Diagnostic and prognostic role of elafin in skin acute graft versus host disease: a systematic review.

BACKGROUND AND OBJECTIVE: Graft versus host disease (GVHD) is the common complication seen after allogeneic hematopoietic stem cell transplantation (HSCT) and a pleomorphic syndrome that resembles autoimmune and other immunologic disorders, leading to profound immune dysregulation and organ dysfunction. The most common targets of GVHD are skin, gastrointestinal tract and liver. GVHD is classified as acute graft versus host disease (aGvHD) if it occurs within the first 100 days after HSCT and chronic graft versus host disease(cGVHD) if it occurs after day 100. The skin is most frequently and earliest affected by aGvHD, followed by the gastrointestinal tract and liver. An ideal biomarker would predict the onset and severity of clinical acute GVHD and help to direct management, and this is an area of active research regarding the use of biomarkers for diagnosis and prognosis of acute GVHD. Recently, elafin has been identified as a potential plasma biomarker for aGVHD. METHOD: We searched the databases PubMed, Cochrane library, and medRxiv for all studies investigating the Diagnostic or prognostic role of elafin in GVHD. We set the search strategy incorporating the search terms, 'elafin', 'graft versus host', and 'GVHD', and operated using the Boolean operators 'AND', and 'OR'. Thus, retrieved articles were then exported on an Excel® sheet, and duplicates were removed. The systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. After selecting the study based on inclusion criteria, data on study characteristics and biomarker description was extracted on a pre-determined data extraction table on the Microsoft Excel version. The quality assessment of the included studies was determined using the QUIPS tool. RESULT: The search revealed 547 studies and 6 studies that met the eligibility criteria of this review have been included. The major finding of our study is the significant elevation of elafin in skin aGVHD. CONCLUSION: Elafin is a significant biomarker for diagnosis and prognosis of skin aGVHD and should be assessed within 2 weeks of the onset of the disease.

Exploring Transthyretin Amyloid Cardiomyopathy: A Comprehensive Review of the Disease and Upcoming Treatments.

Transthyretin amyloid cardiomyopathy (ATTR-CM) is a mutation-based genetic disorder due to the accumulation of unstable transthyretin protein and presents with symptoms of congestive heart failure (CHF) and numerous extracardiac symptoms like carpal tunnel syndrome and neuropathy. Two subtypes of ATTR-CM are hereditary and wild-type, both of which have different risk factors, gender prevalence and major clinical symptoms. Timely usage of imaging modalities like echocardiography, cardiac magnetic imaging resonance, and cardiac scintigraphy has made it possible to suspect ATTR-CM in patients presenting with CHF. Management of ATTR-CM includes appropriate treatment for heart failure for symptomatic relief, prevention of arrhythmias and heart transplantation for nonresponders. With the recent approval of tafamidis in the successful management of ATTR-CM, numerous potential therapeutic points have been identified to stop or delay the progression of ATTR-CM. This article aims to provide a comprehensive review of ATTR-CM and insights into its novel therapeutics and upcoming treatments.

Danazol for the Treatment of Myelodysplastic Syndromes: A Systematic Review.

Purpose To study the potential utility of danazol for treating patients with myelodysplastic syndromes, with a focus on efficacy and adverse effects (AEs). Methods MEDLINE In-Process & Other Non-Indexed Citations, MEDLINE, Embase, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and Scopus were searched for relevant publications from inception June 1, 1950, until June 28, 2022. The studies were screened by title and abstract, followed by full-text screening. The quality of the included studies was assessed via a prespecified set of questionnaires. Data on the efficacy measures and adverse outcomes were extracted and included in a descriptive summary. Results Nine studies consisting of 246 participants were included in our review. The overall quality of the included studies was fair. The age of the participants ranged from 61 to 78 years. In all 9 studies, more male patients had been enrolled than female patients. Overall, a proportion of patients in all the studies reported a desired major response to a danazol dose of 400 to 800 mg/day. Few studies did not observe any improvement in the platelet count. Elevated liver enzyme levels, weight gain, headache, dermatitis, and weakness were the most common AEs observed. One study reported a fatal intracerebral hemorrhage in 1 participant. Conclusions Danazol has been effective in increasing platelet count and hemoglobin level. Despite a few AEs, danazol is a safe drug for the treatment of patients with myelodysplastic syndromes.

Comparison of recent updates in genetics, immunology, biomarkers, and neuroimaging of primary-progressive and relapsing-remitting multiple sclerosis and the role of ocrelizumab in the management of their refractory cases.

