Severe Chronic Respiratory Failure Research Articles

Anesthesia experience in an adult Silver-Russell syndrome: a case report

BackgroundThere are no reports of anesthesia use in adult patients with Silver-Russell syndrome (SRS). Here, we report our experience with anesthesia in an adult patient with SRS complicated by chronic respiratory failure.Case presentationA 33-year-old woman was clinically diagnosed with SRS. She had severe chronic respiratory failure, complicated by superior mesenteric artery syndrome. Percutaneous gastrostomy was scheduled for nutritional management under epidural anesthesia; however, soon after esophagogastroduodenoscopy was started, she lost consciousness and spontaneous respiration. The patient was urgently intubated and converted to general anesthesia. The end-tidal carbon dioxide tension was > 90 mmHg at intubation.ConclusionsAdult SRS patients with chronic respiratory failure have a risk of CO2 narcosis. SRS also requires preparation for difficult airway management during the perioperative period.

High-intensity non-invasive ventilation during exercise-training versus without in people with very severe COPD and chronic hypercapnic respiratory failure: a randomised controlled trial

BackgroundPeople with very severe chronic obstructive pulmonary disease (COPD) using nocturnal non-invasive ventilation (NIV) for chronic hypercapnic respiratory failure (CHRF) experience reduced exercise capacity and severe dyspnoea during exercise training...

P300 AORN A. CARDARELLI NAPOLI/ FEDERICO II UNIVERSITY INTERHOSPITAL PROTOCOL: TAVI IN A COMPLEX SCENARIO

Abstract Rational: In order to provide the best treatment for patients affected by severe aortic stenosis at intermediate–high surgical risk, since more than 3 years an interhospital protocol beetween the Cardiology, UTIC and Cath–lab department of AORN A. Cardarelli of Naples, a center without cardiac surgery, and the cath–lab of the department of Cardiovascular Emergencies, Clinical Medicine and Ageing Medicine of AOU Federico II, is active. The agreement provides the carrying out of a TAVI procedures for patients hospitalized at the A. Cardarelli, by transfering them with a resuscitation ambulance to the Federico II cath–lab, and subsequent return to the department of origin after the execution of the interventional procedure. Case report: 85 year old lady with hypertension, moderate–severe chronic respiratory failure and atrial fibrillation treated with NOAC, is admitted in the emergency room for acute pulmonary edema and contextual diagnosis of severe aortic stenosis is made. After clinical stabilization, the TAVI program is started: both an angio–CT scan and a coronary angiography are carried out, with the detection of a critical calcified stenosis involving left main branch and proximal LAD and a chronic occlusion of Cx branch. Multidisciplinary evaluation and collegial evaluation with the colleagues from the Federico II University are carried out, and indication is given to TAVI with contextual PTCA of LM and LAD critical stenosis. A joint procedure of PTCA with 2 DES and TAVI with Evolute R 29 valve is then carried out in a single session at the Federico II University of Naples and after post–procedural observation, the patient is transferred back to the UTIC of A. Cardarelli for hospitalization. During the hospital stay, no procedural complications are reported and the patient is discharged 4 days after in good clinical conditions; a triple therapy with NOAC and DAPT is started, continuing it for 1 month with subsequent suspension of ASA and carrying on therapy with NOAC + Clopidogrel. At 1 and 6 months clinical follow–up, the patient is stable and well compensated, without significant clinical complications and with NYHA class 2. Conclusions The results of these years–experience, highlight the feasibility and safety of this strategy, making it possible to plan and carry out TAVI procedures even in centers that do not have onsite cardiac surgery, considering the growing indications for TAVI and the subsequent increase of patients who need this procedure

Генетические дисфункции системы сурфактанта у детей: результаты многоцентрового исследования

