Myelodysplastic Syndrome

Pediatric myelodysplastic syndrome (MDS), though rare, constitutes a distinct entity quite different from adult MDS. They have unique clinical features, aggressive clinical course with an overall mean survival of only 9.9 months. A pediatric approach to the WHO classification has become necessary since the WHO classification of MDS has failed to address the uniqueness of pediatric MDS. A new prognostic system also needs to be evolved since the international prognostic system has limited prognostic impact in children. Intensive chemotherapy such as the one used in de novo-acute myeloid leukemia (AML) leads to complete remission in some children and this may be the treatment of choice in pediatric MDS.     

Usability testing of multimodal feedback interface and simulated collision-avoidance power wheelchair for long-term-care home residents with cognitive impairments

Many older adults in long-term-care homes have complex physical and cognitive impairments and have difficulty propelling manual wheelchairs. Power wheelchair use is restricted owing to safety concerns. Power wheelchairs with collision-avoidance features are being developed to enable safe and independent mobility; however, a paucity of information exists on interface features to help users navigate away from obstacles. We developed a system combining an interface with auditory, visual, and haptic feedback and a simulated collision-avoidance power wheelchair. This device allowed the investigator to stop movement of the power wheelchair when users approached obstacles and to deliver feedback to help them navigate. Five long-term-care home residents with mild or moderate cognitive impairments evaluated device usability, which included effectiveness, efficiency, and user satisfaction. Each resident used the device for six 1 h sessions. Observations, feedback interviews, and outcome questionnaires were completed during and after the sessions. We found the device effective in enabling residents to achieve basic driving tasks and self-identified indoor mobility goals. Furthermore, residents perceived workload to be low and were satisfied with the device. Residents also felt that the feedback was useful to help them navigate away from obstacles.     

Tunneled biopsy is an underutilised,simple, safe and efficient method for tissue acquisition from subepithelial tumours

BACKGROUNDTissue acquisition from subepithelial lesions is often attempted by endoscopic ultrasound (EUS)-sampling as conventional endoscopic biopsy usually fails to reach deeper layers of the gastrointestinal wall. AIMTo investigate the utilisation, safety and diagnostic yield of an intensified “bite-on-bite” tunnel biopsy technique. METHODSIn this retrospective cohort study, all patients presenting with subepithelial masses in the upper gastrointestinal tract from March 2013 to July 2019 were included. Data were analysed for size and location of the subepithelial mass, use of intensified tunnel biopsy protocol (more than 10 double bite-on-bite biopsies) or superficial conventional biopsies, histology and imaging results, occurrence of readmission and adverse events after endoscopy.RESULTSTwo hundred and twenty-nine patients with subepithelial lesions were included. Superficial conventional biopsies were taken in 117 patients and were diagnostic only in one lipoma (0.9 %). Tunnel biopsies taken in 112/229 (48.9%) patients were significantly more likely to provide histological diagnosis (53.6%; P < 0.001). For lesions ≥ 10mm the diagnostic yield of tunnel biopsies further increased to 41/67 (61.2%). No immediate or delayed complications were reported. Only 8 of the 51 endoscopists (15.7%) regularly attempted tunnel biopsies. CONCLUSIONTunnel biopsy is a simple, safe and efficient but underutilised diagnostic modality for tissue acquisition in subepithelial masses. It should be routinely attempted at the initial endoscopy.     

Relationship Between Measures of Cerebrovascular Reactivity and Intracranial Lesion Progression in Acute TBI Patients: an Exploratory Analysis

Neurocritical Care - Failure of cerebral autoregulation and progression of intracranial lesion have both been shown to contribute to poor outcome in patients with acute traumatic brain injury...     

Regionally selective atrophy of subcortical structures in prodromal HD as revealed by statistical shape analysis

Huntington disease (HD) is a neurodegenerative disorder that involves preferential atrophy in the striatal complex and related subcortical nuclei. In this article, which is based on a dataset extracted from the PREDICT‐HD study, we use statistical shape analysis with deformation markers obtained through “Large Deformation Diffeomorphic Metric Mapping” of cortical surfaces to highlight specific atrophy patterns in the caudate, putamen, and globus pallidus, at different prodromal stages of the disease. On the basis of the relation to cortico‐basal ganglia circuitry, we propose that statistical shape analysis, along with other structural and functional imaging studies, may help expand our understanding of the brain circuitry affected and other aspects of the neurobiology of HD, and also guide the most effective strategies for intervention. Hum Brain Mapp 35:792–809, 2014. © 2012 Wiley Periodicals, Inc.     

Accuracy of magnetic resonance imaging for preoperative detection of portal vein thrombosis in liver transplant candidates.

The detection of main portal vein thrombosis (PVT) on preoperative imaging of liver transplant candidates has important technical implications for the transplantation procedure. Data are scarce regarding the accuracy of magnetic resonance imaging (MRI) at detecting PVT. The aim of our study was to compare preoperative findings of the portal vein on MRI to operative findings at liver transplantation. Abdominal MRI and clinical records of 172 consecutive patients who received liver transplants between January 1999 and September 2004 were reviewed. Two radiologists independently evaluated the last abdominal magnetic resonance examinations obtained before liver transplantation, blinded to the original reading, operative findings, and clinical data. Findings on MRI were compared with intraoperative findings at transplantation. Main PVT was detected in 12 patients, in whom 8 were found to have thrombus at surgery, with 6 requiring a jump graft or thrombectomy. Sensitivity and specificity of MRI for detecting main PVT were 100% and 98%, respectively. The cause of discordance between findings on MRI and at transplantation in 2 cases was a diminutive caliber of the main portal vein that was interpreted as recanalized chronic thrombosis on MRI. In conclusion, in our study group MRI detected PVT in all liver transplant recipients requiring jump grafts at transplantation. The major reason for a false-positive MRI was a diminutive but patent portal vein.     

Amygdala volume analysis in female twins with major depression.

BACKGROUND: Previous research examining the amygdala volumes in major depressive disorder (MDD) has found conflicting evidence for association. Furthermore, few of these studies have examined differences in individuals with an onset during childhood or adolescence. This study examined amygdala volume and its potential association with early onset major depression. METHODS: A community-based sample of 47 right-handed young adult female monozygotic and dizygotic twin pairs was examined. For 29 twin pairs, one twin per pair had a lifetime history of MDD, while 18 age-matched control twin pairs had no lifetime history of MDD or other Axis I disorder. Core, noncore, and total amygdala volumes were estimated based on a combination of manual tracing, automated segmentation, and expert rater regional boundary definitions. RESULTS: No significant differences were found in amygdala volumes between depressed, high-risk, or control subjects. However, analyses comparing control monozygotic twins to randomly created control subject pairs suggest that there are familial, perhaps genetic, influences on core and total amygdala volumes. CONCLUSIONS: Findings suggest that although there were no significant differences in amygdala volumes between groups, familial factors influence amygdala volumes. Discrepancies between studies measuring amygdala volume in MDD may be due to differences in amygdala boundary definitions.