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Vol. 30. Issue 10.
Pages 479-484 (December 1994)
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Vol. 30. Issue 10.
Pages 479-484 (December 1994)
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Evaluación del tratamiento sustitutivo del enfisema por déficit de alfa-1-antitripsina
Assessment of α1-antitrypsin replacement therapy for emphysema
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M. Miravitlles1, R. Vidal, M. Torrella, J.M. Bofill, J. de Gracia
Servicio de Neumología. Hospital General Universitario Vall d’Hebron. Barcelona
M. Cotrina*
* Servicio de Bioquímica. Hospital General Universitario Vall d’Hebron. Barcelona
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Evaluación del tratamiento sustitutivo en pacientes con enfisema por déficit de alfa-1 antitripsina (AAT). Se valoran las características de los pacientes, los posibles efectos secundarios del tratamiento y su eficacia para mantener valores de AAT adecuados en plasma. También se estudia la evolución funcional de los pacientes.

El protocolo se inicia con 4 dosis semanales de 60 mg/kg de AAT (Prolastin®) intravenosas, seguidas de dosis mensuales de 240 mg/kg. Previamente a cada administración se determinan valores de AAT en plasma. Cada 6 meses se realiza un estudio funcional respiratorio con espirometría, pletismografía y prueba de transferencia del CO.

Desde 1988 han iniciado el tratamiento 13 pacientes con una edad media de 46 años, el FEV1, promedio al inicio era de 0,79 1. Se han infundido más de 250 dosis sin advertir ninguna reacción adversa de importancia. En 3 pacientes los valores de AAT antes de la siguiente infusión eran inferiores a los considerados protectores de 50 mg/dl. La evolución funcional muestra en algunos casos una estabilización de los valores espirométricos.

Existe un retraso importante en el diagnóstico del déficit de AAT que provoca que los pacientes alcancen una alteración funcional importante antes de iniciar tratamiento sustitutivo. No se observan efectos secundarios con el tratamiento. Hasta conseguir establecer el intervalo adecuado entre dosis, es necesario monitorizar los valores de AAT en cada paciente.

Palabra clave:
Enfisema
Alfa-1-antitripsina

Assessment of α1-antitrypsin replacement therapy (AAT) for emphysema. Patient characteristics were analyzed along with the possible side effects of the treatment and its efficacy in maintaining appropriate AAT blood levels. Lung function changes were also studied.

The treatment protocol began with 4 weekly intravenous doses of 60 mg/kg AAT (Prolastin®) and continued with monthly doses of 240 mg/kg. AAT serum levels were measured before each dose. Every 6 months pulmonary function tests (spirometry, pletismography and CO transfer) were performed.

Thirteen patients (mean age 46 yr) have been studied since 1988. Mean initial FEV1, was 0.79 1. Over 250 doses have been infused with no significant side effects reported. AAT levels before treatment in 3 patients were lower than that considered protective (50 mg/dl). Function tests results indicated stabilization of spirometric values in most cases.

Diagnosis of AAT deficiency is delayed considerably, meaning that significant functional deterioration takes place before replacement therapy begins. No side effects of treatment have been observed. Until an appropriate interval between doses has been established, each patient's AAT levels must be monitored.

Key words:
Enphysema
α1-antitrypsin
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Copyright © 1994. Sociedad Española de Neumología y Cirugía Torácica
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