[Recurrent infections of the upper aerodigestive tract in patients with primary immunodeficiency].
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DOI: 10.1007/s00106-019-0683-x
Abstract: BACKGROUND Primary immunodeficiency is a rare disease of humoral and cellular immune defense, which can lead to severe and recurrent infections of different organs. The diagnosis of this disease is often difficult, and its early identification… read more here.
Keywords: immunodeficiency; recurrent infections; ent infections; infections upper ... See more keywords
Low Rates of Poliovirus Antibodies in Primary Immunodeficiency Patients on Regular Intravenous Immunoglobulin Treatment
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DOI: 10.1007/s10875-018-0531-x
Abstract: PurposePoliovirus has been nearly eliminated as part of a world-wide effort to immunize and contain circulating wild-type polio. Nevertheless, poliovirus has been detected in water supplies and represents a threat to patients with humoral immunodeficiencies… read more here.
Keywords: intravenous immunoglobulin; regular intravenous; primary immunodeficiency; patients regular ... See more keywords
Nasal Nitric Oxide in Primary Immunodeficiency and Primary Ciliary Dyskinesia: Helping to Distinguish Between Clinically Similar Diseases
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DOI: 10.1007/s10875-019-00613-8
Abstract: PurposePrimary ciliary dyskinesia (PCD) is a rare disorder of the mucociliary clearance leading to recurrent upper and lower respiratory tract infections. PCD is difficult to clinically distinguish from other entities leading to recurrent oto-sino-pulmonary infections,… read more here.
Keywords: ciliary dyskinesia; primary immunodeficiency; nasal nitric; nitric oxide ... See more keywords
Should MASP-2 Deficiency Be Considered a Primary Immunodeficiency? Relevance of the Lectin Pathway
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DOI: 10.1007/s10875-019-00714-4
Abstract: Mannose-binding lectin (MBL)-associated serine protease-2 (MASP-2) is an indispensable enzyme for the activation of the lectin pathway of complement. Its deficiency is classified as a primary immunodeficiency associated to pyogenic bacterial infections, inflammatory lung disease,… read more here.
Keywords: lectin pathway; deficiency; masp deficiency; primary immunodeficiency ... See more keywords
Health-Related Quality of Life and Emotional Difficulties in Chronic Granulomatous Disease: Data on Adult and Pediatric Patients from Italian Network for Primary Immunodeficiency (IPINet)
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DOI: 10.1007/s10875-019-00725-1
Abstract: Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by life-threatening infections, inflammation, and autoimmunity with an impact on health-related quality of life (HRQoL). Few data are available for children, whereas no study has been… read more here.
Keywords: granulomatous disease; chronic granulomatous; health related; primary immunodeficiency ... See more keywords
Adoptive T Cell Immunotherapy for Patients with Primary Immunodeficiency Disorders
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DOI: 10.1007/s11882-017-0669-2
Abstract: Primary immunodeficiency disorders (PID) are a group of inborn errors of immunity with a broad range of clinical severity but often associated with recurrent and serious infections. While hematopoietic stem cell transplantation (HSCT) can be… read more here.
Keywords: pid patients; immunodeficiency disorders; immunotherapy; primary immunodeficiency ... See more keywords
Hematopoietic stem cell transplantation for pulmonary alveolar proteinosis associated with primary immunodeficiency disease
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DOI: 10.1007/s12185-017-2375-1
Abstract: Pulmonary alveolar proteinosis (PAP) is a rare disorder that is characterized by the excessive accumulation of surfactant-like materials in the alveoli, leading to hypoxemic respiratory failure. We describe two Japanese infants with PAP associated with… read more here.
Keywords: pulmonary alveolar; cell transplantation; primary immunodeficiency; stem cell ... See more keywords
Safety and tolerability of IgPro10 in Japanese primary immunodeficiency patients: a registrational study
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DOI: 10.1007/s12185-021-03106-w
Abstract: Studies investigating the safety of IgPro10 (Privigen®, CSL Behring, King of Prussia, PA, USA) in Japanese patients with primary immunodeficiency (PID) are lacking. This study evaluated safety and tolerability of IgPro10 in Japanese patients with… read more here.
Keywords: igpro10; safety tolerability; safety; primary immunodeficiency ... See more keywords
Primary immunodeficiency diseases in Northern Iran.
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DOI: 10.1016/j.aller.2016.11.001
Abstract: INTRODUCTION Primary immunodeficiency diseases (PID) are a heterogeneous group of inherited disorders, characterised by recurrent severe infections, autoimmunity and lymphoproliferation. Despite impressive progress in identification of novel PID, there is an unfortunate lack of awareness… read more here.
Keywords: northern iran; diseases northern; immunodeficiency diseases; primary immunodeficiency ... See more keywords
Hematological Malignancies Associated With Primary Immunodeficiency Disorders.
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DOI: 10.1016/j.clim.2018.06.011
Abstract: Primary Immunodeficiency disorders (PID) have been increasingly recognized in association with hematologic malignancies. To better appreciate this association, a systemic search of the Ovid MEDLINE database was performed with terms related of hematologic malignancies and… read more here.
Keywords: immunodeficiency; immunodeficiency disorders; primary immunodeficiency; hematologic malignancies ... See more keywords
A novel NEMO/IKBKG mutation identified in a primary immunodeficiency disorder with recurrent atypical mycobacterial infections
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DOI: 10.1016/j.jdcr.2020.10.019
Abstract: EDA-ID: ectodermal dysplasia and immunodeficiency NEMO: nuclear factor-kB (NF-kB) essential modulator NF-kB: nuclear factor-kB PID: primary immunodeficiency disorder INTRODUCTION Hypomorphicmutations in the IKBKG gene, which encodes nuclear factor-kB (NF-kB) essential modulator (NEMO), on the X… read more here.
Keywords: immunodeficiency; primary immunodeficiency; immunodeficiency disorder; ectodermal dysplasia ... See more keywords