A comprehensive study of mutation and phenotypic heterogeneity of childhood mitochondrial leukodystrophies.

OBJECTIVE: Mitochondrial leukodystrophies (MLs) are mainly caused by impairments of the mitochondrial respiratory chains. This study reports the mutation and phenotypic spectrum of a cohort of 41 pediatric patients from 39 distinct families with MLs among 320 patients with a molecular diagnosis of leukodystrophies. METHODS: This study summarizes the clinical, imaging, and molecular data of these patients for five years. RESULTS: The three most common symptoms were neurologic regression (58.5%), pyramidal signs (58.5%), and extrapyramidal signs (43.9%). Because nuclear DNA mutations are responsible for a high percentage of pediatric MLs, whole exome sequencing was performed on all patients. In total, 39 homozygous variants were detected. Additionally, two previously reported mtDNA variants were identified with different levels of heteroplasmy in two patients. Among 41 mutant alleles, 33 (80.4%) were missense, 4 (9.8%) were frameshift (including 3 deletions and one duplication), and 4 (9.8%) were splicing mutations. Oxidative phosphorylation in 27 cases (65.8%) and mtDNA maintenance pathways in 8 patients (19.5%) were the most commonly affected mitochondrial pathways. In total, 5 novel variants in PDSS1, NDUFB9, FXBL4, SURF1, and NDUSF1 were also detected. In silico analyses showed how each novel variant may contribute to ML pathogenesis. CONCLUSIONS: The findings of this study suggest whole-exome sequencing as a strong diagnostic genetic tool to identify the causative variants in pediatric MLs. In comparison between oxidative phosphorylation (OXPHOS) and mtDNA maintenance groups, brain stem and periaqueductal gray matter (PAGM) involvement were more commonly seen in OXPHOS group (P value of 0.002 and 0.009, respectively), and thinning of corpus callosum was observed more frequently in mtDNA maintenance group (P value of 0.042).

Expanding phenotype heterogeneity of NARS2 by presenting subdural hematoma and parenchymal hemorrhage.

BACKGROUND: NARS2 encodes mitochondrial Asparaginyl-tRNA Synthetase 2, which catalyzes the aminoacylation of tRNA-Asn in the mitochondria. To date, 24 variants have been reported in NARS2 gene in 35 patients. The phenotypic variability of NARS2-associated disorder is broad, ranging from neurodevelopmental disorders to hearing loss. In this study, we report some novel imaging findings in an Iranian patient suffering from epileptic encephalopathy, caused by a previously reported variant, c.500A > G; p.(His167Arg), in NARS2. METHODS: The spectrum of clinical manifestations of two Iranian patients was investigated and genetic analysis was performed by Whole-exome sequencing (WES). Additionally, we also reviewed the literature and summarized the phenotypes of previously reported patients with variants in the NARS2 gene. RESULTS: Here, we present the phenotypic and genetic features of 2 unrelated Iranian infants presented with neurodevelopmental delay, seizures, hearing impairment, feeding problems, elevated serum lactate levels in addition to subdural hematoma and cerebral parenchymal hemorrhage in the brain magnetic resonance imaging (MRI) of one of the patients. Genetic analysis revealed a biallelic missense variant in NARS2: c.500A > G; p.(His167Arg). We described the subdural hematoma and cerebral parenchymal hemorrhage of the brain for the first time. CONCLUSIONS: Our study provides new clinical findings, subdural hematoma, and parenchymal hemorrhage, in NARS2-related disorders. Our findings along with previous studies provide more evidence of the clinical presentation of the disease caused by pathogenic variants in NARS2. Expanding the clinical spectrum increases the diagnostic rate of molecular testing and improves the quality of counseling for at-risk couples.

Transforaminal Endoscopic Lumbar Diskectomy versus Open Microdiskectomy for Symptomatic Lumbar Disk Herniation: A Comparative Cohort Study on Costs and Long-Term Outcomes.

