Training, detraining, and retraining: Two 12-week respiratory muscle training regimens in a child with infantile-onset Pompe disease.

BACKGROUND: Respiratory muscle weakness is a primary cause of morbidity and mortality in patients with Pompe disease. We previously described the effects of our 12-week respiratory muscle training (RMT) regimen in 8 adults with late-onset Pompe disease [1] and 2 children with infantile-onset Pompe disease [2]. CASE REPORT: Here we describe repeat enrollment by one of the pediatric participants who completed a second 12-week RMT regimen after 7 months of detraining. We investigated the effects of two 12-week RMT regimens (RMT #1, RMT #2) using a single-participant A-B-A experimental design. Primary outcome measures were maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP). Effect sizes for changes in MIP and MEP were determined using Cohen's d statistic. Exploratory outcomes targeted motor function. RELEVANCE: From pretest to posttest, RMT #2 was associated with a 25% increase in MIP and a 22% increase in MEP, corresponding with very large effect sizes (d= 2.92 and d= 2.65, respectively). Following two 12-week RMT regimens over 16 months, MIP increased by 69% and MEP increased by 97%, corresponding with very large effect sizes (d= 3.57 and d= 5.10, respectively). MIP and MEP were largely stable over 7 months of detraining between regimens. Magnitude of change was greater for RMT #1 relative to RMT #2.

Soporte ventilatorio no invasivo en insuficiencia ventilatoria aguda severa en adolescentes con enfermedad neuromuscular y obesidad: estudio de casos / Non-invasive ventilatory support in severe acute ventilatory failure in adolescents with neuromuscular disease and obesity: study of cases

Noninvasive ventilatory support (NIVS) combined with mechanical cough assist (MI-E) is an effective tool to treat patients with acute ventilatory failure due to neuromuscular disorders (NMD). Airway respiratory infection could be lethal or with risk of endotraqueal intubation, especially when vital capacity (VC) is less than 15 ml/k. We report 2 obese adolescents, aged 11 and 14 years old, with myasthenic crisis (MC) and Duchenne muscular dystrophy (DMD). The last one with a severe cifoescoliosis treated with nocturnal noninvasive ventilation at home. MC girl has been treated with pyridostigmine, prednisolone and mycophenolate. They were admitted for thymectomy and spinal surgery arthrodesis respectively. After admission they developed airway respiratory infection triggering by Methaneumovirus and were treated with oxygen therapy, non-invasive ventilation with low-pressure support and EV immunoglobulin for the MC girl. After 48 h both patients developed severe respiratory failure, Sa/FiO2 < 200, atelectasis of lower lobes and difficulty to swallow, a peak cough flow (PFT)

El soporte ventilatorio no invasivo (SVNI) y la rehabilitación respiratoria con apilamiento de aire más tos asistida manual o mecánica, son efectivas para tratar la insuficiencia ventilatoria aguda en pacientes con enfermedades neuromusculares (ENM) y deterioro progresivo de la bomba respiratoria. Las agudizaciones gatilladas por infecciones respiratorias causan insuficiencia ventilatoria aguda potencialmente mortal y con alto riesgo de intubación, en especial cuando la capacidad vital (CV) es < de 15ml/k. Se reportan 2 adolescentes obesos con ENM de 11 y 14 años con miastenia gravis y distrofia muscular de Duchenne (DMD) con asistencia ventilatoria no invasiva nocturna con baja presión de soporte (AVNI), ingresados para timectomía y artrodesis de columna respectivamente. Una vez ingresados evolucionan con insuficiencia ventilatoria aguda secundaria a una infección respiratoria por Metaneumovirus. Inicialmente fueron manejados con oxigenoterapia, AVNI y gamaglobulina endovenosa en el caso de la paciente con crisis miasténica (CM). A las 48h presentan dificultad respiratoria severa, Sa/FiO2 < 200, atelectasias bibasales y disfagia, CV de 800ml (11ml/k) en el paciente con CM y de 200 ml (2,5ml/k) en el paciente con DMD y un pico flujo tosido (PFT) < 100 l/m. Se cambia a SVNI con equipo Trilogy® y BipapA40®, en modalidad S/T (espontáneo/tiempo) y AVAPS (volumen promedio asegurado en presión de soporte) con altos parámetros ventilatorios; suspendiendo rápidamente la oxigenoterapia, al combinar tos mecánicamente asistida con in-exsufflator (MI-E) en forma intensiva. Ambos pacientes presentan mejoría clínica sostenida, de la CV, PFT y pico flujo exuflado máximo con MI-E (PFE-MI-E). El SVNI más la aplicación sistemática del MI-E hasta lograr SaO2 de al menos 95% con oxígeno ambiental evita la intubación endotraqueal en ENM, a diferencia del agravamiento producido por AVNI y oxigenoterapia con criterios clásicos.

