The association of pro-oxidant/antioxidant balance and blood parameters in patients with beta-thalassemia major: a cross-sectional study.

Background: Oxidative stress due to iron accumulation in patients with beta-thalassemia major (BTM) causes complications such as tissue damage and destruction. This study aimed to assess the association between the serum prooxidant/antioxidant balance (PAB) and blood parameters in patients with BTM. Methods: This cross-sectional study included 92 patients with BTM. In this study, PAB was measured using an enzyme-linked immunosorbent assay (ELISA). Serum ferritin, blood urea nitrogen (BUN), creatinine (Cr), alanine aminotransferase (ALT), aspartate aminotransferase (AST), thyroid-stimulating hormone (TSH), total cholesterol (TC), triglyceride (TG), complete blood cell count (CBC), and history of blood transfusion were recorded. The association of the blood parameters was assessed across the tertiles (T) of serum PAB (highest T vs. lowest T). Results: The results showed that high serum ferritin was directly associated with serum PAB [odds ratio (OR), 12.80; 95% confidence interval (CI), 2.98‒54.91; T3 vs. T1]. Also, direct associations were found for high TC (OR, 4.97; 95% CI, 1.42‒17.32; T3 vs. T1), high ALT (OR, 4.95; 95% CI, 1.33‒18.46; T3 vs. T1) and high TSH (OR, 3.78; 95% CI, 1.10‒13.02; T3 vs. T1). Conclusion: The findings of the present study showed that serum PAB levels were directly associated with ferritin, ALT, TC, and TSH levels. This indicates that improvements in blood parameters, especially ferritin and TSH levels, occur by ameliorating oxidative stress in patients with BTM.

Detection of HSV-1, HSV-2 and VZV Isolated from Cerebrospinal Fluid Samples of Children Suspected to Encephalitis.

Background: Encephalitis is an uncommon, serious brain infection that can cause fatality among children. The cause of most encephalitis remained unknown, yet viruses are the most well-known infectious agents that cause encephalitis. This study aimed to determine varicella-zoster virus (VZV) and herpes simplex virus type 1, 2 (HSV1/2) among individuals who are under 5 years of age in Iran. Materials and Methods: In this study, 149 cerebrospinal fluid samples of suspected patients of encephalitis were analyzed with some symptoms, such as seizure, fever, nausea loss of consciousness, and dizziness from Mofid Children's Hospital in Tehran, Iran. Then, the molecular evaluation of samples was performed using multiplex Polymerase Chain Reaction (PCR) for detecting HSV1/2 and VZV. Results: The mean age of the patients was 1.8 years. Also, 63.4% of children were male and 36.6% were female. Out of 149 tested samples, 11 (7.3%) showed the viral DNA for one of the herpes viruses (7.3%). Nine samples were HSV1 positive (6.0%) and two samples were VZV positive (1.3%). Fever and vomiting were the most frequent symptoms. The mean ± standard deviation (SD) WBC and counts in cerebrospinal fluid (CSF)-positive samples, and all included samples were 298.8 ± 552.7 cells/µL and 131.1 ± 474.6 cells/µL, respectively. Conclusions: Although viral encephalitis is considered a threat to children's health, with accurate diagnosis and appropriate antiviral drugs, death and neurological complications in children can be prevented.

New Presentation of CD27 Deficiency; Coronary Ectasia and COVID-19.

CD27 is a costimulatory receptor involved in the maturation of the innate and adaptive immunity. CD27, through interaction with CD70, plays a role in the control of Epstein-Barr virus (EBV) infection. CD27 deficiency leads to an immune dysregulation disease characterized by EBV susceptibility. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) might put patients with primary immunodeficiency at risk for adverse outcomes. Chromogenic in situ hybridization (CISH) study was performed to detect EBV in the lymphoma tissue. Genetic analysis of the patient was done with Whole Exome Sequencing and detected variant was confirmed with PCR-Sanger sequencing. Here we report a 20-month-old boy with CD27 deficiency who developed lymphoma and coronary artery ectasia and had been infected with SARS-CoV-2. Clinical and laboratory findings were incompatible with atypical Kawasaki syndrome or multisystem inflammatory syndrome in children (MIS-C). As CD27 deficiency is a rare immune defect, publishing clinical data about the identified patient(s) can shed light on our knowledge about the related phenotype and the spectrum of clinical manifestations associated with CD27 deficiency. Thus, our findings expanded the spectrum of manifestations beyond EBV infection, highlighting this unusual cardiac sequela that could be related to EBV infection, lymphoma, or an underlying disease.

