic studies of IBD, with a particular focus on Very Early-Onset IBD (VEO-IBD), which often presents with a more severe phenotype than IBD at an older age.
MethodsThe methods used in this systematic review were performed according to the guidelines of the PRISMA checklist. A search was conducted by two independent researchers in international databases (PubMed, Web of Science, Scopus, and Google Scholar) to find relevant studies published in English.
ResultsPatients with VEO-IBD have rare or novel genes associated with immunodeficiency that may play a role in the pathogenesis of the disease. To date, ten regions for 240 genes, which are usually monogenic, have been identified for this disease, mostly due to mutations. But the most important cause of VEO-IBD is mutation in interleukin 10. It has also been reported that VEO-IBD is associated with increased expression of S100A8 and S100A9 genes in rectal mucosa and serum.
ConclusionConsidering the multifactorial nature of IBD, all the changes that cause protein expression and function should be taken into account; so for early diagnosis and timely treatment of this disease, more extensive phenotypic sequencing is needed to discover new gene loci. And these children can be treated with hematopoietic stem cell transplantation, as the most efficient method.
Keywords: Genetics, Crohn' S Disease, Ulcerative Colitis, Early Inflammatory Disease -
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. We aimed to analyze the safety and complications of large-volume paracentesis in children with or without albumin therapy.
MethodsThis study was conducted on children with severe ascites with chronic liver disease who underwent large-volume paracentesis. They were divided into albumin-infused and albumin non-infused groups. In the case of coagulopathy, no adjustment was made. Albumin was not administered after the procedure. The outcomes were monitored to evaluate the complications. To compare two groups, a t-test was utilized, and the ANOVA test was used to compare several groups. If the requirements for using these tests were not met, Mann-Whitney and Kruskal-Wallis tests were applied.
ResultsDecreased heart rate was observed in all time intervals and was meaningful six days after paracentesis. MAP also decreased statistically at 48 hours and six days after the procedure (P < 0.05). Other variables did not show any meaningful change.
ConclusionChildren having tense ascites with thrombocytopenia, prolonged PT, Child-Pugh class C, and encephalopathy can undergo large-volume paracentesis without any complication. Albumin administration before the procedure in patients with low levels of Albumin (<2.9) can effectively overcome the problems of tachycardia and increased mean arterial pressure. There will be no need for Albumin administration after paracentesis.
Keywords: Paracentesis, Cirrhosis, Albumin, Children, Pediatric, Complications, Ascites -
Objective
Changes in endocrine, nervous, renal, cardiovascular, and respiratory systems during pregnancy have been studied, but changes in liver function have been poorly studied. Therefore, the purpose of this study was to investigate the trend of changes in liver enzymes in normal pregnancy.
Materials and methodsThis prospective longitudinal study included 68 pregnant women who were referred to the Obstetrics and Gynecology Clinic of Amiral Momenin Hospital in Zabol in 2021. In terms of the trimester of pregnancy, the presence of underlying diseases, history of previous pregnancies, disorders of the enzymes of recent patients, the patients were evaluated, and the information from the patients' files was analyzed.
ResultsThe average AST levels in pregnant women in the first, second, and third trimesters were 16.82, 17.47, and 18.00, respectively, which show that garlic consumption is increasing. The average PT in pregnant women decreased in the first, second, and third trimesters. The average direct and total bilirubin levels in pregnant women in the first and second trimesters showed a constant trend. The amount of total protein increased in pregnant women during the first, second, and third trimesters. In the second and third trimesters, the enzyme level was significantly higher in pregnant women than in nonpregnant women. The level of GGT in pregnant women in the first, second, and third trimesters showed a different trend.
ConclusionAccurate evaluation of patients, especially in the third trimester, is necessary from the point of view of increasing enzyme levels in other countries.