Efficacy of low molecular weight heparin for chronic obstructive pulmonary disease and respiratory failure: A protocol of systematic review of randomized controlled trials.

BACKGROUND: Evaluating the efficacy and safety of low molecular weight heparin (LMWH) for patients with chronic obstructive pulmonary disease (COPD) and respiratory failure (RF) is a major purpose of this study. METHODS: The following electronic databases will be comprehensively retrieved from the inception to July 1, 2019: Cochrane Library, PUBMED, EMBASE, Google Scholar, Web of Science, Allied and Complementary Medicine Database, WANGFANG, and China National Knowledge Infrastructure without language restrictions. All randomized controlled trials related to LMWH for COPD and RF will be included. Two authors will carry out study selection, data collection, and risk of bias assessment independently. RESULTS: This study will systematically explore the efficacy and safety of LMWH for COPD and RF. The primary outcome is lung function. The secondary outcomes are severity of dyspnea on exertion, quality of life, body mass index, airflow obstruction; and any expected and unexpected adverse events. CONCLUSION: The findings of this study will provide evidence to judge whether LMWH is an effective treatment for patients with COPD and RF. PROSPERO REGISTRATION NUMBER: PROSPERO CRD42019 139631.

Thyrotoxic periodic paralysis complicated by life-threatening acute hypercapnic respiratory failure in a Chinese male with painless thyroiditis.

CONTEXT: Thyrotoxic periodic paralysis (TPP) is a relatively common complication seen in Asian hyperthyroid patients. However, it is a rare occurrence to find a TPP case comprised of acute hypercapnic respiratory failure in patients with painless thyroiditis. PATIENT: A 29-year-old Chinese man presented with flaccid paralysis of all four limbs and he was brought to emergency room. Severe hypokalemia was found on admission. Although treatment had been initiated with potassium chloride supplementation, he went on to develop acute hypercapnic respiratory failure likely due to muscle fatigue. The patient was intubated for mechanical ventilatory support. Once his serum potassium levels were normalized, he was able to be weaned off ventilator support. Thyroid function tests showed elevated free thyroxine concentration and low thyroid-stimulating hormone concentration. He underwent a thyroid uptake scan with 131I which revealed decreased uptake rate of thyroid area. Based on the patient's clinical presentation and associated findings, we diagnosed him with TPP due to painless thyroiditis. We have reviewed TPP cases caused by painless thyroiditis and TPP cases associated with acute hypercapnic respiratory failure. CONCLUSION: It is important to note that potentially fatal complications such as acute hypercapnic respiratory failure might occur in acute attacks of TPP even in cases of TPP due to painless thyroiditis.

Cutaneous phaeohyphomycosis in a hematopoietic stem cell transplant patient caused by Alternaria rosae: First case report.

Alternaria species have been reported as a rare cause of fungal infection in organ and stem cell transplant recipients, but to date, no reports have been published of infection in humans caused by Alternaria rosae. Here, we report cutaneous A. rosae infection in a 66-year-old farmer with a history of primary myelofibrosis who had undergone allogeneic unrelated donor hematopoietic stem cell transplantation. Forty-nine days post transplant, he presented with a nodule on the thumb with no findings suggestive of disseminated infection. Pathology, culture, and molecular speciation showed the nodule was caused by cutaneous A. rosae. He had been on voriconazole as antifungal prophylaxis, but was found to have a subtherapeutic voriconazole level. He was switched to posaconazole based on published in vitro data showing its superior efficacy in Alternaria treatment. Susceptibility testing showed that the A. rosae isolate was indeed susceptible to posaconazole. His cutaneous lesion remained stable, but he died from respiratory failure secondary to lobar pneumonia. At lung autopsy, A. rosae was not identified in the lungs. We believe this to be the first published report, to our knowledge, of A. rosae infection in humans.