Allogenic Hematopoietic Stem Cell Transplant in Iranian Patients With Congenital Sideroblastic Anemia: A Single-Center Experience.

Congenital sideroblastic anemia is characterized by anemia and intramitochondrial iron accumulation in erythroid precursors that form ring sideroblasts. The most common recessive forms are caused by sequence variations in the ALAS2 and SLC25A38 genes. In patients with transfusion-dependent and pyridoxine- resistant severe congenital sideroblastic anemia, hematopoietic stem celltransplantis the only curative option. Herein, we described successful implementations of allogeneic hematopoietic stem cell transplant in 4 Iranian children with congenital sideroblastic anemia. The patients had presented with clinical manifestations of anemia early in life, and the diagnoses of congenital sideroblastic anemia were established through blood tests and bone marrow aspiration. Congenital sideroblastic anemia was further confirmed by the identification of pathogenic variants in SLC25A38 in 2 patients. All 4 patients received allogeneic hematopoietic stem cell transplant with myeloablative conditioning regimen that included busulfan, cyclophosphamide, andrabbit antithymocyte globulin. A combination of cyclosporine A and methotrexate or mycophenolate mofetil was used for graft-versus-host disease prophylaxis. Bone marrow and peripheral blood from sibling or related donors with fully matched human leukocyte antigen profiles were applied. The outcomes of hematopoietic stem celltransplantin patients with congenital sideroblastic anemia were favorable. Three patients achieved full donor chimerism (>95%, 98%, and 100%), and the other patient showed mixed chimerism (75%). All patients remained transfusion independent. Hemato- poietic stem celltransplantis a curative treatmentthat can provide long-term survival for patients with congenital sideroblastic anemia, particularly when used in a timely manner. There remain ongoing challenges in various aspects of hematopoietic stem celltransplantin patients with congenital sideroblastic anemia, which remain to be elucidated.

Assessment of early and post COVID-19 vaccination antibody response in healthcare workers: a multicentre cross-sectional study on inactivated, mRNA and vector-based vaccines.

In this multicentre study, we compared the status of antibody production in healthcare personnel (HCP) before and after vaccination using different brands of COVID-19 vaccines between March 2021 and September 2021. Out of a total of 962 HCP enrolled in our study, the antibody against the S1 domain of SARS-CoV-2 was detected in 48.3%, 95.5% and 96.2% of them before, after the first and the second doses of the vaccines, respectively. Our results showed post-vaccination infection in 3.7% and 5.9% of the individuals after the first and second doses of vaccines, respectively. The infection was significantly lower in HCP who presented higher antibody titres before the vaccination. Although types of vaccines did not show a significant difference in the infection rate, a lower infection rate was recorded for AstraZeneca after the second vaccination course. This rate was equal among individuals receiving a second dose of Sinopharm and Sputnik. Vaccine-related side effects were more frequent among AstraZeneca recipients after the first dose and among Sputnik recipients after the second dose. In conclusion, our results showed diversity among different brands of COVID-19 vaccines; however, it seems that two doses of the vaccines could induce an antibody response in most of HCP. The induced immunity could persist for 3-5 months after the second vaccination course.

Expression analysis of miRNA-155 level in Helicobacter pylori related inflammation and chronic gastritis.

Background and Objectives: Helicobacter pylori, is a major etiologic agent associated with gastritis. There is more evidence of noncoding microRNAs (miRs) dysregulation in gastrointestinal diseases, including inflammation caused by Helicobacter pylori. Also, the classification of gastrointestinal malignancies using the miRs profile is better than the protein profile. MiRNA-155(miRNA-155) among other miRs plays an important role in control of inflammation and gastric malignancy, so it can be remarkable prognosis marker of gastric cancer in the phase of chronic gastritis. The aim of this study was to compare the expression of miRNA-155 in gastric biopsy and serum samples of adult patients with chronic gastritis. Materials and Methods: Biopsy and blood samples were collected from endoscopy candidates at Taleghani hospital, Tehran, during 2019. H. pylori infection was detected using histology, culture and molecular PCR methods. Based on cagA and vacA genotyping, the toxicity of H. pylori isolates were determined. After RNA extraction, the expression rate of miRNA-155 was evaluated by real-time polymerase chain reaction (RT-PCR) in gastric tissue and serum of adults infected by H. pylori (n = 30) compared with control group without infection (n = 20). RNU6 housekeeping miRNA were used as endogenous control and statistical analyses were performed using SPSS, ANOVA and Student's t-test. Results: miRNA-155 expression in H. pylori infected adult patients increased significantly by 5.61 and 10.11 fold in serum and tissue respectively, compared to that observed in the control group. Evaluation of miRNA-155 expression pattern in relation to bacterial virulence factors showed that the increase in miRNA-155 expression is independent of CagA and VacA toxins. Conclusion: According to the differential expression patterns of miRNA-155 in serum samples of the infected adult patients, miRNA-155 has the potential to evaluate as chronic gastritis marker.

