What Immunological Defects Predispose to Non-tuberculosis Mycobacterial Infections? [PDF]
Nontuberculous mycobacteria (NTM) are categorized as one of the large and diverse groups of environmental organisms which are abundant in water and soil. NTM cause a variety of diseases in humans that mainly affect the lung.
Adcock, IM +7 more
core
Genetic basis of primary immunodeficiencies. Combined immunodeficiencies (review)
Background. Primary immunodeficiencies are a large and heterogeneous group of hereditary disorders characterized by disorders in the development, functioning or regulation of the immune system.
L.V. Bieliaieva +3 more
doaj +1 more source
Efficacy and safety of Privigen® in patients with chronic inflammatory demyelinating polyneuropathy: results of a prospective, single-arm, open-label Phase III study (the PRIMA study) [PDF]
This prospective, multicenter, single-arm, open-label Phase III study aimed to evaluate the efficacy and safety of Privigen (R) (10% liquid human intravenous immunoglobulin [IVIG], stabilized with l-proline) in patients with chronic inflammatory ...
Bauhofer, Artur +12 more
core +1 more source
Human inmmunoglobulin in primary immunodeficiencies
Human immunoglobulin replacement therapy has become a corner- stone in the treatment of patients with primary immunodeficiencies (PID). Currently indicated as first-line therapy for predominantly antibody deficiencies, severe combined immunodeficiencies,
Sheffler Mendoza Selma +2 more
doaj +1 more source
The European internet-based patient and research database for primary immunodeficiencies: results 2006-2008 [PDF]
Primary immunodeficiencies (PID) are rare diseases; therefore transnational studies are essential to maximize the scientific outcome and to improve diagnosis and therapy.
Abuzakouk M. +70 more
core +1 more source
Registry of primary immunodeficiencies in children at a fourth level hospital. Bogota, 2010-2016
Background: Primary immunodeficiencies are inherited diseases that compromise numerous systems and whose clinical manifestation is varied. It is an underdiagnosed pathology in Colombia.
Ángela Pedraza +2 more
doaj +1 more source
Unexpectedly High Prevalence of Common Variable Immunodeficiency in Finland [PDF]
Background: Common variable immunodeficiency (CVID) is the most common primary immunodeficiency. Prevalence varies greatly between countries and studies. Most diagnostic criteria include hypogammaglobulinemia and impaired vaccine response.
Aalto, Arja H. +13 more
core +1 more source
Pemphigus vulgaris in a patient with primary hypogammaglobulinemia: A case report
Pemphigus vulgaris (PV) is a rare autoimmune disorder characterized by blisters on the mucous membranes and skin. Autoimmunity is an important complication developing in predominantly antibody deficiencies, which is a subgroup of primary ...
Uğur Hacı Musabak +2 more
doaj +1 more source
Medical awareness concerning primary immunodeficiency diseases in the city of São Paulo, Brazil [PDF]
OBJETIVO: Avaliar o conhecimento médico sobre as imunodeficiências primárias na cidade de São Paulo (SP). MÉTODOS: Um questionário de 14 questões sobre as imunodeficiências primárias foi aplicado a médicos que trabalhavam em hospitais gerais.
Aranda, Carolina Sanchez +11 more
core +4 more sources
Case Report: Hypomorphic Ligase 4 deficiency – a paradigm of immunodysregulation
DNA Ligase 4 is critical to nonhomologous end joining, necessary for V(D)J recombination in T and B cell development. Ligase 4 deficiency is a rare autosomal recessive disorder caused by hypomorphic mutations in the DNA Ligase 4 gene, that can lead to a ...
Catarina Andrade +8 more
doaj +1 more source

