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04.01.2022 | Original Article

CD40 Ligand Deficiency in Latin America: Clinical, Immunological, and Genetic Characteristics

verfasst von: Tábata Takahashi França, Lucila Akune Barreiros, Ranieri Coelho Salgado, Sarah Maria da Silva Napoleão, Lillian Nunes Gomes, Janáira Fernandes Severo Ferreira, Carolina Prando, Cristina Worm Weber, Regina Sumiko Watanabe Di Gesu, Cecilia Montenegro, Carolina Sanchez Aranda, Gisele Kuntze, Aidé Tamara Staines-Boone, Edna Venegas-Montoya, Juan Carlos Aldave Becerra, Liliana Bezrodnik, Daniela Di Giovanni, Ileana Moreira, Gisela Analia Seminario, Andrea Cecilia Gómez Raccio, Mayra de Barros Dorna, Nelson Augusto Rosário-Filho, Herberto Jose Chong-Neto, Elisa de Carvalho, Milena Baptistella Grotta, Julio Cesar Orellana, Miguel Garcia Dominguez, Oscar Porras, Laura Sasia, Karina Salvucci, Emilio Garip, Luiz Fernando Bacarini Leite, Wilma Carvalho Neves Forte, Fernanda Pinto-Mariz, Ekaterini Goudouris, María Enriqueta Nuñez Nuñez, Magdalena Schelotto, Laura Berrón Ruiz, Diana Inés Liberatore, Hans D. Ochs, Otavio Cabral-Marques, Antonio Condino-Neto

Erschienen in: Journal of Clinical Immunology | Ausgabe 3/2022

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Abstract

CD40 ligand (CD40L) deficiency is a rare inborn error of immunity presenting with heterogeneous clinical manifestations. While a detailed characterization of patients affected by CD40L deficiency is essential to an accurate diagnosis and management, information about this disorder in Latin American patients is limited. We retrospectively analyzed data from 50 patients collected by the Latin American Society for Immunodeficiencies registry or provided by affiliated physicians to characterize the clinical, laboratory, and molecular features of Latin American patients with CD40L deficiency. The median age at disease onset and diagnosis was 7 months and 17 months, respectively, with a median diagnosis delay of 1 year. Forty-seven patients were genetically characterized revealing 6 novel mutations in the CD40LG gene. Pneumonia was the most common first symptom reported (66%). Initial immunoglobulin levels were variable among patients. Pneumonia (86%), upper respiratory tract infections (70%), neutropenia (70%), and gastrointestinal manifestations (60%) were the most prevalent clinical symptoms throughout life. Thirty-five infectious agents were reported, five of which were not previously described in CD40L deficient patients, representing the largest number of pathogens reported to date in a cohort of CD40L deficient patients. The characterization of the largest cohort of Latin American patients with CD40L deficiency adds novel insights to the recognition of this disorder, helping to fulfill unmet needs and gaps in the diagnosis and management of patients with CD40L deficiency.

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Titel: CD40 Ligand Deficiency in Latin America: Clinical, Immunological, and Genetic Characteristics
verfasst von: Tábata Takahashi França
Lucila Akune Barreiros
Ranieri Coelho Salgado
Sarah Maria da Silva Napoleão
Lillian Nunes Gomes
Janáira Fernandes Severo Ferreira
Carolina Prando
Cristina Worm Weber
Regina Sumiko Watanabe Di Gesu
Cecilia Montenegro
Carolina Sanchez Aranda
Gisele Kuntze
Aidé Tamara Staines-Boone
Edna Venegas-Montoya
Juan Carlos Aldave Becerra
Liliana Bezrodnik
Daniela Di Giovanni
Ileana Moreira
Gisela Analia Seminario
Andrea Cecilia Gómez Raccio
Mayra de Barros Dorna
Nelson Augusto Rosário-Filho
Herberto Jose Chong-Neto
Elisa de Carvalho
Milena Baptistella Grotta
Julio Cesar Orellana
Miguel Garcia Dominguez
Oscar Porras
Laura Sasia
Karina Salvucci
Emilio Garip
Luiz Fernando Bacarini Leite
Wilma Carvalho Neves Forte
Fernanda Pinto-Mariz
Ekaterini Goudouris
María Enriqueta Nuñez Nuñez
Magdalena Schelotto
Laura Berrón Ruiz
Diana Inés Liberatore
Hans D. Ochs
Otavio Cabral-Marques
Antonio Condino-Neto
Publikationsdatum: 04.01.2022
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 3/2022
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-021-01182-5

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