Aim: Genetic, clinical, laboratory-instrumental and morphological characteristics of genetic dysfunctions of the surfactant system in children, therapy and outcomes of the disease. Design: Multicentre, ambispective, open-label, descriptive pilot longitudinal study. Materials and methods. We observed 17 children from 16 families with identified mutations in the SFTPC, ABCA3, NKX2-1 genes. Methods used: genealogical, Sanger sequencing, clinical exome sequencing, computed tomography and histological examination of the lungs. Results. The study included 8 children with congenital deficiency of surfactant protein C, 8 children with brain-lung-thyroid syndrome and 1 patient with congenital deficiency of protein ABSA3. Based on the results of a genetic examination of patients, nucleotide variants c.218T>C were identified in 2 out of 8 patients with a mutation in the SFTPC gene, which is the most common according to the literature. In 5 children, the mutations were hereditary. Congenital deficiency of surfactant protein C, ABCA3 protein and brain-lung-thyroid syndrome were characterized by clinical, computed tomography, and morphological signs of interstitial lung disease. Despite complex respiratory, anti-inflammatory therapy, the frequency of deaths in congenital deficiency of surfactant protein C was 37.5%. Conclusion. Children with severe respiratory distress syndrome of newborns, interstitial lung disease with the development of severe chronic respiratory failure, burdened with a family history should undergo genetic testing to detect mutations in the genes SFTPB, SFTPC, ABCA3. The patient's combination of respiratory symptoms with congenital hypothyroidism and neurological pathology is the basis for genetic examination for NKX2-1 gene mutations to exclude the brain-lung-thyroid syndrome. Keywords: genetic dysfunctions of the surfactant system, congenital deficiency of surfactant protein C, congenital deficiency of ABCA3 protein, brain-lung-thyroid syndrome, NKX2-1 gene, children.

0802 Central Sleep Apnea as a Result of Cerebral Cavernous Malformation Hemorrhage in the Pediatric Population

Abstract Introduction Cerebral Cavernous malformations (CCMs) consist of a collection of capillaries in the central nervous system (CNS) that are enlarged and irregular in structure. Patients with CCMs are at increased risk of hemorrhage into the brain or spinal cord, resulting in seizures, focal neurologic deficits, hydrocephalus, and death. Given the importance of the brainstem and central chemoreceptors in regulating respiratory function, rupture of CCMs can also lead to dysregulation of breathing. We present a series of 3 pediatric patients who have a diagnosis of central sleep apnea (CSA) due to rupture of CCMs managed with home ventilators with significant differences in clinical presentation from mild CSA to night time respiratory support for severe CSA and chronic respiratory failure requiring continuous mechanical ventilation. Report of Cases: Subject 1 is a 13 yo M with a history of ruptured posterior fossa arteriovenous malformation (AVM) complicated by post-hemorrhagic hydrocephalus, right hemiparesis and severe central apnea requiring nighttime mechanical ventilation via tracheostomy. Subject 2 is a 19 yo M with a history of mid-pontine cavernoma with multiple episodes of hemorrhage failing surgical resection resulting in right hemiparesis, severe central apnea and hypoventilation requiring continuous mechanical ventilator support via tracheostomy. Subject 3 is a 6 yo F with a history of thoracic cavernous hemangioma resulting in spinal cord injury and mild central sleep apnea requiring mask ventilation via home ventilator during sleep. All of these patients experienced loss of respiratory drive as a result of complications from brainstem or spinal cord lesions with varying degrees of ventilator support requirement and clinical presentation. Conclusion Due to the propensity for CNS bleeds among patients with CCM they are at increased risk of respiratory compromise. Home ventilator support can be used effectively to treat central apneas and chronic respiratory failure but this is a moving paradigm as subsequent bleeds worsen respiratory compromise. Support (If Any) Support (if any):

Anticoagulants in the treatment of superficial vein thrombosis of lower limbs: possibilities and limitations