Lethal hypophosphatasia successfully treated with enzyme replacement from day 1 after birth.

UNLABELLED: Hypophosphatasia (HPP) is a rare metabolic bone disease caused by loss-of-function mutations in the gene ALPL encoding the tissue nonspecific alkaline phosphatase (TNSALP). There is a broad range of severity in the phenotype of HPP, and the most severe form exhibits perinatal lethality without mineralization of the skeleton. Here, we describe a female infant with perinatal lethal HPP diagnosed in utero. She was treated with a recombinant ALP (asfotase alfa) as an enzyme replacement therapy (ERT), which started from 1 day after birth. She required invasive ventilation immediately upon birth and demonstrated severe hypomineralization of whole body bone. Severe respiratory insufficiency was controlled by intensive respiratory care with high-frequency oscillation ventilation and nitric oxide inhalation and deep sedation just after birth. Bone mineralization improved with treatment; improvements were visible by 3 weeks of age and continued with treatment. Serum calcium levels decreased following treatment, resulting in hypocalcemia and convulsion, and calcium supplementation was required until 3 months of treatment. She was weaned from mechanical ventilation and has now survived more than 1 year. CONCLUSION: This case demonstrates the success of ERT in treating the severest HPP and highlights the importance of early diagnosis and intervention for these patients.

Effects of early inhaled nitric oxide therapy and vitamin A supplementation on the risk for bronchopulmonary dysplasia in premature newborns with respiratory failure.

OBJECTIVE: To assess whether the combination of early inhaled nitric oxide (iNO) therapy and vitamin A supplementation lowers the incidence of bronchopulmonary dysplasia (BPD) in premature newborns with respiratory failure. STUDY DESIGN: A total of 793 mechanically ventilated infants (birth weight 500-1250 g) were randomized (after stratification by birth weight) to receive placebo or iNO (5 ppm) for 21 days or until extubation (500-749, 750-999, or 1000-1250 g). A total of 398 newborns received iNO, and of these, 118 (30%) received vitamin A according to their enrollment center. We compared patients who received iNO + vitamin A with those who received iNO alone. The primary outcome was a composite of death or BPD at 36 weeks postconceptual age. RESULTS: BPD was reduced in infants who received iNO + vitamin A for the 750-999 g birth weight group compared with iNO alone (P = .01). This group also showed a reduction in the combined outcome of BPD + death compared with iNO alone (P = .01). The use of vitamin A did not change the risk for BPD in the placebo group. Overall, the use of vitamin A was low (229 of 793 patients, or 29%). Combined therapy improved Bayley Scales of Infant Development II Mental and Psychomotor Developmental Index scores at 1 year compared with infants treated solely with iNO for the 500-749 g birth weight group. CONCLUSIONS: In this retrospective analysis of the nonrandomized use of vitamin A, combined iNO + vitamin A therapy in preterm infants with birth weight 750-999 g reduced the incidence of BPD and BPD + death and improved neurocognitive outcomes at 1 year in the 500-749 g birth weight group.

Pulmonary sequelae of severe H1N1 infection treated with high frequency oscillatory ventilation.

During the recent influenza A (H1N1) pandemic, due to severe respiratory failure many patients required treatment with alternative ventilator modalities including High Frequency Oscillatory Ventilation (HFOV). We present four such patients treated with HFOV at an academic, tertiary referral hospital in Ireland. We detail outcomes of clinical examination, pulmonary function testing, quality of life assessment and radiographic appearance on CT Thorax at follow-up at 6 months. Further clinical assessment and pulmonary function testing were performed at median 19 months (range 18-21 months) post-discharge. At initial review all patients were found to have reduced gas transfer (median predicted DLCO 74%) with preservation of lung volumes and normal spirometrical values at 6 months (median FVC 5.42L [101% predicted] and FEV14.5L [101.2% predicted] respectively), with improvements in gas transfer (median predicted DLCO 83%)at subsequent testing. Post-inflammatory changes on CT thorax at 6 months were seen in all 4 cases. To our knowledge this is the first report to document the long-term effects of severe H1N1 infection requiring high frequency oscillation on respiratory function. We conclude that the effects on respiratory function and pulmonary radiological appearance are similar to those observed following conventional treatment of Acute Respiratory Distress Syndrome [ARDS].

