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We describe a 34 year old male patient with systemic lupus erythematosus (SLE) who developed pulmonary hypertension (PHT) associated with anticardiolipin antibodies. Ten years after diagnosis, an helical CT scan revealed thrombosis of the major pulmonary arteries, which was confirmed by digital pulmonary angiography. An inferior caval vein filter was placed and oral anticoagulation was begun. The patient refused pulmonary thromboendarterectomy and died soon thereafter. Even though PHT is widely described associated with SLE, proximal thrombosis of pulmonary arteries is exceptional. We describe such a patient review the different etiologies, incidence and diagnostic approach of PHT in SLE, focusing on those patients with an effective treatment.

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We describe a 34 year old male patient with systemic lupus erythematosus (SLE) who developed pulmonary hypertension (PHT) associated with anticardiolipin antibodies. Ten years after diagnosis, an helical CT scan revealed thrombosis of the major pulmonary arteries, which was confirmed by digital pulmonary angiography. An inferior caval vein filter was placed and oral anticoagulation was begun. The patient refused pulmonary thromboendarterectomy and died soon thereafter. Even though PHT is widely described associated with SLE, proximal thrombosis of pulmonary arteries is exceptional. We describe such a patient review the different etiologies, incidence and diagnostic approach of PHT in SLE, focusing on those patients with an effective treatment.

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We studied the strength developed by the diaphragm during progressive ascitis induction (40 up to 280 ml/kg wt) and the changes in the radium of curvature in relation with its strength in 6 anesthetized dogs. Force generation of the diaphragm was assessed with the Pdi obtained with bilateral phrenic nerve stimulation at 60 Hz. In relation to increases in the abdominal liquid, the thoraco-pulmonary compliance decreases (p < 0.05) from 10.1 to 6.8 ml/kg/cm H2O. The radius of curvature increases 158% from the basal values; the calculated basal tension was 347 +/- 43, final 448 +/- 32 cm H2O/cm. The diaphragmatic length in percent of the basal value was 138%. The X-rays findings showed cephalic displacement, diaphragmatic flattening and stretching. The Pdi obtained should be the result of a complex interaction between the diaphragmatic geometry, the length, the degree of stretching during the contraction and preload. We can describe the changes in Pdi in 2 steps: initial increase and final decrease, associated to high Pga at the beginning and a low one later. First the Pdi increases by the Pga without significant changes in the Pes. The fact is that an increase in Pga can be related with cephalic displacement of the diaphragm with stretching and shortening of the radius of curvature and decreases in the abdominal compliance. The final decreases in the Pdi could be related with overstretching and decreases of the radius of curvature.

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We studied the strength developed by the diaphragm during progressive ascitis induction (40 up to 280 ml/kg wt) and the changes in the radium of curvature in relation with its strength in 6 anesthetized dogs. Force generation of the diaphragm was assessed with the Pdi obtained with bilateral phrenic nerve stimulation at 60 Hz. In relation to increases in the abdominal liquid, the thoraco-pulmonary compliance decreases (p < 0.05) from 10.1 to 6.8 ml/kg/cm H2O. The radius of curvature increases 158

from the basal values; the calculated basal tension was 347 +/- 43, final 448 +/- 32 cm H2O/cm. The diaphragmatic length in percent of the basal value was 138

. The X-rays findings showed cephalic displacement, diaphragmatic flattening and stretching. The Pdi obtained should be the result of a complex interaction between the diaphragmatic geometry, the length, the degree of stretching during the contraction and preload. We can describe the changes in Pdi in 2 steps: initial increase and final decrease, associated to high Pga at the beginning and a low one later. First the Pdi increases by the Pga without significant changes in the Pes. The fact is that an increase in Pga can be related with cephalic displacement of the diaphragm with stretching and shortening of the radius of curvature and decreases in the abdominal compliance. The final decreases in the Pdi could be related with overstretching and decreases of the radius of curvature.

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El estridor larígeo de origen emocional puede fácilmente confundirse con el broncoespasmo. En esta paciente la presencia de disnea episódica y sibilancias llevó al diagnóstico inicial de asma bronquial. El examen físico, la curva volumen-tiempo normal, los test de broncoprovocación negativos y la falta de respuesta al tratamiento, permitieron excluir ese diagnóstico. La observación directa de la laringe mediante la fibrobroncoscopía asi como las curvas flujo-volumen permitieron descartar la presencia de estenosis orgánica de la vía aérea superior. Durante el examen endoscópico se observó la aducción de las cuerdas vocales no sólo en espiración sino también durante la inspiración. La estrechez funcional de la glotis y el volumen corriente desplazado hacia el volumen residual en la curva flujo-volumen ponen en evidencia una espiración activa y forzada aún durante el reposo, hallazgos del síndrome de sibilancia laríngeas emocionales. Dos mecanismos (la respiración forzada a bajo volumen pulmonar y la aducción inspiratoria de las cuerdas vocales) parecen estar presente en este caso. El asma no es la única causa de sibilancias y cuando se han descartado otras etiologías las causa funcionales deben ser consideradas

