[Analysis of factors associated with chronic hypercapnia in patients with myotonic dystrophy]. / Análisis de factores relacionados con hipercapnia crónica en la distrofia miotónica.

Myotonic dystrophy (DM) is the most common dystrophy in adults. Several factors may explain the chronic CO2 retention. The selection of patients, different clinical stages and evaluation forms may explain the differing results obtained. Our objectives were to characterize respiratory function and to evaluate factors associated with chronic retention of CO2 in DM. We included 27 consecutive ambulatory and stable patients who were allocated into normocapnic and hypercapnic groups (PaCO2 ≥ 43 mmHg). Forced vital capacity (FVC), maximum static pressure, voluntary apnea time, Epworth scale and arterial blood gases were measured. The CO2 chemosensitivity was assessed using CO2 rebreathing (Read method). The slope ΔP0.1/ΔPCO2 expressed the CO2 chemosensitivity. A 59.3% (16/27) presented hypercapnia. FVC and respiratory muscle strength were normal or showed mild to moderate decrease. No significant differences in these variables were found in both groups. Inadequate response to CO2 (slope ΔP0.1/ΔPCO2 low (< 0.1 cm H2O/mmHg) or flat) was associated with hypercapnia (p < 0.005). Chronic retention of CO2 represented 11.56 times higher risk of inadequate response to CO2. The group with low-flat slope ΔP0.1/ΔPCO2 showed higher PaCO2 (p = 0.0017) and more prolonged voluntary apnea time (p = 0.002). We conclude that in our patients with DM, chronic CO2 retention was associated with the presence of abnormalities of the central control of breathing. Our results allow explaining previous reports describing the striking frequency of postoperative respiratory failure and difficulties in the process of weaning from mechanical ventilation.

Análisis de factores relacionados con hipercapnia crónica en la distrofia miotónica / Analysis of factors associated with chronic hypercapnia in patients with myotonic dystrophy

La distrofia miotónica (DM) es la distrofia muscular más común en adultos. Diversos factores pueden explicar la retención crónica de CO2. La selección de pacientes, diferentes estadios evolutivos y formas de evaluación, pueden explicar los resultados disímiles al respecto. Nuestros objetivos fueron caracterizar la función respiratoria y analizar los factores relacionados con la retención crónica de CO2 en la DM. Se incluyeron 27 pacientes ambulatorios consecutivos, estables clínicamente y se los agrupó como normocápnicos e hipercápnicos (PaCO2 ≥ 43 mm Hg). Se determinaron capacidad vital forzada (FVC), presiones estáticas máximas, tiempo de apnea voluntaria, escala de Epworth y gases arteriales. La quimiosensibilidad al CO2 se evaluó mediante la reinhalación de CO2 (método de Read). La pendiente ∆P0.1/∆PCO2 expresa la quimiosensibilidad al CO2. El 59.3% tenían hipercapnia. La FVC y la fuerza muscular respiratoria fueron normales o mostraron disminución leve a moderada, sin diferencias significativas en ambos grupos. La inadecuada respuesta al CO2 (pendientes ∆P0.1/∆PCO2 bajas (< 0.1 cmH2O/mm Hg) o planas) se asoció con hipercapnia (p < 0.005) y ésta significó un riesgo 11.6 veces mayor de inadecuada respuesta al CO2. El grupo con pendiente ∆P0.1/∆PCO2 baja-plana mostró mayor PaCO2 (p = 0.0017) y tiempo de apnea voluntaria más prolongado (p = 0.002). Concluimos que, en nuestros pacientes con DM, la hipercapnia crónica se asoció a la presencia de anomalías del control central de la respiración. Estos resultados permiten explicar los informes previos que describen la llamativa ocurrencia de insuficiencia respiratoria postoperatoria y las dificultades en el proceso de desvinculación de asistencia ventilatoria mecánica en estos pacientes.

