Authors:Tubagus Djumhana Atmakusuma Abstract: Background: People living with transfusion dependent thalassemia have high risk of getting infected by COVID-19. This can be caused by both several factors. Chronic complications of thalassemia also render them more vulnerable to infectious diseases, including COVID-19. However, anecdotal data shows that Thalassemia patients less incidence of COVID-19 compared to the general population. Patients and methods: This research used a cross sectional design. The study was conducted at the Division of Haematology and Medical Oncology of the Cipto Mangunkusumo Hospital in Jakarta from May 2020 – August 2021. Total sampling method was used involving all Thalassemia Major patients who had been infected with COVID-19 obtained directly from medical record and through the thalassemia patients-parents foundation Results: From 10,397 patients with thalassemia, 72 (0.69%) people were infected by COVID-19 and 7 (9.7%) were deceased. Meanwhile, the incidence of COVID-19 in general population of Indonesia was 0.87%, more than in the thalassemia population. This means that thalassemia might provide protection against COVID-19 due to several mechanism. Conclusion: Indonesia and other countries with high prevalence of thalassemia have lower COVID-19 incidence than countries with low prevalence of thalassemia. Thalassemia might have protective effect against COVID-19. Well designed studies are needed to provide better evidence on the protective effect of thalassemia on COVID-19. PubDate: 2022-01-24 DOI: 10.4081/hr.2021.9379 Issue No:Vol. 13, No. 4 (2022)
Authors:Orapan Sripichai, Woratree Kaewsakulthong, Phitchapa Pongpaksupasin, Tiwaporn Nualkaew, Suradej Hongeng, Suthat Fucharoen, Natee Jearawiriyapaisarn Abstract: Induction of fetal hemoglobin (HbF) ameliorates the clinical severity of β-thalassemias. Histone methyltransferase LSD1 enzyme removes methyl groups from the activating chromatin mark histone 3 lysine 4 at silenced genes, including the γ-globin genes. LSD1 inhibitor RN-1 induces HbF levels in cultured human erythroid cells. Here, the HbF-inducing activity of RN-1 was investigated in erythroid progenitor cells derived from β0-thalassemia/HbE patients. The significant and reproducible increases in γ-globin transcript and HbF expression upon RN-1 treatment was demonstrated in erythroid cells with divergent HbF baseline levels, the average of HbF induction was 17.7 + 0.8%. RN-1 at low concentration did not affect viability and proliferation of erythroid cells, but decreases in cell number was observed in cells treated with RN-1 at high concentration. Delayed terminal erythroid differentiation was revealed in β0-thalassemia/HbE erythroid cells treated with RN-1 as similar to other compounds that target LSD1 activity. Downregulation of repressors of γ-globin expression; NCOR1 and SOX6, was observed in RN-1 treatment. These findings provide a proof of concept that a LSD1 epigenetic enzymes is a potential therapeutic target for β0-thalassemia/HbE patients. PubDate: 2021-11-26 DOI: 10.4081/hr.2021.9215 Issue No:Vol. 13, No. 4 (2021)
Authors:Andrea Duminuco, Elisa Mauro, Giuseppe A. M. Palumbo, Bruno Garibaldi, Marina Parisi, Francesco Di Raimondo, Cinzia Maugeri, Calogero Vetro Abstract: Fungal infections occurring in immunocompromised patients after immuno-chemotherapy treatment, are often difficult to eradicate and capable of even being fatal. Systemic mycoses affecting severely immunocompromised patients often manifest acutely with rapidly progressive pneumonia, fungemia, or manifestations of extrapulmonary dissemination. Opportunistic fungal infections (mycoses) include several pathogens elements, as Candidiasis, Aspergillosis, Mucormycosis (zygomycosis) and Fusariosis. Prompt diagnosis and effective therapy are needed to improve the associated morbidity and mortality, especially in cases with non-canonical fungal localizations and not responsive to the available antifungal drugs. PubDate: 2021-11-26 DOI: 10.4081/hr.2021.9329 Issue No:Vol. 13, No. 4 (2021)
Authors:Samuel sarmiento Doncel, Gina Alejandra Diaz Mosquera, Javier Mauricio Cortes , Nelson Ramirez, Francisco Javier Meza, Carol Agudelo Rico Abstract: Introduction: In recent decades, hemophilia A treatment has been focused on body weight, without taking pharmacokinetic parameters into account. Previous research has shown that the individual pharmacokinetic response is more effective in predicting the required dose of clotting factor. We want to evaluate the impact on reducing the frequency of bleeding in patients treated with recombinant factor VIII, based on a personalized comprehensive management program. Objective: Our aim was to compare the results of a standard comprehensive treatment program (stage I) vs. a personalized pharmacokinetic - based treatment program (stage II) in a cohort of 60 patients with severe hemophilia without inhibitors. Results:The median age was 15.5 years (3 - 68). The ABR was 1.03 (62 episodes) in the first stage and 0.58 (35 episodes) in the second one, (p = 0.004). By type of bleeding, the impact of the intervention differs significantly in spontaneous bleeding (p = 0.007) and a 73% reduction in the first stage. There were no significant differences in traumatic bleeding. Conclusions: The use of pharmacokinetics for personalized dosing of patients with severe hemophilia A, significantly reduces ABR and spontaneous bleeding, improving the patient's quality of life and costs for the health system. PubDate: 2021-11-26 DOI: 10.4081/hr.2021.8904 Issue No:Vol. 13, No. 4 (2021)
Authors:Thilina Gunawardena Abstract: Thrombin inhibitors and direct factor Xa inhibitors represent a major breakthrough in the field of anticoagulation pharmacotherapy. These novel agents have replaced warfarin as the oral anticoagulant of choice in certain indications, as they possess equal or superior efficacy and better safety profiles. They have a quick onset of action, predictable pharmacokinetic properties and minimal drug and food interactions. So they do not require frequent blood monitoring and dose adjustments as with warfarin. Considering all the advantages, there seems to be a rapid increase in the number of patients who are started on these novel anticoagulants. In this review, we highlight the pharmacology of these direct oral anticoagulants and the evidence-based indications for their use. We aim to provide a clinical overview for the non-specialist who may be called upon to manage a patient who is currently on one of these novel anticoagulants. PubDate: 2021-11-26 DOI: 10.4081/hr.2021.9239 Issue No:Vol. 13, No. 4 (2021)
Open Access journal
[52 journals]