BACKGROUND: Transforaminal endoscopic lumbar diskectomy (TELD) is considered an effective treatment for lumbar disk herniation (LDH). There is a paucity of studies comparing in detail the costs and long-term clinical outcomes of TELD and open microdiskectomy (MD), especially in developing countries. Thus, we sought to provide a multidimensional insight into this matter by comparing the direct costs and long-term outcomes of TELD with those of MD. METHODS: The electronic health records of 434 patients with LDH who underwent either TELD or MD were collected from February 2011 to October 2014. Within a 7-year follow-up period, 412 patients, comprising 203 patients treated with TELD and 209 patients treated with MD, were fully evaluated. Patient characteristics, operative time, intraoperative blood loss (IBL), postoperative hospital stay, time to return to work (RTW), perioperative complications, and direct costs were collected. Clinical outcomes were assessed using the Visual Analog Scale (VAS), Oswestry Disability Index (ODI), and modified MacNab criteria. RESULTS: The postoperative ODI and VAS scores improved significantly in both groups (p < 0.001). In accordance with the modified MacNab criteria, the rate of excellent and good outcomes was 88.67 and 88.03% in the TELD and MD groups, respectively. There were no significant differences between the groups in the clinical outcomes and perioperative complications. However, IBL, hospital stay, and RTW were significantly reduced in the TELD group (p < 0.05). Twenty-one cases in the TELD group and nine in the MD group underwent reoperation due to recurrence (p < 0.05). Total inpatient cost per patient was $1,596 in the TELD group and $1,990 in the MD group (p < 0.05). CONCLUSION: TELD for the treatment of symptomatic LDH could be an affordable strategy, providing certain advantages of minimally invasive procedures such as shorter hospital stay and earlier recovery along with comparable clinical outcomes to the conventional surgical method.

High genetic heterogeneity of leukodystrophies in Iranian children: the first report of Iranian Leukodystrophy Registry.

Leukodystrophies (LDs) are a heterogeneous group of progressive neurological disorders and characterized by primary involvement of white matter of the central nervous system (CNS). This is the first report of the Iranian LD Registry database to describe the clinical, radiological, and genomic data of Persian patients with leukodystrophies. From 2016 to 2019, patients suspicious of LDs were examined followed by a brain magnetic resonance imaging (MRI). A single gene testing or whole-exome sequencing (WES) was used depending on the neuroradiologic phenotypes. In a few cases, the diagnosis was made by metabolic studies. Based on the MRI pattern, diagnosed patients were divided into cohorts A (hypomyelinating LDs) versus cohort B (Other LDs). The most recent LD classification was utilized for classification of diagnosed patients. For novel variants, in silico analyses were performed to verify their pathogenicity. Out of 680 registered patients, 342 completed the diagnostic evaluations. In total, 245 patients met a diagnosis which in turn 24.5% were categorized in cohort A and the remaining in cohort B. Genetic tests revealed causal variants in 228 patients consisting of 213 variants in 110 genes with 78 novel variants. WES and single gene testing identified a causal variant in 65.5% and 34.5% cases, respectively. The total diagnostic rate of WES was 60.7%. Lysosomal disorders (27.3%; GM2-gangliosidosis-9.8%, MLD-6.1%, KD-4.5%), amino and organic acid disorders (17.15%; Canavan disease-4.5%, L-2-HGA-3.6%), mitochondrial leukodystrophies (12.6%), ion and water homeostasis disorders (7.3%; MLC-4.5%), peroxisomal disorders (6.5%; X-ALD-3.6%), and myelin protein disorders (3.6%; PMLD-3.6%) were the most commonly diagnosed disorders. Thirty-seven percent of cases had a pathogenic variant in nine genes (ARSA, HEXA, ASPA, MLC1, GALC, GJC2, ABCD1, L2HGDH, GCDH). This study highlights the most common types as well as the genetic heterogeneity of LDs in Iranian children.

An unusual site of a pericardial cyst in a child: A case report.