This article presents a critical analysis of the results of three prospective randomized controlled trials of anticoagulant use in patients with superficial vein thrombosis of lower limbs CALISTO using fondaparinux sodium, STEFLUX using different doses of parnaparin sodium, and SURPRISE comparing the effectiveness and safety of fondaparinux sodium and the preventive dose of the oral anticoagulant rivaroxaban.Evidence from these clinical trials suggests that low-dose anticoagulants should be used for at least 1.5 months in superficial vein thrombosis of lower limbs with thrombus localization beyond 3 cm from the saphenofemoral and/or saphenopopliteal junction. However, anticoagulant treatment does not appear to prevent the progression of venous thrombosis after withdrawal, so in patients with persisting risk factors, it is reasonable to consider extended treatment. This seems to apply primarily to patients with superficial thrombophlebitis a history of superficial or deep venous thrombosis in the lower extremities, a family history of venous thromboembolic complications, and possibly a broader contingent of high-risk patients, not represented in the CALISTO and STEFLUX studies (with active malignancy, ongoing anticancer therapy, severe chronic heart or respiratory failure, inability to withdraw hormone replacement therapy, autoimmune disease). According to the accumulated evidence, subcutaneous injections of fondaparinux sodium, intermediate doses of low molecular weight heparin, and direct oral anticoagulant rivaroxaban in the dose of 10 mg once a day are effective and sufficiently safe in the treatment of superficial vein thrombosis of lower limbs with localization beyond 3 cm from the saphenofemoral and/or saphenopopliteal junction. Questions about the feasibility of increasing the doses of anticoagulants to further reduce the rate of superficial thrombophlebitis - progression during treatment and the optimal duration of their use remain open.

Regional Anesthesia for Abdominal Surgery in a Patient with Severe Chronic Respiratory Failure: A Case Report

General anesthesia is associated with a risk for postoperative pulmonary complications. The risk is even higher in patients with chronic respiratory failure, and postoperative mortality rates are high. Proper perioperative anesthetic management is important in such patients. Therefore, it is essential to optimize the patient’s physical status before anesthesia and to determine the optimal anesthesia technique based on the pre-anesthesia evaluation of the patient’s pulmonary function. We successfully performed abdominal surgery under spinal anesthesia in a patient with severe chronic respiratory failure.

WILLIAMS-CAMPBELL SYNDROME: A RARE CAUSE OF CYSTIC LUNG DISEASE

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Williams-Campbell syndrome (WCS), also known as bronchomalacia, is a rare form of bronchiectasis that develops due to markedly diminished or absent cartilage around the sub-segmental bronchi, resulting in dilation of the subsequent bronchi. Affected patients will have a normal trachea and central bronchi caliber with cystic bronchiectasis affecting the fourth to sixth-order bronchi. Although WCS has been best described in children with recurrent pneumonia and broncho-obstructive symptoms, there have been recent descriptions in adults due to delayed diagnosis [1]. CASE PRESENTATION: A 69-year-old non-smoking female with a history of severe bronchiectasis and chronic hypoxemic respiratory failure presented with complaints of progressive dyspnea and productive cough. Her respiratory symptoms started about 20 years ago with no clear inciting etiology and have gradually worsened. Chest x-ray (CXR) at that time showed multiple areas of cystic degeneration (Fig. 1). She was transferred to us after being diagnosed with acute on chronic hypoxic respiratory failure from rhinovirus infection. Physical examination revealed bilateral coarse crackles with rhonchi. Arterial blood gas showed hypoxic and hypercapnic respiratory failure. CXR was significant for cystic changes with bilateral pulmonary infiltrates and a small right-sided effusion. Computed tomographic (CT) scan of the chest showed cystic lesions with patchy infiltrates suggestive of bronchopneumonia (Fig. 2). On hospital day 2, she developed copious hemoptysis requiring transfusion of packed red blood cells. Her respiratory distress was managed conservatively with non-invasive mechanical ventilation. Bronchoscopy was not pursued due to her DNI status and an increased risk of ventilator dependence after the procedure. When her overall clinical condition improved after steroid and antibiotic treatment, she was discharged with home health therapy. DISCUSSION: WCS is a rare cause of congenital bronchiectasis usually diagnosed in childhood when patients present with recurrent pneumonia, cough and wheezing. However, adults have been diagnosed with WCS later in life because of either a subclinical presentation or an acquired form secondary to adenovirus exists [1]. Multi-splice spiral CT and CT bronchoscopy can be used to diagnose WCS. A lack of cartilage rings, as determined by an absence of ring impressions, will be seen on imaging [1]. Treatment is generally supportive with prophylactic antibiotics, inhaled corticosteroids, and exercise or inspiratory muscle training [3]. CONCLUSIONS: To prevent a delayed diagnosis, William-Campbell Syndrome should be considered in adults presenting with cystic lung disease with bronchiectasis, productive cough, and recurrent infections. Reference #1: Aldave AP, Saliski DW. The clinical manifestations, diagnosis and management of Williams-Campbell syndrome. North American Journal of Medical Sciences. 2014 Sep;6(9):429-432. Reference #2: Shoemark A, Ozerovitch L, Wilson R. Aetiology in adult patients with bronchiectasis. Respiratory Medicine. 2007 Jun 1;101(6):1163-70. Reference #3: ten Hacken NH, van der Molen T. Bronchiectasis. BMJ. 2010 Jul 14;341:c2766. DISCLOSURES: My spouse/partner as a Consultant relationship with Abbvie Please note: $1-$1000 Added 05/27/2020 by Ebtesam Islam, source=Web Response, value=Consulting fee No relevant relationships by Kenneth Iwuji, source=Web Response No relevant relationships by Lisa Saa, source=Web Response

