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30-Year Review of Pediatric- and Adult-Onset CVID: Clinical Correlates and Prognostic Indicators

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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.

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Abbreviations

CVID:

Common variable immunodeficiency disorders

ESID:

European Society for Immunodeficiencies

GLILD:

Granulomatous lymphocytic interstitial lung disease

GERD:

Gastroesophageal reflux disease

LOCID:

Late-onset combined immune deficiency

MRSA:

Methicillin-resistant Staphylococcus aureus

USIDNET:

United States Immunodeficiency Network

VRE:

Vancomycin-resistant Enterococcus

IRB:

Institutional review board

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Funding

All phases of this study were financially supported by 1T32HG008955-01A1 and the Steifel Family Immunology Fund.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Duke University Medical Center, Durham, NC, USA

    Carolyn Baloh, Michele Henson, Rebecca Buckley & Patricia Lugar

  2. Duke University Medical Center, Durham, NC, USA

    Carolyn Baloh, Anupama Reddy, Michele Henson, Katherine Prince, Rebecca Buckley & Patricia Lugar

  3. Center for Genomic and Computational Biology, Duke University Medical Center, Durham, NC, USA

    Anupama Reddy

  4. Department of Internal Medicine, Duke University Medical Center, Durham, NC, USA

    Katherine Prince & Patricia Lugar

  5. Department of Immunology, Duke University Medical Center, Durham, NC, USA

    Rebecca Buckley

  6. 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

Authors
  1. Carolyn Baloh
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  2. Anupama Reddy
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  3. Michele Henson
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  4. Katherine Prince
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  5. Rebecca Buckley
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  6. Patricia Lugar
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Corresponding author

Correspondence to Patricia Lugar.

Ethics declarations

This study was approved by the Duke University Institutional Review Board, and as this was a retrospective chart review involving existing data and posing no more than minimal risks to subjects, the need for individual consent was waived.

Conflict of Interest

The authors declare that they have no conflict of interest.

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Baloh, C., Reddy, A., Henson, M. et al. 30-Year Review of Pediatric- and Adult-Onset CVID: Clinical Correlates and Prognostic Indicators. J Clin Immunol 39, 678–687 (2019). https://doi.org/10.1007/s10875-019-00674-9

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  • DOI: https://doi.org/10.1007/s10875-019-00674-9

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