Myotonic dystrophy (DM) is the most common dystrophy in adults. Several factors may explain the chronic CO2 retention. The selection of patients, different clinical stages and evaluation forms may explain the differing results obtained. Our objectives were to characterize respiratory function and to evaluate factors associated with chronic retention of CO2 in DM. We included 27 consecutive ambulatory and stable patients who were allocated into normocapnic and hypercapnic groups (PaCO2 ≥ 43 mmHg). Forced vital capacity (FVC), maximum static pressure, voluntary apnea time, Epworth scale and arterial blood gases were measured. The CO2 chemosensitivity was assessed using CO2 rebreathing (Read method). The slope ΔP0.1/ΔPCO2 expressed the CO2 chemosensitivity. A 59.3% (16/27) presented hypercapnia. FVC and respiratory muscle strength were normal or showed mild to moderate decrease. No significant differences in these variables were found in both groups. Inadequate response to CO2 (slope ΔP0.1/ΔPCO2 low (< 0.1 cm H2O/mmHg) or flat) was associated with hypercapnia (p < 0.005). Chronic retention of CO2 represented 11.56 times higher risk of inadequate response to CO2. The group with low-flat slope ΔP0.1/ΔPCO2 showed higher PaCO2 (p = 0.0017) and more prolonged voluntary apnea time (p = 0.002). We conclude that in our patients with DM, chronic CO2 retention was associated with the presence of abnormalities of the central control of breathing. Our results allow explaining previous reports describing the striking frequency of postoperative respiratory failure and difficulties in the process of weaning from mechanical ventilation.

Análisis de factores relacionados con hipercapnia crónica en la distrofia miotónica / Analysis of factors associated with chronic hypercapnia in patients with myotonic dystrophy

La distrofia miotónica (DM) es la distrofia muscular más común en adultos. Diversos factores pueden explicar la retención crónica de CO2. La selección de pacientes, diferentes estadios evolutivos y formas de evaluación, pueden explicar los resultados disímiles al respecto. Nuestros objetivos fueron caracterizar la función respiratoria y analizar los factores relacionados con la retención crónica de CO2 en la DM. Se incluyeron 27 pacientes ambulatorios consecutivos, estables clínicamente y se los agrupó como normocápnicos e hipercápnicos (PaCO2 ≥ 43 mm Hg). Se determinaron capacidad vital forzada (FVC), presiones estáticas máximas, tiempo de apnea voluntaria, escala de Epworth y gases arteriales. La quimiosensibilidad al CO2 se evaluó mediante la reinhalación de CO2 (método de Read). La pendiente ∆P0.1/∆PCO2 expresa la quimiosensibilidad al CO2. El 59.3% tenían hipercapnia. La FVC y la fuerza muscular respiratoria fueron normales o mostraron disminución leve a moderada, sin diferencias significativas en ambos grupos. La inadecuada respuesta al CO2 (pendientes ∆P0.1/∆PCO2 bajas (< 0.1 cmH2O/mm Hg) o planas) se asoció con hipercapnia (p < 0.005) y ésta significó un riesgo 11.6 veces mayor de inadecuada respuesta al CO2. El grupo con pendiente ∆P0.1/∆PCO2 baja-plana mostró mayor PaCO2 (p = 0.0017) y tiempo de apnea voluntaria más prolongado (p = 0.002). Concluimos que, en nuestros pacientes con DM, la hipercapnia crónica se asoció a la presencia de anomalías del control central de la respiración. Estos resultados permiten explicar los informes previos que describen la llamativa ocurrencia de insuficiencia respiratoria postoperatoria y las dificultades en el proceso de desvinculación de asistencia ventilatoria mecánica en estos pacientes.(AU)

Myotonic dystrophy (DM) is the most common dystrophy in adults. Several factors may explain the chronic CO2 retention. The selection of patients, different clinical stages and evaluation forms may explain the differing results obtained. Our objectives were to characterize respiratory function and to evaluate factors associated with chronic retention of CO2 in DM. We included 27 consecutive ambulatory and stable patients who were allocated into normocapnic and hypercapnic groups (PaCO2 ≥ 43 mmHg). Forced vital capacity (FVC), maximum static pressure, voluntary apnea time, Epworth scale and arterial blood gases were measured. The CO2 chemosensitivity was assessed using CO2 rebreathing (Read method). The slope ΔP0.1/ΔPCO2 expressed the CO2 chemosensitivity. A 59.3% (16/27) presented hypercapnia. FVC and respiratory muscle strength were normal or showed mild to moderate decrease. No significant differences in these variables were found in both groups. Inadequate response to CO2 (slope ΔP0.1/ΔPCO2 low (< 0.1 cm H2O/mmHg) or flat) was associated with hypercapnia (p < 0.005). Chronic retention of CO2 represented 11.56 times higher risk of inadequate response to CO2. The group with low-flat slope ΔP0.1/ΔPCO2 showed higher PaCO2 (p = 0.0017) and more prolonged voluntary apnea time (p = 0.002). We conclude that in our patients with DM, chronic CO2 retention was associated with the presence of abnormalities of the central control of breathing. Our results allow explaining previous reports describing the striking frequency of postoperative respiratory failure and difficulties in the process of weaning from mechanical ventilation.(AU)

