[Social and housing conditions of patients with chronic respiratory failure treated in the ANTADIR Federation]. / La vie quotidienne et sociale des patients insuffisants respiratoires chroniques sévères pris en charge dans le réseau ANTADIR.

INTRODUCTION: Chronic respiratory failure (CRF) has an impact on quality of life because of respiratory handicap. METHODS: The purpose of this study was to analyse the daily life and social activities of patients with CRF. A questionnaire was addressed to over 9000 patients being cared for in the ANTADIR homecare network, with over a 60% response rate. RESULTS: The data showed that patients were old, and frequently had comorbidity. The predominant diagnosis was chronic obstructive airways disease (COPD) and the number of women in this population continues to increase. Respiratory handicap led to a loss of autonomy, a reduction in social activity and mobility of patients, but the impact differed markedly according to the cause of the respiratory failure, as well as age and social class. A typological study of behavioural characteristics revealed three groups of patients with contrasting profiles. CONCLUSION: These results lead to recommendation for better management of social aspects of patient care in the ANTADIR network.

[Familial diffuse interstitial fibrosis of prolonged onset worsening during pregnancy]. / Pneumopathie interstitielle diffuse fibrosante familiale d'évolution prolongée aggravée au cours d'une grossesse.

INTRODUCTION: Familial idiopathic interstitial pulmonary fibrosis is rare. In this case report the diagnosis was confirmed histologically in four members of the same family. CASE REPORT: A woman whose father and two paternal uncles had developed pulmonary fibrosis was hospitalised from birth on account of delayed growth and dyspnoea. At the age of one year an increase in dyspnoea and the development of hypoxaemia and diffuse interstitial shadowing led to a surgical lung biopsy. The histological diagnosis was idiopathic interstitial fibrosis. Immunosuppressive treatment for one year led to clinical improvement with relief of the hypoxaemia but persistence of the interstitial shadowing. A pneumothorax at the age of 15 required pleurectomy. The clinical state remained stable with a restrictive ventilatory defect up to the age of 26 when respiratory insufficiency developed in the course of pregnancy. The outcome following delivery was severe respiratory failure complicated by pulmonary arterial hypertension leading to death the following year. CONCLUSIONS: This case is distinguished by a histological diagnosis in four members of a family of whom one was an infant, the prolonged stabilisation after immunosuppressive therapy and the possible role of pregnancy in the progression.

[Diffuse pulmonary lymphangiomatosis in a young adult]. / Lymphangiomatose pulmonaire diffuse chez un adulte jeune.

We report a new case of diffuse pulmonary lymphangiomatosis in a 22 year-old man with a dysmorphic syndrome. The disease started with dyspnea which became rapidly disabling. The diagnosis was established from a pulmonary biopsy. Our patient developed severe chronic respiratory failure. Diffuse pulmonary lymphangiomatosis is a very uncommon disease. It is seen predominantly in children, exceptionally in adults, and affects both sexes equally. Symptoms like dyspnea and cough, pulmonary function with restrictive pattern, and interstitial syndrome, are not specific. Only pathology is evocative, characterized primarily by multifocal proliferation of pulmonary lymphatic vessels and increased number of complex anastomosing channels. These channels tend to dilate with time. The prognosis is poor and the treatment essentially palliative.

[Diagnosis and evaluation of chronic respiratory insufficiency]. / Diagnostic et évaluation de l'insuffisance respiratoire chronique.

Chronic respiratory failure is defined by the inability of the respiratory system to carry out the gas exchange needed to fulfill the body needs. Its diagnosis is based on arterial blood gas measurements. It is possible, however, for the physician to assume it from clinical symptoms. Measuring peak expiratory flow allows one to evaluate the degree of insufficiency. The pneumologist confirms the diagnosis with arterial blood gases measurement and determines its severity. Exercise tests evaluate the incapacity. A quality of life questionnaire assesses the respiratory handicap resulting from the patient's life comfort deficit. After diagnosis, the respiratory failure follow-up is performed by the general practitioner, with specialized investigations as often as necessary according to the severity of the respiratory failure.

Interpretation of high wedge pressure on exercise in patients with chronic obstructive pulmonary disease.

