Diffuse alveolar haemorrhage as a rare complication of antiphospholipid syndrome.

Diffuse alveolar haemorrhage (DAH) is a rare complication of antiphospholipid syndrome. With a mortality rate of 46%, early diagnosis and management remain an ongoing challenge. Case reports are limited, and management guidelines are not yet definitive. In this case report, we present a 43-year-old male with DAH who required high-dose oral steroids, intravenous methylprednisolone cyclophosphamide and rituximab over 18 months to control life-threatening episodes of pulmonary bleeding.

Computerized tomography image correlation of His bundle/deep septal pacing location and outcomes: an analysis from the Canberra HIs bundle/deep septal Pacing Study (CHIPS).

BACKGROUND: Localisation of the conduction system under fluoroscopy is not easy and the ideal location of the pacing leads in physiological pacing is still being debated. OBJECTIVE: The primary aim was to assess the lead locations using cardiac CT scan. Secondary aims were clinical outcomes including success and safety of the procedure and lead performance. METHODS: Of the 100 consecutive patients who received physiological pacing, 34 patients underwent follow-up cardiac CT scan. The four different types of pacing were identified as His bundle (HBP), para-Hisian, left bundle branch (LBBP), and deep septal pacing. RESULTS: Most patients had successful HBP via the right atrium (RA) (87.5%) as compared to the right ventricle (RV) (12.5%). Lower thresholds were observed when leads were placed within 2 mm of the junction of the membranous and muscular ventricular septum. Unlike HBP, LBBP was possible at a wide region of the septum and selective capture of individual fascicles was feasible. LBBP showed deeper penetration of leads into the septum, as compared to deep septal pacing (70% vs. 45%). Approximately, 80% of patients did not have an intra-ventricular portion of the membranous septum. CONCLUSIONS: The anterior part of the atrio-ventricular (AV) septum at the junction between the membranous and muscular septum via RA appeared to be the best target to successfully pace His bundle. LBBP was possible at a wide region of the septum and selective capture of individual fascicle was feasible. Adequate depth of penetration of lead was very important to capture the left bundle.

Computational investigation of particle penetration and deposition pattern in a realistic respiratory tract model from different types of dry powder inhalers.

The aim of this study was to evaluate the device performance of a new design by comparing with a typical commercial DPI. Computational fluid dynamics (CFD) coupled with the discrete element method (DEM) collision has been utilized in this study to characterize and examine the flow field and particle transportation, respectively. A typical commercial DPI and an in-house designed novel DPI with distinct design features were compared to explore their dispersion capabilities and suitability for delivery to the respiratory tract. For this exploration, realistic oral to larynx and tracheobronchial airway models consisting of bio-relevant features were adopted to enhance practical feasibility. Distinct aerosol performances were observed between the two DPIs in the respiratory tract, where the in-house DPI, in comparison with the commercial DPI, has shown approximately 30% lower deposition fraction in the mouth-throat region with approximately 7% higher escape rate in the tracheobronchial region under the identical inhalation condition. This observation demonstrates that a novel in-house designed DPI provides higher device efficiency over the selected typical commercial DPI.

Fronto-striatal atrophy correlates of neuropsychiatric dysfunction in frontotemporal dementia (FTD) and Alzheimer's disease (AD) / Correlatos de atrofia fronto-estriatal e disfunção neuropsiquiátrica em demência frontotemporal (DFT) e doença de ALZHEIMER (DA)

Behavioural disturbances in frontotemporal dementia (FTD) are thought to reflect mainly atrophy of cortical regions. Recent studies suggest that subcortical brain regions, in particular the striatum, are also significantly affected and this pathology might play a role in the generation of behavioural symptoms. OBJECTIVE: To investigate prefrontal cortical and striatal atrophy contributions to behavioural symptoms in FTD.METHODS: One hundred and eighty-two participants (87 FTD patients, 39 AD patients and 56 controls) were included. Behavioural profiles were established using the Cambridge Behavioural Inventory Revised (CBI-R) and Frontal System Behaviour Scale (FrSBe). Atrophy in prefrontal (VMPFC, DLPFC) and striatal (caudate, putamen) regions was established via a 5-point visual rating scale of the MRI scans. Behavioural scores were correlated with atrophy rating scores. RESULTS: Behavioural and atrophy ratings demonstrated that patients were significantly impaired compared to controls, with bvFTD being most severely affected. Behavioural-anatomical correlations revealed that VMPFC atrophy was closely related to abnormal behaviour and motivation disturbances. Stereotypical behaviours were associated with both VMPFC and striatal atrophy. By contrast, disturbance of eating was found to be related to striatal atrophy only. CONCLUSION: Frontal and striatal atrophy contributed to the behavioural disturbances seen in FTD, with some behaviours related to frontal, striatal or combined fronto-striatal pathology. Consideration of striatal contributions to the generation of behavioural disturbances should be taken into account when assessing patients with potential FTD.