Effect of therapeutic massage on insomnia and climacteric symptoms in postmenopausal women.

INTRODUCTION: Physiological and psychological alterations in the climacteric period frequently influence women's quality of life. Hot flushes, nocturia, mood alterations, respiratory disturbances, insomnia and restless leg syndrome all affect sleep, and the altered hormonal state in this period impacts the aging process. As hormonal therapy is not indicated in some cases, the search for complementary therapies, such as massage therapy, to improve insomnia in the climacteric period is increasing. OBJECTIVE: To evaluate the effect of therapeutic massage on insomnia and climacteric symptoms in postmenopausal women. METHODS: Forty-four volunteers were randomly distributed into three groups: therapeutic massage (TM), passive movement (PM) and control (CTL). The women received 32 therapeutic massage sessions and passive movement twice a week. Questionnaires were given in the pre-trial and the 16th and 32nd sessions. The Insomnia Severity Index (ISI), Beck Anxiety Inventory (BAI), Beck Depression Inventory (BDI), Menopause Quality of Life questionnaire (MENQOL), Kupperman Menopausal Index and Lipp Symptoms of Stress Inventory were assessed. In addition, the women underwent polysomnography at baseline and post-treatment. Statistical analyses were calculated using Friedman and Wilcoxon non-parametric tests. The level of significance was fixed at p ≤ 0.05. RESULTS: There was an improvement in ISI in the TM group (p = 0.000) and in the PM group (p = 0.001). A decrease in the BDI occurred in the TM group (p = 0.004), and the MENQOL improved in the TM group (p = 0.015). Furthermore, there were no significant differences in polysomnography parameters in the TM group, with only an increase in minimal saturation (p = 0.053). CONCLUSION: The TM group exhibited improved subjective data considering the changes in symptoms according to the ISI and the MENQOL and a decrease in symptoms according to the BDI.

Successful treatment of septic shock and respiratory failure due to leptospirosis and scrub typhus coinfection with penicillin, levofloxacin, and activated protein C.

Leptospirosis and scrub typhus are common zoonoses and coinfection with both diseases has been reported sporadically, particularly in tropical and subtropical areas. A 53-year-old male presented with acute hypoxemic respiratory failure and septic shock due to leptospirosis and scrub typhus coinfection confirmed by serological assessments. Antibiotics, including intravenous penicillin and levofloxacin, were administered and human recombinant activated protein C was added because of a high risk of death due to septic shock with multiple organ failure. The patient's hemodynamics and hypoxemia substantially improved 4 days later and he had a complete recovery from the disease after 10 days of hospitalization. Coinfection of leptospirosis and scrub typhus may easily go unrecognized by physicians in febrile travelers or patients in the region where both diseases are endemic. In severe and critical cases of leptospirosis, scrub typhus, or coinfection with both, the use of APC in addition to appropriate antibiotic treatment and standard critical care might provide a greater chance for survival and a favorable outcome.

Multimodal nutritional rehabilitation improves clinical outcomes of malnourished patients with chronic respiratory failure: a randomised controlled trial.

BACKGROUND: In chronic respiratory failure (CRF), body composition strongly predicts survival. METHODS: A prospective randomised controlled trial was undertaken in malnourished patients with CRF to evaluate the effects of 3 months of home rehabilitation on body functioning and composition. 122 patients with CRF on long-term oxygen therapy and/or non-invasive ventilation (mean (SD) age 66 (10) years, 91 men) were included from eight respiratory units; 62 were assigned to home health education (controls) and 60 to multimodal nutritional rehabilitation combining health education, oral nutritional supplements, exercise and oral testosterone for 90 days. The primary endpoint was exercise tolerance assessed by the 6-min walking test (6MWT). Secondary endpoints were body composition, quality of life after 3 months and 15-month survival. RESULTS: Mean (SD) baseline arterial oxygen tension was 7.7 (1.2) kPa, forced expiratory volume in 1 s 31 (13)% predicted, body mass index (BMI) 21.5 (3.9) kg/m2 and fat-free mass index (FFMI) 15.5 (2.4) kg/m2. The intervention had no significant effect on 6MWT. Improvements (treatment effect) were seen in BMI (+0.56 kg/m2, 95% CI 0.18 to 0.95, p=0.004), FFMI (+0.60 kg/m2, 95% CI 0.15 to 1.05, p=0.01), haemoglobin (+9.1 g/l, 95% CI 2.5 to 15.7, p=0.008), peak workload (+7.2 W, 95% CI 3.7 to 10.6, p<0.001), quadriceps isometric force (+28.3 N, 95% CI 7.2 to 49.3, p=0.009), endurance time (+5.9 min, 95% CI 3.1 to 8.8, p<0.001) and, in women, Chronic Respiratory Questionnaire (+16.5 units, 95% CI 5.3 to 27.7, p=0.006). In a multivariate Cox analysis, only rehabilitation in a per-protocol analysis predicted survival (HR 0.27, 95% CI 0.07 to 0.95, p=0.042). CONCLUSIONS: Multimodal nutritional rehabilitation aimed at improving body composition increased exercise tolerance, quality of life in women and survival in compliant patients, supporting its incorporation in the treatment of malnourished patients with CRF. Clinical Trial number NCT00230984.