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RESUMEN

El estridor larígeo de origen emocional puede fácilmente confundirse con el broncoespasmo. En esta paciente la presencia de disnea episódica y sibilancias llevó al diagnóstico inicial de asma bronquial. El examen físico, la curva volumen-tiempo normal, los test de broncoprovocación negativos y la falta de respuesta al tratamiento, permitieron excluir ese diagnóstico. La observación directa de la laringe mediante la fibrobroncoscopía asi como las curvas flujo-volumen permitieron descartar la presencia de estenosis orgánica de la vía aérea superior. Durante el examen endoscópico se observó la aducción de las cuerdas vocales no sólo en espiración sino también durante la inspiración. La estrechez funcional de la glotis y el volumen corriente desplazado hacia el volumen residual en la curva flujo-volumen ponen en evidencia una espiración activa y forzada aún durante el reposo, hallazgos del síndrome de sibilancia laríngeas emocionales. Dos mecanismos (la respiración forzada a bajo volumen pulmonar y la aducción inspiratoria de las cuerdas vocales) parecen estar presente en este caso. El asma no es la única causa de sibilancias y cuando se han descartado otras etiologías las causa funcionales deben ser consideradas (AU)

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Laryngeal wheezing caused by emotional stress is usually confused with that caused by bronchospasm and diagnosed as asthma, a well known emotionally influenced entity. Therefore, it is treated with bronchodilators, including corticosteroids, frequently resulting in a iatrogenic Cushing's syndrome. This case report concerns a patient initially considered to have bronchial asthma. Physiological and endoscopic studies allowed us to exclude this disease, as well as any organic obstruction of the upper and lower airway. Flow-volume curve showed that the tidal volume (VT) loop was displaced towards RV during the crisis and the expiratory flow of the VT reached the envelope of the maximal expiratory flow (Fig. 1). Direct larynx observation during fiberoptic bronchoscopy showed not only expiratory but also inspiratory vocal cords adduction. A diagnosis of emotional laryngeal wheezing was made. Excluding asthma, bronchodilators were progressively discontinued. She started to receive alprazolam and psychotherapy and during one year of follow-up she remained symptomless. Two mechanisms may be present in our patient: partial inspiratory adduction of vocal cords and breathing at low lung volume. Despite reported dissimilarities between these two mechanisms both seem to have a similar emotional origin.

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Laryngeal wheezing caused by emotional stress is usually confused with that caused by bronchospasm and diagnosed as asthma, a well known emotionally influenced entity. Therefore, it is treated with bronchodilators, including corticosteroids, frequently resulting in a iatrogenic Cushings syndrome. This case report concerns a patient initially considered to have bronchial asthma. Physiological and endoscopic studies allowed us to exclude this disease, as well as any organic obstruction of the upper and lower airway. Flow-volume curve showed that the tidal volume (VT) loop was displaced towards RV during the crisis and the expiratory flow of the VT reached the envelope of the maximal expiratory flow (Fig. 1). Direct larynx observation during fiberoptic bronchoscopy showed not only expiratory but also inspiratory vocal cords adduction. A diagnosis of emotional laryngeal wheezing was made. Excluding asthma, bronchodilators were progressively discontinued. She started to receive alprazolam and psychotherapy and during one year of follow-up she remained symptomless. Two mechanisms may be present in our patient: partial inspiratory adduction of vocal cords and breathing at low lung volume. Despite reported dissimilarities between these two mechanisms both seem to have a similar emotional origin.

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A 62-year-old woman with a bilateral carotid body paraganglioma presented, 2 years after the removal of the right one, with signs of right-heart failure. Hypoxemia, hypercapnia, polycythemia and pulmonary hypertension with normal ventilatory capacity were found. Central alveolar hypoventilation was diagnosed on the basis of absence of ventilatory response and sensation to provoked hypercapnia, prolonged breath-holding time and correction of hypercapnia by voluntary hyperventilation.

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Nine obese patients (OB) and seven normal subjects (N) were studied in order to determine diaphragmatic strength reserve, measured in terms of diaphragm Tension/Time Index (TTdi). This index was measured with the patients awake and during the obstructive apnea (OA) episodes. TTdi was 2.7 times superior in the OB (p < 0.0005) and was related with a lower Pdi Max (102 vs 202 cm H2O; p < 0.005) and with a higher Pdi (9 vs 6 cm H2O; p < 0.05). During OA the TTdi was higher than the threshold value to develop diaphragmatic fatigue (0.15-0.20) in three patients. This value was not exceeded in one patient because of striking paradoxical diaphragmatic movements. As for anthropometric data, DPI% range was higher and wider in OB (Table 1). The pCO2 in OB was 40 +/- 6 mmHg. In all patients (Table 3), severe hypoxia and hypercapnial were observed. TTdi evolution in an OA in 4 patients can be appreciated in Figure 3. It can be concluded that the diaphragmatic strength reserve is reduced in OB so that they are more susceptible to develop diaphragmatic fatigue. A fatigant respiratory pattern was also registered during the OA.

