Spinal subdural hematomas in a normal child without trauma history: a case report

Acute Spinal Subdural Hematoma (ASSH) is a rarely recognized condition that may result in severe irreversible neurologic complication. We presented a 7-year old girl was transferred to our hospital with limping and pain in lower extremities and acute paraplegia without history of direct trauma. The patient's lower limbs weakened. She was unable to bear weight. Deep Tendon Reflexes(DTR) in lower extremities had increased. Her MRI showed spinal subdural hematoma we reextended from T2 to T6. We performed laminectomy from T2 to T5 and about 70 cc of subdural hematoma was evacuated. After a month after the operation, the patient's neurological deficit resolved completely. The case illustrates the need of attention to clinical manifestation of acute spinal subdural hematoma and early diagnosis to prevent irreversible neurologic complication

Prevalence of Nephrolithiasis in 7-11 year-old Students: A Multicenter Study

Introduction: Renal diseases can be asymptomatic even in progressive disorders; therefore, detecting urine and ultrasound abnormalities may help facilitate early diagnosis and prevention of renal diseases. This study was conducted to investigate random urine parameters and urinary system ultrasonography findings in 7-11 year-old students.Materials and Methods: Healthy students from Tehran and Qom, Iran were enrolled in a prospective descriptive study and their sex, age, weight, height, and BMI were measured. Then, a fresh clean urine sample was collected and ultrasonography of the urinary tract was done. The urine specimen was tested for urine Ca/Cr, urine oxalate/Cr, and urine citrate/Cr.Results: Of 932 students, 47.9% were female and 52.1% were male. The age range of the students was 7-11 years with a mean age of9.08 years. A history of renal disease and UTI was positive in 1.1% and 9.9% of the students, respectively. Ultrasound was normal in78% and abnormal in 22% of the students. Abnormal findings included hydronephrosis in 1.1%, fullness of the urinary tract in 0.1%, urinary system duplication in 3%, urolithiases in 0.7%, decreased kidney size in 0.4%, increased bladder thickness in 8.9%, and other abnormal findings in 7.8% of the subjects. Abnormal urine findings included hypercalciuria, in 10.9%, urine hyperuricosuria in 5.4%, urine hyperoxaluria in 12.8%, and hypocitraturia in 96.9% of the students.Conclusions: According to the results, nephrolithiasis may be due to hyperoxaluria, hypercalciuria, and hyperuricosuria in a normal population. Genetics and nutrition are more important risk factors. Therefore, some nutritional interventions for decreasing urine oxalate, calcium, and uric acid may be beneficial. Keywords: Urinalysis; Ultrasonography; Hypercalciuria; Hyperuricosuria; Hyperoxaluria; Child

Succinate Dehydrogenase Deficiency, a Treatable Neurometabolic Disorder

Succinate dehydrogenase (SDH) deficiency is a rare autosomal recessive neurometabolic disorder that causes brain insult, neurodevelopmental delay, exercise intolerance, and cardiomyopathy. A 25-month-old boy was referred to our neurometabolic center due to developmental regression after injecting the influenza vaccine when he was 10 months old. Magnetic resonance imaging (MRI) showed high signal changes in the brain white matter, and magnetic resonance spectroscopy (MRS) detected a high succinate peak at 2.4 parts per million (ppm). The evaluation of urine organic acids showed a significant elevated succinic acid and whole exome sequencing, confirming SDH. Treatment with a mitochondrial cocktail was initiated, and remarkable improvement was observed. SDH deficiency as a treatable neurometabolic disorder should be considered in any patients with developmental disorders, accompanied by hyperintensity in white matter (as similar to leukodystrophia). Further evaluation is recommended since outcomes depend on early diagnosis and treatment. &nbsp

Primary spinal tumors and masses in children: a study of 37 cases

Abstract Objectives Spinal cord tumors are a rare diagnosis in children, mostly presented with unspecific symptoms which may pose a problem due to their possible malignancy and further complications. Yet there is little data regarding spinal cord lesions in our country.The aim of this study is to present a series of 37 cases of primary spinal tumors treated at the same institution and briefly review their pathology, symptoms and site of occurrence. Materials & Methods  37 cases of spinal cord tumors and masses were selected from March 2007 to 2017, excluding spinal dysraphism. Data regarding age, sex, clinical presentation, location of the mass, and pathology were retrospectively collected. Results Mean age at diagnosis was 5 years and 8 months (standard deviation: 4.1 years). 21 were male and 16 were female (male-to-female ratio: 1.31). Pathological findings were 9 Neuroepitheliomas (6 Neuroblastoma, 2 Ganglioneuroma, 1 Ganglioneuroblastoma/Ganglioneuroma), 4 Ependymomas, 3 Primitive Neuroectodermal Tumors, 3 Glial tumors, 4 Neurodevelopmental tumors, 3 Lymphomas, 1 Hemangiopericytoma and 1 Neurofibroma. 26 patients had Motor symptoms (74.2%), 14 had pain (40%), 6 showed sensory symptoms (16.6%) and 4 had urinary symptoms (11.4%). The most common location of occurrence was the lumbosacral region.  conclusion While differing in pathological composition and location of tumors in comparison to other papers, our study presents possible presentations and/or expected pathologies in pediatric spinal cord tumors. &nbsp

