Incidence and Temporal Trends of Primary Immunodeficiency: A Population-Based Cohort Study

Joshi, Avni Y.; Iyer, Vivek N.; Hagan, John B.; St. Sauver, Jennifer L.; Boyce, Thomas G.
January 2009
Mayo Clinic Proceedings;Jan2009, Vol. 84 Issue 1, p16
Academic Journal
OBJECTIVE: To determine the Incidence and temporal trends of primary immunodeficiency diseases (PIDs) and examine whether an association exists between delayed diagnosis and increased morbidity. PATIENTS AND METHODS: We performed a historical cohort study to describe the epidemiology of PIDs in Olmsted County, Minnesota, during a 31-year period from January 1, 1976, through December 31, 2006, using the Rochester Epidemiology Project. Incidence and trends over time, presence of comorbid conditions, and trends In management were determined. RESULTS: During the 31-year study period, 158 new cases of PIDs were diagnosed, with an overall Incidence rate of 4.6 per 100,000 person-years. The rate of PIDs from 2001 through 2006 (10.3 per 100,000 person-years) was nearly 5 times higher than that from 1976 through 1980 (2.4 per 100,000 person-years). The associations between continuous variable(s) and categorical outcome(s) were assessed by using the Wilcoxon rank sum test. Longer delay in diagnosis was significantly associated with recurrent sinusitis (P<.001), recurrent pneumonia (P=.03), and subsequent treatment with immunoglobulins (P<.001). On the basis of Kaplan-Meier survival estimates, the proportion of patients surviving at 10 years after diagnosis was 93.5% (95% confidence interval, 85.9%-97.1%). However, older age at diagnosis was significantly associated with mortality (P=.01). CONCLUSION: This Is one of the first population-based studies to examine the temporal trends of PIDs. The incidence of PIDs increased markedly between 1976 and 2006. In this cohort, a delay In diagnosis was common and was associated with Increased morbidity. Despite substantial morbidity, most patients with PIDs can expect a normal life span.


Related Articles

  • Nanobodies: The ultrasmall antibodies. Nicholls, Henry // New Scientist;10/6/2007, Vol. 196 Issue 2624, p50 

    The article describes the antibodies that have been found in the blood of camels. These antibodies are smaller than the conventional sort, which means that they can exist in greater quantity, perform a higher number of beneficial tasks and are less expensive to manufacture. These antibodies are...

  • CSF oligoclonal band status informs prognosis in multiple sclerosis: a case control study of 100 patients. F G Joseph // Journal of Neurology, Neurosurgery & Psychiatry;Mar2009, Vol. 80 Issue 3, p292 

    OBJECTIVE: Oligoclonal band (OCB) negative multiple sclerosis (MS) is well recognised but uncommon, studied in only a few usually small case series. These reached differing conclusions on whether its clinical features or course differ from OCB positive disease. The study hypothesis was that a...

  • Evaluation of serum IgA levels in Iranian patients with type 1 diabetes mellitus. Sayarifard, Fatemeh; Aghamohammadi, Asghar; Haghi-Ashtiani, Mohammad; Rajab, Asadollah; Irani, Heshmat; Ahmadian, Javad; Zaridoost, Ahya; Parvaneh, Nima; Rezaei, Nima; Rabbani, Ali // Acta Diabetologica;Apr2012, Vol. 49 Issue 2, p131 

    An increased prevalence of immunoglobulin (Ig) A deficiency has been documented in a number of autoimmune diseases; however, its association with type 1 diabetes mellitus (DM1) is a subject of debate. This study was performed to evaluate serum IgA levels in a group of pediatric patients with...

  • Monoclonal Antibodies: an Introduction. Rizvi, Syed A. // Medicine & Health Rhode Island;Nov2011, Vol. 94 Issue 11, p316 

    The article focuses on the monoclonal antibodies (MCA), which are antibodies that are same and came from one type of immune cell. It mentions that the antibodies main nature makes MCA extraordinary and gives way the potential of revolutionary applications like focused therapy and other...

  • Assessment of a novel, capsid-modified adenovirus with an improved vascular gene transfer profile. White, Katie M.; Alba, Raul; Parker, Alan L; Wright, Audrey F.; Bradshaw, Angela C.; Delles, Christian; McDonald, Robert A.; Baker, Andrew H. // Journal of Cardiothoracic Surgery;2013, Vol. 8 Issue 1, p1 

    Background: Cardiovascular disorders, including coronary artery bypass graft failure and in-stent restenosis remain significant opportunities for the advancement of novel therapeutics that target neointimal hyperplasia, a characteristic of both pathologies. Gene therapy may provide a successful...

  • Clusterin expression can be modulated by changes in TCF1-mediated Wnt signaling. Schepeler, Troels; Mansillar, Francisco; Christensenr, Lise L.; ├śrntoftr, Torben F.; Andersenr, Claus L. // Journal of Molecular Signaling;2007, Vol. 2, p6 

    Background: Clusterin (CLU) is an enigmatic molecule associated with various physiological processes and disease states. Different modes of cellular stress lead to increased CLU levels, and additionally numerous growth factors and cytokines affect the expression of the CLU gene. APC and c-MYC,...

  • Viral checkmate. de Souza, Natalie // Nature Methods;Sep2007, Vol. 4 Issue 9, p686 

    Researchers use the tremendous combinatorial diversity of recombinant antibody libraries to enable mapping of viral trajectories in vitro.

  • Serum Immunoglobulins (IgG, IgA, IgM) levels in Preeclampsia and Eclampsia Pregnancies. Ahsan, Touhida; Wahab, Farzana; Kamal, Md. Mustafa; Islam, Sheikh. Nazrul // Internet Journal of Third World Medicine;2009, Vol. 8 Issue 1, p16 

    Bacground: Preeclampsia is the most common pregnancy-specific complication. It is thought to be associated with modulation of immune response. Objective: The aim of this study was to investigate the serum levels of immunoglobulins-IgG, IgA and IgM in pre-eclampsia and eclampsia, and to examine...

  • Immunopathology of Psoriasis: A Comparison with Other Parakeratotic Lesions. Weiss, Virginia C.; van Den Broek, Hans; Barrett, Shari; West, Dennis P. // Journal of Investigative Dermatology;Mar1982, Vol. 78 Issue 3, p256 

    Characteristic in vivo binding of IgG and C3 to the stratum corneum of psoriatic lesions has been implicated as a significant event in the pathogenesis of the disease. Immunofluorescent findings of psoriatic lesions are compared with other parakeratotic and nonparakeratotic lesions....


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics