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| DOI | 10.32641/RCHPED.V91I2.1364 | ||||||
| Año | 2020 | ||||||
| Tipo | artículo de investigación |
Citas Totales
Autores Afiliación Chile
Instituciones Chile
% Participación
Internacional
Autores
Afiliación Extranjera
Instituciones
Extranjeras
The development of anti-factor VIII neutralizing antibodies in hemophilia A is the most severe complication related to treatment. Immune tolerance induction (ITI) is the only known treatment for eradicating inhibitors. A successful ITI allows using factor VIII (FVIII) again for the treatment or prophylaxis of hemorrhagic events. Objective: To report the experience of pediatric patients who underwent ITI in the country's public health care network. Patients and Method: Retrospective and descriptive analysis of 13 pediatric patients with severe Hemophilia A and high-titer inhibitors persistence who underwent ITI and complete follow-up. Plasma-derived FVIII concentrate was used at 70-180 IU/kg/day doses. The success of the treatment is defined by achieving a negative titer and a half-life recovery of the FVIII. The results were expressed in median (range). Results: In 13 patients, the inhibitor was identified at an average age of 17.6 months, after 35.2 days of exposure to the FVIII. 11 patients (84.6%) recovered the half-life of FVIII after 49.6 months of treatment. In the patients who responded to treatment, the inhibitor titer was negative at 6 months on average. Conclusions: ITI is the treatment of choice for patients with hemophilia A and inhibitors persistence. ITI must be personalized since the time response is variable in each patient.
| Ord. | Autor | Género | Institución - País |
|---|---|---|---|
| 1 | SOTO-ARELLANO, VERONICA | Mujer |
Hospital de Niños Dr Roberto del Rio - Chile
Hospital de Niños Roberto del Río - Chile Hospital de Niños Robeto del Rio - Chile |
| 2 | Cortez S., Daniela | Mujer |
Hospital de Niños Dr Roberto del Rio - Chile
Hospital de Niños Roberto del Río - Chile Hospital de Niños Robeto del Rio - Chile |
| 3 | GONZALEZ-VERGARA, MARISEL ANGELICA | Mujer |
Universidad de Chile - Chile
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