Effects of a comprehensive, inpatient pulmonary rehabilitation programme in a cachectic patient with very severe COPD and chronic respiratory failure.

A cachectic patient with very severe COPD and chronic respiratory failure may benefit from comprehensive and personalised pulmonary rehabilitation including neuromuscular electrical stimulation, noninvasive ventilation and anabolic steroids http://bit.ly/31Ss7WZ

Non-invasive ventilation in palliative care: a systematic review

An ageing population and steady increase in the rates of neoplasms and chronic degenerative diseases poses a challenge for societies and their healthcare systems. Because of the recent and continued advances in therapies, such as the development and widespread use of non-invasive ventilation (NIV), survival rates have increased for these pathologies. For patients with end-stage chronic respiratory diseases, the use of NIV following the onset of acute or severe chronic respiratory failure is a valid option when intubation has been excluded. The following electronic databases were searched from their inception to January 2000 to December 2017: MEDLINE, EMBASE, CINHAIL, CENTRAL (Cochrane Central register of Controlled Trials), DARE (Database of Abstracts of Reviews of Effectiveness), the Cochrane Database of Systematic Reviews, ACP Journal Club database. The available evidence strongly supports the use of NIV in patients presenting with an exacerbation of chronic obstructive pulmonary disease, as well end-stage neuromuscular disease. Few studies support the use of NIV in end-stage interstitial lung disease and in morbid obesity patients. In patients with cancer has been recommend offering NIV as palliative care to improve dyspnea. The decision regarding the treatment should be made by the patient, ideally before reaching the terminal stage and after having a frank dialogue with healthcare professionals and family members.

ВЛИЯНИЕ ДЛИТЕЛЬНОЙ КИСЛОРОДОТЕРАПИИ НА КЛИНИЧЕСКОЕ ТЕЧЕНИЕ ЗАБОЛЕВАНИЯ И ФУНКЦИЮ ДИАФРАГМЫ У БОЛЬНЫХ ХРОНИЧЕСКОЙ ОБСТРУКТИВНОЙ БОЛЕЗНЬЮ ЛЕГКИХ С ГИПОКСЕМИЧЕСКОЙ ДЫХАТЕЛЬНОЙ НЕДОСТАТОЧНОСТЬЮ (ОПЫТ ТРЕХЛЕТНЕГО НАБЛЮДЕНИЯ)

The objective: to determine the effect of prolonged oxygen therapy (POT) on changes in the clinical and functional status of patients with chronic obstructive pulmonary disease (COPD) and severe hypoxemic chronic respiratory failure. Subjects and methods. 30 patients with COPD were examined during the period of remission of the disease, the average age was 64.8 ± 6.9 years. After the initial examination, patients were prescribed POT at home for 16 hours/day. As a basic therapy, all patients received a combined bronchodilator of long-acting indacaterol/glycopyrronium at a dose of 110/50 μg once a day in combination with beclomethasone dipropionate (BDP) at the dose of 200-400 μg/day. The patients were examined repeatedly 3 times with a 1-year interval. Clinical indicators, gas composition of arterial blood, results of spirometry and ultrasound examination of the diaphragm were evaluated. Results. Initially, disorders of the functional state of the diaphragm were detected in COPD patients with hypoxemia (decrease in the thickening fraction and relaxation rate). After the first year of using POT, an increase in the rates of contraction and relaxation of the diaphragm muscle under tidal respiration was observed. On the 2nd and 3rd year of using POT, it was noted that indicators of the functional state of the diaphragm stabilized. As a result of POT, the number of exacerbations went down and mortality in COPD patients with hypoxemia decreased. Against the background of basic therapy with indacaterol/glycopyrronium in combination with low doses of BDP, no adverse events were observed for the entire period of follow-up. Conclusions. POT in combination with a double long-acting bronchodilator and low-dose inhaled glucocorticosteroids in COPD patients with hypoxemic chronic respiratory failure helps to reduce dyspnea, the number of severe exacerbations, as well as improve pulmonary ventilation functions, blood gas content, and the diaphragm function during the first year of its use. Further, the indicators continued to stabilize.

