Primary Immunodeficiencies in Costa Rican Adults

CASE SERIES OF PRIMARY IMMUNODEFICIENCIES IN ADULTS DIAGNOSED IN TWO HOSPITALS OF THE TERTIARY LEVEL OF CARE OF THE COSTA RICAN SOCIAL SECURITY Fund, 2017-2018

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.

Study Overview

• Status: Completed
• Conditions: Primary Immune Deficiency in Adults

• Study Type: Observational
• Enrollment (Actual): 137

Contacts and Locations

Study Locations

• Costa Rica
  • San José, Costa Rica, 00000
    • Hospital Calderon Guardia

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 12 years to 100 years (ADULT, OLDER_ADULT, CHILD)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

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.

Description

Inclusion Criteria:

• All patients from adult primary immunodeficiency clinics will be included.

Exclusion Criteria:

• Patients with HIV
• Proven secondary immunodeficiencies.

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
characterization of immunodeficient patients	Characterize immunodeficient patients using sociodemographic and health variables.	1 year
Concordances between clinical manifestations and literature	•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

Collaborators and Investigators

• Sponsor: Caja Costarricense de Seguro Social

Study record dates

Study Major Dates

• Study Start (ACTUAL): November 11, 2018
• Primary Completion (ACTUAL): November 11, 2019
• Study Completion (ACTUAL): November 11, 2019

Study Registration Dates

• First Submitted: July 6, 2021
• First Submitted That Met QC Criteria: July 14, 2021
• First Posted (ACTUAL): July 15, 2021

Study Record Updates

• Last Update Posted (ACTUAL): July 15, 2021
• Last Update Submitted That Met QC Criteria: July 14, 2021
• Last Verified: July 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Immune System Diseases; Genetic Diseases, Inborn; Immunologic Deficiency Syndromes; Primary Immunodeficiency Diseases
• Other Study ID Numbers: R017-SABI-00148.

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No