Pulmonary rehabilitation at home guided by telemonitoring and access to healthcare facilities for respiratory complications in patients with neuromuscular disease.

BACKGROUND: Pulmonary complications are the main cause of morbidity and mortality in neuromuscular patients. Aim of this study was to evaluate the feasibility of a home follow-up program combining telemonitoring and chest physiotherapy (CPT) in preventing acute respiratory episodes. DESIGN: Prospective observational study in a period of 24 months, and comparison with preintervention data of the same patients. SETTING: Outpatients and community. POPULATION: Neuromuscular patients. Enrolment criteria were: reduced efficacy of cough, high family support, long home-to-hospital distance. METHODS: Caregivers and patients had to register daily respiratory signs and symptoms. Each patient was equipped with a pulse oximeter with a modem for transmitting data to a remote control center, in charge of alerting the pulmonologist in case of sign and symptom deterioration. CPT interventions at home were planned after indication by the pulmonologist. The number of emergency room admissions or hospitalization following respiratory exacerbations were registered. RESULTS: Thirteen patients were enrolled. In the first year of monitoring, 18 alerts were transmitted to the pulmonologist, average 1.38±1.38 alert/patient. In the second year, the number of alerts were 5, average 0.38±0.65 alert/patient (P<0.01). In 24 months, 241 respiratory therapists' interventions were conducted on 11 patients. In the first 12 months there were four episodes of hospitalisation, none in the following 12 months. In the year prior to the project, there were seven cases of hospitalisation and one case of emergency room admission. CONCLUSION: The combination of telemonitoring and CPT at home is feasible in the long-term for patients with neuromuscular disease. CLINICAL REHABILITATION IMPACT: An apparent reduction of hospitalisation and emergency room admissions for respiratory complications can justify a randomized control trial to confirm efficacy and effectiveness.

Heart and lungs in COPD. Close friends in real life--separate in daily medical practice?

Chronic obstructive pulmonary disease (COPD) is associated with a 2-3 times higher rate of cardiovascular disorders (CVD) which is independent of other risk factors. A low FEV1 is a specific predictor of mortality as a result of cardiac causes, even stronger than increased cholesterol: for each 10% reduction of FEV1, cardiovascular mortality increases by 28%. The main causes of death among COPD patients are of cardiovascular origin. COPD and CVD have two major risk factors in common - advanced age and tobacco smoking. The search for a pathogenetic link between the two conditions focuses mainly on systemic extension of pulmonary inflammation. Despite such a frequent association, pulmonologists and cardiologists in both the clinical and the research settings often underestimate the importance of a correct diagnosis and severity stratification of the two combined conditions. Spirometry, in particular, is largely underprescribed. Missed diagnosis and severity stratification, incomplete knowledge of adverse drug events and lack of resources lead to undertreatment of patients combining COPD and CVD, and in particular, the underuse of beta-blockers, inhaled bronchodilators and rehabilitation. Clinical studies focusing on this group of patients should be promoted in the future to test therapies and manage options. Furthermore, efforts must be made to improve the present standards of care, which falls short of recommended levels, starting from the often-neglected use of spirometry to confirm a diagnosis of COPD.

Prediction of FEV1 reductions in patients undergoing pulmonary resection.

In the assessment of functional operability of lung cancer the simple calculation of predicted postoperative forced expiratory volume in one second (ppoFEV1) based on the number of the bronchopulmonary segments removed, has recently been found to underestimate the actual postoperative forced expiratory volume in one second (FEV1). We checked whether this could be accounted for by lesions causing uneven ventilation or perfusion. We performed a retrospective study of 12 patients with atelectasis, hilar disease or endobronchial involvement (Group A) versus 24 patients with peripheral lung cancer (Group B). Baseline and postoperative FEV1 were measured and ppoFEV1 and ppoFEV1/actual postoperative FEV1 were calculated. In all subjects in Group A ppoFEV1 grossly underestimated the measured postoperative FEV1 (mean (SD) ratio between the two parameters: 67.0 (12.1)%). In Group B, ppoFEV1 was almost identical to the measured value in all but three patients, in whom it only slightly underestimated the actual postoperative value (mean ratio between the two parameters: 94.9 (12.6) %). In conclusion, predicted postoperative forced expiratory volume in one second is useful in the preoperative assessment of patients undergoing lung resection for lesions other than those causing uneven ventilation or perfusion. When this condition is suspected, before declaring a given case ineligible for surgery or at high risk of complications, more sophisticated procedures, such as radionuclide perfusion studies, should be performed.

Pulmonary complications and respiratory function changes after bone marrow transplantation in children.

We prospectively assessed the frequency of pulmonary complications and the natural course of lung function after bone marrow transplantation (BMT), as well as the effect of several risk factors in a homogeneous group of 39 children who underwent allogeneic or autologous BMT for haematological malignancies between 1992 and 1995. Four patients developed pneumonia within the first 3 months and three 3-6 months after BMT. A considerable percentage of acute bronchitis was recorded throughout the follow-up. Three patients died after the 6 month visit because of pneumonia (two patients) and pulmonary aspergillosis (one patient). No patients had obstructive lung disease syndrome. At 3 months after BMT, forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and transfer factor of the lung for carbon monoxide (TL,CO) significantly decreased, but FEV1/FVC ratio and maximal expiratory flow at 25% of FVC remained unchanged, suggesting a restrictive defect with diffusion impairment. At 18 months, there was a progressive recovery in lung function, although only 11 patients had normalized. Seropositivity for cytomegalovirus had a significant effect on lung function whereas graft-versus-host disease also had an effect, although it was not statistically significant. Baseline respiratory function, type of transplant, type of conditioning regimen and respiratory infections did not significantly affect the outcome of BMT. The high frequency of severe lung function abnormalities found in this study, suggests a careful functional monitoring in all subjects undergoing bone marrow transplantation, even in the absence of respiratory symptoms.