Pericardial cysts are rare congenital anomalies, often clinically silent and incidentally found on imaging. However, patients with pericardial cysts may present with chest pain, tachypnea, and, rarely, symptoms secondary to cardiac tamponade. Echocardiography (transthoracic or transesophageal) and chest computed tomography (CT) scan with contrast are diagnostic modalities of choice in patients with pericardial cysts. Conservative management is justified in asymptomatic patients, while a surgical approach is recommended in symptomatic patients. Here, we describe the case of a 12-year-old boy who underwent imaging during the coronavirus disease 2019 (COVID-19) pandemic and was incidentally found to have a pericardial cyst.

Expanding the neuroimaging findings of guanidinoacetate methyltransferase deficiency in an Iranian girl with a homozygous frameshift variant in the GAMT.

Guanidinoacetate methyltransferase deficiency (GAMTD) is a treatable neurodevelopmental disorder with normal or nonspecific imaging findings. Here, we reported a 14-month-old girl with GAMTD and novel findings on brain magnetic resonance imaging (MRI).A 14-||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||month-old female patient was referred to Myelin Disorders Clinic due to onset of seizures and developmental regression following routine vaccination at 4 months of age. Brain MRI, prior to initiation of treatment, showed high signal intensity in T2-weighted imaging in bilateral thalami, globus pallidus, subthalamic nuclei, substantia nigra, dentate nuclei, central tegmental tracts in the brainstem, and posterior periventricular white matter which was masquerading for mitochondrial leukodystrophy. Basic metabolic tests were normal except for low urine creatinine; however, exome sequencing identified a homozygous frameshift deletion variant [NM_000156: c.491del; (p.Gly164AlafsTer14)] in the GAMT. Biallelic pathogenic or likely pathogenic variants cause GAMTD. We confirmed the homozygous state for this variant in the proband, as well as the heterozygote state in the parents by Sanger sequencing.MRI features in GAMTD can mimic mitochondrial leukodystrophy. Pediatric neurologists should be aware of variable MRI findings in GAMTD since they would be misleading to other diagnoses.

A Practical Index to Distinguish Backwash Ileitis From Crohn's Terminal Ileitis in MR Enterography.

BACKGROUND: Differentiating ulcerative colitis-associated "backwash" ileitis (BWI) from Crohn's terminal ileitis (CTI) is a diagnostic challenge and highly affects patient's management. This study aimed to investigate magnetic resonance enterography (MRE) features including ileocecal valve patency index (ICPI) in patients with BWI and CTI and distinguish these entities based on MRE findings. METHODS: After obtaining institutional review board approval, we reviewed 1654 MREs; 60 patients with pathologically proven BWI (n = 30) and CTI (n = 30) were enrolled. Two radiologists who were blinded to the clinical diagnosis analyzed MREs. We evaluated bowel wall thickness and enhancement pattern, ileocecal valve (ICV) diameter, and lip thickness. Ileocecal valve patency index-T and ICPI-C were calculated to normalize the ICV diameter with respect to terminal ileum (TI) and cecum, respectively. An additional group of non-BWI-UC patients (n = 30) was also included to validate indices. RESULTS: Circumferential mural thickening (90% vs 1%, P < .001) and inner-wall enhancement (P < .001) of TI were more frequent in BWI patients than CTI. Serosal irregularity (53% vs 13%, P = .002), higher mural thickness (5mm vs 3mm, P < .001), and asymmetric hyperenhancement (P < .001) of TI were more prevalent in CTI than BWI. Ileocecal valve patency and lip atrophy were significantly higher in BWI than CTI and non-BWI-UC groups (both P < .001). Ileocecal valve patency indices-C and ICPI-T indices were able to accurately distinguish BWI from CTI (area under the ROC curve [AUC], 0.864 and 0.847 for ICPI-T and ICPI-C, respectively) and non-BWI-UC (AUC, 0.777 and 0.791 for ICPI-T and ICPI-C, respectively). Ileocecal valve patency indices-T  ≥31.5% were 100% specific to distinguish BWI from CTI, but sensitivity was 63%. CONCLUSIONS: Magnetic resonance enterography features of ICV and TI can accurately differentiate BWI from CTI. Two practical indices introduced in this study showed high specificity to distinguish BWI from CTI.