New non invasive ventilator strategy applied to COPD patients in acute ventilator failure

IntroductionThere is no evidence in the literature regarding the combined use of positive ventilation with negative ventilation. A recent study reported that the two techniques can be combined in patients with ARDS, who undergo ventilatory support for severe acute respiratory failure (ARF). There is experience of non-invasive ventilation in patients with chronic respiratory diseases and ARF.The aim of this study was to test the efficacy of a non-invasive ventilatory strategy based on the combined use of negative (N) and positive ventilation (P) in bi-level mode (PN). MethodsWe enrolled 8 patients with severe COPD exacerbations and exacerbated chronic respiratory failure admitted in a monitored setting of an intermediate-intensive respiratory Unit. ResultsPatients underwent combined positive/negative ventilation and at different times, in place of the two singular ventilation modes (P and N).After each cycle, in the combined P/N ventilatory mode, gas exchanges were significantly increased compared to the two singular P/N mode: pH (7.42 vs 7.40 and 7.40); PCO2 (85.01 vs 72.05 and 66.81 mmHg); FiO2/PO2 (488.75 vs 352.62 and 327.87).All patients well tolerated the application of the double ventilation mode. ConclusionsIn conclusion, the use of dual mode ventilation appears well tolerated and superior to the individual modes in patients with COPD exacerbations and ARF.

Respiratory home care: take the pulmonary specialist out of the hospital!

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Episodic medical home interventions in severe bedridden Chronic Respiratory Failure patients: a 4 year retrospective study

Home care for respiratory patients includes a complex array of services delivered in an uncontrolled setting. The role of a respiratory specialist inside the home healthcare team has been scarcely studied up to now. Our aims were to analyse the number and quality of episodic home visits performed by respiratory physicians to severe bedridden Chronic Respiratory Failure (CRF) patients, and also to evaluate the safety of tracheotomy tube substitutions at home. 231 home interventions (59.8/year) in 123 CRF patients (59 males; age 63 +/- 17 y, 24 on oxygen therapy, 35 under non invasive mechanical ventilation, 46 under invasive ventilation, 74 with tracheostomy) located 35 +/- 16 km far from referred hospital, were revised in a period of 4 years (2005-2008). Chronic Obstructive Pulmonary Disease (COPD) (31%) and amyotrophic lateral sclerosis (ALS) (28%) were the more frequent diagnoses. Interventions were: tracheotomy tube substitution (64%) presenting 22% of minor adverse events and 1.4% of major adverse events; change or new oxygen prescription (37%); nocturnal pulsed saturimetric trend prescription (24%); change in mechanical ventilation (MV) setting (4%); new MV adaptation (7%). After medical intervention, new home medical equipment devices (oxygen and MV) were prescribed in 36% of the cases while rehabilitative hospital admission and home respiratory physiotherapy prescription was proposed in 9% and 6% of the cases respectively. Patient/caregiver's satisfaction was reported on average 8.48 +/- 0.79 (1 = the worst; 10 = the higher). The local health care system (HCS) reimbursed 70 euros for each home intervention. Families saved 42 +/- 20 euros per visit for ambulance transportation. Home visits performed by a respiratory physician to bedridden patients with chronic respiratory failure: 1. include predominantly patients affected by COPD and ALS; 2. determine a very good satisfaction to patients/caregivers; 3. allow money saving to caregivers; 4. are predominantly made up to change tracheotomy tube without severe adverse events.