Distúrbios de comportamento na demência frontotemporal (DFT) parecem refletir principalmente atrofia de regiões corticais. Estudos recentes sugerem que regiões cerebrais subcorticais, em particilar o estriado, são também são significativamente afetados e esta patologia pode ter um papel na geração dos sintomas comportamentais. OBJETIVO: Investigar a contribuição da atrofia cortical prefrontal e estriatal para os sintomas da DFT. MÉTODOS: 182 participantes (87 pacienjtes com DFT, 39 pacientes com DA e 56 controles) foram incluídos. Os perfis cognitivos foram estabelecidos usando o Cambridge Behavioural Inventory Revised (CBI-R) e Frontal System Behaviour Scale (FrSBe). Atrofia nas regiões prefrontal (VMPFC, DLPFC) e estriatal (caudado e putamen) foi estabelecida através de uma escala visual de 5 pontos nas imagens de ressonância magnética. Os escores de comportamento foram correlacionados aos escores de atrfoia. RESULTADOS: Os resultados comportamentais e de atrofia demonstraram que os pacientes estavam significativamente mais comprometidos do que os controles, com os pacientes com DFT mais gravemente afetados. As correlações anátomo-comportamentais revelaram que a atrofia do VMPFC foi intimamente relacionada ao comportamento anormal e distúrbios de motivação. Comportamentos estereotipados estiveram associados com atrofia do VMPFC e estriatal. Em contraste, distúrbios da alimentação foram relacionados somente a atrofia estriatal. CONCLUSÃO: A atrofia frontal e estriatal contribuíram para os distúrbios vistos na DFT, com alguns comportamentos relacionados a patologia frontal, estriatal ou combinadas. Considerações quanto à contribuição estriatal na gênese dos distúrbios de comportamento devem ser levados em conta quando se avalia pacientes com DFT em potencial.

Fronto-striatal atrophy correlates of neuropsychiatric dysfunction in frontotemporal dementia (FTD) and Alzheimer's disease (AD).

Behavioural disturbances in frontotemporal dementia (FTD) are thought to reflect mainly atrophy of cortical regions. Recent studies suggest that subcortical brain regions, in particular the striatum, are also significantly affected and this pathology might play a role in the generation of behavioural symptoms. OBJECTIVE: To investigate prefrontal cortical and striatal atrophy contributions to behavioural symptoms in FTD. METHODS: One hundred and eighty-two participants (87 FTD patients, 39 AD patients and 56 controls) were included. Behavioural profiles were established using the Cambridge Behavioural Inventory Revised (CBI-R) and Frontal System Behaviour Scale (FrSBe). Atrophy in prefrontal (VMPFC, DLPFC) and striatal (caudate, putamen) regions was established via a 5-point visual rating scale of the MRI scans. Behavioural scores were correlated with atrophy rating scores. RESULTS: Behavioural and atrophy ratings demonstrated that patients were significantly impaired compared to controls, with bvFTD being most severely affected. Behavioural-anatomical correlations revealed that VMPFC atrophy was closely related to abnormal behaviour and motivation disturbances. Stereotypical behaviours were associated with both VMPFC and striatal atrophy. By contrast, disturbance of eating was found to be related to striatal atrophy only. CONCLUSION: Frontal and striatal atrophy contributed to the behavioural disturbances seen in FTD, with some behaviours related to frontal, striatal or combined fronto-striatal pathology. Consideration of striatal contributions to the generation of behavioural disturbances should be taken into account when assessing patients with potential FTD.

Distúrbios de comportamento na demência frontotemporal (DFT) parecem refletir principalmente atrofia de regiões corticais. Estudos recentes sugerem que regiões cerebrais subcorticais, em particilar o estriado, são também são significativamente afetados e esta patologia pode ter um papel na geração dos sintomas comportamentais. OBJETIVO: Investigar a contribuição da atrofia cortical prefrontal e estriatal para os sintomas da DFT. MÉTODOS: 182 participantes (87 pacienjtes com DFT, 39 pacientes com DA e 56 controles) foram incluídos. Os perfis cognitivos foram estabelecidos usando o Cambridge Behavioural Inventory Revised (CBI-R) e Frontal System Behaviour Scale (FrSBe). Atrofia nas regiões prefrontal (VMPFC, DLPFC) e estriatal (caudado e putamen) foi estabelecida através de uma escala visual de 5 pontos nas imagens de ressonância magnética. Os escores de comportamento foram correlacionados aos escores de atrfoia. RESULTADOS: Os resultados comportamentais e de atrofia demonstraram que os pacientes estavam significativamente mais comprometidos do que os controles, com os pacientes com DFT mais gravemente afetados. As correlações anátomo-comportamentais revelaram que a atrofia do VMPFC foi intimamente relacionada ao comportamento anormal e distúrbios de motivação. Comportamentos estereotipados estiveram associados com atrofia do VMPFC e estriatal. Em contraste, distúrbios da alimentação foram relacionados somente a atrofia estriatal. CONCLUSÃO: A atrofia frontal e estriatal contribuíram para os distúrbios vistos na DFT, com alguns comportamentos relacionados a patologia frontal, estriatal ou combinadas. Considerações quanto à contribuição estriatal na gênese dos distúrbios de comportamento devem ser levados em conta quando se avalia pacientes com DFT em potencial.