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Multidisciplinary supportive care in systemic light chain amyloidosis.

The immunoglobulin light-chain amyloidosis is a multisystemic disease which manifests by damage to the vital organs by light chain-derived amyloid fibril. Traditionally, the treatment has been directed to the underlying plasma cell clone with or without high dose chemotherapy followed by autologous stem cell transplantation using melphalan based conditioning. Now with the approval of highly tolerable anti-CD38 monoclonal antibody daratumumab based anti-plasma cell therapy in 2021, high rates of hematologic complete responses are possible even in patients who are otherwise deemed not a candidat

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… | Intervention Anasarca due to heart failure and/or nephrotic syndrome | Salt restriction and diuretics are the mainstay of treatment, tracking daily weight, collaboration and co-management with cardio-oncology and nephron-oncology when available Ventricular or supra-ventricular tachy-arrhythmia | Amiodarone … Among the various available antiarrhythmic drugs, only amiodarone and (occasionally dofelitide) are tolerated and are therefore recommended for treatment of arrythmias. However, the use of anti-arrhythmic drugs did not show any survival benefit when prophylactically used [ 16 ]. …

Cardiotoxicity after cancer treatment: a process map of the patient treatment journey.

Background/aimCardiotoxicity is a potential complication of anticancer therapy. While guidelines have been developed to assist practitioners, an effective, evidence based clinical pathway for the treatment of cardiotoxicity has not yet been developed. The aim of this study was to describe the journey of patients who developed cardiotoxicity through the healthcare system in order to establish baseline data to inform the development and implementation of a patient-centred, evidence-based clinical pathway.MethodsMixed-methods design with quantitative and qualitative components using process mappi

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… Paclitaxel 429.0 3 (7) Vincristine a 12 (26) Monoclonal Antibodies Rituximab 655.0 11 (24) Trastuzumab 378.1 7 (15) Radiation therapy 28 (61) a Dose not recorded b Each patient’s regimen comprised individualised composites and doses of the above cytotoxic agents c Other drugs taken include: amiloride, amiodarone … zoledronic acid and vancomycin Table 3 Cancer diagnosis/type, chemotherapy agent class and radiation therapy (Medical Record Review cohort only) a Dose not recorded b Each patient’s regimen comprised individualised composites and doses of the above cytotoxic agents c Other drugs taken include: amiloride, amiodarone …

Ранняя потеря "Macular Ganglion Cell" в Leber Hereditary Optic Neurophy — биомаркер оптической совместимости для дифференцирования оптического неврита.

Фон/цели: Наследственная оптическая нейропатия Лебера (LHON) часто неправильно диагностируется на ранних этапах как идиопатический единичный оптический неуриит (SION) или многоклерозовый оптический неврал (MS-ON) из-за раннего возраста пациентов, потери субакутного зрения и нарушения центрального визуального поля. Цель этого ретроспективного исследования состояла в том, чтобы оценить изменения в перипиллярном RNFL и GCLT с течением времени у пациентов с ранним LHON, MS-ON и SION, с тем чтобы провести различие между наследственной нейропатией (LHON) и оптическим неуриитом (ON) на ранних стадиях

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… general incomplete data sets, missing follow-up examinations, artefacts in OCT imaging, other ophthalmological or neurological diseases, systemic conditions that could affect the visual system, medication intake of drugs with known toxic side effects to the retina or optic nerve (e.g., chloroquine, amiodarone …

Brown Tumor From Secondary Hyperparathyroidism Mimicking Metastatic Renal Cell Carcinoma in a Patient With End-Stage Renal Disease.

Brown tumors (also known as osteitis fibrosa cystica) are rare complications of end-stage renal disease (ESRD) and secondary hyperparathyroidism (HPT), characterized by focal bone lesions that resemble neoplasms. They are often misdiagnosed as metastatic bone disease, especially in patients with a history of malignancy. We present a case of a 60-year-old man with a history of renal cell carcinoma (RCC), and ESRD on hemodialysis (HD), who developed diffuse bone lesions on imaging with osteolytic/osteoblastic appearance concerning metastases, but on further workup was found to have brown tumors.

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… The patient had an extensive past medical history of atrial fibrillation on amiodarone, hypertension, gout, and ESRD secondary to occult vesicoureteral reflux complicated by severe pyelonephritis requiring left nephrectomy six years ago, prior to left living donor renal transplant (LDKT) about 30 years …

DHCR24 in Tumor Diagnosis and Treatment: A Comprehensive Review.

