Prevalence of the C282Y and H63D mutations of the HFE hemochromatosis gene in Azerian major β-thalassemia and iron overload

In this study, we have determined the allele frequency of HFE mutations H63D and C282Y in a group of Azerian beta-thalassemia major patients. These two mutations are implicated in hereditary hemochromatosis among Caucasians. In this study, we wanted to outbreak these mutations with the iron status in major beta-thalassemia patients. Sixty Azerian major beta-thalassemias were screening for the C282Y and H63D by digestion of polymerase chain reaction products (PCRP). Serum ferritin level was measured by enzyme-linked immunosorbent assay (ELISA). The allele frequency of H63D mutation was 20 . C282Y mutation was not present in our studied patients. No statistically significant difference of serum ferritin level was found between major beta-thalassemia with and without HFE mutations. Our data suggest that H63D mutation is so frequent in Azerian major beta-thalassemia patients than in the general population and that the coinheritance of H63D mutation does not influence the severity of iron overload in these patients. © 2015, Springer-Verlag London

Intra-operative total bowel washing: A new method to improve the outcome of gastroschisis primary repair

Background: Gastroschisis is an abdominal wall defect that is managed by surgical reduction of herniated bowel into the abdominal cavity and abdominal wall reconstruction. Loss of abdominal domain is the main challenge that may complicate the process of gastroschisis management. Objectives: This article is about innovative manure called total bowel washing (TBW) that may improve the outcome of gastroschisis primary repair. Methods: All neonates with gastroschisis who met the study inclusion criteria between 2006-2019 were enrolled and divided into two groups of conventional and TBW method of gastroschisis management. In TBW group, bowls were washed with warm saline and after a gentle enterolysis, the whole gastrointestinal tract was irrigated via a gastric tube and evacuated completely from thick meconium until the watery stool started to come out of anus slightly. Primary abdominal wall closure was performed after loop by loop bowel reduction. Gastroschisis management outcome was compared between the two groups. Results: 15 neonates were allocated in each group. Demographic and anthropometric variables were compared and any significant difference wasn�t reported between the two groups. We observed a significantly better outcome in terms of faster GI rehabilitation, shorter time to oral feeding tolerance, less need to silo placement and shorter NICU and hospital stay in TBW method. Operation time was slightly longer in TBW group while the difference was not significant statistically. Conclusions: Total bowel washing and complete evacuation of gastrointestinal tract from thick meconium will increase the success rate of primary repair and improve the outcome of gastroschisis management. © 2020, Author(s)

Pre-operative hormone stimulation in hypospadias repair: A facilitator or a confounder

Introduction: Preoperative hormone stimulation (PHS) is popular in hypospadias reconstructive surgery. While still controversial, it is performed to have a more developed gross anatomy of the glans and penis for improvement of the surgical condition and outcome. Objective: In this study we assessed morphometric and histologic effects of pre-operative testosterone therapy on the penis and prepuce in patients with hypospadias. Study design: 18 patients with hypospadias who received 3 monthly doses of 25 mg testosterone injections were compared with 23 patients with hypospadias who were managed without pre-operative androgen therapy. Penile morphometry and hormone side effects were assessed in monthly pre-operative visits. Intra operative observations and preputial histopathology were also compared between the groups. Results: Glans diameter enlarged significantly after the first dose of testosterone. (P < 0.001) while morphologic changes were not significant by further injections. 72.2 showed one or more hormone related side effects that were mostly mild. Severe side effects such as thick pubic hair growth, frequent erections or considerable penis hyper-sensation were observed in 27.8. Glans to corpus ratio was 0.2 ± 0.75 in PHS group compare to 0.3 ± 0.17 in control group. (P < 0.001). Hemorrhage needing tourniquet placement was reported in 44.4 of the PHS patients compared to 26.1 among the control group. Histopathologic studies revealed increased vascularity and less inflammation of the prepuce in PHS group compared to controls. Discussion: The glans and penis enlargement wasn't significant in 22.2 of patients who underwent PHS. We identified the first dose of testosterone as the most effective one. This finding supports the idea of close clinical monitoring to stop PHS when a significant response is observed and the expected goals are reached. Androgen side effects were not uncommon among our patients although they were mainly mild. Intraoperative assessments showed a decreased glans wing thickness to corporal body ratio in PHS group compared to controls. This finding caused more complex glanuloplasty while wrapping the glans wings over neo urethra and enlarged corporal bodies. Conclusion: We suggest limiting PHS to carefully selected cases considering the observed anatomical and histological changes and the side effects. Monthly monitoring during PHS is recommended to stop androgen therapy as soon as the minimum defined targets are reached. This may decrease the rate of androgen side effects while providing better surgical conditions. Table presented © 2020 Journal of Pediatric Urology Compan