An atypical imaging characteristic of calvarial metastasis of neuroblastoma as multiple multi-loculated cystic masses with internal blood-fluid levels: a case report.

BACKGROUND: As the third most common malignancy of childhood, Neuroblastoma has a great propensity to metastasize to multiple organs. The most common site of metastasis is the bone and bone marrow. Concerning the central nervous system, neuroblastoma usually involves the calvarium and the external dural surface. The skull metastases may show different appearances, including: multiple lytic bone lesions, bone thickening, hair-on-end periosteal reaction, irregular suture widening and/or plaque like epidural deposits. Here we present a case of metastatic neuroblastoma, appearing as multiple multi-loculated cystic epidural masses with internal blood-fluid levels as a rare imaging manifestation of calvarial metastasis. CASE PRESENTATION: An 8-year-old boy with known history of autism, presented to the emergency department with a 3-month history of intermittent fever, malaise and myalgia and headache along with significant weight loss. Laboratory examination revealed elevated ESR and CRP and anemia. On Abdomino-Pelvic imaging a well-defined, 45*30*24 mm, solid-cystic mass was observed, replacing the normal left adrenal gland. On brain MRI, multiple multi-loculated cystic, lentiform masses were observed on the external surface of cerebral hemispheric dura. Multiple fluid-fluid levels were noted in the locules in some of which the dependent fluid was hyperintense on T1w and FLAIR and hypointense on T2w sequences, compatible with blood, representing blood-fluid level. The wall and septa of the masses, enhanced after contrast administration. Associated abnormal marrow signal and aggressive type periosteal reactions were identified in the overlying bone. All of the lesions had increased uptake in MIBG scan. Bone marrow biopsy revealed small round cells, diagnostic for neuroblastoma. The patient underwent chemotherapy treatment. All calvarial/epidural metastatic lesions resolved after chemotherapy and residual adrenal tumor was resected. CONCLUSION: Cystic epidural lesions, especially when associated with adjacent abnormal bone marrow signal, or periosteal reaction and containing blood-fluid level should raise the suspicion of a calvarial metastasis.

The clinical significance of biliary findings in magnetic resonance enterography of patients with inflammatory bowel disease.

Purpose: Given the association of inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC), we aimed to investigate the clinical relevance of abnormal hepatobiliary findings on magnetic resonance enterography (MRE) of IBD patients considering the risk of over- or underestimation of PSC at MRE. Material and methods: Using the MRE dataset of patients referring to a tertiary hospital and the National Registry of Crohn's and Colitis, 69 MREs, including 23 IBD-PSC, 23 IBD-without PSC, and 23 healthy controls (HC), were retrospectively reviewed by 2 experienced radiologists blinded to the clinical data, to evaluate hepatobiliary abnormalities. Sensitivity, specificity, and likelihood ratios were calculated. Results: Bile duct irregularities were the most common finding in the IBD-PSC group, with a frequency of 91%. Intra- and extrahepatic bile duct (IHBD and EHBD) irregularities were observed in 87% and 78% of PSC patients, respectively. Higher frequency of IHBD and EHBD wall thickening, bile duct dilation, EHBD stricture, and periportal oedema were observed in the IBD-PSC group. Peribiliary T2-weighted hyperintensities and contrast-enhancement were significantly more common in the IBD-PSC group than in the IBD and HC groups (48% and 35%, respectively) (p < 0.001). Detection of biliary irregularities on MRE had a specificity of 94% (95% CI: 82-99%), a sensitivity of 91% (95% CI: 72-99%), and a positive likelihood ratio of 14.0 (95% CI: 4.7-42.1) for the diagnosis of PSC. Conclusions: This study emphasizes the importance of assessing and reporting hepatobiliary abnormalities visible in the MRE of patients with IBD to avoid a delayed diagnosis of PSC.

