Impact of SARS-CoV-2 Pandemic on Patients with Primary Immunodeficiency
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ORIGINAL ARTICLE
Impact of SARS-CoV-2 Pandemic on Patients with Primary Immunodeficiency Samaneh Delavari 1 & Hassan Abolhassani 1,2 & Farhad Abolnezhadian 3 & Fateme Babaha 1,4 & Sara Iranparast 5 & Hamid Ahanchian 6 & Nasrin Moazzen 6 & Mohammad Nabavi 7 & Saba Arshi 7 & Morteza Fallahpour 7 & Mohammad Hassan Bemanian 7 & Sima Shokri 7 & Tooba Momen 8 & Mahnaz Sadeghi-Shabestari 9 & Rasol Molatefi 10 & Afshin Shirkani 11 & Ahmad Vosughimotlagh 12 & Molood Safarirad 12 & Meisam Sharifzadeh 13 & Salar Pashangzadeh 1 & Fereshte Salami 1 & Paniz Shirmast 1 & Arezou Rezaei 1 & Tannaz Moeini Shad 1 & Minoo Mohraz 14 & Nima Rezaei 1 & Lennart Hammarström 2 & Reza Yazdani 1 & Asghar Aghamohamamdi 1 Received: 23 August 2020 / Accepted: 16 November 2020 # The Author(s) 2020
Abstract Although it is estimated that COVID-19 life-threatening conditions may be diagnosed in less than 1:1000 infected individuals below the age of 50, but the real impact of this pandemic on pediatric patients with different types of primary immunodeficiency (PID) is not elucidated. The current prospective study on a national registry of PID patients showed that with only 1.23 folds higher incidence of infections, these patients present a 10-folds higher mortality rate compared to population mainly in patients with combined immunodeficiency and immune dysregulation. Therefore, further management modalities against COVID-19 should be considered to improve the survival rate in these two PID entities using hematopoietic stem cell transplantation and immunomodulatory agents. Keywords COVID-19 . Primary immunodeficiency . Severe viral infection . Mortality rate
Introduction The novel coronavirus disease, known as coronavirus disease 2019 (COVID-19), is an acute infectious respiratory disease that first emerged in Wuhan, China, in late 2019 and is characterized as a pandemic in mid-March 2020 by World Health Organization [1]. It has been found that COVID-19 causes severe acute respiratory syndrome (SARS, therefore coined as SARS-CoV-2) similar to two other RNA viruses from the Coronoviridea family SARS-CoV-1 and Middle East Samaneh Delavari and Hassan Abolhassani contributed equally to this work. Article Summary Line Patients with combined immunodeficiency and immune dysregulation are at a high risk of mortality due to SARS-CoV-2 compared to other types of primary immunodeficiency. * Hassan Abolhassani [email protected] * Asghar Aghamohamamdi [email protected] Extended author information available on the last page of the article
respiratory syndrome coronavirus (MERS-CoV). SARSCoV-2 main route of entry to a human host is the angiotensin-converting enzyme 2 (ACE2) receptor. ACE2 receptor is ubiquitously expressed on the surfaces of various cell types, including cells of the airway epithelium which is the major site of infection [2]. Besides the ACE2 receptor, SARSCoV-2 uses the transmembrane serine protease 2 (TMPRSS2), a cellular serine protease, for the host cell entry, which activates the SARS-CoV-2 spike protein by cleaving the Furin
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