Pulmonary function in patients with systemic sclerosis.

The lungs are frequently affected in systemic sclerosis (SSc), a generalized connective tissue disorder. We evaluated the prevalence of respiratory functional abnormalities and their correlation with symptoms and radiograph features in a group of 34 patients who fulfilled the American Rheumatism Association criteria for the diagnosis of systemic sclerosis. Patients were submitted to a specific respiratory questionnaire and to lung function tests. Measurements were performed according to the European Coal and Steel Community (ECSC) recommendations and results expressed as a SD score, an accurate method that, taking into account the dispersion of the parameters in the reference population, allows precise definition of pathological subjects. Of the patients examined, 38% reported dyspnoea at rest or on exertion. No other respiratory symptoms were reported. Fifty percent had a normal chest radiograph. This study documents the high prevalence of respiratory functional abnormalities in patients with SSc. A restrictive pattern was found in 41% and an isolated diffusion impairment in 18%. No significant relationship was found between the isolated impairment of transfer factor of the lungs for carbon monoxide (TL,CO) and the mean duration of the scleroderma: thus, it does not seem to represent an early sign of severe restrictive disease. No bronchial or bronchiolar obstructive patterns were observed: it can be stated that small airways dysfunction is not a characteristic manifestation of SSc as considered previously. A significant association was found between the group of subjects with chest radiographic abnormalities and that with a restrictive pattern or isolated TL,CO alteration (p = 0.018). Chest radiographic abnormalities were also found in 29% and dyspnoea in 35% of the patients with normal respiratory function. The mean duration of scleroderma was not significantly different between the groups with and without abnormalities on chest radiography, between the groups with and without a restrictive pattern or isolated diffusion impairment, and between the groups of patients with and without dyspnoea. In conclusion, an accurate evaluation of respiratory function is recommended in the assessment of patients with systemic sclerosis, since the functional involvement of the lung cannot be predicted on the basis of the chest radiograph and the respiratory symptoms.

Pulmonary function in children with systemic lupus erythematosus.

BACKGROUND: Abnormalities of pulmonary function have been found in children with systemic lupus erythematosus (SLE) even in the absence of clinical or radiographic evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they are associated with disease activity. METHODS: After a mean of 4.5 years, respiratory function (forced vital capacity (FVC) and single breath gas transfer factor (TLCO)) and disease activity were reexamined in 13 of 15 previously studied children with SLE. Disease activity was assessed by a validated index of SLE activity (SLE activity measure (SLAM)). RESULTS: In spite of the high prevalence of abnormalities of respiratory function at the baseline investigation, no chest radiographic abnormalities or overt clinical signs of lung disease were found at baseline, in the interval between the two investigations, or at the re-evaluation in any patient. From baseline to the second investigation the mean value of SLAM decreased and there was a trend toward an improvement in FVC and TLCO. TLCO was more severely impaired than FVC, being found as an isolated abnormality in a high percentage of patients (45% at baseline and 35% at follow up). There was a relationship between baseline TLCO and disease activity, expressed as a SLAM score. Moreover, there was a correlation between the changes in the SLAM score from baseline to the second investigation and the corresponding changes in the TLCO value, but not with the corresponding changes in the FVC value. CONCLUSIONS: In this series of patients the decrease in SLE activity from the first to the second investigation was associated with an improvement in pulmonary function. The presence of early isolated functional abnormalities was not associated with subsequent development of lung disease.

Reference values of arterial oxygen tension in the middle-aged and elderly.

The lack of available reference values of arterial PO2, particularly for elderly persons, led us to study a sample of 194 normal nonsmoking subjects, equally distributed over all age ranges from 40 to 90 yr. The radial artery was punctured and blood samples were taken and analyzed on an automated, computerized gas-analyzer. The trend of the mean values of PaO2 in the 5-yr class intervals of age showed a clear decline up to the 70- to 74-yr class, and then an inversion. The two regression lines intersecting at this point provided a better fit to the data than did a single regression line (R22 - R12 = 0.918 - 0.678 = 0.24; F = 20.49, p = 0.0027). The relationship of PaO2 with age was thus subsequently considered for the two subgroups (40 to 74 yr; > or = 75 yr) identified on the basis of this cutoff. Because of the significant influence on Pao2 of age, body-mass index (BMI), and PaCO2 in the group 40 to 74 yr of age, the following reference equation was constructed: Pao2 (mm Hg) = 143.6 - (0.39 . age) - (0.56 . BMI) - (0.57 . PaCO2); R2 = 0.28; SEE = 7.48; p < 0.0001. For subjects > or = 75 yr old, for whom there was no correlation with age, BMI, or PaCO2, only the mean +/- SD and 5th percentile of PaO2 were reported (83.4 +/- 9.15 mm Hg and 68.4 mm Hg, respectively). PaCO2 values were not correlated with either age or BMI; the mean +/- SD was 35.79 +/- 3.87 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)