In a retrospective review of patients subjected to right heart catheterization, case records from 109 consecutive patients with chronic obstructive pulmonary disease (COPD) and wedge pressure > or =15 mm Hg on exercise were analyzed. Patients were separated into group 1, resting wedge pressure (P macro w) <15 mm Hg and difference between P macro w and right atrial pressure (P macro ra) change on exercise <5 mm Hg (n = 54), group 2, same P macro w at rest but increase in macro on exercise > or =5 mm Hg, being higher than that of P macro ra (n = 34), and group 3, P macro w > or =15 mm Hg at rest (n = 21). The occurrence of left heart disease increased from group 1 to group 3 (19, 53 and 71%, respectively), and the slope of the P macro w/stroke index relationship was lowest in group 3. High P macro w on exercise can be explained by the pressure rise in the cardiac fossa associated with lower lobe gas trapping in group 1, which showed the most severe airflow limitation, decreased left ventricular compliance in group 2, and heart failure in group 3.

[Home ventilation after intensive care]. / La ventilation à domicile après un séjour en réanimation.

In the course of chronic respiratory insufficiency, acute episodes often require the use of mechanical ventilation. Failure of weaning, or worsening of alveolar hypoventilation, results in long term ventilatory assistance with the need of overall care of the patient. The pneumologist has a key role in the choice of indications, devices and mode of home mechanical ventilation. Thanks to the non-invasive ventilation with a facial or nasal mask, tracheostomy is less often needed. Respiratory failure due to lung restriction is the best indication of mechanical ventilation. The results in COPD are questionable. Whatever to the technique of ventilation and the underlying disease, the pneumologist has to ascertain the steady state of the medical condition, patient and family education, and social situation, all factors to be taken into account before the patient can be discharged. Knowing the specific needs in these patients'care, the pneumologist plays a key role at each stage of home return and follow-up. He co-ordinates the different aspects of pneumological care, like rehabilitation and acute episodes treatment. The overall care of the patients should be improved by the development of health networks in the field of chronic respiratory insufficiency.

[Sinobronchial syndrome]. / Le syndrome sinobronchique.

The sinobronchial syndrome consists of chronic sinusitis and chronic nonspecific inflammation of the lower airways, typically chronic bronchitis, bronchiectasis and diffuse panbronchiolitis. This airway disease of unknown etiology is not related to smoking. It is common in Japan and is poorly described in western countries. We report a case in a non-Asian patient. The known efficacy of long-term low-dose erythromycin therapy justifies careful diagnosis of sinobronchial syndrome in patients of non-Asian ethnic origin.

Endurance training in patients with chronic obstructive pulmonary disease: a comparison of high versus moderate intensity.

PURPOSE: To create a maximum tolerated 45-minute aerobic training program for patients with chronic obstructive pulmonary disease (COPD) and to compare its outcomes with those of commonly prescribed moderate exercise. DESIGN: Prospective, randomized trial. SETTING: A work physiology laboratory. PATIENTS AND METHODS: The maximum exercise intensities that 7 COPD patients could sustain for 45 minutes were determined on a bilevel exercise ergometer. The patients then exercised 45 minutes daily, 5 days a week for 6 weeks, working 2.03+/-0.4 kJ/kg per session. They were matched with 6 COPD patients who pushed an O2 cart for 45 minutes daily, 5 days a week for 6 weeks, working 1.44+/-.35 kJ/kg per session. RESULTS: A 45 minute maximal regimen was established by alternating 1-minute peak exercise at peak VO2-levels with 4 minutes at the ventilatory anaerobic threshold or at 40% of peak VO2. Maximal bilevel training significantly decreased dyspnea at rest (p< or =.01) and the blood lactate level during submaximal exercise (p<.001), and increased peak VO2 and total physical work (p<.01), maximum inspiratory and expiratory pressures (p<.01), and grip and forearm strength and endurance (p<.01). The training also increased maximum voluntary ventilation while decreasing the ventilatory equivalent during exercise (p<.001). The O2 cart pushers significantly improved only on the 12-minute walk (p<.05). CONCLUSIONS: A maximally intense anaerobic exercise program can be created for most COPD patients that can significantly improve both skeletal and respiratory muscle strength and endurance as well as dyspnea and physiologic parameters.

[Lung transplantation: indications, techniques and results]. / La transplantation pulmonaire: indications, techniques et résultats.

