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In 2021, Suwala et al. described Primary Mismatch Repair Deficient IDH‐mutant Astrocytoma (PMMRDIA) as a distinct group of gliomas. In unsupervised clustering, PMMRDIA forms distinct cluster, separate from other IDH‐mutant gliomas, including IDH‐mutant gliomas with secondary mismatch repair (MMR) deficiency. In the published cohort, three patients received treatment with an immune checkpoint blocker (ICB), yet none exhibited a response, which aligns with existing knowledge about the decreased immunogenicity of IDH‐mutant gliomas in comparison to IDH‐wildtype. In the case of PMMRDIA, the inherent resistance to the standard‐of‐care temozolomide caused by MMR deficiency is an additional challenge. It is known that a gain‐of‐function mutation of IDH1/2 genes produces the oncometabolite R‐2‐hydroxyglutarate (R‐2‐HG), which increases DNA and histone methylation contributing to the characteristic glioma‐associated CpG island methylator phenotype (G‐CIMP). While other factors could be involved in remodeling the tumor microenvironment (TME) of IDH‐mutant gliomas, this systematic review emphasizes the role of R‐2‐HG and the subsequent G‐CIMP in immune suppression. This highlights a potential actionable pathway to enhance the response of ICB, which might be relevant for addressing the unmet therapeutic challenge of PMMRDIA. [ABSTRACT FROM AUTHOR]

Objective The goal of this study was to obtain population-based data on the incidence, clinical presentation, management, imaging features, and long-term outcomes of patients with all types of neonatal stroke (NS). Methods Full-term neonates with NS born between January 2007 and December 2013 were identified through the Nova Scotia Provincial Perinatal Follow-up Program Database. Perinatal data and neonatal course were reviewed. Neurodevelopmental outcomes were assessed at 18 and 36 months of age using standardized testing. Results Twenty-nine neonates with NS were identified during the study period, giving an incidence of 47 per 100,000 live births in Nova Scotia. Arterial ischemic stroke was the most common stroke type (76%), followed by neonatal hemorrhagic stroke (17%), then cerebral sinovenous thrombosis (7%). The majority of neonates presented with seizures (86%) on the first day of life (76%). At 36 months of age, 23 (79%) of the children had a normal outcome, while 3 (10%) were diagnosed with cerebral palsy (2 with neonatal arterial stroke and one with neonatal hemorrhagic stroke) and 3 (10%) had recurrent seizures (1 patient from each stroke subtype group). Conclusion The incidence of NS in Nova Scotia is higher than what has been reported internationally in the literature. However, the neurodevelopmental outcomes at 3 years of age are better. Further studies are required to better understand the reasons for these findings. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this article is to determine whether hepatic artery (HA) Doppler parameters can be used to predict pediatric liver graft status in the immediate postoperative (< 10 days) period and in the first year after transplant., Materials and Methods: A retrospective review of the liver transplant database was performed to review operative, clinical, laboratory, histopathologic, and ultrasound findings at three time points after surgery: 3 days, 3 months, and 1 year. The association between HA velocity and resistive index (RI) with graft status was assessed., Results: Of 120 children (54 girls and 66 boys; mean age, 3 years 2 months) enrolled, 70 had satisfactory graft status at the 1-year follow-up examination. HA velocity of 50-200 cm/s at 3 days was associated with normal graft status during the immediate postoperative period (p = 0.003), at 3 months (p = 0.0653), and at the 1-year follow-up examination (p = 0.0268). Vascular and biliary complications in the immediate postoperative period were more frequent at an HA velocity of 201-300 cm/s (p = 0.0024). There was a significant association between RI at 3 days and graft status in the immediate postoperative period (p = 0.0308), with an RI less than 0.5 associated with vascular complications (p = 0.0116)., Conclusion: An RI less than 0.5 is associated with vascular complications. An HA velocity of 50-200 cm/s and an RI of 0.5-0.8 are associated with normal graft status in children within the first year after transplant. Values outside this range, or in this range along with a constellation of other ultrasound findings and clinical status, support the initiation of additional more-specific investigations that may help in early treatment to prevent complications.

Objective: The objective of this study was to assess the diagnostic agreement between bowel sonography and MR enterography in children with suspected or confirmed inflammatory bowel disease (IBD)., Subjects and Methods: We prospectively studied 33 children undergoing clinically indicated MR enterography. Bowel sonography was performed within 2 hours before MR enterography. The reliability of each modality was assessed by computing the interreader kappa agreement on nine inflammatory markers in eight bowel segments and the interreader intraclass correlation coefficients (ICC) in each bowel segment. The agreement between both modalities in each bowel segment was also calculated using the Kendall coefficient of concordance (KCC) in addition to the sensitivity and specificity of sonography using those of MR enterography as the reference standard., Results: Sonographic readings are substantially reliable in the colon and terminal and distal ileum (ICC = 0.79-0.88) but are highly unreliable in the mid and proximal small bowel. MR enterography has moderate to almost perfect reliability for all bowel segments (ICC = 0.43-0.96). Sonography was in substantial agreement with MR enterography in most of the large bowel (KCC = 0.67-0.83) and terminal and distal ileum (KCC = 0.75) and in moderate agreement in the remaining bowel segments (KCC = 0.45-0.48). Sonographic findings are specific for disease using MR enterography as the reference standard in all bowel segments (0.83-1.00), but sensitivity markedly varies according to disease location., Conclusion: Sonography is highly specific and shows substantial agreement with MR enterography in detecting inflammatory and damage changes in the colon and terminal and distal ileum, the segments most likely to be affected by IBD. Therefore, sonography can be a good complement to MR enterography in evaluations of patients with known or suspected IBD, particularly if MR enterography access is limited.

Objectives: To present our experience in operated meningioma cases regarding their prevalence, anatomical location, multiplicity, presenting signs and symptoms, and the possible correlation between MRI signal intensity and histological grades to set criteria for radio-pathological diagnosis., Methods: In this retrospective study, operated meningioma cases in the Department of Neurosurgery, Jordan University Hospital (JUH), Amman, Jordan between January 1997 and January 2007 were reviewed. Our study included 90 cases, and their medical records, histopathological reports, and neuroimages were analyzed thoroughly., Results: Meningioma was more common in females than males with a ratio of 2.2:1. Para-sagittal meningiomas were the most common (23.3%). Multiple intracranial meningiomas were found in 4.4% of the cases. Most cases were of benign histopathology and exhibited iso-intense signals on T1 and T2, and appeared with hyper-intense signals on FLAIR with vivid enhancement., Conclusion: The prevalence of meningioma among genders and its anatomical location at JUH corresponds to the published medical literature worldwide. There was no correlation between signal intensities (as seen on T1WI, T2WI, and FLAIR sequences), enhancement pattern on one side, and histological grades on the other side.