Análisis de factores relacionados con hipercapnia crónica en la distrofia miotónica. / [Analysis of factors associated with chronic hypercapnia in patients with myotonic dystrophy].

Myotonic dystrophy (DM) is the most common dystrophy in adults. Several factors may explain the chronic CO2 retention. The selection of patients, different clinical stages and evaluation forms may explain the differing results obtained. Our objectives were to characterize respiratory function and to evaluate factors associated with chronic retention of CO2 in DM. We included 27 consecutive ambulatory and stable patients who were allocated into normocapnic and hypercapnic groups (PaCO2 ÔëÑ 43 mmHg). Forced vital capacity (FVC), maximum static pressure, voluntary apnea time, Epworth scale and arterial blood gases were measured. The CO2 chemosensitivity was assessed using CO2 rebreathing (Read method). The slope ÎöP0.1/ÎöPCO2 expressed the CO2 chemosensitivity. A 59.3

(16/27) presented hypercapnia. FVC and respiratory muscle strength were normal or showed mild to moderate decrease. No significant differences in these variables were found in both groups. Inadequate response to CO2 (slope ÎöP0.1/ÎöPCO2 low (< 0.1 cm H2O/mmHg) or flat) was associated with hypercapnia (p < 0.005). Chronic retention of CO2 represented 11.56 times higher risk of inadequate response to CO2. The group with low-flat slope ÎöP0.1/ÎöPCO2 showed higher PaCO2 (p = 0.0017) and more prolonged voluntary apnea time (p = 0.002). We conclude that in our patients with DM, chronic CO2 retention was associated with the presence of abnormalities of the central control of breathing. Our results allow explaining previous reports describing the striking frequency of postoperative respiratory failure and difficulties in the process of weaning from mechanical ventilation.

Elimination of ventilator dead space during synchronized ventilation in premature infants.

BACKGROUND: Mainstream airflow sensors used in neonatal ventilators to synchronize mechanical breaths with spontaneous inspiration and measure ventilation increase dead space and may impair carbon dioxide (CO(2)) elimination. OBJECTIVE: To evaluate a technique consisting of a continuous gas leakage at the endotracheal tube (ETT) adapter to wash out the airflow sensor for synchronization and ventilation monitoring without CO(2) rebreathing in preterm infants. DESIGN: Minute ventilation (V'(E)) by respiratory inductance plethysmography, end-inspiratory and end-expiratory CO(2) by side-stream microcapnography, and transcutaneous CO(2) tension (TcPCO(2)) were measured in 10 infants (body weight, 835+/-244 g; gestational age, 26+/-2 weeks; age, 19+/-9 days; weight, 856+/-206 g; ventilator rate, 21+/-6 beats/min; PIP, 16+/-1 centimeters of water (cmH(2)O); PEEP, 4.2+/-0.4 cmH(2)O; fraction of inspired oxygen (FIo(2)), 0.26+/-0.6). The measurements were made during four 30-minute periods in random order: IMV (without airflow sensor), IMV+Sensor, SIMV (with airflow sensor), and SIMV+Leak (ETT adapter continuous leakage). RESULTS: Airflow sensor presence during SIMV and IMV+Sensor periods resulted in higher end-inspiratory and end-expiratory CO(2), Tcpco(2), and spontaneous V'(E) compared with IMV. These effects were not observed during SIMV+Leak. CONCLUSIONS: The significant physiologic effects of airflow sensor dead space during synchronized ventilation in preterm infants can be effectively prevented by the ETT adapter continuous leakage technique.

