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Immunol Res ; 69(5): 436-444, 2021 10.
Artículo en Inglés | MEDLINE | ID: mdl-34427868

RESUMEN

The aim of the study was to determine the TREC/KREC levels in the patients diagnosed with ataxia-telangiectasia (AT) and to establish their informative value for early diagnosis of this pathology. TRECs and KREC assay was performed using real-time polymerase chain reaction on the DNA of 25 patients diagnosed with AT aged 3 to 14 years and of 173 healthy individuals of the control group aged 1 to 12 years. Clinical and laboratory characteristics of patients were ascertained using their medical records. In the patients with AT, the mean level of TRECs was 542.84 per 106 cells, ranging from 4 to 4720, while mean level of KRECs was 1317.64 per 106 cells, ranging from 146 to 9300. In 84% of the patients, TREC levels were less than 1000, which was significantly lower than in the control group, while KREC levels were reduced in 48% of the patients. A correlation was found between the levels of TREC and the absolute values of CD4 (r = 0.5455). Measurement of TREC/KREC levels opens new opportunities for early AT detection in children as a part of the newborn screening. Reduced time to diagnosis will allow to carry out timely in-depth immunological and genetic testing, prevent the development of severe infections, and improve quality of life.


Asunto(s)
Ataxia Telangiectasia/etiología , Biomarcadores , ADN Circular/genética , Cadenas kappa de Inmunoglobulina/genética , Receptores de Antígenos de Linfocitos T/genética , Adolescente , Alelos , Ataxia Telangiectasia/diagnóstico , Proteínas de la Ataxia Telangiectasia Mutada/genética , Linfocitos B/inmunología , Linfocitos B/metabolismo , Recuento de Linfocito CD4 , Niño , Preescolar , Diagnóstico Diferencial , Susceptibilidad a Enfermedades , Femenino , Dosificación de Gen , Genotipo , Humanos , Lactante , Masculino , Mutación , Fenotipo , Pronóstico , Reacción en Cadena en Tiempo Real de la Polimerasa , Linfocitos T/inmunología , Linfocitos T/metabolismo
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