Primary immunodeficiency diseases in adulthood

Internal Medicine Journal
D S Riminton, S Limaye

Abstract

Occasionally, patients present with clinical episodes of infectious disease that seem to fall beyond the scope of normal immunocompetence. Examples include infections that are unusually persistent, recurrent or resistant to treatment, or those involving unexpected dissemination of disease or atypical pathogens. Recent cellular and molecular advances in immunology help to inform the investigation and management of these cases and provide opportunities for family surveillance and disease prevention. More than 100 separate primary (inherited) immunodeficiency disease (PID) states have now been defined, each with a range of syndromic manifestations. Many of these are rare diseases of childhood, but some, either in atypical or attenuated forms, emerge for the first time in adulthood. We outline a diagnostic approach to five groups of PID presenting in adulthood and describe the recently launched web-based PID Register of Australia and New Zealand--a new mechanism for improving knowledge and service delivery to Australasian patients and their clinical carers.

References

Sep 22, 1975·JAMA : the Journal of the American Medical Association·M J ChusidA S Fauci
Oct 6, 1997·The Journal of Allergy and Clinical Immunology·K W BaumgartD Roberton
Mar 4, 1999·The New England Journal of Medicine·B GrimbacherJ M Puck
May 3, 2000·Clinical and Experimental Immunology·L HammarströmD Webster
Sep 6, 2000·Clinical and Experimental Immunology·D A LammasD S Kumararatne
Dec 9, 2000·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Y MinegishiM E Conley
Feb 24, 2001·Immunological Reviews·Q Pan, L Hammarström
Feb 24, 2001·Australian and New Zealand Journal of Public Health·N DonnellyW Hall
Jan 10, 2002·Genes and Immunity·D M FruchtF Candotti
Feb 28, 2002·Annual Review of Immunology·Jean-Laurent Casanova, Laurent Abel
Apr 20, 2002·Current Opinion in Allergy and Clinical Immunology·J ReichenbachJ L Casanova
Apr 20, 2002·Current Opinion in Allergy and Clinical Immunology·A Durandy
Sep 3, 2002·Nature Genetics·Patrick Concannon
Dec 25, 2002·Journal of Clinical Pathology·P Kelleher, S A Misbah
Feb 11, 2003·Nature Immunology·Bodo GrimbacherHans H Peter
Mar 11, 2003·Journal of Medical Virology·M HurmeM Helminen

❮ Previous
Next ❯

Citations

Dec 13, 2006·Clinical & Developmental Immunology·Arvind KumarM Eric Gershwin
Sep 1, 2005·Journal of Clinical Immunology·Davood MansouriStuart E Turvey
Sep 29, 2011·Bioinformatics·Nico AdamsChristian Hennig
Sep 25, 2012·Molecular Immunology·David C Kilpatrick
Sep 1, 2010·Surgical Pathology Clinics·Pierre Russo
Jul 29, 2006·Pediatric Clinics of North America·James W Verbsky, William J Grossman
Jul 15, 2015·The Journal of Allergy and Clinical Immunology. in Practice·Vincent Robert Bonagura
May 20, 2014·Vaccine·Nicola Principi, Susanna Esposito
Nov 12, 2016·The Journal of Allergy and Clinical Immunology. in Practice·Elli RosenbergJudah A Denburg

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.