Masoud Tahani

A low Apgar score may indicate a higher risk of neonatal mortality and adverse neurological outcomes in the years following birth. It plays an essential role in diagnosing asphyxia, which can lead to hypoxic-ischemic liver injury. Objective. To explore the association between Apgar scores and liver marker variations in newborns. Patients and methods. In this study, 50 newborns with Apgar scores below 7, admitted to the Neonatal Intensive Care Unit (NICU), were selected alongside a control group of 50 newborns with Apgar scores above 7. Liver markers, including aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), lactate dehydrogenase (LDH), albumin, and total protein levels, were recorded on the first and fourth days after birth. The average liver marker levels between low and normal Apgar groups were compared using a statistical software package version 26. Results. Newborns with low Apgar scores at birth were found to have significantly higher levels of AST, ALP, albumin, total protein, and LDH in serum compared to those with normal Apgar scores (p < 0.05). However, there was no significant difference in ALT levels between two groups (p = 0.460). By the fourth day, all liver markers showed a significant decrease from birth; however, the average levels of AST, ALP, albumin, and LDH remained higher in newborns with abnormal Apgar scores (p < 0.05). Conclusion. This study underscores the prospective significance of Apgar score as an early marker of probable liver failure, which may have clinical ramifications for initial screening and prompt management in neonates with low scores. Key words: Apgar score, liver diseases, infants, perinatology, biomarkers, asphyxia

Achalasia is a primary esophageal motility disorder that impairs the relaxation of the lower esophageal sphincter. Objective. To evaluate the diagnostic and therapeutic approaches to the treatment of achalasia in children at Namazi Hospital, to assess the efficacy of different treatment modalities used at the center, and to explore whether there is a need to introduce peroral endoscopic myotomy (POEM) in pediatric practice. Patients and methods. This retrospective cross-sectional study included children diagnosed with achalasia between 2016 and 2023 at Namazi Hospital. Patients were selected from the hospital’s archives using the ICD-10 code (K22.0). Data on demographic characteristics, diagnostic findings, and therapeutic interventions were collected. Results. A total of 41 patients were included in the study, with a mean age of 7.17 (±5.41) years at the initial presentation. Among these patients, 36.6% were female and 63.4% were male. Significant differences were observed in the age of onset between children with and without failure to thrive (FTT) (p = 0.006). However, there were no significant differences in the duration of the disease prior to diagnosis (p = 0.839) or in the change in Eckardt score (p = 0.095). The Wilcoxon signed-rank test showed a significant reduction in Eckardt scores following an intervention, with a Z-value of -5.53 and a p-value of less than 0.001. The Mann–Whitney U test indicated no significant difference in Eckardt scores before the intervention between both genders (p = 0.524). Conclusion. The study revealed that earlier onset of achalasia is associated with FTT, and interventions significantly reduce Eckardt scores. No significant gender differences were observed in Eckardt scores prior to treatment. Further research with a larger sample and a longitudinal approach is recommended to expand these findings. Key words: achalasia, Heller myotomy, Eckardt score, pediatrics

Dehghani SM, et al. Pediatr Gastroenterol Hepatol Nutr. 2025 May;28(3):199-200. https://doi.org/10.5223/pghn.2025.28.3.199

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. Methods: In 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. Results: The 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. Conclusion: The 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.

Background: There are ongoing concerns about the safety of endoscopic retrograde cholangiopancreatography (ERCP) in children, as recorded cases have shown complications. Objectives: The objective of this study is to investigate the indications, complications, and outcomes of ERCP in pediatric patients. Methods: This 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. Results: During 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%. Conclusions: The infrequent occurrence of failures and complications highlights the substantial benefit of ERCP in treating biliary and pancreatic illnesses in children.

Background: Endoscopic retrograde cholangiopancreatography (ERCP), a standard procedure used for diagnosing and treating pancreaticobiliary disorders, has the highest rate of complications among endoscopic procedures. We aimed to evaluate the association of post-ERCP pain onset and its severity with the development of acute complications. Methods: This cross-sectional study included 172 candidates for ERCP who were referred to Namazi Hospital, Shiraz, from January 21, 2021, to January 21, 2022. Demographic features of the participants, including age and gender, were recorded. ERCP indications, complications during and after ERCP, and narcotic requirements were also noted. Post-ERCP pain severity was evaluated using a 10-point visual analogue scale (VAS), with 0 indicating no pain and 10 indicating the worst pain. Pain severity was evaluated twice: once by the physician and once by the patient. The interval between the procedure and the onset of pain was also recorded. Results: Out of the 172 participants of this study with a mean age of 53.77 ± 20.20 years, 98 (57%) were male. The most typical indication of ERCP was common bile duct stone (36%). Complications during and after ERCP occurred in 2.3% and 2.9%, respectively, with retroperitoneal/sphincterotomy perforation (1.2%) being the most common post-ERCP complication. Post-ERCP pain score (both by patient and physician) was significantly higher in patients with complications compared to those without complications (P < 0.001). The interval between the ERCP procedure and the onset of pain was significantly shorter in patients with post-ERCP complications (P = 0.003). Also, a significantly higher percentage of patients with complications required narcotics (40% vs 1.2%, P = 0.004). Conclusions: Although the presence of post-ERCP pain may not necessarily be indicative of complications, post-ERCP pain severity and onset, as well as narcotic requirement, appear to be associated with the development of post-ERCP complications.

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. Objectives: The aim of the present study is to compare the lipid profile in liver transplant recipients from living-related (LR) and deceased donors (DDs). Methods: This 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. Results: A 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. Conclusions: Patients who received a liver from an LR donor have a significantly lower TG, higher HDL, and a lower cardiovascular risk.

Dehghani SM, et al. Pediatr Gastroenterol Hepatol Nutr. 2024 Sep;27(5):286-297. https://doi.org/10.5223/pghn.2024.27.5.286

Introduction: COVID-19 is an epidemic viral infection that has especially caused great concern for those with chronic diseases. Few studies have been conducted in Asian countries on the prevalence and severity of COVID-19 complications among celiac patients. This study aimed to determine the frequency, severity, and prevalence of COVID-19 complications among celiac patients in Sistan region. Methods: In this descriptive cross-sectional study, a total of 200 patients including 40 celiac patients and 160 matched controls were studied from September to October 2021. All celiac patients in our registry were contacted and a detailed questionnaire was administered to collect required data. In addition, polymerase chain reaction (PCR) was performed on patients to detect COVID-19. Recorded variables were compared between celiac patients and controls using t-test and Chi-square test. Statistical analysis was carried out using Stata 14 software. Results: Out of 200 patients, 25% of celiac patients and 18.13% of controls were infected with COVID-19. Additionally, 60% of COVID-19 infected celiac patients had severe gastrointestinal symptoms and none were hospitalized, while 31% of COVID-19 infected controls had gastrointestinal symptoms and 3.45% were hospitalized. The frequency of Marsh score above 2 was significantly lower among COVID-19 infected celiac patients compared with uninfected celiac patients. Conclusion: The presence of celiac disease posed no additional risk for COVID-19 infection in terms of prevalence and frequency. However, in comparison to the general population, celiac patients experienced more severe gastrointestinal complications when infected with COVID-19.

Background: The 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. Methods: The 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. Results: Out 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. Conclusion: The 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.