Application of diagnostic and treatment criteria for common variable immunodeficiency disorder
| Autor: | Peter Storey, See-Tarn Woon, Anthony Jordan, Russell Barker, Rohan Ameratunga, Wikke Koopmans |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
Adult
Male 0301 basic medicine Pediatrics medicine.medical_specialty Transmembrane Activator and CAML Interactor Protein Immunology Asymptomatic Hypogammaglobulinemia 03 medical and health sciences Humans Immunology and Allergy Medicine Genetic Predisposition to Disease Bronchiectasis business.industry Common variable immunodeficiency Primary immune deficiency disorder Immunoglobulins Intravenous Middle Aged medicine.disease Pedigree Common Variable Immunodeficiency 030104 developmental biology Asymptomatic Diseases Female medicine.symptom business New Zealand |
| Zdroj: | Expert Review of Clinical Immunology. 12:257-266 |
| ISSN: | 1744-8409 1744-666X |
| DOI: | 10.1586/1744666x.2016.1126509 |
| Popis: | Common variable immunodeficiency disorder (CVID) is the most frequent symptomatic primary immune deficiency disorder in adults. It probably comprises a spectrum of polygenic disorders, with hypogammaglobulinemia being the overarching feature. While the majority of patients with CVID can be identified with relative ease, a significant proportion can present with minimal symptoms in spite of profound laboratory abnormalities. Here we discuss three patients who were presented to the Auckland Hospital immunoglobulin treatment committee to determine if they qualified for immunoglobulin replacement. Two were asymptomatic with profound laboratory abnormalities while the third patient was severely ill with extensive bronchiectasis. The third patient had less severe laboratory abnormalities compared with the two asymptomatic patients. We have applied four sets of published diagnostic and treatment criteria to these patients to compare their clinical utility. We have chosen these patients from the broad phenotypic spectrum of CVID, as this often illustrates differences in diagnostic and treatment criteria. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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