Acute Pancreatitis as a Possible Unusual Manifestation of COVID-19 in Children

Coronavirus disease-2019 (COVID-19) which is caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has spread throughout the world causing problems for millions of people. Symptoms of COVID-19 in pediatric patients include both respiratory and gastrointestinal symptoms. The most common symptoms are fever, cough, and fatigue. In this report, we describe a case of a previously well 14-year-old boy, who presented to our emergency department with a complaint of abdominal pain, nausea, and vomiting without fever or respiratory symptoms. He was diagnosed with acute pancreatitis based on an abnormal amylase level and abdomen computed tomography (CT) and later found to be infected by SARS-CoV-2, by a positive reverse transcriptase-polymerase chain reaction (RT-PCR) test

Stroke Modifies Drug Consumption in Opium Addicts:Role of the Insula

Introduction: Addiction imposes a large medical, social and economic burden on societies. Currently, there is no effective treatment for addiction. Our struggle to decipher the different mechanisms involved in addiction requires a proper understanding of the brain regions which promote this devastating behavior. Previous studies have shown a pivotal role for insula in cigarette smoking. In this study we investigated the change in opium consumption after CVA. Methods: This study took place in three referral academic hospitals affiliated to Tehran University of Medical Sciences. Patients who suffered a CVA and were addicted to opium were recruited during their hospitalization or visit to the neurology clinic in this study. Age, sex and the route and mean amount of opium use of each patient before CVA and 1, 3 and 6 months post-CVA was asked using a questionnaire. The patients were divided into three groups based on the location of brain ischemia (insula, basal ganglia and non-insula non-basal ganglia group). Results: Seventy five percent of the patients with ischemia of the insula changed the route or amount of opium use after CVA and 37.5% of them stopped opium use after CVA. These values were significantly higher than patients with non-insula non-basal ganglia ischemia (p values 0.005 and 0.03 for change in route or amount and stopping opium use, respectively). This was not true in patients with ischemia of the basal ganglia. Younger patients were more likely to change the route or amount of opium use and stop opium use after CVA (p values 0.002 and 0.026, respectively). Discussion: The results of the present study indicate a possible role for the insula in opium addiction, especially in younger individuals

Childhood Guillain–Barre syndrome in the SARS‐CoV‐2 era: Is there any causative relation?

Abstract We reported an association between SARS‐CoV‐2 infection and Guillain–Barre syndrome (GBS). From 37 patients with GBS, previous SARS‐CoV‐2 clinical clues, including fever, cough, and diarrhea, were recorded in 18 patients. Among them, SARS‐CoV‐2 IgG was detected in seven patients, considered confirmed as cases. SARS‐CoV‐2 PCR was positive in just one patient. Although we found no increase in patient recruitment during the pandemic compared to previous years, our study indicated that SARS‐CoV‐2 is associated with poorer outcomes regarding GBS disability scale and hospital stay

The genetic basis of early-onset hereditary ataxia in Iran: results of a national registry of a heterogeneous population

Abstract Background To investigate the genetics of early-onset progressive cerebellar ataxia in Iran, we conducted a study at the Children’s Medical Center (CMC), the primary referral center for pediatric disorders in the country, over a three-year period from 2019 to 2022. In this report, we provide the initial findings from the national registry. Methods We selected all early-onset patients with an autosomal recessive mode of inheritance to assess their phenotype, paraclinical tests, and genotypes. The clinical data encompassed clinical features, the Scale for the Assessment and Rating of Ataxia (SARA) scores, Magnetic Resonance Imaging (MRI) results, Electrodiagnostic exams (EDX), and biomarker features. Our genetic investigations included single-gene testing, Whole Exome Sequencing (WES), and Whole Genome Sequencing (WGS). Results Our study enrolled 162 patients from various geographic regions of our country. Among our subpopulations, we identified known and novel pathogenic variants in 42 genes in 97 families. The overall genetic diagnostic rate was 59.9%. Notably, we observed PLA2G6, ATM, SACS, and SCA variants in 19, 14, 12, and 10 families, respectively. Remarkably, more than 59% of the cases were attributed to pathogenic variants in these genes. Conclusions Iran, being at the crossroad of the Middle East, exhibits a highly diverse genetic etiology for autosomal recessive hereditary ataxia. In light of this heterogeneity, the development of preventive strategies and targeted molecular therapeutics becomes crucial. A national guideline for the diagnosis and management of patients with these conditions could significantly aid in advancing healthcare approaches and improving patient outcomes