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Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center

INTRODUCTION: Early diagnosis and treatment are keys to improve survival of patients with primary immunodeficiency diseases (PID). The clinical characteristics of these patients in Thailand were not well defined. OBJECTIVE: This study aimed to determine the clinical characteristics and outcomes of p...

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Autores principales: Benjasupattananan, P., Simasathein, T., Vichyanond, P., Leungwedchakarn, V., Visitsunthorn, N., Pacharn, P., Jirapongsananuruk, O.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7102384/
https://www.ncbi.nlm.nih.gov/pubmed/19184381
http://dx.doi.org/10.1007/s10875-008-9273-5
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author Benjasupattananan, P.
Simasathein, T.
Vichyanond, P.
Leungwedchakarn, V.
Visitsunthorn, N.
Pacharn, P.
Jirapongsananuruk, O.
author_facet Benjasupattananan, P.
Simasathein, T.
Vichyanond, P.
Leungwedchakarn, V.
Visitsunthorn, N.
Pacharn, P.
Jirapongsananuruk, O.
author_sort Benjasupattananan, P.
collection PubMed
description INTRODUCTION: Early diagnosis and treatment are keys to improve survival of patients with primary immunodeficiency diseases (PID). The clinical characteristics of these patients in Thailand were not well defined. OBJECTIVE: This study aimed to determine the clinical characteristics and outcomes of patients with PID in Thailand. METHODS: Medical records of PID patients in the past 18 years were reviewed. RESULTS: Sixty-seven children were registered. Antibody deficiencies were the most common PID (52.2%), followed by combined T cell and B cell immunodeficiencies (25.4%), other well-defined immunodeficiency syndromes (11.9%), and phagocytic defects (10.4%). The most common presentations of antibody deficiencies, combined T cell and B cell immunodeficiencies, and phagocytic defects were infection in the upper respiratory tract (74.3%), gastrointestinal tract (82.4%), and skin (85.7%), respectively. The highest mortality rate (52.9%) was found in severe combined immunodeficiency. CONCLUSION: These results provide clinical features of PID in Thailand. Knowing these features will lead to prompt diagnosis and appropriate management.
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spelling pubmed-71023842020-03-31 Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center Benjasupattananan, P. Simasathein, T. Vichyanond, P. Leungwedchakarn, V. Visitsunthorn, N. Pacharn, P. Jirapongsananuruk, O. J Clin Immunol Article INTRODUCTION: Early diagnosis and treatment are keys to improve survival of patients with primary immunodeficiency diseases (PID). The clinical characteristics of these patients in Thailand were not well defined. OBJECTIVE: This study aimed to determine the clinical characteristics and outcomes of patients with PID in Thailand. METHODS: Medical records of PID patients in the past 18 years were reviewed. RESULTS: Sixty-seven children were registered. Antibody deficiencies were the most common PID (52.2%), followed by combined T cell and B cell immunodeficiencies (25.4%), other well-defined immunodeficiency syndromes (11.9%), and phagocytic defects (10.4%). The most common presentations of antibody deficiencies, combined T cell and B cell immunodeficiencies, and phagocytic defects were infection in the upper respiratory tract (74.3%), gastrointestinal tract (82.4%), and skin (85.7%), respectively. The highest mortality rate (52.9%) was found in severe combined immunodeficiency. CONCLUSION: These results provide clinical features of PID in Thailand. Knowing these features will lead to prompt diagnosis and appropriate management. Springer US 2009-01-28 2009 /pmc/articles/PMC7102384/ /pubmed/19184381 http://dx.doi.org/10.1007/s10875-008-9273-5 Text en © Springer Science+Business Media, LLC 2009 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.
spellingShingle Article
Benjasupattananan, P.
Simasathein, T.
Vichyanond, P.
Leungwedchakarn, V.
Visitsunthorn, N.
Pacharn, P.
Jirapongsananuruk, O.
Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center
title Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center
title_full Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center
title_fullStr Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center
title_full_unstemmed Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center
title_short Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center
title_sort clinical characteristics and outcomes of primary immunodeficiencies in thai children: an 18-year experience from a tertiary care center
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7102384/
https://www.ncbi.nlm.nih.gov/pubmed/19184381
http://dx.doi.org/10.1007/s10875-008-9273-5
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