Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry.
This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras, Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reveals that from a total of 3321 patients registered, the most common form of primary immunodeficiency disease was predominantly antibody deficiency (53.2%) with IgA deficiency reported as the most frequent phenotype. This category was followed by 22.6% other well-defined ID syndromes, 9.5% combined T- and B-cell inmunodeficiency, 8.6% phagocytic disorders, 3.3% diseases of immune dysregulation, and 2.8% complement deficiencies. All countries that participated in the first publication in 1998 reported an increase in registered primary immunodeficiency cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic granulomatous disease, and severe combined immunodeficiency between the first report and the present one shows an increase in the reporting of these diseases in all countries. In this report, the estimated minimal incidence of chronic granulomatous disease was between 0.72 and 1.26 cases per 100,000 births in Argentina, Chile, Costa Rica, and Uruguay and the incidence of severe combined immunodeficiency was 1.28 and 3.79 per 100,000 births in Chile and Costa Rica, respectively. However, these diseases are underreported in other participating countries. In addition to a better diagnosis of primary immunodeficiency diseases, more work on improving the registration of patients by each participating country and by countries that have not yet joined LAGID is still needed.
Leiva
Lily E
LE
Department of Pediatrics, LSU Health Sciences Center, New Orleans, Louisiana, USA. lleiva@lsuhsc.edu
Zelazco
Marta
M
Oleastro
Matías
M
Carneiro-Sampaio
Magda
M
Condino-Neto
Antonio
A
Costa-Carvalho
Beatriz Tavares
BT
Grumach
Anete Sevciovic
AS
Quezada
Arnoldo
A
Patiño
Pablo
P
Franco
José Luis
JL
Porras
Oscar
O
Rodríguez
Francisco Javier
FJ
Espinosa-Rosales
Francisco Javier
FJ
Espinosa-Padilla
Sara Elva
SE
Almillategui
Diva
D
Martínez
Celia
C
Tafur
Juan Rodríguez
JR
Valentín
Marilyn
M
Benarroch
Lorena
L
Barroso
Rosy
R
Sorensen
Ricardo U
RU
D001723:Birth Rate; D003625:Data Collection; D003710:Demography; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000145:classification / Q000453:epidemiology* / Q000276:immunology; D007843:Latin America / Q000453:epidemiology; D008297:Male; D010641:Phenotype; D015995:Prevalence; D012042:Registries; D011795:Surveys and Questionnaires