RETRACTED ARTICLE: Prevalence of the C282Y and H63D mutations of the HFE hemochromatosis gene in Azerian major β-thalassemia and iron overload (Comparative Clinical Pathology, (2016), 25, 1, (151-154), 10.1007/s00580-015-2156-2)

This article has been retracted at the request of the Editorin- Chief per the Committee on Publication Ethics guidelines. The article shows evidence of irregularities in authorship during the submission process, there is strong reason to believe that the peer review process was compromised and the article contains patchwork plagiarism from a variety of sources. © 2016, Springer-Verlag London

Bioavailability of rectal acetaminophen in children following anorectal surgery

Background: Acetaminophen is widely used as an analgesic and antipyretic agent in pediatrics. Although bioavailability of rectal acetaminophen is unpredictable, rectal route is a usual and acceptable method of prescription. Major anorectal surgery may alter the normal structure of the surgical site, especially the vascular elements and the normal connections between port and systemic vessels. As a result the pharmacokinetics of rectal medications might also be altered. Based on this hypothesis, we decided to study acetaminophen plasma concentration among children who underwent these types of surgeries to determine the pharmacokinetic of absorption, plasma concentration, safety, and efficacy of rectal acetaminophen. Materials and methods: The study included 20 cases with previous history of pull-through procedure owing to Hirschsprung's disease (HD), 20 cases with imperforate anus (IA) reconstructive surgeries who were admitted for colostomy closure, and 20 otherwise healthy cases of inguinal herniotomy. Venus blood sampling was done 4, 8 and 12 hrs after a single loading dose of rectal acetaminophen (40 mg/kg), and plasma acetaminophen concentration was compared between groups. Results: Mean serum acetaminophen levels of the HD group were significantly higher than those of the herniotomy group (36.3 ± 6.79, 27.4 ± 8.42, 16.8 ± 7.62 versus 25.9 ± 9.12, 16.7 ± 6.74, 8.1 ± 5.79 (μg/ml) at 4, 8 and 12 hrs after drug administration and P < 0.05). The IA group had higher concentrations of plasma acetaminophen compared to the herniotomy group; however, the p values were not statistically significant. (31.4 ± 10.39, 21.5 ± 9.12, 13.3 ± 6.79 versus 25.9 ± 9.12, 16.7 ± 6.74, 8.1 ± 5.79 (μg/ml) at 4, 8 and 12 hrs after drug administration). Serum concentrations of acetaminophen in IA and HD patients were above the therapeutic range four hours after administering the loading dose (31.4 ± 10.39 and 36.3 ± 6.79 versus 5�20 μg/ml). Conclusion: Bioavailability of rectal acetaminophen might get altered after major anorectal surgery in children. Rectal acetaminophen should be administered with special caution among infants with history of anorectal operations. Repeated dose of rectal acetaminophen may cause the drug blood concentration to reach toxic levels in these patients. Type of study: Prospective comparative study. Level of evidence: Level II. © 2020 Elsevier Inc

Coronavirus disease 2019 (COVID-19) outbreak in pediatrics and the role of pediatricians: A systematic review

