Survival and Predictors of Death Among Primary Immunodeficient Patients: A Registry-Based Study

Al‐Herz, Waleed; Moussa, Mohamed

doi:10.1007/s10875-011-9636-1

Cited by 39 publications

(30 citation statements)

References 19 publications

(18 reference statements)

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“…In individuals with various congenital immunodeficiencies, particularly severe combined immunodeficiency (SCID) syndrome, severe and recurrent pulmonary HAdV infections, and even lethal disseminated disease, are not uncommon (20,144), with reported fatality rates reaching up to 55% (43). In contrast, life-threatening disease currently appears to be relatively rare in acquired immunodeficiency associated with HIV infection (145,146), where HAdV infections are mostly associated with acute diarrhea only (147).…”

Section: Incidence Of Hadv Infections In Immunocompromised Adult and mentioning

confidence: 99%

Adenovirus Infections in Immunocompetent and Immunocompromised Patients

2014

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SUMMARY Human adenoviruses (HAdVs) are an important cause of infections in both immunocompetent and immunocompromised individuals, and they continue to provide clinical challenges pertaining to diagnostics and treatment. The growing number of HAdV types identified by genomic analysis, as well as the improved understanding of the sites of viral persistence and reactivation, requires continuous adaptions of diagnostic approaches to facilitate timely detection and monitoring of HAdV infections. In view of the clinical relevance of life-threatening HAdV diseases in the immunocompromised setting, there is an urgent need for highly effective treatment modalities lacking major side effects. The present review summarizes the recent progress in the understanding and management of HAdV infections.

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Section: Incidence Of Hadv Infections In Immunocompromised Adult and mentioning

confidence: 99%

Adenovirus Infections in Immunocompetent and Immunocompromised Patients

2014

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“…Infections are often severe and caused by unusual pathogens, but also recurrent respiratory infections caused by RSV and other widespread respiratory viruses are often a presenting feature of undiagnosed T-cell immunodeficiencies at a median age of four months [46, 47]. …”

Section: Immune Response To Rsv Infections In Infancymentioning

confidence: 99%

Respiratory Syncytial Virus Infections in Infants Affected by Primary Immunodeficiency

Lanari¹,

Vandini

Capretti

et al. 2014

Journal of Immunology Research

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Primary immunodeficiencies are rare inherited disorders that may lead to frequent and often severe acute respiratory infections. Respiratory syncytial virus (RSV) is one of the most frequent pathogens during early infancy and the infection is more severe in immunocompromised infants than in healthy infants, as a result of impaired T- and B-cell immune response unable to efficaciously neutralize viral replication, with subsequent increased viral shedding and potentially lethal lower respiratory tract infection. Several authors have reported a severe clinical course after RSV infections in infants and children with primary and acquired immunodeficiencies. Environmental prophylaxis is essential in order to reduce the infection during the epidemic season in hospitalized immunocompromised infants. Prophylaxis with palivizumab, a humanized monoclonal antibody against the RSV F protein, is currently recommended in high-risk infants born prematurely, with chronic lung disease or congenital heart disease. Currently however the prophylaxis is not routinely recommended in infants with primary immunodeficiency, although some authors propose the extension of prophylaxis to this high risk population.

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“…PID patients with a history of parental consanguinity have been found to present with more severe PID phenotypes, as documented by the high rates of complications and unusual infections [26,27] , poor overall performance status [28] , and a higher risk of death [29] . This could be due to an overrepresentation of more severe early-onset PIDs in these populations.…”

Section: Impact Of Parental Consanguinity On Pidsmentioning

confidence: 99%

Consanguinity and Primary Immunodeficiencies

et al. 2014

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Primary immunodeficiencies (PIDs) are a heterogeneous group of genetic disorders caused by defects in the immune system that predispose patients to infections, autoimmune diseases, lymphoproliferation and malignancies. Most PIDs are inherited in an autosomal recessive pattern; therefore, they are more common in areas with high rates of consanguineous marriage. Reports about PIDs from these areas have demonstrated a peculiar prevalence of more severe forms of diseases compared to other regions, and patients born to consanguineous parents have increased rates of morbidity and mortality compared to other patients. Individuals at high risk of having a child with a PID who wish to have a healthy child have limited options, these include prenatal diagnosis and pre-implantation genetic diagnosis. However, these options require a collaborative team of specialists and may not always be implemented due to geographic, religious, financial or social factors. The recent introduction of newborn-screening programs for a number of T and B lymphocyte deficiencies will facilitate early diagnosis and therapeutic interventions, which may include hematopoietic stem cell transplantation and intravenous immunoglobulin treatment. There is a need for the implementation of strategies to increase public awareness of the health risks associated with consanguineous marriage. It should be stressed that genetic counseling should be an important component of the care of patients with PIDs as well as their families.

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Survival and Predictors of Death Among Primary Immunodeficient Patients: A Registry-Based Study

Cited by 39 publications

References 19 publications

Adenovirus Infections in Immunocompetent and Immunocompromised Patients

Adenovirus Infections in Immunocompetent and Immunocompromised Patients

Respiratory Syncytial Virus Infections in Infants Affected by Primary Immunodeficiency

Consanguinity and Primary Immunodeficiencies

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