As an important nutrient in the human body, cholesterol can not only provide structural components for the body's cells, but also can be transformed into a variety of active substances to regulate cell signaling pathways. As an important cholesterol synthase, DHCR24 participates in important regulatory processes in the body. The application of DHCR24 in tumor clinical diagnosis and treatment also attracts much attention. This article reviews the structure and regulatory characteristics of DHCR24, and the research of DHCR24 on tumor progression. We summarize the possible mechanisms of DHCR24 pr

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… Amiodarone disrupts cholesterol biosynthesis pathway and causes accumulation of circulating desmosterol by inhibiting 24-dehydrocholesterol reductase. J Intern Med. 2020;288(5):560-569. doi: 10.1111/joim.13095 179. Allen LB, Genaro-Mattos TC, Anderson A, et al. … Amiodarone alters cholesterol biosynthesis through tissue-dependent inhibition of emopamil binding protein and dehydrocholesterol reductase 24. ACS Chem Neurosci. 2020;11(10):1413-1423. doi: 10.1021/acschemneuro.0c00042 180. Yang XB, Zu HB, Zhao YF, et al. …

Case Report: AL Amyloidosis Severe Restrictive Cardiomyopathy Associated With Multiple Myeloma-Diagnostic Difficulties.

BackgroundCardiac AL amyloidosis as a complication of multiple myeloma (MM) is a formidable life-threatening condition. The first-line therapy for both MM and systemic AL amyloidosis is proteasome inhibitors (PIs). Unfortunately, the use of PIs may lead to cardiovascular toxicity development, which requires specific cardio-oncology supervision.Case reportA 57-year-old woman was admitted to a university hospital with clinical manifestation of progressive chronic heart failure. The patient had hypertension and no history of diabetes mellitus, myocardial infarction (MI), stroke, and arrhythmias.

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… There was an attempt to administer amiodarone, but it was not tolerated by the patient (extreme systemic hypotension, nausea, vomiting, and dizziness). Thus, sotalol was the only option in this case with regular ECG control (throughout the whole follow-up period no QTc prolongation was registered). …

Forecasting drug utilization and expenditure in a metropolitan health region.

BackgroundNew pharmacological therapies are challenging the healthcare systems, and there is an increasing need to assess their therapeutic value in relation to existing alternatives as well as their potential budget impact. Consequently, new models to introduce drugs in healthcare are urgently needed. In the metropolitan health region of Stockholm, Sweden, a model has been developed including early warning (horizon scanning), forecasting of drug utilization and expenditure, critical drug evaluation as well as structured programs for the introduction and follow-up of new drugs. The aim of this

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… In 2010, a new antiarrythmic agent, dronedarone, will be introduced as an alternative to amiodarone for patients with atrial fibrillation. … However, the greater tolerability of dronedarone compared to amiodarone is achieved at the expense of lesser efficacy [ 33 ], and the target population for dronedarone has not yet been defined. …

Arrhythmias, conduction disorders and sudden cardiac death in cancer patients and survivors: expert opinion of the working groups on cardio-oncology and on electrophysiology of the hellenic cardiac society.

Advances in cancer therapeutics have significantly improved patient survival; however, cardiotoxic effects-including arrhythmias-have emerged as a growing clinical concern. This expert opinion, developed by the Working Groups on Cardio-Oncology and Electrophysiology of the Hellenic Cardiological Society, aims to raise awareness of the increasing burden of arrhythmias in cancer patients and survivors. This document explores the multifactorial etiology of arrhythmias in this population, including patient-related factors, the direct effects of malignancy, and the arrhythmogenic potential of thera

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… Propafenone | Angiogenesis inhibitor (pomalidomide) Anthracycline (doxorubicin) EGFR TKI (erlotinib, gefitinib) SERM (tamoxifen) | Common metabolism in CYP1A2 and CYP2D6 | Alternative antiarrhythmic agent or regular monitoring for signs of toxicity such as bradycardia, QTc prolongation, PR or QRS widening Amiodarone … inhibitor (everolimus) PARP inhibitor (Olaparib) PI3Kδ inhibitor (duvelisib, idelalisib) Proteasome inhibitor (ixazomib) SERM (tamoxifen, toremifene) Taxane (paclitaxel) Thalidomide Trk inhibitor (Larotrectinib) TS inhibitor (capecitabine) | Common metabolism in CYP2C8, CYP3A4, P-gp/ ABCB1 | Avoid amiodarone …

2005 American Heart Association (AHA) guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal patients: pediatric basic life support.

This publication presents the 2005 American Heart Association (AHA) guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiov … , but it can be used for treatment of ventricular fibrillation/pulseless ventricular tachycardia if amiodarone is not available. Induced hypothermia (32-34 degrees C for 12-24 hours) may be considered if the child remains comatose after resuscitation. Indications for the use of inodilators are mentioned in the postresuscitation section. Termination of resusc …

Sensory neuropathy in patients with Pompe disease: a case series in Iran.