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RESUMEN

Nine obese patients (OB) and seven normal subjects (N) were studied in order to determine diaphragmatic strength reserve, measured in terms of diaphragm Tension/Time Index (TTdi). This index was measured with the patients awake and during the obstructive apnea (OA) episodes. TTdi was 2.7 times superior in the OB (p < 0.0005) and was related with a lower Pdi Max (102 vs 202 cm H2O; p < 0.005) and with a higher Pdi (9 vs 6 cm H2O; p < 0.05). During OA the TTdi was higher than the threshold value to develop diaphragmatic fatigue (0.15-0.20) in three patients. This value was not exceeded in one patient because of striking paradoxical diaphragmatic movements. As for anthropometric data, DPI

range was higher and wider in OB (Table 1). The pCO2 in OB was 40 +/- 6 mmHg. In all patients (Table 3), severe hypoxia and hypercapnial were observed. TTdi evolution in an OA in 4 patients can be appreciated in Figure 3. It can be concluded that the diaphragmatic strength reserve is reduced in OB so that they are more susceptible to develop diaphragmatic fatigue. A fatigant respiratory pattern was also registered during the OA.

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RESUMEN

Nine obese patients (OB) and seven normal subjects (N) were studied in order to determine diaphragmatic strength reserve, measured in terms of diaphragm Tension/Time Index (TTdi). This index was measured with the patients awake and during the obstructive apnea (OA) episodes. TTdi was 2.7 times superior in the OB (p < 0.0005) and was related with a lower Pdi Max (102 vs 202 cm H2O; p < 0.005) and with a higher Pdi (9 vs 6 cm H2O; p < 0.05). During OA the TTdi was higher than the threshold value to develop diaphragmatic fatigue (0.15-0.20) in three patients. This value was not exceeded in one patient because of striking paradoxical diaphragmatic movements. As for anthropometric data, DPI

range was higher and wider in OB (Table 1). The pCO2 in OB was 40 +/- 6 mmHg. In all patients (Table 3), severe hypoxia and hypercapnial were observed. TTdi evolution in an OA in 4 patients can be appreciated in Figure 3. It can be concluded that the diaphragmatic strength reserve is reduced in OB so that they are more susceptible to develop diaphragmatic fatigue. A fatigant respiratory pattern was also registered during the OA.

RESUMEN

Nine obese patients (OB) and seven normal subjects (N) were studied in order to determine diaphragmatic strength reserve, measured in terms of diaphragm Tension/Time Index (TTdi). This index was measured with the patients awake and during the obstructive apnea (OA) episodes. TTdi was 2.7 times superior in the OB (p < 0.0005) and was related with a lower Pdi Max (102 vs 202 cm H2O; p < 0.005) and with a higher Pdi (9 vs 6 cm H2O; p < 0.05). During OA the TTdi was higher than the threshold value to develop diaphragmatic fatigue (0.15-0.20) in three patients. This value was not exceeded in one patient because of striking paradoxical diaphragmatic movements. As for anthropometric data, DPI

range was higher and wider in OB (Table 1). The pCO2 in OB was 40 +/- 6 mmHg. In all patients (Table 3), severe hypoxia and hypercapnial were observed. TTdi evolution in an OA in 4 patients can be appreciated in Figure 3. It can be concluded that the diaphragmatic strength reserve is reduced in OB so that they are more susceptible to develop diaphragmatic fatigue. A fatigant respiratory pattern was also registered during the OA.

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Se presenta una paciente con distrofia miotónica (enfermedad de Steinert) con debilidad muscular generalizada, disnea de decúbito, parálisis diafragmática bilateral, incapacidad ventilatoria severa e insuficiencia respiratoria con hipoxia e hipercapnea. La PImáx y Pdimáx fueron muy inferiores a los valores normales así como la respuesta a la inhalación de CO2. Luego de aminofilina desapareció la ortopnea, mejoraron Pdimáx, PImáx y respuesta al CO2, normalizándose la PaCO2 en posición sentada. Estos resultados, de confirmarse, sugieren el uso de aminofilina en la debilidad muscular respiratoria de la enfermedad de Steinert

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Se presenta una paciente con distrofia miotónica (enfermedad de Steinert) con debilidad muscular generalizada, disnea de decúbito, parálisis diafragmática bilateral, incapacidad ventilatoria severa e insuficiencia respiratoria con hipoxia e hipercapnea. La PImáx y Pdimáx fueron muy inferiores a los valores normales así como la respuesta a la inhalación de CO2. Luego de aminofilina desapareció la ortopnea, mejoraron Pdimáx, PImáx y respuesta al CO2, normalizándose la PaCO2 en posición sentada. Estos resultados, de confirmarse, sugieren el uso de aminofilina en la debilidad muscular respiratoria de la enfermedad de Steinert (AU)

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