The coronavirus disease 2019 (COVID-19) outbreak initiated in Wuhan, China and has spread rapidly all around the world and labeled as a pandemic with almost 168,000 infected cases and 6,500 deaths globally up to March 16, 2020. It is believed that children are less likely than adults to be infected with COVID-19. In this review, we discuss different aspects of COVID-19 infection in pediatrics. COVID-19 in pediatrics occurs in the early stages of its outbreak at a high rate with a family cluster pattern mainly. Children infected with COVID-19 are mostly asymptomatic carriers and the main potential causes of the spread and transmission of the disease in com-munities. Asymptomatic children with no underlying disease or red flags should follow home isolation protocols. Children with red flags, comorbidities and risk factors or those with severe pneumonia must be admitted to the hospitals. Children�s hospitals should be equipped with the acute respiratory diseases ward, quarantine rooms, and intensive care unit to protect other patients and health care staff during the COVID-19 outbreak. © 2020, Author(s)

Pediatric renal neoplasms (A clinicopathological study in pathology departments of Dr Sheikh children hospital and Imam Reza hospital in Mashhad during 15 years)

Background&Objective: Pediatric renal tumors represent approximately 7% of all childhood cancers and are completely different from those occurring in adults. The aim of this study was to make an analysis of clinical and pathological characteristics of these tumors. Materials&Methods: In this descriptive study all of the pediatric patients diagnosed as having renal neoplasm in departments of pathology of Dr Sheikh children hospital and Imam Reza hospital from 1991-2006 were evaluated. Results: 52 patients including 27 boys and 25 girls with mean age of 40.63 months were studied. Tumors involved each kidney in 24 (45.3%) and were bilateral in 5 (9.4%) patients. Abdominal mass was the commonest clinical symptom and sign. Congenital anomalies were presented in 6 (14.6%) patients. Histopathological examination showed Wilms tumor in 46 (86.8%), cystic partially differentiated nephroblastoma in 2 (3.8%), mesoblastic nephroma in 2 (3.8%) rhabdoid tumor of the kidney in 1 (1.9%), metanephric adenofiroma and low-grade tubulopapillary carcinoma in 1 (1.9%) and clear cell sarcoma of the kidney in 1 (1.9%) patient. 11 (20.8%) cases were in stage I, 16 (30.2%) stage II, 13 (24.5%) stage III, 8 (15.1%) stage IV and 5 (9.4%) stage V. Conclusion: Although Wilms tumor is the commonest renal neoplasm in childhood there are also recently described entities such as metanephirc tumors and juvenile renal carcinoma that must be considered in histopathological evaluation of a pediatric renal neoplasm. Role of molecular and cytogenetic methods is increasing for classification and treatment of childhood renal neoplasms

Diagnostic Accuracy of Polyclonal Stool Antigen for Detection of Helicobacter Pylori Infection in Children

Introduction: Helicobacter pylori infection has various clinical features. One of the most common presentations of this infection is upper abdominal pain. Complications such as gastritis, gastric ulcer, gastric carcinoma and MALT lymphoma mandate early diagnosis of H.P infection by a low cost and non invasive manner. The aim of this study was to evaluate the diagnostic role of H.P stool antigen detection as a simple and non invasive method for diagnosis of this infection.   Materials and Methods: Upper endoscopy with gastric biopsy was done on all patients between 6 months to 18 years old with upper abdominal pain. Stool test was done by polyclonal anti-h.p antibody. Results of stool Test were compared with results of RUT and histologic examination   Results: Overall 110 patients (57 boys,53 girls) were studied, 32 patients were Helicobacter pylori positive and 78 patients were Helicobacter pylori negative based on RUT and histologic examination. Stool antigen testing was positive for 39 patients. Sensitivity and specificity of stool Ag were 100% and 91% respectively.   Conclusion: Stool Ag test has high sensitivity and negatity predictive value for diagnosis of Helicobacter pylori infection.   Keywords: Helicobacter pylori, Sensitivity, Stool antigen test, Specificity