The value of bronchocele attenuation in pulmonary computed tomography in assessment of allergic bronchopulmonary aspergillosis in the background of cystic fibrosis: A cross-sectional study.

Background: There is no specific test in the definitive diagnostic approach to Allergic bronchopulmonary aspergillosis (ABPA) especially in the background of cystic fibrosis, but comprehensive and simultaneous clinical, radiological and serological examination will be the basis of ABPA diagnosis. The increasing in attenuation of bronchoceles in imaging has recently been proposed as a valuable diagnostic criterion. Purpose: The present study aimed to assess bronchocele attenuation in pulmonary CT scan of patients with complicated cystic fibrosis for diagnosis of ABPA. Methods: This cross-sectional study was performed on 74 consecutive patients aged 3-18 years suffering cystic fibrosis presented with exacerbation of pulmonary symptoms and were suspected of having ABPA. All were examined by 16 Slice CT Scan and the density of bronchoceles above 5 mm in diameter were measured in Hounsfield unit. The total serum IgE titer, skin prick test for aspergillus and anti-aspergillus IgG and IgE level were obtained for all subjects and both cutoff values of IgE level (>500 IU/mL and >1000 IU/mL) were considered as the criteria for ABPA diagnosis. Results: Considering IgE level of greater than 500 IU/mL and 1000 IU/mL as the diagnostic criteria, 24.3% and 10.8% had evidence of ABPA, respectively. Considering the two pointed diagnostic IgE ranges and based on the analysis of the area under the ROC curve, bronchocele attenuation could effectively predict the presence of ABPA with the best cutoff values of 37.25 (with a sensitivity of 70.6% and a specificity of 66.7%) and 40.00 (with a sensitivity of 85.7% and a specificity of 65.1%), respectively. Conclusion: The presence of bronchocele and an increase in its attenuation on CT scan will be diagnostic for the occurrence of ABPA.

COVID-19 Pandemic Experiences in Pediatric Intensive Care Unit: An Iranian Referral Hospital-Based Study.

Introduction: In late February 2020, after we had informed about the presence of some cases of COVID-19 in Iran and its rapid spread throughout the country, we decided to make the necessary arrangements for patients with critical conditions in Pediatric Intensive Care Unit (PICU) at Children's Medical Center. There are a little data on critically ill children with COVID-19 infection with ICU requirements. The aim of this study was to describe clinical characteristics, laboratory parameters, treatment, and outcomes of the pediatrics population infected by SARS-CoV-2 admitted to PICU. Materials and Methods: This study was performed between February 2020 and May 2020 in the COVID PICU of the Children's Medical Center Hospital in Tehran, Iran. Patients were evaluated in terms of demographic categories, primary symptoms and signs at presentation, underlying disease, SARS-CoV-2 RT-PCR test result, laboratory findings at PICU admission, chest X-ray (CXR) and lung CT findings, and treatment. Moreover, the need to noninvasive ventilation (NIV) or mechanical ventilation, the length of hospital stay in the PICU, and outcomes were assessed. Results: In total, 99 patients were admitted to COVID PICU, 42.4% (42 patients) were males, and 66 patients had positive SARS-CoV-2 real-time reverse transcriptase-polymerase chain reaction (RT-PCR). There was no statistically significant difference in the frequency of clinical signs and symptoms (except for fever) among patients with positive SARS-CoV-2 RT-PCR and negative ones. Among all admitted patients, the presence of underlying diseases was noticed in 81 (82%) patients. Of 99 patients, 34 patients were treated with NIV during their admission. Furthermore, 35 patients were intubated and treated with mechanical ventilation. Unfortunately, 11 out of 35 mechanically ventilated patients (31%) passed away. Conclusion: No laboratory and radiological findings in children infected with COVID-19 were diagnostic in cases with COVID-19 admitted to PICU. There are higher risks of severe COVID-19, PICU admission, and mortality in children with comorbidities.

