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Clinical manifestations and expression of CD18 to guide the diagnosis of leukocyte adhesion deficiency type 1
Fuentes-Lara, Elma Isela; Arce-Estrada, Gabriel Emmanuel; Bojalil-Cabildo, Abner; Yamazaki-Nakashimada, Marco Antonio; Espinosa-Padilla, Sara Elva; Gamez-Gonzalez, Luisa Berenise; Nideshda Ramirez-Uribe, Rosa María; Saucedo-Ramirez, Omar Josue; Berron-Ruiz, Laura.
Affiliation
  • Fuentes-Lara, Elma Isela; National Institute of Pediatrics. Immunodeficiency Laboratory. Mexico City. Mexico
  • Arce-Estrada, Gabriel Emmanuel; National Institute of Pediatrics. Immunodeficiency Laboratory. Mexico City. Mexico
  • Bojalil-Cabildo, Abner; National Institute of Pediatrics. Clinical Immunology Service. Mexico City. Mexico
  • Yamazaki-Nakashimada, Marco Antonio; National Institute of Pediatrics. Clinical Immunology Service. Mexico City. Mexico
  • Espinosa-Padilla, Sara Elva; National Institute of Pediatrics. Immunodeficiency Laboratory. Mexico City. Mexico
  • Gamez-Gonzalez, Luisa Berenise; Chihuahua Children's Specialty Hospital. Chihuahua. Mexico
  • Nideshda Ramirez-Uribe, Rosa María; The National Pediatrics Institute. Bone Marrow Transplant Unit. México City. Mexico
  • Saucedo-Ramirez, Omar Josue; Children’s Hospital of Mexico “Federico Gómez,. Clinical Immunology Service. Mexico City. Mexico
  • Berron-Ruiz, Laura; National Institute of Pediatrics. Immunodeficiency Laboratory. Mexico City. Mexico
Allergol. immunopatol ; 51(6): 89-96, 2023. ilus, graf, tab
Article in English | IBECS | ID: ibc-227312
Responsible library: ES1.1
Localization: ES15.1 - BNCS
ABSTRACT

Background:

Leukocyte adhesion deficiency type 1 (LAD-1) is an inborn error of immunity characterized by a defect in leukocyte trafficking.

Methods:

Patients with clinical suspicion of LAD-1 were referred to our institution. Complete blood count and flow cytometric analysis, to identify the expression of CD18, CD11b, and the lymphocyte population phenotyping, were performed, and statistical analysis was completed.

Results:

We report clinical manifestations and immunological findings of six Mexican patients diagnosed with LAD-1. The diagnosis was based on typical clinical presentation, combined with laboratory demonstration of leukocytosis, and significant reduction or near absence of CD18 and its associated molecules CD11a, CD11b, and CD11c on leukocytes. We found atypical manifestations, not described in other countries, such as early-onset autoimmunity or infections caused by certain microorganisms.

Conclusions:

Patients with LAD-1 may present with atypical manifestations, making flow cytometry an indispensable tool to confirm the diagnosis. We present the first report of LAD-1 patients in a Latin American country (AU)
Subject(s)


Full text: Available Collection: National databases / Spain Database: IBECS Main subject: Leukocyte-Adhesion Deficiency Syndrome / CD18 Antigens / Leukocytes Limits: Female / Humans / Infant / Male Country/Region as subject: Mexico Language: English Journal: Allergol. immunopatol Year: 2023 Document type: Article Institution/Affiliation country: Chihuahua Children's Specialty Hospital/Mexico / Children’s Hospital of Mexico “Federico Gómez,/Mexico / National Institute of Pediatrics/Mexico / The National Pediatrics Institute/Mexico

Full text: Available Collection: National databases / Spain Database: IBECS Main subject: Leukocyte-Adhesion Deficiency Syndrome / CD18 Antigens / Leukocytes Limits: Female / Humans / Infant / Male Country/Region as subject: Mexico Language: English Journal: Allergol. immunopatol Year: 2023 Document type: Article Institution/Affiliation country: Chihuahua Children's Specialty Hospital/Mexico / Children’s Hospital of Mexico “Federico Gómez,/Mexico / National Institute of Pediatrics/Mexico / The National Pediatrics Institute/Mexico
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