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Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent

Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,...

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Autores principales: Jindal, Ankur Kumar, Sil, Archan, Aggarwal, Ridhima, Vinay, Keshavamurthy, Bishnoi, Anuradha, Suri, Deepti, Rawat, Amit, Kumaran, Muthu Sendhil, Saikia, Biman, Sarkar, Rashmi, Gupta, Lalit, Kumar, D Dinesh, Jindal, Rashmi, Sukumaran, T U, Ouseph, Jose, Longhurst, Hilary, Pawankar, Ruby, Singh, Surjit, Dogra, Sunil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10287105/
https://www.ncbi.nlm.nih.gov/pubmed/37388810
http://dx.doi.org/10.5415/apallergy.0000000000000100
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author Jindal, Ankur Kumar
Sil, Archan
Aggarwal, Ridhima
Vinay, Keshavamurthy
Bishnoi, Anuradha
Suri, Deepti
Rawat, Amit
Kumaran, Muthu Sendhil
Saikia, Biman
Sarkar, Rashmi
Gupta, Lalit
Kumar, D Dinesh
Jindal, Rashmi
Sukumaran, T U
Ouseph, Jose
Longhurst, Hilary
Pawankar, Ruby
Singh, Surjit
Dogra, Sunil
author_facet Jindal, Ankur Kumar
Sil, Archan
Aggarwal, Ridhima
Vinay, Keshavamurthy
Bishnoi, Anuradha
Suri, Deepti
Rawat, Amit
Kumaran, Muthu Sendhil
Saikia, Biman
Sarkar, Rashmi
Gupta, Lalit
Kumar, D Dinesh
Jindal, Rashmi
Sukumaran, T U
Ouseph, Jose
Longhurst, Hilary
Pawankar, Ruby
Singh, Surjit
Dogra, Sunil
author_sort Jindal, Ankur Kumar
collection PubMed
description Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,000 to 135,000 patients with HAE in India at present. The majority of these, however, remain undiagnosed. Replacement of plasma-derived or recombinant C1-esterase inhibitor (C1-INH) protein, administered intravenously, is the treatment of choice during the management of acute episodes of angioedema (i.e., “on-demand treatment”) and is also useful for short-term prophylaxis (STP) and long-term prophylaxis (LTP). This has been found to be effective and safe even in young children and during pregnancy. Until recently, none of the first-line treatment options were available for “on-demand treatment,” STP or LTP in India. As a result, physicians had to use fresh frozen plasma for both “on-demand treatment” and STP. For LTP, attenuated androgens (danazol or stanozolol) and/or tranexamic acid were commonly used. These drugs have been reported to be useful for LTP but are associated with a significant risk of adverse effects. Intravenous pd-C1-INH, the first-line treatment option, is now available in India. However, because there is no universal health insurance, access to pd-C1-INH is a significant challenge. HAE Society of India has developed these consensus guidelines for India and other resource-constrained settings where plasma-derived C1-INH therapy is the only available first-line treatment option for the management of HAE and diagnostic facilities are limited. These guidelines have been developed because it may not be possible for all patients to access the recommended therapy and at the recommended doses as suggested by the international guidelines. Moreover, it may not be feasible to follow the evaluation algorithm suggested by the international guidelines.
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spelling pubmed-102871052023-06-29 Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent Jindal, Ankur Kumar Sil, Archan Aggarwal, Ridhima Vinay, Keshavamurthy Bishnoi, Anuradha Suri, Deepti Rawat, Amit Kumaran, Muthu Sendhil Saikia, Biman Sarkar, Rashmi Gupta, Lalit Kumar, D Dinesh Jindal, Rashmi Sukumaran, T U Ouseph, Jose Longhurst, Hilary Pawankar, Ruby Singh, Surjit Dogra, Sunil Asia Pac Allergy Original Study Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,000 to 135,000 patients with HAE in India at present. The majority of these, however, remain undiagnosed. Replacement of plasma-derived or recombinant C1-esterase inhibitor (C1-INH) protein, administered intravenously, is the treatment of choice during the management of acute episodes of angioedema (i.e., “on-demand treatment”) and is also useful for short-term prophylaxis (STP) and long-term prophylaxis (LTP). This has been found to be effective and safe even in young children and during pregnancy. Until recently, none of the first-line treatment options were available for “on-demand treatment,” STP or LTP in India. As a result, physicians had to use fresh frozen plasma for both “on-demand treatment” and STP. For LTP, attenuated androgens (danazol or stanozolol) and/or tranexamic acid were commonly used. These drugs have been reported to be useful for LTP but are associated with a significant risk of adverse effects. Intravenous pd-C1-INH, the first-line treatment option, is now available in India. However, because there is no universal health insurance, access to pd-C1-INH is a significant challenge. HAE Society of India has developed these consensus guidelines for India and other resource-constrained settings where plasma-derived C1-INH therapy is the only available first-line treatment option for the management of HAE and diagnostic facilities are limited. These guidelines have been developed because it may not be possible for all patients to access the recommended therapy and at the recommended doses as suggested by the international guidelines. Moreover, it may not be feasible to follow the evaluation algorithm suggested by the international guidelines. Lippincott Williams & Wilkins 2023-06-06 2023-06 /pmc/articles/PMC10287105/ /pubmed/37388810 http://dx.doi.org/10.5415/apallergy.0000000000000100 Text en Copyright © 2023. Asia Pacific Association of Allergy, Asthma and Clinical Immunology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Original Study
Jindal, Ankur Kumar
Sil, Archan
Aggarwal, Ridhima
Vinay, Keshavamurthy
Bishnoi, Anuradha
Suri, Deepti
Rawat, Amit
Kumaran, Muthu Sendhil
Saikia, Biman
Sarkar, Rashmi
Gupta, Lalit
Kumar, D Dinesh
Jindal, Rashmi
Sukumaran, T U
Ouseph, Jose
Longhurst, Hilary
Pawankar, Ruby
Singh, Surjit
Dogra, Sunil
Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent
title Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent
title_full Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent
title_fullStr Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent
title_full_unstemmed Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent
title_short Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent
title_sort management of hereditary angioedema in resource-constrained settings: a consensus statement from indian subcontinent
topic Original Study
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10287105/
https://www.ncbi.nlm.nih.gov/pubmed/37388810
http://dx.doi.org/10.5415/apallergy.0000000000000100
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