Lung transplantation is proposed for young patients with a severe disease that can be expected to be fatal within less than two or three years. The main indications are chronic respiratory failure induced by chronic obstructive lung disease, cystic fibrosis or pulmonary fibrosis, and severe primary or secondary (Eisenmenger syndrome) pulmonary hypertension. The type of transplantation, determined after an exhaustive work-up ruling out all contraindications, is generally a single lung transplantation if there is no bronchial infection or two-lung transplantation or heart-lung transplantation in case of bronchiectasis or pulmonary artery hypertension. Survival at 1, 3 and 5 years is 72, 57 and 43% respectively. Transplantation improves exercise capacity, quality of life and lung function. It normalizes hematosis in case of chronic respiratory failure and pulmonary hemodynamics in case of pulmonary hypertension. The risk of complications, dominated by infections and rejections, requires careful clinical, functional and endoscopic follow-up. Bacterial infections are frequent during the first weeks. The frequency of opportunistic infections can be reduced by anti-infectious prophylaxis strategies. The rejection can occur as an acute episode, frequent during the first 100 days, or is sometimes asymptomatic. Chronic rejection or obliterating bronchiolitis is the main mid-term complication after lung transplantation and is responsible for the low long-term survival rate with recurrent secondary infections due to frequent bronchial colonization with Pseudomonas aeruginosa. Improved prognosis of lung transplantation requires the development of new immunosuppressive agents with lower risk of infection and chronic rejection observed with current treatments.

[Hepatopulmonary syndrome: physiopathology of impaired gas exchange]. / Syndrome hépatopulmonaire. Physiopathologie des anomalies des échanges gazeux.

The hepatopulmonary syndrome (HPS) consists of a triad of liver dysfunction, increased alveolar-arterial oxygen gradient and intrapulmonary vascular dilations. The mechanisms of impaired arterial oxygenation are still debated but the multiple inert gases elimination technique and more recently contrast echocardiography, greatly facilitated the investigation of such mechanisms. Subsequently the cause of hypoxemia can be attributed to several mechanisms such as ventilation-perfusion mismatch, right-to-left intrapulmonary shunts and alveolar-to-capillary diffusion defect, variously implicated in the severity of the disease. SHP may result from intrapulmonary vascular dilations and angiogenesis but the pathogenesis of such abnormalities is not completely explained. The hypothesis of an imbalance in vasoactive mediators and angiogenic factors has been put forward. Increasing data support the theory that the increase in synthesis and release of nitric oxide (NO) is the key factor modulating vascular tone. If this hypothesis is true, the use of compettive inhibitors of NO synthesis should restore pulmonary vascular tone, reversing the hemodynamic changes and gas exchange impairment of HPS.

Validity of transcutaneous oxygen/carbon dioxide pressure measurement in the monitoring of mechanical ventilation in stable chronic respiratory failure.

The accuracy and precision of transcutaneous pressure measurements of oxygen (Ptc,O2) and carbon dioxide (Ptc,CO2) in the monitoring of nocturnal assisted ventilation in adult patients were evaluated. Transcutaneous measurements obtained with two analysers, Radiometer TINATCM3 (R) and Kontron MicroGas-7650 (K), were compared with arterial blood gases analysed in blood samples withdrawn simultaneously in 10 patients. Sensors were heated to 43 degrees C. Measurements of transcutaneous blood gases and arterial blood gases were collected six times at 1-h intervals. The data obtained with both instruments were similar and did not significantly change over the 5 h test period. Measurement of Ptc,O2 underestimated arterial oxygen tension (Pa,O2) and this underestimation increased with the level of Pa,O2 (p<0.01). Measurements of Ptc,CO2 overestimated arterial carbon dioxide tension (Pa,CO2) and this overestimation increased with the level of Pa,CO2 (p<0.05). These errors suggested an instrumental bias. Mathematical correction of this bias neutralized the error in accuracy and improved the precision (SD of the differences transcutaneous blood gases - arterial blood gases). An additional correction, suppressing the between-subject scattering, improved the actual precision: precision was reduced from 1.9 to 0.8 kPa (14.4 to 5.7 mmHg) (R) and from 1.7 to 0.5 kPa (13.1 to 3.7 mmHg) (K) for oxygen, and from 1.0 kPa (7.8 mmHg) (R) and 0.7 kPa (5.6 mmHg) (K) to 0.4 kPa (3.2 mmHg) for carbon dioxide (R and K). In conclusion, with these two successive corrections, transcutaneous oxygen and carbon dioxide provide a reliable estimation of blood gases to monitor nocturnal ventilation in adults with chronic respiratory failure.

