Journal Information
Vol. 24. Issue 5.
Pages 221-224 (September - October 1988)
Share
Share
Download PDF
More article options
Vol. 24. Issue 5.
Pages 221-224 (September - October 1988)
Full text access
Hipoventilacion alveolar cronica primaria
Visits
21517
J.A. Garrido, E. Llorca, N. González Mangado, J.M. Castrillo
Fundación Jiménez Díaz. Madrid
This item has received
Article information

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.

Full text is only aviable in PDF
Bibliografia
[1.]
G.G. Rhoads, J.S. Brody.
Idiopathic alveolar hypoventilation: Clinical spectrum.
Ann Intern Med, 71 (1969), pp. 271-278
[2.]
R.M. Senior, A.P. Fishman.
Disturbances of alveolar ventilation.
Med Clin North Am, 51 (1967), pp. 403-425
[3.]
J.B. West.
Regulation of ventilation, Chap. 3. En Pulmonary gas exchange.
Academic Press, (1980), pp. 107-112
[4.]
W.A. Whitelaw.
Disorders of the respiratory pump.
En Pulmonary Medicine, pp. 193-234
[5.]
R.B. Mellins, H.H. Balfour, G.M. Turino, R.W. Winters.
Failure of automatic control of ventilation (Ondine's curse).
Medicine (Balt.), 19 (1970), pp. 487-504
[6.]
O. Ratto, W.A. Briscoe, J.W. Morton.
Anoxemia secondary to polycytemia polycytemia secondary to anoxemia.
Am J Med, 1 (1955), pp. 958-965
[7.]
K.E. Schaefer.
Respiratory patterns and respiratory response to CO2.
J Appl Physiol, 13 (1958), pp. 1-14
[8.]
R.V. Lourenco, J.M. Miranda.
Drive and perfomance of the ventilatory apparatus in chronic obstructive lung disease.
N Eng J Med, 279 (1968), pp. 53-59
[9.]
R.H. Hyland, N.L. Jones, A.C.P. Powles, et al.
Primary alveolar hypoventilation treated with nocturnal electrophrenic respiration.
Am Rev Respir Dis, 117 (1978), pp. 165-172
[10.]
A.J. Berger, R.A. Mitcell, Y.W. Severinghaus.
Regulation of respiration.
N Eng J Med, 297 (1977), pp. 194-201
[11.]
E. Monsó, R. Vidal, L. Galdós, et al.
Apneas prolongadas como forma de presentación de un síndrome de hipoventilación alveolar central. A propósito de un caso.
Med Clin, 85 (1985), pp. 415-417
[12.]
J. Naughton, R. Block, M. Welch.
Central alveolar hypoventilation Case report.
Am Rev Respir Dis, 103 (1971), pp. 557-565
[13.]
T. Richter, J.R. West, A.P. Fishman.
The syndrome of alveolar hypoventilation and diminished sensitivity of the respiratory center.
N Eng J Med, 256 (1957), pp. 1.165-1.170
[14.]
M.A. Moskowitz, Y.N. Fisher, M.D. Simpser, D.J. Strieder.
Periodic apnea, exercise hypoventilation and hypothalamic dysfunction.
Ann Intern Med, 84 (1976), pp. 171-173
[15.]
P.B. Oliva, M.H. Williams, S.S. Park.
Alveolar hypoventilation syndrome.
Am Rev Respir Dis, 96 (1967), pp. 805-811
[16.]
F. Plum, A.G. Swanson.
Abnormalities in central regulation of respiration, in acute and convalescent poliomyelitis.
Arch Neurol Psychiat, 80 (1958), pp. 267-285
[17.]
S.J. Sarnoff, J.L. Whittemberger, J.E. Affeldt.
Hypoventilation syndrome in bulbar polimyelitis.
JAMA, 147 (1951), pp. 30-34
[18.]
W.W.L. Glenn, W.G. Holcomb, R.K. Shaw, et al.
Long term ventilatory support by diaphragm pacing in quadriplegia.
Ann Surg, 183 (1976), pp. 566-577
Copyright © 1988. Sociedad Española de Neumología y Cirugía Torácica
Archivos de Bronconeumología
Article options
Tools

Are you a health professional able to prescribe or dispense drugs?