Symptomatic care of late-onset Alexander disease presenting with area postrema-like syndrome with prednisolone; a case report.

BACKGROUND: Alexander disease (AxD) is classified into AxD type I (infantile) and AxD type II (juvenile and adult form). We aimed to determine the potential genetic cause(s) contributing to the AxD type II manifestations in a 9-year-old male who presented area postrema-like syndrome and his vomiting and weight loss improved after taking prednisolone. CASE PRESENTATION: A normal cognitive 9-year-old boy with persistent nausea, vomiting, and a significant weight loss at the age of 6 years was noticed. He also experienced an episode of status epilepticus with generalized atonic seizures. He showed non-febrile infrequent multifocal motor seizures at the age of 40 days which were treated with phenobarbital. He exhibited normal physical growth and neurologic developmental milestones by the age of six. Occasionally vomiting unrelated to feeding was reported. Upon examination at 9 years, a weak gag reflex, prominent drooling, exaggerated knee-deep tendon reflexes (3+), and nasal tone speech was detected. All gastroenterological, biochemical, and metabolic assessments were normal. Brain magnetic resonance imaging (MRI) revealed bifrontal confluent deep and periventricular white matter signal changes, fine symmetric frontal white matter and bilateral caudate nucleus involvements with garland changes, and a hyperintense tumefactive-like lesion in the brain stem around the floor of the fourth ventricle and area postrema with contrast uptake in post-contrast T1-W images. Latter MRI at the age of 8 years showed enlarged area postrema lesion and bilateral middle cerebellar peduncles and dentate nuclei involvements. Due to clinical and genetic heterogeneities, whole-exome sequencing was performed and the candidate variant was confirmed by Sanger sequencing. A de novo heterozygous mutation, NM_001242376.1:c.262 C > T;R88C in exon 1 of the GFAP (OMIM: 137,780) was verified. Because of persistent vomiting and weight loss of 6.0 kg, prednisolone was prescribed which brought about ceasing vomiting and led to weight gaining of 3.0 kg over the next 3 months after treatment. Occasional attempts to discontinue prednisolone had been resulting in the reappearance of vomiting. CONCLUSIONS: This study broadens the spectrum of symptomatic treatment in leukodystrophies and also shows that R88C mutation may lead to a broad range of phenotypes in AxD type II patients.

Cholecysto-hepatic duct serving as the only drainage pathway of bile from the intrahepatic to the extrahepatic biliary system in an infant: a case report.

BACKGROUND: Cholecystohepatic duct is a rare anomaly of the biliary system which involves drainage of bile into the gallbladder which may be associated with agenesis of the common hepatic duct or common bile duct. CASE PRESENTATION: A 2.5-month-old infant presented to our emergency department with icterus. He had a history of esophageal atresia and imperforate anus which had been treated surgically by thoracotomy, esophagostomy, gastrostomy and colostomy placement. Following imaging studies by ultrasound and MRCP, the diagnosis of common hepatic duct agenesis was made. Cholecystohepatic duct was present as the solitary drainage pathway of bile from the intrahepatic to extrahepatic biliary system. CONCLUSIONS: Cholecystohepatic ducts need a high index of suspicion to be diagnosed on preoperative hepatobiliary imaging. As they may be asymptomatic, they are predisposed to iatrogenic injury during hepatobiliary surgeries.

Fetal corpus callosum abnormalities: Ultrasound and magnetic resonance imaging role.

The corpus callosum (CC) is the major interhemispheric commissure and its abnormalities include agenesis, hypoplasia, and hyperplasia. The CC anomalies are typically related to other central nervous system (CNS) or extra-CNS malformations. The antenatal diagnosis of complete CC agenesis is easy after mid-trimester by ultrasound (US) even in the axial plane. The non-visualization of cavum septum pellucidum and colpocephaly are critical signs in the axial view. More subtle findings (i.e., hypoplasia and partial agenesis) might also be recognized antenatally. In this review, the focus was given on the prenatal diagnosis of CC abnormalities in US and magnetic resonance imaging.

