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Vol. 24. Issue 5.
Pages 221-224 (September - October 1988)
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Vol. 24. Issue 5.
Pages 221-224 (September - October 1988)
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Hipoventilacion alveolar cronica primaria
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J.A. Garrido, E. Llorca, N. González Mangado, J.M. Castrillo
Fundación Jiménez Díaz. Madrid
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Presentamos el caso de una mujer de 36 años con un síndrome de hipoventilación alveolar primaria. Comenzó sus síntomas en la infancia con cianosis durante el sueño, siendo diagnosticada de cardiopatía congénita cianógena, añadiéndose después cianosis diurna, astenia, púrpura y edema en miembros inferiores. No tenía antecedentes de enfermedad neurológica. Destacaba poliglobulia con estudio de hemoglobinas normal. Un estudio hemodinámico descartó cortocircuitos y lesiones valvulares, existiendo hipertensión pulmonar moderada. Sus pruebas funcionales respiratorias no mostraron alteraciones y presentaba acidosis respiratoria con hipoxemia, que se corregían con la hiperventiladón y con el ejercicio. El estudio del sueño no apoyaba el diagnóstico de «Sleep-apnea». Demostramos la ausencia de respuesta ventilatoria adecuada frente a la hipoxia. La presencia de hipercapnia en ausenda de enfermedad pulmonar, neuromuscular o de la caja torácica, reflejaba disminución de la sensibilidad central al CO2. Los hallazgos permiten sugerir una afectación mixta de quimiorreceptores centrales y periféricos con indemnidad de los mecanismos reflejos al esfuerzo.

We present the case of a 36-year-old woman with a primary alveolar hypoventilation syndrome. She began presenting symptoms at infancy, with cyanosis during the sleep, being diagnosed of cyanotic congenital malformation of the heart; she subsequently developed diurnal cyanosis, fatigue, purpura, and lower limbs edema. She gave no history of neurological disease. As laboratory data, there was polyglobulia and the hemoglobins study was normal. A hemodynamic exploration ruled out circulatory shunts and valvular lesions, confirming a moderate pulmonary hypertension. The tests of respiratory function did not reveal any alteration; the patient presented respiratory acidosis with hypoxemia, which normalized with hyperventilation and physical exercise. The sleep study did not support the diagnosis of sleep apnea. We confirmed the lack of an adequate ventilatory response to hypoxia. The presence of hypercapnia in the absence of pulmonary, neuromuscular and thoracic cage diseases reflected the decrease in the central sensitivity to CO2. The findings may suggest an alteration in the central and peripheral chemorreceptors with sparing of the reflex machanisms to physical effort.

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Copyright © 1988. Sociedad Española de Neumología y Cirugía Torácica
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