Hyperoxia sensing: from molecular mechanisms to significance in disease.

Oxygen therapy using mechanical ventilation with hyperoxia is necessary to treat patients with respiratory failure and distress. However, prolonged exposure to hyperoxia leads to the generation of excessive reactive oxygen species (ROS), causing cellular damage and multiple organ dysfunctions. As the lungs are directly exposed, hyperoxia can cause both acute and chronic inflammatory lung injury and compromise innate immunity. ROS may contribute to pulmonary oxygen toxicity by overwhelming redox homeostasis, altering signaling cascades that affect cell fate, ultimately leading to hyperoxia-induced acute lung injury (HALI). HALI is characterized by pronounced inflammatory responses with leukocyte infiltration, injury, and death of pulmonary cells, including epithelia, endothelia, and macrophages. Under hyperoxic conditions, ROS mediate both direct and indirect modulation of signaling molecules such as protein kinases, transcription factors, receptors, and pro- and anti-apoptotic factors. The focus of this review is to elaborate on hyperoxia-activated key sensing molecules and current understanding of their signaling mechanisms in HALI. A better understanding of the signaling pathways leading to HALI may provide valuable insights on its pathogenesis and may help in designing more effective therapeutic approaches.

Perry syndrome due to the DCTN1 G71R mutation: a distinctive levodopa responsive disorder with behavioral syndrome, vertical gaze palsy, and respiratory failure.

Perry syndrome is a rare form of autosomal dominant Parkinsonism with respiratory failure recently defined as being due to mutations in the DCTN1 gene. We describe a new family carrying a G71R mutation in the DCTN1 gene. The proband displayed a series of distinctive features not previously described in Perry syndrome: a disorder of vertical downward saccades accompanied by progressive midbrain atrophy, predominant nonmotor symptoms responsive to levodopa, distinctive craniocervical levodopa induced dyskinesias, and a good response to high-dose levodopa therapy and respiratory support. The family was initially thought to have autosomal dominant behavioral variant frontotemporal dementia with Parkinsonism. This report expands the clinical definition of this distinctive syndrome.

In vitro evaluation of an active heat-and-moisture exchanger: the Hygrovent Gold.

BACKGROUND: To improve the heat and humidification that can be achieved with a heat-and-moisture exchanger (HME), a hybrid active (ie, adds heat and water) HME, the Hygrovent Gold, was developed. We evaluated in vitro the performance of the Hygrovent Gold. METHODS: We tested the Hygrovent Gold (with and without its supplemental heat and moisture options activated), the Hygrobac, and the Hygrovent S. We measured the absolute humidity, using a test lung ventilated at minute volumes of 5, 10, and 15 L/min, in normothermic (expired temperature 34 degrees C) and hypothermic (expired temperature 28 degrees C) conditions. We also measured the HMEs' flow resistance and weight after 24 h and 48 h. RESULTS: In its active mode the Hygrovent Gold provided the highest absolute humidity, independent of minute volume, in both normothermia and hypothermia. The respective normothermia and hypothermia absolute humidity values at 10 L/min were 36.3 + 1.3 mg/L and 27.1 + 1.0 mg/L with the active Hygrovent Gold, 33.9 + 0.5 mg/L and 24.2 + 0.8 mg/L with the passive Hygrovent Gold, 33.8 + 0.56 mg/L and 24.4 + 0.4 mg/L with the Hygrobac, and 33.9 + 0.8 mg/L and 24.6 + 0.6 mg/L with the Hygrovent S. The efficiency of the tested HMEs did not change over time. At 24 h and 48 h the increase in weight and flow resistance was highest in the active Hygrovent Gold. CONCLUSIONS: The passive Hygrovent Gold provided adequate heat and moisture in normothermia, but the active Hygrovent Gold provided the highest humidity, in both normothermia and hypothermia.