BackgroundPompe disease is a glycogen storage disease primarily affecting striated muscles. Despite its main manifestation in muscles, patients with Pompe disease may exhibit non-muscle symptoms, such as hearing loss, suggesting potential involvement of sensory organs or the nervous system due to glycogen accumulation.AimsThis study aimed to evaluate the presence of concomitant small and large fiber neuropathy in patients with Pompe disease.MethodsIn this case series study, nine patients with Pompe disease without complaints of neuropathy were evaluated. Small fiber neuropathy was assessed usi

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… neurophysiological findings; 7 Excluded by appropriated anamnesis and medical record study; 8 Excluded by anamnesis and blood tests Inherited factors 1 | Neoplastic factors 6 HNPP | Monoclonal gammopathy CMT | Primary amyloidosis Metabolic 2 | Multiple myeloma Prediabetes state | Drugs 7 Diabetes | Amiodarone …

Регенеративная терапия на основе коллагена в критическом ишемическом диабиотическом ульце: доклад по делу о лимб-алкоголе.

58-летний мужчина с диабетом, конечной стадией почечных заболеваний и тяжелой периферической артерией страдал от хронической ишемической язвы ступни до одного года традиционной терапии. Инъекционная регенеративная терапия на основе коллагена привела к полной эпителиализации в течение трех месяцев и к долговременному спасению конечностей даже после техногенного рецидива, требующего реваскуляции.

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… The patient was maintained on long‐term cardiovascular and antiplatelet therapy, including aspirin, bisoprolol, nifedipine, amiodarone, and an oral hypoglycemic agent. …

AL type cardiac amyloidosis: a devastating fatal disease.

IntroductionCardiac amyloidosis is a rare entity with a grave prognosis. Due to the low index of suspicion secondary to non-specific symptoms, it is often diagnosed at an advanced stage with multi-organ involvement.MethodsWe report a case of systemic AL amyloidosis with predominant cardiac and renal involvement associated with multiple myeloma.Case summaryA 60-year-old male presented with progressive anasarca, orthopnea and weight gain over 8 months. On clinical examination, 3+ pitting edema was found in bilateral extremities and scrotum. Serum N-type proBNP and troponin T were elevated, and E

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… Ibutilide and amiodarone are commonly used for atrial fibrillation management in this group [ 12 ]. Due to excessive amyloid deposition in cardiac conduction pathways, the patient can experience symptomatic bradycardia and heart block which can be managed with a pacemaker. …

Progress and challenges in the treatment of cardiac amyloidosis: a review of the literature.

Cardiac amyloidosis is a restrictive cardiomyopathy determined by the accumulation of amyloid, which is represented by misfolded protein fragments in the cardiac extracellular space. The main classification of systemic amyloidosis is determined by the amyloid precursor proteins causing a very heterogeneous disease spectrum, but the main types of amyloidosis involving the heart are light chain (AL) and transthyretin amyloidosis (ATTR). AL, in which the amyloid precursor is represented by misfolded immunoglobulin light chains, can involve almost any system carrying the worst prognosis among amyl

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… Because pharmacological rhythm control options are limited, amiodarone is the most commonly administered drug in CA with arrhythmias and is relatively well tolerated. 22 , 33 Other options used in clinical practice are sotalol and dofetilide, if well tolerated. … Ventricular arrhythmias also appear to be prevalent in CA, including premature ventricular contractions, and non‐sustained and sustained ventricular tachycardias. 34 Moreover, they hold a prognostic role, being associated to sudden cardiac death (SCD). 34 Amiodarone is indicated for the pharmacological …

Primary Health Care approach to managing chronic kidney disease.

Chronic kidney disease (CKD) is a growing global health issue among ageing populations, driven by increasing diabetes and hypertension. Managing advanced stages of CKD with dialysis or transplants is costly. Early stages often have no symptoms, making early detection important for prevention. Primary care providers can help identify and manage CKD early by performing annual screening for high-risk patients, including blood pressure checks, urine tests for albuminuria or proteinuria and estimating glomerular filtration rate from serum creatinine. For those with CKD, renal protective measures ar

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… 130 mmHg) Glycaemic control (Targeted HbA1c of 6.5% – 7.0%) Lipid-lowering therapy e.g. statin Antiplatelet only as secondary prevention 5 | Atrial fibrillation | Kidney adjusted dose of oral anticoagulant after a bleeding risk score Beta blockers (carvedilol) Cardioversion Antiarrhythmic therapy (amiodarone …

Inpatient Medicine

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Сфингосин-1-Фосфатная модуляция в неврологических расстройствах: Отчёты от MS и Stroke.

