Carolyn Baloh1,2, Anupama Reddy2,3, Michele Henson1,2, Katherine Prince2,4, Rebecca Buckley1,2,5, Patricia Lugar6,7,8,9. 1. Department of Pediatrics, Duke University Medical Center, Durham, NC, USA. 2. Duke University Medical Center, Durham, NC, USA. 3. Center for Genomic and Computational Biology, Duke University Medical Center, Durham, NC, USA. 4. Department of Internal Medicine, Duke University Medical Center, Durham, NC, USA. 5. Department of Immunology, Duke University Medical Center, Durham, NC, USA. 6. Department of Pediatrics, Duke University Medical Center, Durham, NC, USA. Patricia.lugar@duke.edu. 7. Duke University Medical Center, Durham, NC, USA. Patricia.lugar@duke.edu. 8. Department of Internal Medicine, Duke University Medical Center, Durham, NC, USA. Patricia.lugar@duke.edu. 9. Departments of Internal Medicine and Pediatrics, Division of Pulmonary, Allergy and Critical Care, Duke University Medical Center, 1821 Hillandale Road, Suite 25A, Durham, NC, 27705, USA. Patricia.lugar@duke.edu.
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.
PURPOSE: To evaluate mortality risk factors in pediatric-onset common variable immunodeficiency disorders (CVID), we evaluated the largest single-institution cohort of pediatric-onset CVIDpatients. 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 immunodeficientpatients.
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