Persistent elevation of aspartate aminotransferase in a child after incomplete Kawasaki disease: a case report and literature review.

BACKGROUND: Interpretation of abnormalities in liver function tests, especially in asymptomatic children, is a common problem faced by clinicians. Isolated elevation of aspartate aminotransferase may further puzzle physicians. Macro-aspartate aminotransferase (AST) results from complexes AST produces with other plasma components, such as immunoglobulin. To our knowledge, this is the first report on a case of macro-AST-associated incomplete Kawasaki disease (KD). It is to make physicians aware of this benign condition and help to prevent extensive, unnecessary investigations and invasive workups. CASE PRESENTATION: A 16-month old boy with a 7-day history of fever was admitted to our pediatric ward for pyrexia workup. After complete investigations, KD was confirmed by a pediatric rheumatologist. During his admission and serial follow-up tests, an isolated AST elevation was noted. Comprehensive tests were performed and using the polyethylene glycol (PEG) precipitation method, macro-AST was confirmed. The patient has been followed up for 3 years, and so far, the benign nature of this condition has been confirmed. CONCLUSION: Clinicians should consider testing for macro-AST when elevated AST is the only abnormal lab finding. Although an uncommon finding, macro-AST may be seen in both children and adults. There are many reasons for this phenomenon, including resolved acute hepatitis or in some cases, inflammatory bowel disease, hepatic malignancy, monoclonal gammapathy, celiac disease, or KD; however, it may be observed in asymptomatic healthy children as well. Using the PEG precipitation method, a definitive diagnosis can be made. In none of these conditions does macro-AST have any prognostic significance. An appreciation of macro-AST may prevent the need for more invasive investigations to which patients may be unnecessarily subjected. It is important to recognize this condition as benign and assure patients that no specific treatment is required.

Absence of Human Papillomavirus in Benign and Malignant Breast Tissue.

BACKGROUND & OBJECTIVE: Malignant breast tumors, which are one of the most important deadly cancers in women, like many other cancers, are proposed to be related to viruses etiologically. Proper management of breast carcinoma necessitates an identification of the etiological factors. Human Papillomavirus is considered to have an etiological role in breast carcinoma. We carried out this study to find out if Human Papillomavirus-DNA is present in the malignant and benign breast tissue in our patients. METHODS: Seventy-five paraffin-embedded breast cancer tissues and 75 normal breast tissues and benign breast lesions were examined in this study (case-control) to look for Human Papillomavirus-DNA employing Nested Polymerase Chain reaction. The tissues were examined over a period of ten years in the pathology department of the Pathobiology Laboratory Center of Tehran. RESULTS: No Human Papillomavirus-DNA was found in any of the malignant or control group specimens. CONCLUSION: Our results showed no evidence of Human Papillomavirus in cancerous and benign tissues, which is consistent with some other studies in English medical literature. More investigations using more specimens from different parts of the country are required to confirm the presence or absence of any connection between Human Papillomavirus and development of breast carcinoma in Iran.

Neonatal neuroblastoma presented with respiratory distress, a case report.

BACKGROUND: The most common neonatal tumor is neuroblastoma and adrenal gland is the most common site of involvement. 11-26% of this tumor is seen in the thorax of neonates. Due to a similar appearance of the mediastinal tumor with pneumonia, a high index of suspicion is necessary for early diagnosis of this disorder. CASE REPORT: A 17-day -old female and term neonate delivered by caesarian section was reported in the study. The mother had a normal pregnancy without any reported complications. The newborn admitted to a neonatal intensive care unit (NICU) in her hometown, because of respiratory distress, stridor, and tachypnea which was started from 7 days of life and due to lack of recovery and persistent respiratory symptoms, the patient referred to our hospital. In chest X-ray, opacity in the right upper lobe of the lung was seen. In barium study, a mass like lesion in the size of 35.34 mm adjacent to upper intra-thoracic esophagus with mild mass effect was observed and in CTS a posterior mediastinal mass with severe compression on the trachea and extension to the spinal canal was reported. Due to severe obstruction on the airway, the baby underwent emergency surgery and a mediastinal mass with adhesion and involvement of the ribs and spinal cord was resected. The final diagnosis of poorly differentiated neuroblastoma was confirmed by biopsy. CONCLUSION: In every neonate with persistent respiratory distress, stridor and abnormal chest X-ray, diagnosis of neonatal neuroblastoma should be considered.

