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Primary immunodeficiencies represent a underdiagnosed group of rare diseases which if diagnosed well and in time can be treated in an efficient manner and prevent complications that may affect the quality of patients life in an severe manner. Neither in Costa Rica nor in Central America studies or national registries regarding the prevalence and characterization of primary immunodeficiencies in adult patients exist up to now. This study reflects for the first time the epidemiologic situation of primary immunodeficiencies in a Central American country, characterizing adult patients diagnosed with primary and idiopathic immune disorders treated in two specialized immunodeficiency clinics in Costa Rica.
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| Measure | Description | Time Frame |
|---|---|---|
| Clinical manifestations of all adult patients diagnosed with primary and idiopathic immune defects | Describe the clinical manifestations of all adult patients diagnosed with primary and idiopathic immune defects attended in the primary immunodeficiency clinics of the Hospitals Mexico and Dr. Rafael Ángel Calderón Guardia during the study period. | 1 year |
| Measure | Description | Time Frame |
|---|---|---|
| characterization of immunodeficient patients | Characterize immunodeficient patients using sociodemographic and health variables. | 1 year |
| Concordances between clinical manifestations and literature |
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Inclusion Criteria:
Exclusion Criteria:
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All cases of primary immunodeficiency and idiopathic defects of immunity detected in the primary immunodeficiency clinics of the Hospitals Mexico and Dr. Rafael Ángel Calderón Guardia between November 2017 and May 2018.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hospital Calderon Guardia | San José | 00000 | Costa Rica |
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•Establish concordances between the clinical manifestations of the group of patients under study with those described in the literature according to the different pathologies.
| 1 year |
| organic complications | Record organic complications secondary to immunodeficiency status. | 1 year |
| type and degree of immunodeficiency | Differentiate the type and degree of immunodeficiency of each patient. | 1 year |
| pathonges that cause infectious | Report isolated pathogens that cause infectious manifestations in patients. | 1 year |
| bacteria present in upper airways | •Report the colonizing bacteria present in the cultures of the upper airways of the patients requested in the initial approach. | 1 year |