Background: Primary-progressive multiple sclerosis (PPMS) and relapsing-remitting multiple sclerosis (RRMS) are two frequent multiple sclerosis (MS) subtypes that involve 10%-15% of patients. PPMS progresses slowly and is diagnosed later in life. Both subtypes are influenced by genetic and environmental factors such as smoking, obesity, and vitamin D insufficiency. Although there is no cure, ocrelizumab can reduce symptoms and delay disease development. RRMS is an autoimmune disease that causes inflammation, demyelination, and disability. Early detection, therapy, and lifestyle changes are critical. This study delves into genetics, immunology, biomarkers, neuroimaging, and the usefulness of ocrelizumab in the treatment of refractory patients of PPMS. Method: In search of published literature providing up-to-date information on PPMS and RRMS, this review conducted numerous searches in databases such as PubMed, Google Scholar, MEDLINE, and Scopus. We looked into genetics, immunology, biomarkers, current breakthroughs in neuroimaging, and the role of ocrelizumab in refractory cases. Results: Our comprehensive analysis found considerable advances in genetics, immunology, biomarkers, neuroimaging, and the efficacy of ocrelizumab in the treatment of refractory patients. Conclusion: Early detection, timely intervention, and the adoption of lifestyle modifications play pivotal roles in enhancing treatment outcomes. Notably, ocrelizumab has demonstrated potential in symptom control and mitigating the rate of disease advancement, further underscoring its clinical significance in the management of MS.

A case report on role of hypertonic saline solution in management of parotid fistula.

Introduction: Parotid fistula is an uncommon consequence of surgical or non-surgical trauma to the parotid gland or the area surrounding it. To treat it, a variety of pharmacological medicines and surgical techniques are used, each with their own set of benefits, drawbacks, and patient preferences. Case Presentation: We present the successful care of a young female with post-traumatic parotid fistula using hypertonic saline injections into the parotid substance, which is a simple yet efficient approach of treating this abnormality. Discussion: Thermodynamic and physicochemical calculations suggest that hypertonic saline solutions work to close parotid fistula by causing conformational denaturation of the cell membrane proteins in situ and saline can be diluted to a point where there will be no cellular toxicity. It is advised that temperature of the saline should be raised above body temperature to enhance the fibrosing property of physiologic saline. Conclusion: The use of hypertonic hot saline injections combined with compression dressing is a cost-effective, patient-friendly, and almost complication-free approach of treating parotid fistulas with promising results.

A rare case of atrial and biventricular thrombi with dilated cardiomyopathy as a delayed presentation in a patient with COVID-19.

Introduction: Coronavirus 2019 (COVID-19) can cause cardiovascular manifestations including myocardial injury and thromboembolic events. Case presentation: Here, we report a case of a 27-year-old female with dilated cardiomyopathy, right atrial and biventricular thrombi infected with COVID-19. Discussion: There are several complex coagulation abnormalities in COVID-19 patients that have been suggested to create a hypercoagulable state. Evidence have shown that endothelial injury potentially leading to thromboembolic events is caused by direct invasion of endothelial cell by SARS-CoV-2 and complement activation contributed by the virus spike protein. Conclusion: DCM can be complicated by atrial and biventricular thrombi due to coagulation abnormalities that are likely to persist after recovery from COVID-19. Thus, long-term careful monitoring of cardiac function is necessary after recovery of COVID-19.

ANCA-associated vasculitis following Johnson and Johnson COVID-19 vaccine.

Introduction: Antineutrophil cytoplasmic autoantibodies associated vasculitis (AAV) is characterized by antibodies against antigens in cytoplasmic granules of neutrophils and predominantly affects small vessels. AAV after COVID-19 mRNA vaccination has been reported. Case presentation: We report a rare case of AAV in a patient who presented with rapidly progressive glomerulonephritis (RPGN) after Johnson & Johnson COVID-19 vaccine administration. Discussion: The temporal causal association between autoimmune manifestations like AAV and COVID-19 vaccines can be explained by hypothesized mechanisms like molecular mimicry, defective neutrophilic apoptosis, polyclonal activation, and systemic proinflammatory cytokine response. These mechanisms are likely to trigger autoimmune responses in genetically susceptible individuals. Still there are many research going on to fill the research gap on the development of ANCA associated with COVID-19 vaccines. Conclusion: Increasing reports of rare adverse effects like AAV following COVID-19 vaccination warrants the further study and evaluation of immune responses induced by those vaccines. Considering the potential severity of COVID-19 and the rarity of the above-mentioned adverse effects, COVID-19 vaccination should not be withheld.