Motor neuron disease (MND) is a neurodegenerative disease and manifested as progressive decline in physical, respiratory, swallowing and communication function, and ultimately death. Traditional model of care was fragmented and did not match with multifacet needs of patients and carers. Furthermore, there could be lack of integrated care at end of life for patients with MND in most lower- and middle-income countries or in places with inadequate palliative care (PC) coverage. In view of this, a special workgroup for patients with MND, which includes neurologist, respiratory physician, rehabilitation specialist, and PC physician was formed in Hong Kong since year 2011. In various disease phase, each specialty team plays a leading role in coordinated care of patients with MND. From July 2011 to June 2017, a total of 52 patients with MND were referred for PC; 41 deceased patients with MND were included into data analysis. Major cause of death remains pneumonia (54.8%) and respiratory failure (40.5%). Most of the patients with MND (66.7%) died in acute ward and neurology units, with only 11.9% dying in PC units and hospices. The PC team plays a major role in advance care planning (ACP), and most patients had their ACP documented at second or third PC clinic visit (93.8%). Patients with MND often have limitations in mobility, swallowing difficulty, respiratory insufficiency requiring ventilator support, and various psychosocial needs. This highlighted the importance of early PC referral.
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http://dx.doi.org/10.1177/1049909117735832 | DOI Listing |
Muscle Nerve
December 2024
Department of Psychological Medicine, Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, UK.
Introduction/aims: A previous randomized controlled trial showed that guided self-help acceptance and commitment therapy plus standard medical care (ACT+SMC) was superior to standard medical care alone (SMC) for improving quality of life (QoL) and mood at 9-weeks post randomization in a sample of people with muscle disorders (MD). This follow-up study evaluated whether these effects were maintained in the longer term alongside individual patterns of response.
Methods: The original study was a two-arm parallel group randomized controlled trial, which compared ACT+SMC to SMC.
Thromb Haemost
December 2024
Pharmacology, Chulalongkorn University, Bangkok, Thailand.
Wiskott-Aldrich syndrome (WAS) is a severe X-linked disorder caused by loss-of-function mutations in the WAS gene, responsible for encoding WASP, a key regulator of actin cytoskeleton in all hematopoietic cells except red blood cells. The mechanism underlying microthrombocytopenia, a distinctive feature of WAS and a major contributor to mortality, remains not fully elucidated. In this study, using different gene editing strategies, we corrected mutations in patient-derived WAS-induced pluripotent stem cell lines, generating isogeneic WAS iPSC lines.
View Article and Find Full Text PDFJ Orthop Surg Res
December 2024
Department of Orthopaedic Surgery, Henry Ford Hospital, Henry Ford Health, 2799 W. Grand Blvd CFP-6, Detroit, MI, 48202, USA.
Background: Socioeconomic status has been recognized as a crucial social determinant of health influencing patient outcomes. Area Deprivation Index (ADI) is a validated measure of an area's socioeconomic status. Limited data exists on the impact of ADI and clinical outcomes and complications following rotator cuff repair (RCR).
View Article and Find Full Text PDFClin Exp Metastasis
December 2024
Department of Radiation Oncology, University Hospital Schleswig-Holstein Campus Kiel, Arnold-Heller-Str.3, 24105, Kiel, Germany.
Metastasis-directed therapy (MDT) for oligometastatic breast cancer (≤ 5 metastases) has shown little effect in specific scenarios of randomized trials. Therefore, we aimed to assess outcomes after metastasis-directed stereotactic radiotherapy (SRT) in various clinical scenarios. We conducted an international retrospective cohort study in thirteen centers including breast cancer patients receiving SRT to any metastatic site.
View Article and Find Full Text PDFJ Assoc Physicians India
December 2024
Resident, Department of Neurosurgery, Ravindra Nath Tagore Medical College, Udaipur, Rajasthan, India.
Introduction: Syringomyelia is a slowly progressive degenerative disorder of the spinal cord. Clinical features of syringomyelia vary from weakness in limbs to positive sensory symptoms and dissociative sensory loss. Thus, early and prompt diagnosis becomes crucial for reducing the morbidity associated with the disease.
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