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Dutch-beta thalassemia: A rare mutation from India

INTRODUCTION: COVID-19 pandemic imposed challenges towards management of transfusion-dependent thalassemia patients (TDT). The need for regular blood transfusions and iron chelation therapy in these patients added further uncertainty about managing COVID-19 in this subset of patients. AIMS: To descr...

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Autores principales: Sanghvi, Nirali, Aggarwal, Priyanka, Singh, Vineeta, Gupta, Vineeta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Pediatric Hematology Oncology Chapter of Indian Academy of Pediatrics. Publishing Services by Elsevier B.V. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9360999/
http://dx.doi.org/10.1016/j.phoj.2022.06.001
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author Sanghvi, Nirali
Aggarwal, Priyanka
Singh, Vineeta
Gupta, Vineeta
author_facet Sanghvi, Nirali
Aggarwal, Priyanka
Singh, Vineeta
Gupta, Vineeta
author_sort Sanghvi, Nirali
collection PubMed
description INTRODUCTION: COVID-19 pandemic imposed challenges towards management of transfusion-dependent thalassemia patients (TDT). The need for regular blood transfusions and iron chelation therapy in these patients added further uncertainty about managing COVID-19 in this subset of patients. AIMS: To describe the clinical manifestations of SARS-CoV2 infection in patients with TDT and to evaluate feasibility of home management for patients with mild disease. MATERIALS AND METHODS: The study involved TDT patients registered with thalassemia day care center, DMCH, who tested positive for COVID-19 by RTPCR. The demographics, clinical characteristics and baseline investigations were recorded. Patients with mild disease were managed at home and others were hospitalized. The daily home monitoring and the hospital course were noted and analyzed. RESULTS: The study involved 14 TDT patients who were infected with SARS-CoV2 with a mean age of 18.9 ± 6.7 years and a male to female ratio of 6:1. Five patients each were in low and high-risk groups and 4 patients were in highest risk group. The symptoms reported by these patients were fever, fatigue, sore throat etc. Two patients were hospitalized with one patient requiring oxygen therapy. He was discharged after 48 hours. The other patient had severe cardiac iron overload and diabetes mellitus. His iron chelation therapy was with held during hospitalization. He presented with a cardiac arrhythmia later and was cardioverted. Thus, all other patients were continued on iron chelation with deferasirox. Twelve patients were successfully managed at home with regular telephonic monitoring. CONCLUSION: Patients with thalassemia do not necessarily need hospitalization for management of COVID-19. Home management can be offered to patients with mild disease in a resource limited setting. Iron chelation with deferasirox can be continued safely.
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spelling pubmed-93609992022-08-09 Dutch-beta thalassemia: A rare mutation from India Sanghvi, Nirali Aggarwal, Priyanka Singh, Vineeta Gupta, Vineeta Pediatric Hematology Oncology Journal Article INTRODUCTION: COVID-19 pandemic imposed challenges towards management of transfusion-dependent thalassemia patients (TDT). The need for regular blood transfusions and iron chelation therapy in these patients added further uncertainty about managing COVID-19 in this subset of patients. AIMS: To describe the clinical manifestations of SARS-CoV2 infection in patients with TDT and to evaluate feasibility of home management for patients with mild disease. MATERIALS AND METHODS: The study involved TDT patients registered with thalassemia day care center, DMCH, who tested positive for COVID-19 by RTPCR. The demographics, clinical characteristics and baseline investigations were recorded. Patients with mild disease were managed at home and others were hospitalized. The daily home monitoring and the hospital course were noted and analyzed. RESULTS: The study involved 14 TDT patients who were infected with SARS-CoV2 with a mean age of 18.9 ± 6.7 years and a male to female ratio of 6:1. Five patients each were in low and high-risk groups and 4 patients were in highest risk group. The symptoms reported by these patients were fever, fatigue, sore throat etc. Two patients were hospitalized with one patient requiring oxygen therapy. He was discharged after 48 hours. The other patient had severe cardiac iron overload and diabetes mellitus. His iron chelation therapy was with held during hospitalization. He presented with a cardiac arrhythmia later and was cardioverted. Thus, all other patients were continued on iron chelation with deferasirox. Twelve patients were successfully managed at home with regular telephonic monitoring. CONCLUSION: Patients with thalassemia do not necessarily need hospitalization for management of COVID-19. Home management can be offered to patients with mild disease in a resource limited setting. Iron chelation with deferasirox can be continued safely. Pediatric Hematology Oncology Chapter of Indian Academy of Pediatrics. Publishing Services by Elsevier B.V. 2022-09 2022-06-09 /pmc/articles/PMC9360999/ http://dx.doi.org/10.1016/j.phoj.2022.06.001 Text en © 2022 Pediatric Hematology Oncology Chapter of Indian Academy of Pediatrics. Publishing Services by Elsevier B.V. Since January 2020 Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on the novel coronavirus COVID-19. The COVID-19 resource centre is hosted on Elsevier Connect, the company's public news and information website. Elsevier hereby grants permission to make all its COVID-19-related research that is available on the COVID-19 resource centre - including this research content - immediately available in PubMed Central and other publicly funded repositories, such as the WHO COVID database with rights for unrestricted research re-use and analyses in any form or by any means with acknowledgement of the original source. These permissions are granted for free by Elsevier for as long as the COVID-19 resource centre remains active.
spellingShingle Article
Sanghvi, Nirali
Aggarwal, Priyanka
Singh, Vineeta
Gupta, Vineeta
Dutch-beta thalassemia: A rare mutation from India
title Dutch-beta thalassemia: A rare mutation from India
title_full Dutch-beta thalassemia: A rare mutation from India
title_fullStr Dutch-beta thalassemia: A rare mutation from India
title_full_unstemmed Dutch-beta thalassemia: A rare mutation from India
title_short Dutch-beta thalassemia: A rare mutation from India
title_sort dutch-beta thalassemia: a rare mutation from india
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9360999/
http://dx.doi.org/10.1016/j.phoj.2022.06.001
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AT guptavineeta dutchbetathalassemiaararemutationfromindia