The safety and efficacy of umbilical cord blood mononuclear cells in individuals with spastic cerebral palsy: a randomized double-blind sham-controlled clinical trial.

INTRODUCTION: The current multi-center, randomized, double-blind study was conducted among children with cerebral palsy (CP) to assess the safety and efficacy of umbilical cord blood mononuclear cell (UCB-MNC). We performed the diffusion tensor imaging to assess the changes in the white matter structure. METHODS: Males and females aged 4 to 14 years old with spastic CP were included. Eligible participants were allocated in 4:1 ratio to be in the experimental or control groups; respectively. Individuals who were assigned in UCB-MNC group were tested for human leukocyte antigen (HLA) and fully-matched individuals were treated with UCB-MNCs. A single dose (5 × 106 /kg) UCB-MNCs were administered via intrathecal route in experimental group. The changes in gross motor function measure (GMFM)-66 from baseline to one year after treatment were the primary endpoints. The mean changes in modified Ashworth scale (MAS), pediatric evaluation of disability inventory (PEDI), and CP quality of life (CP-QoL) were also evaluated and compared between groups. The mean changes in fractional anisotropy (FA) and mean diffusivity (MD) of corticospinal tract (CST) and posterior thalamic radiation (PTR) were the secondary endpoints. Adverse events were safety endpoint. RESULTS: There were 72 included individuals (36 cases in each group). The mean GMFM-66 scores increased in experimental group; compared to baseline (+ 9.62; 95%CI: 6.75, 12.49) and control arm (ß: 7.10; 95%CI: 2.08, 12.76; Cohen's d: 0.62) and mean MAS reduced in individuals treated with UCB-MNCs compared to the baseline (-0.87; 95%CI: -1.2, -0.54) and control group (ß: -0.58; 95%CI: -1.18, -0.11; Cohen's d: 0.36). The mean PEDI scores and mean CP-QoL scores in two domains were higher in the experimental group compared to the control. The imaging data indicated that mean FA increased and MD decreased in participants of UCB-MNC group indicating improvements in white matter structure. Lower back pain, headaches, and irritability were the most common adverse events within 24 h of treatment that were related to lumbar puncture. No side effects were observed during follow-up. CONCLUSIONS: This trial showed that intrathecal injection of UCB-MNCs were safe and effective in children with CP. TRIAL REGISTRATION: The study was registered with ClinicalTrials.gov ( NCT03795974 ).

Abdominal Imaging Findings in Patients with COVID-19 Part 2: Solid Organs.

Since severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) first appeared in China in December 2019, the globe has been dealing with an ever-increasing incidence of coronavirus disease 2019 (COVID-19). In addition to respiratory disorders, 40% of patients present with gastrointestinal (GI) involvement. Abdominal pain is the most common indication for computed tomography (CT) and ultrasonography. After GI tract involvement, solid visceral organ infarction is the most prevalent abdominal abnormality in COVID-19. This review aims to gather the available data in the literature about imaging features of solid abdominal organs in patients with COVID-19. Gallbladder wall thickening and distension, cholelithiasis, hyperdense biliary sludge, acalculous cholecystitis, periportal edema, heterogeneous liver enhancement, and liver hypodensity and infarction are among hepatobiliary imaging findings in CT, particularly in patients admitted to ICU. Pancreatic involvement can develop as a result of direct SARS-CoV2 invasion with signs of acute pancreatitis in abdominal CT, such as edema and inflammation of the pancreas. Infarction was the most prevalent renal and splenic involvement in patients with COVID-19 who underwent abdominal CT presenting with areas of parenchymal hypodensity. In conclusion, although solid abdominal organs are rarely affected by COVID-19, clinicians must be familiar with the manifestations since they are associated with the disease severity and poor outcome.