Acute chest syndrome in adult Afro-Caribbean patients with sickle cell disease. Analysis of 81 episodes among 53 patients.

BACKGROUND: To evaluate the frequency, presentation, and course of the acute chest syndrome (ACS) in adult Afro-Caribbean patients with sickle cell disease (SCD). PATIENTS AND METHODS: Retrospective cohort study during a 12-year period in patients with SCD at least 14 years of age, discharged with a diagnosis of ACS from the only hospital on the Caribbean island of Curaçao, where 109 patients with SCD (62 HbSS, 47 HbSC) were observed. RESULTS: Eighty-one episodes of ACS occurred (57 in 34 patients with HbSS and 24 in 19 patients with HbSC). The risk (odds ratio, 1.80; P = .13) and incidence (7.6 vs 4.2 per 100 patient-years; P > .2) of ACS did not differ between patients with HbSS and HbSC, but recurrent ACS affected patients with HbSS more (odds ratio, 2.96; P = .09). Abnormal chest sounds (mainly bilateral crepitations) were found in 91% of cases at diagnosis, but 48% had normal chest roentgenograms at that time and had delayed development (5.4 +/- 3.4 days) of radiologic abnormalities. Patients with HbSS and HbSC had similar clinical presentations. Mortality (6%) and hospital stay (20 days) were not influenced by the use of transfusions or anticoagulation. All five nonsurviving female patients with HbSS had had more previous admissions for SCD and ACS. CONCLUSIONS: Acute chest syndrome occurs in 42% of adult Afro-Caribbean patients with SCD; patients with HbSS are more prone to recurrences. Delayed development of radiologic infiltrates is common. Interventions apart from supportive care do not influence the course of ACS. Fatal ACS occurs in patients with a more severe form of SCD.

[Evaluation of the hypophyseal-hypothalamo-adrenal axis in patients undergoing chronic corticotherapy at suppressive doses]. / Valoración del eje hipófisis-hipotálamo-adrenal en pacientes con corticoterapia crónica a dosis supresivas.

A cortisol curve was practiced on 32 healthy persons with an average age of 38.7 years, being the morning amount at 8 o'clock of 10 mg/100 ml o higher. Later we practiced the same study in 42 patients with an average age of 41.4 years, that were under a corticosteroid therapy higher than 10 mg per day of prednisone during more than three months of an uninterrupted prescription and said treatment was stopped 48 previous hours to the study. We found that 27 of them (64.2%) had lowe morning amount of cortisol to those of the control group, and the patients with rheumatoid arthritis being the lowest. It is concluded that the type of systemic disease could be another factor in the suppression of the axis H-H-A of patients under a higher dosis than 10 mg of prednisone and a durability longer than three continuous months.

Pulse oximetry for continuous oxygen monitoring in sick newborn infants.

We studied 54 neonates with acute cardiorespiratory illness and 21 infants with bronchopulmonary dysplasia, to evaluate the accuracy of a nonheated pulse oximeter in predicting arterial oxygen saturation (SaO2). We also studied the accuracy of transcutaneous oxygen tension (tcPO2) in estimating arterial oxygen tension (PaO2) in infants with bronchopulmonary dysplasia. We compared pulse oximeter SaO2 with simultaneously measured SaO2 (range 78% to 100%) using a co-oximeter. Over a wide range of values for heart rate, blood pressure, hematocrit, PO2, PCO2, and pH, linear regression analysis revealed a close correlation between in vivo pulse oximeter readings and in vitro SaO2 measurements in patients with acute (r = 0.86, Y = 29.64 + 0.68X) and chronic (r = 0.91, Y = 6.29 + 0.96X) disease. Regression analysis of tcPO2 versus PaO2 showed an r value of 0.76 in infants with bronchopulmonary dysplasia. In these patients the mean difference between pulse oximeter SaO2 and in vitro SaO2 was 2.9% +/- 1.8% (SD), whereas the mean difference between tcPO2 and PaO2 was -14.5 +/- 11.1 mm Hg. Fetal hemoglobin ranged from 4.3% to 95%. We conclude that pulse oximetry is an appropriate alternative to tcPO2 for continuous oxygen monitoring in newborn infants with acute cardiorespiratory illnesses and chronic lung disease.