Keywords: Liver Function Tests, Pregnancy, Alanine Aminotransferase (ALT), Aspartate Aminotransferase(AST), Bilirubin -
BackgroundThe presence of autoantibodies is a prerequisite for the diagnosis of autoimmune hepatitis (AIH). However, most autoantibodies are not disease-specific, and serological overlap between AIH and other chronic liver diseases is common. Since the prognostic parameters of AIH are limited, this study aimed to investigate the relationship between histopathological findings on liver biopsy with different types of autoantibodies associated with AIH and how autoantibodies can predict the severity and extent of disease.MethodsThe present study was performed on 30 patients with a definite diagnosis of AIH according to the International Autoimmune Hepatitis Group (IAIHG) criteria. Pediatric AIH patients underwent liver tissue examinations at the time of diagnosis at accession, which confirmed characteristic histological changes. AIH-related serologic major and minor autoantibodies were measured using indirect immunofluorescence assays and ELISA kit (EUROIMMUN, Germany), respectively, and were compared within all patients, and the results were recorded. Finally, the obtained data were analyzed using SPSS V25 software.ResultsOut of 30 patients, 17 (56.66%) were female, and the age range of patients was 17-11 years (8.46 ± 6.95). Anti-nuclear antibody (ANA) (73.3%), smooth muscle antibody (SMA)-anti-smooth muscle actin antibody (ASMA) (70%), perinuclear anti- neutrophilic cytoplasmic antibodies (p-ANCA) (63%), and liver kidney microsomal (LKM) (43.3%) were the most common autoantibodies found in children with AIH. There was a significant relation between the severity of histological findings and the presence of LKM antibodies (P < 0.05). The highest sensitivity for predicting severe AIH based on histopathological findings was ANA autoantibody positivity and the presence of at least two primary autoantibodies (LKM and SMA-ASMA). On the other hand, positive LKM antibodies had the highest specificity and positive predictive value (PPV) in AIH severity prediction.ConclusionThe results of the present study suggested that there might be a significant correlation between the presence of primary LKM autoantibodies and biopsy results, so it can possibly act as an accurate autoantibody for predicting the severity of AIH, while other AIH-related autoantibodies did not seem to have a significant correlation with biochemical and histological findings.Keywords: Autoimmune hepatitis, Autoantibody, Serology, histology, children
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Background
Although the invention of more specialized duodenoscopes helped to develop Endoscopic Retrograde Cholangiopancreatography (ERCP) as a Therapeutic Method in children, there is a general perception that ERCP is practically challenging and dangerous in children. This has limited its widespread use in children. The aim of this meta-analysis is to update the previous reviews and evaluate more recent outcomes and complications of therapeutic ERCP among children with HPB disease.
MethodsA systematic literature search based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) was performed in Web of Science, Embase, PubMed, and Cochrane library databases to find and evaluate all articles regarding therapeutic ERCP in children with HPB diseases published from January 2017 to July 2022. The analysis included studies that evaluated patients less than 18 years old undergoing ERCP with the objective of HPB diseases, and had reported the success rate and side effects. The main outcome measures were the success and complication rate of therapeutic ERCP. This meta-analysis was performed using STATA version 16 (StataCorp LP, College Station, Texas).
ResultsA total of 10 articles consisting of 1924 ERCP, performed on 958 children, were included. Out of all ERCPs, 96% (95%CI: 96-97, I2:95.8%) were conducted with therapeutic goals. The overall success rate among therapeutic ERCPs was 91% (95CI:90-92%, I2:97.1%) ranging from 65% to 95% among different studies. An overall of 9% (95%CI: 8-10, I2:97.8%) of all therapeutic ERCPs were associated with different adverse effects such as postoperative pancreatitis (6%) and Hemorrhage (1%).
ConclusionOur analysis suggested ERCP as a safe therapeutic method for pediatric patients suffering HPB patients, having a success rate of 91% and a complication rate of 9%, none of which led to in-hospital mortality.
Keywords: Endoscopic Retrograde Cholangiopancreatography (ERCP), Success rate, Adverse Effect Rate, Pediatric -
Journal of Advances in Medical and Biomedical Research, Volume:31 Issue: 144, Jan-Feb 2023, PP 57 -63Background and Objective
Since the distribution of HBV genotypes in patients with hepatitis B indicates the predominant genotypes in specific geographical area, this study aimed to investigate the prevalence of S gene mutations in high-risk children with maternal HBV in southeastern Iran.
Materials and MethodsThis cross-sectional prospective study was performed from March 2019 to March 2020. We studied 26 patients infected with hepatitis B virus through vertical route. Serological markers of HBV were tested for HBs Ag, anti-HBs, HBe Ag, anti-HBe, and anti-HBc through ELISA. HBV-positive DNA-HBV samples were examined by nested PCR. The correlation between categorical variables was estimated using Spearmen correlation coefficient. All statistical analyses were performed using SPSS version 22 software.
ResultsOur study showed that no S gene mutation occurred in studied children. However, 4 (15.4%) of the children were HBe Ag positive, while the frequency of positive HBe antibody positive in the serum of children was 18 (69.2%).
ConclusionIn general, since different types of Pre-S / S variants are predominantly identified in patients with chronic HBV that can affect the progression of liver disease, it is therefore necessary to evaluate the types of Pre-S / S variants regularly in HBV. Carriers should be identified to help discover people at higher risk for liver disease. Our study, as a single center study in southeastern Iran, indicated no mutation in this gene in hepatitis B patients.