A multicenter retrospective study of clinical features, laboratory characteristics, and outcomes of 166 hospitalized children with coronavirus disease 2019 (COVID-19): A preliminary report from Iranian Network for Research in Viral Diseases (INRVD).

BACKGROUND: The objectives of this study were to analyze the clinical features and laboratory profiles and risk factors associated with critical illness of children with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). METHODS: One hundred and sixty-six coronavirus disease 2019 (COVID-19) Iranian pediatric patients were recruited through a collaborative research network between March and May 2020. Demographics, clinical, laboratory, and radiological results were obtained from patient files. RESULTS: Of 166 patients, 102 (61%) and 64 (39%) were males and females, respectively. Ninety-six (57.8%) and 70 (42.2%), had moderate and severe conditions, respectively. Thirty (18%) of patients died. The common symptoms were fever (73%), cough (54%), and shortness of breath, headache decrease in neutrophil and platelet counts; increase values in lactate dehydrogenase, decrease in the blood pH and HCO3 were significantly associated with the disease severity. 54% and 56% of patients showed abnormal radiographic appearance in Chest X-ray and in chest computed tomography scan, respectively. Sixty-one (36.7%) of patients were referred to intensive care unit (ICU). The coexistence of comorbidity was the main factor associated with ICU admission, shock, arrhythmia, acute kidney injury, acute respiratory distress syndrome, acute cardiac injury, and death. CONCLUSIONS: We describe a higher than previously recognized rate of COVID-19 mortality in Iranian pediatric patients. Epidemiological factors, such as the relatively high case fatality rate in the country and the presence of underlying diseases were the main factors for the high death rate.

Role of Percutaneous Laser Disc Decompression in Patients with Lumbar Disc Herniation on Pain Relief: A Quasi-Experimental Pilot Study:.

Background: Disc herniation is broadly defined as a localized or focal displacement of disc material beyond the limits of the intervertebral disc space. The disc material may be the nucleus, cartilage, fragmented apophyseal bone, annular tissue, or any combination thereof. Laser surgery is one of the treatment modalities for treating patients with lumbar disc herniation. This study aims to examine the effect of Percutaneous Laser Disc Decompression (PLDD) in patients with lumbar disc herniation. Materials and Methods: This study was conducted on 58 patients who underwent PLDD (optical fiber inserted through an 18G needle, 8 joules, and 8 watts). Individuals were monitored before and after treatment using the comparing visual analog scale (VAS) pain score (from 0 [no pain] to 10 [severe pain]). Results: The mean age of participants was 63.19±13.48 years. Regarding gender, 24 patients (41.4%) were female. The mean VAS score before surgery was 8.73±1.29, and VAS score after surgery was 55.2±2.71, which means pain was significantly reduced (P0.001). Conclusion: The patients' post-PLDD pain may decrease; hence, PLDD can use as an appropriate method for treating lumbar disc herniation.

Findings of Abdominal Imaging in Patients with COVID-19 - Part 1: Hollow Organs.

Since COVID-19 has spread worldwide, the role of imaging for early detection of the disease has become more prominent. Abdominal symptoms in COVID-19 are common in addition to respiratory manifestations. This review collected the available data about abdominal computed tomography (CT) and ultrasonography indications in hollow abdominal organs in patients with COVID-19 and their findings. Since abdominal imaging is less frequently used in COVID-19, there is limited information about the gastrointestinal findings. The most common indications for abdominal CT in patients with COVID-19 were abdominal pain and sepsis. Bowel wall thickening and fluid-filled colon were the most common findings in abdominal imaging. Acute mesenteric ischemia (AMI) was one of the COVID-19 presentations secondary to coagulation dysfunction. AMI manifests with sudden abdominal pain associated with high morbidity and mortality in admitted patients; therefore, CT angiography should be considered for early diagnosis of AMI. Ultrasonography is a practical modality because of its availability, safety, rapidity, and ability to be used at the bedside. Clinicians and radiologists should be alert to indications and findings of abdominal imaging modalities in COVID-19 to diagnose the disease and its potentially serious complications promptly.