30-Year Review of Pediatric- and Adult-Onset CVID: Clinical Correlates and Prognostic Indicators.
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2019-10
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Abstract
Purpose
To evaluate mortality risk factors in pediatric-onset common variable immunodeficiency disorders (CVID), we evaluated the largest single-institution cohort of pediatric-onset CVID patients. Previous publications on CVID have provided valuable descriptive data, but lack risk stratification to guide physicians in management of these patients.Methods
Retrospective chart review of 198 subjects with CVID at a single institution, of whom 91 had disease onset at a pediatric age. Clinical and laboratory data were collected at diagnosis and in follow-up. Odds ratios and Fisher tests were utilized to examine trends. This study was approved by an institutional review board.Results
Clinical features and laboratory results for subjects diagnosed with CVID at a pediatric age are similar to those who had adult-onset CVID. However, majority of the deceased subjects (13/18) were at a pediatric age at CVID symptom onset. These subjects had a lower age at mortality, multiple comorbidities, and often depression. The most common cause of death was infection. Lung disease (OR 5, p < 0.05) and infection with severe/opportunistic organisms (OR 9, p < 0.05) are directly related to increased mortality. Delay in diagnosis of CVID is also correlated with mortality. Intermediary markers correlating with mortality include anemia, GERD, and depression.Conclusions
There are many similarities between patients with pediatric- and adult-onset CVID; however, the mortality of pediatric CVID in our cohort is striking. This is the first study to identify specific factors correlated with mortality in pediatric-onset CVID to guide pediatricians and subspecialists in managing these immunodeficient patients.Type
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Baloh, Carolyn, Anupama Reddy, Michele Henson, Katherine Prince, Rebecca Buckley and Patricia Lugar (2019). 30-Year Review of Pediatric- and Adult-Onset CVID: Clinical Correlates and Prognostic Indicators. Journal of clinical immunology, 39(7). pp. 678–687. 10.1007/s10875-019-00674-9 Retrieved from https://hdl.handle.net/10161/32136.
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Rebecca Hatcher Buckley
The overall emphasis of Dr. Buckley's research is in human T,B and NK cell development and in aberrations in their development and regulation. The work involves three particular areas of investigation: 1) the cellular and molecular bases of genetically-determined human immunodeficiency diseases, 2) the use of bone marrow stem cells to cure genetically-determined immunodeficiency diseases, and 3) the use of human SCID bone marrow stem cell chimeras to study human thymic education, T and B cell ontogeny, tolerance induction and MHC restriction mechanisms. Methodology includes monoclonal antibody (mAb) analyses of lymphocyte phenotypes, a variety of T cell and natural killer (NK) cell functional assays, studies of thymic output by T cell receptor recombination excision circle measurement, studies of T cell diversity by spectratyping, studies of T cell longevity by telomere analysis and assessment of B cell differentiation and function. A unique resource available for her studies is the largest population of patients with genetically-determined immunodeficiency diseases in the U.S., which includes the largest population in the world of longterm SCID chimeras treated at a single center, some of whom have been studied and followed for more than 37 years. The administration of rigorously T cell depleted haploidentical bone marrow stem cells to SCID recipients without pre-transplant conditioning or post-transplant use of immunosuppressive drugs to prevent GVHD provides an unmanipulated system for studying human thymic education, T and B cell ontogeny, MHC restriction mechanisms and tolerance induction. Studies to identify mutations in patients with primary immunodeficiency are continuing, particularly in those with SCID.
Patricia Lynne Lugar
I have a strong interest and research background in the study of dysregulated B lymphocytes, B cells, that characterize clinical disease such as autoimmune disease and humoral immune deficiency. My current research projects are aimed at the study of humoral immune deficiency, specifically common variable immune deficiency or CVID and the dysregulation of B cells in the generation of lymphoma and autoimmune disease.
Other ongoing research projects include characterizing the molecular and cellular events that cause a common disease, chronic idiopathic urticaria or CIU and rare disorders idiopathic anaphylaxis and idiopathic angioedema.
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