Keywords: Mutation, Hepatitis B, HBV, Children -
Background
Infection with hepatitis B, C, and delta viruses (HBV/HCV/HDV) caused by mother-to-child transmission (MTCT) is still a serious health problem worldwide. Coinfection with HBV/HDV or HCV has been shown to accelerate the progression of chronic HBV infection, resulting in higher mortality.
ObjectivesThe aim of this study was to evaluate the prevalence of anti-HCV, anti-HDV, and anti-HIV antibodies in mothers with chronic HBV infection and their infants.
MethodsThis cross-sectional study was performed on 100 mothers with chronic HBV (hepatitis B surface antigen-positive [HBsAg+]/hepatitis B core antibody-positive [HBcAb+]) and their infants between 2020 and 2021 in northeastern Iran. The presence of serological markers of HBV, anti-HCV, anti-HDV, and HIV antibodies, as well as the liver function tests, were evaluated in all mothers. The obtained data were analyzed using SPSS version 20, and the level of the statistical significance was set at a P-value < 0.05.
ResultsThe mean age in all individuals was 37.45 ± 17.95 years. In mothers with chronic HBV and their infants, 3, 11, and 15% were serologically positive for anti-HCV, HCV/HDV, and anti-HDV, respectively. Also, all participants were serologically negative for anti-HIV. A statistical analysis showed a significant correlation of anti-HDV (95% CI, 0.113 - 0.332) with education, place of residence, and anti-HCV (95% CI, 0.313 - 0.416) in mothers with chronic HBV. The percentages of mothers and infants with simultaneous infection HBV were reported to be 1, 2, and 1% when anti-HCV, anti-HDV, and anti-HCV/HDV antibodies were measured, respectively.
ConclusionsIntellectual strategies in the diagnosis of HCV, HIV, and HDV in patients with chronic HBV have challenged public and global health, and preventing viral infections in newly born infants is the most effective way to control the HBV/HCV or HDV epidemic.
Keywords: HIV, Coinfection, Hepatitis C Antibody, Chronic Hepatitis B -
Background
Coronavirus disease 2019 (COVID-19) is caused by an infection in the respiratory tract leading to extrapulmonary manifestations, including dysregulation of the immune system and hepatic injury.
ObjectivesGiven the high prevalence of viral hepatitis and a few studies carried out on severe acute respiratory syndrome coronavirus 2 and hepatitis B virus (HBV), this study investigated the impact of COVID-19 on chronic hepatitis B (CHB) patients in the northeast region of Iran.
MethodsIn this cross-sectional study, the blood samples were collected from 93 CHB patients registered in the Patient Detection Data Bank of Golestan University of Medical Sciences, Gorgan, Iran, and 62 healthy individuals as controls. Reverse transcription-polymerase chain reaction was adopted to detect COVID-19 infection in all the participants’ nasopharyngeal samples. All the participants were subjected to anti-hepatitis C virus, anti-hepatitis delta virus, and liver function tests. Then, HBV deoxyribonucleic acid load was detected in CHB patients. The collected data were analyzed by statistical tests using SPSS software (version 20). A P-value less than 0.05 was considered statistically significant.
ResultsIn this study, 14% (13/93) and 32.25% (20/62) of CHB patients and control individuals were infected with COVID-19, respectively. The mean age of CHB patients was 39.69 ± 19.58 years, and 71% of them were female. The risk of developing COVID-19 in healthy controls was observed to be 2.3 times higher than in patients with CHB (0.95% confidence interval: 1.242 - 4.290). On the other hand, the mean values of aspartate aminotransferase, alanine aminotransferase, and alkaline phosphatase in CHB patients superinfected with COVID-19 were higher than other participants. Out of 35.4% of patients with viral hepatitis B that were taking antiviral drugs, only 5.4% had COVID-19.
ConclusionsAlthough CHB infection did not predispose COVID-19 patients to more severe outcomes, the data of this study suggest that antiviral agents also decreased susceptibility to COVID-19 infection. Alternatively, careful assessment of hepatic manifestations and chronic viral hepatitis infections in COVID-19 patients can lead to more favorable health outcomes.
Keywords: Antiviral Agents, Coinfection, Hepatitis B, COVID-19 -
Background
Functional constipation (FC) is one of the most common complaints in childhood. The aim of this study was the evaluation of the quality of life in children with chronic FC and their family members.
MethodsIn this study, 100 children from 2-18 years of age (57 males, 43 females) with chronic FC were selected. The Child Health Questionnaire - Parent Form 50 questionnaire was used for quality of life assessment in children and their family members. Quality of life scores were compared with 100 healthy children from 2-18 years of age. The questionnaire consists of 15 domains, each of which ranges in score from zero to 100. A higher score represents a better quality of life.
ResultsQuality of life scores in all 15 domains were significantly lower in children with FC than in healthy children (P<0.001). Scores of physical functioning, behavior, and mental health were lower in children with FC and fecal incontinence compared to children with FC alone (P<0.05).
ConclusionFC impacts on quality of life of the affected children and their family members. Practitioners should be aware of its effects on quality of life to improve the disease course with early assessment and treatment.
Keywords: Constipation, Quality of life, Children -
زمینه و اهداف
جهش های مقاومت دارویی عناصر کلیدی در شکست درمان طولانی مدت عفونت های ویروس هپاتیت B (HBV) و ویروس نقص ایمنی انسانی (HIV) هستند. جهش در موتیف YMDD در ژن P ویروس HBV مهمترین عامل در مقاومت دارویی ضد ویروسی (به ویژه لامیوودین) است. این مطالعه با هدف ارزیابی موتیف YMDD و سایر جهش های ژن پلیمراز در افراد مبتلا به عفونت همزمان HBV و HIV انجام شد.
مواد و روش کارهمه بیماران وارد مطالعه شده تحت درمان با لامیوودین بودند. نمونه خون از 37 بیمار HBV/HIV مثبت جمع آوری شد و DNA استخراج شد. ژن P با روش PCR با پرایمرهای مناسب تکثیر شد. محصولات PCR برای تشخیص جهش در ژن P بهMacrogen (کره جنوبی) ارسال شد. برای بررسی جهش های ژن P، توالی های به دست آمده با ژن پلیمراز توالی استاندارد (Okamoto) HBV در بانک ژن (شماره دسترسیAB033559) مقایسه شدند.
یافته هامیانگین سنی بیماران 5/7±34/1 سال بود که 59/5 درصد آن ها مرد و 40/5 درصد زن بودند. از مجموع بیماران 56 درصد سوء مصرف کننده مواد، 35 درصد رفتارهای جنسی پرخطر، 56 درصد سابقه زندان و 33 درصد همسران معتاد داشتند. 37 نمونه استخراج شده با موفقیت تعیین توالی شدند. از بین نمونه های مورد مطالعه (37 نفر)، 28 بیمار دارای جهش همزمان YIDD و FLMAQ، 1 بیمار YINN و FLIPH و 1 بیمار YIDD و FSLAQ داشتند.
نتیجه گیریبه طور خلاصه، انواع مقاوم به دارو در اکثر بیماران مبتلا به هپاتیت مزمن (CHB) B و HIV قابل تشخیص است. در نتیجه جهش، راهبردهای درمانی گاهی موثر نیستند. بنابراین، شناسایی و نظارت بر جهش های مقاومت دارویی بسیار مهم است.
کلید واژگان: HBV، HIV، عفونت همزمان، جهش، ژن PBackground and ObjectiveThe drug resistance mutations are key elements in the failure of long-term treatment of Hepatitis B virus (HBV) and human immunodeficiency virus (HIV) infections. The mutation in the YMDD motif in the P gene of HBV is the most critical factor in antiviral drug (especially lamivudine) resistance. This study aimed to assess the YMDD motif and other polymerase gene mutations in individuals with HBV/HIV coinfection.
Materials and MethodsAll enrolled patients were under lamivudine treatment. Blood samples were collected from 37 HBV/HIV-positive patients, and DNA was extracted. The P gene was amplified by the PCR method with appropriate primers. The PCR products for detecting mutations in the P gene were sent to the Macrogen. To investigate the P gene mutations, the obtained sequences were compared with the polymerase gene of the HBV standard sequence in the GeneBank (accession number AB033559).
ResultsThe mean age of the patients was 34.1±5.7 years, of which 59.5% were male, and 40.5% were female. Of all patients, 56% were drug abusers, 35% had risky sexual behavior, 56% had prison history, and 33% had addicted wives. The 37 extracted samples were sequenced successfully. Among the studied samples (n =37), 28 patients had simultaneous mutations of YIDD and FLMAQ, 1 patient had YINN and FLIPH and 1 patient had YIDD and FSLAQ.
ConclusionIn summary, drug-resistant variants were detectable in most coinfected patients with chronic Hepatitis B (CHB) and HIV. As a result of mutations, therapeutic strategies sometimes are not effective. Therefore, recognition and monitoring of drug resistance mutations are critical.
Keywords: Coinfection, HBV, HIV, Lamivudine, Mutation, P gene
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