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Vol. 27. Issue 1.
Pages 43-45 (January - February 1991)
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Vol. 27. Issue 1.
Pages 43-45 (January - February 1991)
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Síndrome de young. ¿Una forma larvada de fibrosis quística?
Young syndrome. An undeveloped form of cystic fibrosis?
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C. Ramos, S. Bello, M. Vila, J.M. Artigas, A. García Aranda, A. Hernández
Servicio de Neumología. Departamento de Medicina Interna. Hospital Miguel Servet. Zaragoza
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Se ha observado en múltiples ocasiones la asociación de patología pulmonar crónica e infertilidad en el varón (síndrome de Young, fibrosis quística, síndrome de Kartagener y discinesia ciliar primaria).

El síndrome de Young es una entidad de etiología desconocida caracterizada por la asociación de infertilidad por azoospermia obstructiva, afectación sinopulmonar crónica y hallazgo de frecuentes masas quísticas palpables en el epipídimo de uno o ambos testículos. Clásicamente se ha diferenciado de la fibrosis quística por la edad más avanzada, el test de sudor negativo y la ausencia de insuficiencia pancreática. Se ha demostrado que existen formas menores de fibrosis quística en adultos que llegan a pasar desapercibidas, así como la ausencia de positividad del test de sudor o insuficiencia pancreática en algunos enfermos afectados.

Describimos el caso de un varón de 50 años que sindrómicamente podía ser catalogado de síndrome de Young, pero que presentaba un test de sudor dudosamente positivo y discreta esteatorrea. Reflexionamos sobre los puntos comunes y diferenciadores para fibrosis quística y síndrome de Young y apuntamos la posibilidad de que el mismo pueda ser una forma larvada de fibrosis quística en el adulto.

The association of chronic pulmonary disease and infertility in man has been observed in several instances (Young syndrome, cystic fibrosis, Kartagener syndrome, and primary ciliary dyscinesia).

Young syndrome is a clinical picture of unknown origin characterized by infertility due to obstructive azoospermia, chronic pulmonary involvement, and palpable cystic masses at the epididymus of one or two testes. Classically cystic fibrosis was identified by the presence of advanced age, negative sweat test, and by the absence of pancreatic insufficiency. It has been demonstrated that minor clinical forms of cystic fibrosis do exist in adult patients and can be unnoticed. It has also been reported that in some affected patients the sweat test might be negative and pancreatic insufficiency absent.

We report the case of a 50 years old man who had the clinical picture of Young syndrome but who presented a slight positive sweat test and mild steatorrhea. We discuss the common and distinctive clinical findings of cystic fibrosis and Young syndrome. We suggest that Young syndrome is a undeveloped form of cystic fibrosis in adult patients.

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Copyright © 1991. Sociedad Española de Neumología y Cirugía Torácica
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