Association of hypophosphatemia with failure-to-wean from mechanical ventilation.

Severe hypophosphatemia is known to be associated with respiratory failure, but there are few studies that specifically examine the relationship between serum phosphorus concentration and failure to wean patients from mechanical ventilation. This study investigated the association between hypophosphatemia and weaning failure in patients in two medical intensive care units (ICU). The study was conducted in a prospectively developed cohort of 66 patients being treated with ventilatory support and in whom 193 weaning trials were attempted. Ultimately, all 66 subjects were successfully weaned. A cross-sectional analysis was conducted on serum phosphorus levels and success or failure to wean the patients from ventilators. At the time of the successful weaning attempts (n = 66), the subjects' serum phosphorus concentrations (mean +/- SD) were 1.18 +/- 0.27 mmol/L, whereas at all failed weaning attempts (n = 127) serum phosphorus concentrations averaged 1.06 +/- 0.31 mmol/L (p = 0.008). Subjects with phosphorus concentrations below the reference interval (RI) in our laboratory (<0.80 mmol/L) had greater risk for weaning failure compared to subjects with phosphorus concentrations at or above the RI (relative risk = 1.18; 95% confidence interval = 1.06 to 1.32; p = 0.01). Serum calcium concentrations were not significantly different at the time of successful weaning compared to those at failed weaning attempts. This study indicates that there is an association between hypophosphatemia and failure-to-wean from mechanical ventilation in ICU patients on ventilatory support.

Proton pump inhibitor suppression of calcium absorption presenting as respiratory distress in a patient with bilateral laryngeal paralysis and hypocalcemia.

We report an unusual case of hypocalcemia and respiratory distress related to acid-suppressive therapy. The patient was a 50-year-old woman with bilateral laryngeal paralysis and hypoparythyroidism resulting from a thyroidectomy performed more than 30 years previously. She required large doses of calcium supplementation to maintain a normal calcium level. Her airway had been marginally adequate. A few weeks prior to presentation, she began to experience increasing dyspnea. Examination was suggestive of laryngopharyngeal reflux, and she was started on a therapeutic trial of esomeprazole 40 mg twice daily. Three days later, she presented to the emergency room with airway distress. Laboratory studies indicated that the patient had hypocalcemia. The esomeprazole was discontinued, and she was treated with intravenous calcium; her symptoms resolved. We attribute the airway distress to tetany in synkinetically reinnervated laryngeal adductor muscles. We recommend that acid-suppressive therapy should be used with caution in patients with hypoparathyroidism or hypocalcemia.

[Effects of home pulmonary rehabilitation in patients with chronic respiratory failure and nutritional depletion]. / IRAD2: Insuffisant respiratoire à domicile 2 (2e étude). Effets d'une réhabilitation à domicile chez l'insuffisant respiratoire chronique dénutri.

STATE OF THE ART: The IRAD2 trial is evaluating a 3-month home intervention which includes education, oral supplements, exercise and androgenic steroids in undernourished patients with chronic respiratory failure. The main objective is to increase the six-minute walking distance by more than 50 m with an improvement in health-related quality-of-life. Secondary end-points include a reduction in exacerbation rates by 25%, a reduction in health-related costs and an increase in survival during the year following intervention. MATERIAL AND METHODS: This interventional, multi-centre, prospective, two-armed parallel, controlled trial is being conducted in 200 patients. In both groups, "Control" and "Rehabilitation", 7 home visits are scheduled during the 3-month intervention for education purpose. In the "Rehabilitation" group, patients will receive 160 mg/d of oral testosterone undecanoate in men, 80 mg/d in women, oral dietary supplements (563 kcal/d) and exercises on an ergometric bicycle 3 to 5 times a week. EXPECTED RESULTS: In the event of significant responses to intervention, this trial would validate a comprehensive and global home-care for undernourished patients with chronic respiratory failure combining therapeutic education, oral supplements, androgenic substitution and physical activity.