[Association of sarcoidosis and hemorrhagic rectocolitis in an insulin-dependent diabetic]. / Association d'une sarcoïdose et d'une rectocolite hémorragique chez un diabétique insulino-dépendant.

Ulcerative colitis and sarcoidosis are seldom associated. A 33-year-old diabetic man developed simultaneously a rectitis and a non-productive cough leading to the diagnosis of ulcerative colitis and sarcoidosis. These diagnoses are discussed because of the possible gastrointestinal lesions from sarcoidosis or the respiratory disorders due to ulcerative colitis. Some pathophysiological evidence can be found in this case in favor of a non-fortuitous association.

Health-related quality of life in long-term oxygen-treated chronic obstructive pulmonary disease patients.

Chronic obstructive pulmonary disease (COPD) induces changes in daily activities and mood. Health-related quality of life (HRQL) measures are of particular relevance in the management of such patients, but predictors of HRQL have rarely been investigated. The aim of this study was to seek factors predicting HRQL in severe COPD patients under long term oxygen therapy (LTOT). The pulmonary function parameters at the start of LTOT were studied as potential predictors of future HRQL. HRQL was assessed after an average of 40 months follow-up by the Duke Health Profile (Duke) and by the St. George Respiratory Questionnaire (SGRQ). Sixty-one patients (47 males and 14 females) with a mean age of 66 years were included in the study. Stepwise multiple regression analyses conducted in each HRQL dimension identified few significantly predictive factors. By the Duke, higher Self-esteem scores were associated with higher Pao2 (p < 0.01) and with older age (p < 0.05); higher Social Health scores were associated with older age (p < 0.005), and higher Pain scores were associated with a higher FEV1/FVC ratio (p < 0.05). By the SGRQ, the Activity score correlated with FEV1/FVC (p < 0.05). The proportion of the variance in each score accounted for by covariates in the model did not exceed 10%. No other significant regression models could be identified using the other HRQL dimensions. Our findings demonstrated weak relations between lung function at the start of LTOT and some dimensions of HRQL measured by the Duke and the SGRQ at the end of follow-up.

Frequency dependence of specific airway resistance in a commercialized plethysmograph.

Specific airway resistance (sRaw) measured by body plethysmography has been shown to decrease markedly with decreasing breathing frequency when the inspired air is not conditioned to body temperature, atmospheric pressure and saturation with water vapour (BTPS). The phenomenon has been attributed to noninstantaneous gas warming and wetting in the airways. The aim of this investigation was to assess whether the phenomenon was also present in a commercialized plethysmograph featuring an "electronic BTPS correction". Airway resistance (Raw) and sRaw were measured in 15 healthy subjects at six breathing frequencies ranging 0.25-3 Hz, using a constant volume plethysmograph in which a correction for non-BTPS gas conditions was applied by electronically flattening the box pressure-airway flow loop (Jaeger Masterscreen Body, version 4.0). The temperature and water vapour saturations in the box averaged 26.5 +/- 1.3 degrees C and 59 +/- 6%, respectively. Raw and sRaw exhibited a clear positive frequency dependence in all but one subject. From 0.25 to 3 Hz Raw increased from (mean+/-SD) 0.62 +/- 0.55 to 1.71 +/- 0.76 hPa x s x L-1 (p<0.001), and sRaw from 2.34 +/- 1.90 to 7.55 +/- 3.08 hPa x s (p<0.001). The data are consistent with a simple model, in which gas conditioning in the airways and external dead space occurred with a time constant of 0.39 s. We conclude that the electronic BTPS correction of the instrument was inadequate, probably because it is assumed that gas conditioning in the airways is instantaneous. We recommend that, with similar instruments, airway resistance be measured using as high a panting frequency as feasible.

Effects of rhDNase on purulent airway secretions in chronic bronchitis.