Enterobacter Meningitis Due To Dermoid Cyst Manipulation.

Gram-negative meningitis can occurssubsequent to dura-arachnoid barrier disruption because of trauma, surgery and rarely an infected dermoid cyst. Association of neurosurgical procedures with Gram-negative meningitis was described for the first time in 1940. Intracranial infections from gram-negative bacilli like Enterobacter are serious and difficult to treat as many antibiotics fail to achieve bactericidal concentrations in the cerebrospinal fluid. Here in, we report a rare case of pediatric Enterobacter meningitis in a patient with a dermoid cyst that had been manipulated. She was managed with antibiotic therapy plus surgical removal of the infected cyst.

Sporadic Insulinoma as a Rare Cause of Recurrent Hypoglycemia in Children.

Insulinoma is a rare pancreatic tumor in children and adolescents. As a result of insulin hypersecretion, signs and symptoms are more commonly consequences of the pathophysiologic responses to hypoglycemia. According to rarity of this tumor in children and nonspecificity of clinical presentations, diagnosis of insulinoma in this group of patients is usually delayed. Early diagnosis is very important for preventing neurologic damage. In this case report, we present the case of a 10-year-old boy with signs and symptoms of hypoglycemia and final diagnosis of insulinoma.

Correlation between Gleason Scores in Needle Biopsy and Corresponding Radical Prostatectomy Specimens: A Twelve-Year Review.

BACKGROUND: Presence of discordance between the Gleason score on needle biopsy and the score of radical prostatectomy specimen is common and universal. In this study, we determined the accuracy of Gleason grading of biopsies in predicting histological grading of radical prostatectomy specimens and the degree of overgrading and undergrading of prostatic adenocarcinoma in our center, which is one of the referral centers in Tehran. METHODS: In this retrospective study, we analyzed the results of prostate needle biopsies and subsequent prostatectomies diagnosed at the Pathobiology Laboratory Center, Tehran, Iran in 45 patients between 2002 and 2013. Preoperative clinical data and the information from biopsy and prostatectomy specimens were collected. The accuracy, sensitivity, specificity, and positive and negative predictive values of different grades and groups were assessed. Pearson and Spearman correlation coefficient were used to determine the relation of different variables. RESULTS: The biopsy Gleason score was identical to the scores in prostatectomy specimens in 68.2% cases, while 31.8% were discrepant by 1 or 2 Gleason score. We had 9.1% downgrading and 22.7% cases upgraded after prostatectomy. The sensitivity and positive predictive value was 86% and 79% for low grade, 67% and 75% for moderate grade, and 80% and 80% for high-grade tumors, respectively. CONCLUSION: Overall, the reliability of Gleason grading of needle biopsies in predicting final pathology was satisfavory. Moderate grade group was the most difficult to diagnose in needle biopsy.

Ciliated hepatic foregut cyst: two case reports in children and review of the literature.

Ciliated hepatic foregut cyst (CHFC) is a rare lesion which originates from detached hepatic diverticulum or from detachment and migration of buds from the esophageal and bronchial regions of the foregut which subsequently get entrapped by the liver during the early embryonic development of the foregut. CHFCs are mostly seen in adults and are rarely reported in children, with only about 10 cases reported in this age group. Hereby, we present two cases of CHFC in two 3.5-year-old boys; one of them had cystic lesion at medial segment of left lobe of liver (common site), and in the other one it was located at right lobe of liver (less common site). Histologically, both cysts had four layers composed of inner ciliated, pseudostratified, columnar epithelium; subepithelial connective tissue; smooth muscle layer; and an outer fibrous layer.

Primary intrarenal neuroblastoma with hypertension and disseminated intravascular coagulation.

The primary intrarenal neuroblastoma (IRNB) is a rare condition. Intrarenal neuroblastoma typically results from direct renal invasion from an adrenal neuroblastoma, but true intrarenal neuroblastoma originates either sequestered adrenal rests during the fetal life or intrarenal sympathetic ganglia. Clinical, radiological, and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. The distinction of this rare tumor from Wilms' tumor is an important challenge since both tumors have major differences in prognostic and therapeutic response. We present a 3-year-old boy of primary intrarenal neuroblastoma with extensive abdominal and mediastinal mass, persistent hypertension, and disseminated intravascular coagulation (DIC).