Journal of Clinical Immunology, Vol. 27, No. 1, January 2007 ( C  2006) DOI: 10.1007/s10875-006-9052-0 Primary Immunodeficiency Diseases in Latin America: The Second Report of the LAGID 1 Registry LILY E. LEIVA, 2,18 MARTA ZELAZCO, 3 MAT ´ IAS OLEASTRO, 3 MAGDA CARNEIRO-SAMPAIO, 4 ANTONIO CONDINO-NETO, 4 BEATRIZ TAVARES COSTA-CARVALHO, 5 ANETE SEVCIOVIC GRUMACH, 6 ARNOLDO QUEZADA, 7 PABLO PATI ˜ NO, 8 JOS ´ E LUIS FRANCO, 8 OSCAR PORRAS, 9 FRANCISCO JAVIER RODR ´ IGUEZ, 10 FRANCISCO JAVIER ESPINOSA-ROSALES, 11 SARA ELVA ESPINOSA-PADILLA, 11 DIVA ALMILLATEGUI, 12 CELIA MART ´ INEZ, 13 JUAN RODR ´ IGUEZ TAFUR, 14 MARILYN VALENT ´ IN, 15 LORENA BENARROCH, 16 ROSY BARROSO, 17 and RICARDO U. SORENSEN 2 Received July 24, 2006; accepted October 17, 2006 Published online: 27 December 2006 This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with pri- mary immunodeficiency diseases in 12 Latin American coun- tries: 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 1 Latin American Group for Primary Immunodeficiency Diseases. 2 Department of Pediatrics, LSU Health Sciences Center, New Orleans, Louisiana. 3 Hospital Nacional de Pediatr´ ıa “Juan P. Garrahan”, Buenos Aires, Argentina. 4 Instituto de Ciˆ encias Biom´ edicas da Universidade de S˜ ao Paulo, S˜ ao Paulo, Brazil. 5 Departamento de Pediatria da UNIFESP-EPM, S˜ ao Paulo, Brazil. 6 Department of Dermatology, University of Sao Paulo, Primary im- munodeficiency Outpatient Group, Sao Paulo, Brazil. 7 Departamento de Pediatria, Facultad de Medicina, Universidad de Chile, Santiago, Chile. 8 Facultad de Medicina, Universidad de Antioquia, Medell´ ın, Colombia. 9 Hospital Nacional de Ni˜ nos “Dr. Carlos Saenz Herrera”, San Jose, Costa Rica. 10 Departamento de Pediatr´ ıa, Instituto Nacional de Seguridad Social, Tegucigalpa, Honduras. 11 Instituto Nacional de Pediatr´ ıa, Mexico City, Mexico. 12 Servicio de Inmunolog´ ıa y Alergia, Hospital de Ni˜ nos de Panam´ a, El Dorado, Panam´ a. 13 Instituto de Investigaci´ on de Ciencias de la Salud, Asunci ´ on, Paraguay. 14 Facultad de Medicina, Universidad Nacional Mayor de San Marcos, Lima, Peru. 15 Hospital Pedi´ atrico “Pereira Rossell”, Montevideo, Uruguay. 16 Hospital de Cl´ ınicas, Caracas, Venezuela. 17 Cl´ ınica “Leopoldo Aguerrevere”, Caracas, Venezuela. 18 To whom correspondence should be addressed to The Research Insti- tute for Children, Children’s Hospital, 200 Henry Clay Avenue, New Orleans, Louisiana, 70118; e-mail: lleiva@lsuhsc.edu. 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 pri- mary immunodeficiency cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic granulomatous disease, and se- vere combined immunodeficiency between the first report and the present one shows an increase in the reporting of these dis- eases in all countries. In this report, the estimated minimal inci- dence 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 immunode- ficiency 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. KEY WORDS: Primary immunodeficiency; Latin America; LAGID; Immunodeficiency epidemiology. INTRODUCTION Although primary immunodeficiencies (PID) are rare in- herited diseases, intense research studies in recent years have contributed to the understanding of the immunologi- cal defects and the genes involved in these disorders. Since the first case was reported in 1952, more than 100 types of PID are now known and their genetic defects have been identified (1). During the last decade, significant advances 101 0271-9142/07/0100-0101/0 C  2006 Springer Science+Business Media, LLC