Recombinant human deoxyribonuclease (rhDNase) has been demonstrated to reduce in vitro the viscosity and to improve the transport capacity of purulent respiratory mucus in cystic fibrosis. During episodes of exacerbation of chronic bronchitis, the patients generally expectorate purulent mucus. Purulence of mucus is associated with an increased deoxyriboneucleic acid (DNA) concentration. We analyzed in vitro the potential effect of rhDNase on chronic bronchitis mucus transport by the ciliary activity (frog palate model) and by simulated cough (cough machine model), as well as the effect on mucus viscosity (controlled stress rheometer) and surface properties (contact angle). Purulent sputa collected from patients with chronic bronchitis (n = 15) during an episode of exacerbation were incubated for 30 min at 37 degrees C with either rhDNase at two different concentrations (final concentration 2 or 4 micrograms.mL-1) or placebo. The median mucociliary transport rate was significantly improved by rhDNase from 0.68 with placebo to 0.79 and 0.83 with 2 and 4 micrograms.mL-1 of rhDNase, respectively. A significant improvement in mucus cough transport was also induced by rhDNase from 25.5 mm with placebo to 27.0 mm with either 2 or 4 micrograms.mL-1 rhDNase. These improvements in mucus transport capacity were associated with alterations in the physical properties of the mucus. The mucus median control viscosity (511.4 Pa.s) and median contact angle (0.85 rd) significantly decreased to 112.5 Pa.s and 0.74 rd, respectively, in the presence of 4 micrograms.mL-1 of rhDNase. These findings demonstrate that recombinant deoxyribonuclease may exert a beneficial effect on mucus clearance in vitro by altering the viscosity and surface properties of the purulent chronic bronchitic sputum samples.

Predictors of survival in patients receiving domiciliary oxygen therapy or mechanical ventilation. A 10-year analysis of ANTADIR Observatory.

STUDY OBJECTIVE: To analyze predictors of survival for patients receiving home long-term oxygen therapy (LTOT) or prolonged mechanical ventilation (PMV) according to the cause of chronic respiratory insufficiency (CRI) and the patients physiologic data. DESIGN: Analysis of a nationwide database (ANTADIR Observatory). SETTING: The national nonprofit network for home treatment of patients with CRI Association Nationale pour le Traitement a Domicile de lInsuffisance Respiratoire Chronique (ANTADIR); founded in France in the 1980s. PATIENTS: There were 26,140 patients receiving LTOT or PMV (noninvasive or via tracheostomy) between January 1, 1984 and January 1, 1993 (chronic bronchitis, 12,043; asthma, 1,755; bronchiectasis, 1,556; emphysema, 551; tuberculosis sequelae, 4,147; kyphoscoliosis, 1,574; neuromuscular diseases, 1,097; pneumoconiosis, 919; and fibrosis, 2,498. MEASUREMENTS AND RESULTS: Survival analysis was performed using the actuarial and the Cox's semiparametric model. The mean survival for patients with chronic bronchitis is 3 years. Survival is slightly better for patients with bronchiectasis and asthma and worse for those with emphysema. Patients with kyphoscoliosis and a neuromuscular disease have the longest survival (8 and 6.5 years, respectively). Patients with CRI due to tuberculosis sequelae experience the same survival as COPD patients (3 years). Prognosis is the worst in patients with pneumoconiosis or fibrosis: 50% of these patients die during the year following the beginning of home treatment. The association of an obstructive lung disease worsens the prognosis of patients with kyphoscoliosis or neuromuscular disease and tends to bring the survival rate of the patients with pneumoconiosis or fibrosis closer to that of COPD patients. In COPD, male sex, older age, lower body mass index (BMI),FEV1 percent predicted,PaO2,and PaCO2 are independent negative prognostic factors. For tuberculous sequelae and kyphoscoliosis, female sex, younger age, a high BMI, PaO2 and PaCO2 (and for kyphoscoliosis a higher FEV1/vital capacity [VC] ratio) are all independent favorable prognostic factors. In pulmonary fibrosis, a lower PaO2 and PaCO2 values, a lower VC percent predicted, and a higher FEV1/VC ratio are negative prognostic factors. CONCLUSIONS: The ANTADIR Observatory allows a unique opportunity to analyze long-term survival of a large population with CRI treated at home.