Множественный склероз — это хроническое аутоиммунное заболевание, при котором иммунная система атакует защитную оболочку или миелин, покрывающую нервные волокна, что влияет на способность мозга общаться с другими частями тела. Эта аномальная иммунная реакция привлекает подстрекательские вещества, которые выглядят как повреждения мозга и спинного мозга. Инсульт характеризуется внезапным ухудшением неврологической функции в результате потери или ограничения кровотока в результате острого повреждения локального участка центральной нервной системы, включая мозг, сетчатку или спинной мозг. В то вре

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… Major adverse events reported in the combination group included two incidences of atrial fibrillation, which resolved with the administration of amiodarone, and two incidences of thrombocytopenia, which resolved without intervention. …

Interleukin-17 plays a role in dental pulp inflammation mediated by zoledronic acid: a mechanism unrelated to the Th17 immune response?

ObjectiveTo evaluate the influence of RORγT inhibition by digoxin on inflammatory changes related to interleukin-17 (IL-17) in the pulp of rats treated with zoledronate (ZOL).MethodologyForty male Wistar rats were divided into a negative control group (NCG) treated with saline solution, a positive control group (PCG) treated with ZOL (0.20 mg/kg), and three groups treated with ZOL and co-treated with digoxin 1, 2, or 4 mg/kg (DG1, 2, and 4). After four intravenous administrations of ZOL or saline solution in a 70-day protocol, the right molars were evaluated by histomorphometry (number of bloo

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… Huh JR Leung MW Huang P Ryan DA Krout MR Malapaka RR et al Digoxin and its derivatives suppress TH17 cell differentiation by antagonizing RORγt activity Nature 2011 472 7344 486 490 10.1038/nature09978 21441909 PMC3172133 37 Rejnmark L Vestergaard P Mosekilde L Fracture risk in patients treated with amiodarone …

Cardiac Amyloidosis: Tribulations and New Frontiers.

This review article aims to provide an overview of the pathophysiology, diagnosis, and contemporary management of cardiac amyloidosis (CA) as well as identify the knowledge gaps and areas of potential research. CA can be divided into two main groups: transthyretin cardiac amyloidosis (ATTR-CA) and light chain cardiac amyloidosis (AL-CA). The former further separates into wild-type transthyretin (ATTRwt) and hereditary transthyretin (ATTRv). African Americans, males, and people older than 75 are the most common demographics affected by this disease. Thanks to an increased understanding of this

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… While amiodarone is well tolerated at low doses, it does not provide a mortality benefit [ 105 ]. … Multiple retrospective and observational trials support the efficacy of rhythm control strategies such as amiodarone, cardioversion, and catheter ablation over rate control, especially when used early in the disease course [ 105 , 106 , 107 ]. …

Crosslink between atrial fibrillation and cancer: a therapeutic conundrum.

Atrial fibrillation (AF) is more common in patients with malignancies than in general population. The pathophysiological processes include the pro-inflammatory condition and the exaggerated inflammatory reaction to chemotherapy, radiotherapy, and surgery interventions. Thus, it is pivotal to decrease morbidity and mortality in this group by providing appropriate care and prevention. In this subset, the risk of thromboembolic and bleeding events is high and the common risk score such as CHA2DS2-VASc and HAS-BLED employed in non-oncologic patients have limited evidence in cancer patients. A pauc

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… In non-cancer patients, current ESC guidelines on management of patients with AF recommend prophylactic treatment with beta-blocker or amiodarone to prevent POAF in patients at higher risk of AF onset, considering potential adverse events and ambiguous effects on major adverse events [ 32 , 33 ]. … Amiodarone is often used to manage cancer-related arrhythmias, especially in individuals with concomitant CV diseases. This drug could raise the levels of other CT drugs due to actions on CYP3A4 and P-glycoprotein. …

Arrhythmic Risk Stratification in Cardiac Amyloidosis: A Review of the Current Literature.

Cardiac amyloidosis is the most frequent infiltrative disease caused by the deposition of misfolded proteins in the cardiac tissue, leading to heart failure, brady- and tachyarrhythmia and death. Conduction disorders, atrial fibrillation (AF) and ventricular arrhythmia (VA) significantly impact patient outcomes and demand recognition. However, several issues remain unresolved regarding early diagnosis and optimal management. Extreme bradycardia is the most common cause of arrhythmic death, while fast and sustained VAs can be found even in the early phases of the disease. Risk stratification an

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… Among the antiarrhythmic drugs, amiodarone is preferred and could be a valuable alternative for rate control, because of the absence of a negative chronotropic effect [ 52 ]. … Dofetilide and sotalol have been proposed as alternatives to amiodarone [ 45 , 70 ]. 3.3. …