Antiphospholipid syndrome, also known as ‘Hughes Syndrome’, is an autoimmune disease characterised by a set of clinical manifestations, almost all of which are direct or indirect sequelae of a hypercoagulable state involving the venous, and to a lesser extent the arterial vasculature. The incidence and prevalence of antiphospholipid syndrome are estimated at approximately 5 de novo cases per 100 000 per year and 40–50 cases per 100 000 individuals, respectively. The clinical spectrum of antiphospholipid syndrome involves haematological (thrombocytopaenia, venous thrombosis), obstetrical (recurrent pregnancy loss), neurological (stroke, transient ischaemic attack, migraine, seizures, cognitive dysfunction, chorea, transverse myelitis, multiple sclerosis), cardiovascular (cardiac valve disease), dermatological (livedo reticularis and racemosa, skin ulceration and necrosis), renal (glomerulonephritis, renal thrombotic microangiopathy) and orthopaedic (avascular necrosis of bones, non-traumatic fractures) manifestations, among others. In addition to the classical antiphospholipid antibodies, namely anticardiolipin antibodies and lupus anticoagulant, new autoantibodies and antibody complexes of different immunoglobulin subtypes (IgA, IgG, IgM) are now recognised as significant contributors to the pathogenesis of antiphospholipid syndrome. Anticoagulation remains the cornerstone in the management of antiphospholipid syndrome; nevertheless, new drugs and therapeutic strategies are being tested, and some have been found effective for the primary and secondary thromboprophylaxis in antiphospholipid syndrome.
Thalassemia intermedia (TI), also known as nontransfusion dependent thalassemia (NTDT), is a type of thalassemia where affected patients do not require lifelong regular transfusions for survival but may require occasional or even frequent transfusions in certain clinical settings and for defined periods of time. NTDT encompasses three distinct clinical forms: β-thalassemia intermedia (β-TI), Hb E/β-thalassemia, and α-thalassemia intermedia (Hb H disease). Over the past decade, our understanding of the molecular features, pathophysiology, and complications of NTDT particularly β-TI has increased tremendously but data on optimal treatment of disease and its various complications are still lacking. In this paper, we shall review a group of commonly encountered complications in β-TI, mainly endocrine and bone complications.
Multiple sclerosis (MS) and antiphospholipid syndrome (APS) share common clinical, laboratory and radiological features. We reviewed all the English papers on MS and APS published in the literature from 1965 to 2014 using PubMed and Google Scholar. We found that APS can mimic antiphospholipid antibodies (aPL)-positive MS in many ways in its clinical presentation. Nevertheless, APS diagnosis, clinical manifestations and management differ from those of MS. aPL were found in MS patients with titers ranging from 2% to 88%. The distribution and volume of lesions on magnetic resonance imaging (MRI) may help to differentiate MS from primary APS. In addition, atypical MS presentation can guide physicians toward an alternative diagnosis, especially when features of thrombosis and/or history of connective tissue disease are present. In that case, an anticoagulation trial could be worthwhile.
BACKGROUND: Neurosurgical services have been affected by the 2019 novel coronavirus disease (COVID-19) pandemic, and several departments have reported their experiences and responses to the COVID-19 crisis in an attempt to provide insights from which other impacted departments can benefit. The goals of this study were to report the load and variety of emergent/urgent neurosurgical cases after implementing the "Battle Plan" at an academic tertiary referral center during the COVID-19 pandemic and to compare these variables with previous practice at the same institution.-METHODS: The clinical data of all patients who underwent a neurosurgical intervention between March 23, 2020, and April 20, 2020, were obtained from a prospectively maintained database. Data of the control group were retrospectively collected from the medical records to compare the types of surgeries/interventions performed by the same neurosurgical service before the COVID-19 pandemic started.-RESULTS: Over a 4-week period during the COVID-19 pandemic, 91 patients underwent emergent, urgent, and essential neurosurgical interventions. Patient screening at teleclinics identified 11 urgent surgical cases. The implementation of the Battle Plan led to a significant decrease in the caseload, and the variation of cases by subspecialty was evident when compared with a control group comprising 214 patients.-CONCLUSIONS: Delivery of optimal care and safe practice and education at an academic neurosurgical department can be well maintained with proper execution of crisis protocols. Teleclinics proved to be efficient in screening patients for urgent neurosurgical conditions, but in-person clinic visits may still be necessary for some cases in the immediate postoperative period.
Background Antiphospholipid syndrome (APS) or 'Hughes syndrome' is a prothrombotic disease characterized by thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). More than three decades have passed, and experts are still uncovering new pieces of this disease complex pathogenesis and management.
Background Every aspect of the medical field has been heavily affected by the coronavirus disease 2019 (COVID-19) pandemic, and neurosurgical services are no exception. Several departments have reported their experiences and protocols to provide insights for others impacted. The goals of this study are to report the load and variety of neurosurgical cases and clinic visits after discontinuing the COVID-19 Battle Plan at an academic tertiary care referral center to provide insights for other departments going through the same transition. Methods The clinical data of all patients who underwent a neurosurgical intervention between May 4, 2020, and June 4, 2020 were obtained from a prospectively maintained database. Data of the control group were retrospectively collected from the medical records to compare the types of surgeries/interventions and clinic visits performed by the same neurosurgical service before the COVID-19 pandemic started. Results One hundred sixty-one patients underwent neurosurgical interventions, and seven-hundred one patients were seen in clinic appointments, in the 4-week period following easing back from our COVID-19 “Battle Plan.” Discontinuing the “Battle Plan” resulted in increases in case load to above-average practice after a week but a continued decrease in clinic appointments throughout the 4 weeks compared with average practice. Conclusions As policy-shaping crises like pandemics abate, easing back to “typical” practice can be completed effectively by appropriately allocating resources. This can be accomplished by anticipating increases in neurosurgical volume, specifically in the functional/epilepsy and brain tumor subspecialties, as well as continued decreases in neurosurgical clinic volume, specifically in elective spine.
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