Abstract 11744: The Association Between Plasma Levels of Anti-angiogenic Isoform of Vascular Endothelial Growth Factor and Clinical Profiles of Peripheral Artery Disease

Background: Although circulating levels of vascular endothelial growth factor (VEGF), known as a stimulator of angiogenesis, were elevated in peripheral artery disease (PAD), patients with PAD is often associated with poor collateral formation, resulting in critical limb ischemia. Recently, it has been reported that VEGF165b, a major splicing variant of human VEGF-A, inhibits angiogenesis in some types of cancer and acts as anti-angiogenic. However, the association between circulating levels of VEGF165b and clinical manifestation of patients with PAD has not been fully examined. Methods and Results: This study included 45 patients with PAD who received endovascular therapy and 20 control subjects without PAD in our hospital. PAD was diagnosed according to the PAD management guidelines, such as computed tomography, angiography, and/or ankle-brachial index. We excluded patients with malignancy, end-stage renal disease on dialysis, severe chronic respiratory failure, and active inflammatory disease in consideration of influence on concentrations of VEGF. We measured circulating concentrations of VEGF165b in peripheral blood samples using enzyme-linked immunosorbent assay (ELISA). Plasma levels of VEGF165b were significantly higher in the PAD group than in the control group (91.7 ± 47.8 pg/ml vs. 30.4 ± 18.3 pg/ml, P<0.001). There was no association between the plasma levels of VEGF165b and the symptom grade (Fontaine class), ankle-brachial index and the lesion morphology based on angiographical findings (TASC-II classification, presence of below knee lesion or chronic total occlusion lesion) in PAD group. In the multiple regression analysis, the independent factors to determine plasma levels of VEGF165b were age (ß 0.258, P=0.031), presence of PAD (ß 0.587, P<0.001), hypertension (ß 0.295, P=0.013), dyslipidemia (ß 0.385, P=0.001), history of stroke (ß 0.245, P=0.041), and coronary artery disease (ß 0.292, P=0.016). Conclusion: Plasma levels of VEGF165b was elevated in PAD, but there was no association between the severity of PAD and levels of VEGF165b. VEGF165b was related to age, hypertension, dyslipidemia, history of cerebral and coronary artery disease as well as PAD.

Changes in the endurance shuttle walk test in COPD patients with chronic respiratory failure after pulmonary rehabilitation: the minimal important difference obtained with anchor- and distribution-based method.

BackgroundAlthough the endurance shuttle walk test (ESWT) has proven to be responsive to change in exercise capacity after pulmonary rehabilitation (PR) for COPD, the minimally important difference (MID) has not yet been established. We aimed to establish the MID of the ESWT in patients with severe COPD and chronic hypercapnic respiratory failure following PR.MethodsData were derived from a randomized controlled trial, investigating the value of noninvasive positive pressure ventilation added to PR. Fifty-five patients with stable COPD, GOLD stage IV, with chronic respiratory failure were included (mean (SD) FEV1 31.1 (12.0) % pred, age 62 (9) y). MID estimates of the ESWT in seconds, percentage and meters change were calculated with anchor based and distribution based methods. Six minute walking distance (6MWD), peak work rate on bicycle ergometry (Wpeak) and Chronic Respiratory Questionnaire (CRQ) were used as anchors and Cohen’s effect size was used as distribution based method.ResultsThe estimated MID of the ESWT with the different anchors ranged from 186–199 s, 76–82% and 154–164 m. Using the distribution based method the MID was 144 s, 61% and 137 m.ConclusionsEstimates of the MID for the ESWT after PR showed only small differences using different anchors in patients with COPD and chronic respiratory failure. Therefore we recommend using a range of 186–199 s, 76–82% or 154–164 m as MID of the ESWT in COPD patients with chronic respiratory failure. Further research in larger populations should elucidate whether this cut-off value is also valid in other COPD populations and with other interventions.Trial registrationClinicalTrials.Gov (ID NCT00135538).Electronic supplementary materialThe online version of this article (doi:10.1186/s12931-015-0182-x) contains supplementary material, which is available to authorized users.

Long-term ventilation in children: Ten years later

IntroductionHome mechanical ventilation (HMV) represents a treatment option for patients with chronic respiratory failure and has changed prognosis and survival of many disorders in children. The aim of this study was to characterize a group of children on long-term mechanical ventilation (LTMV) for a period longer than 10 years. MethodsA retrospective analysis was carried out including patients on LTMV for more than 10 years (LTMV-10) in a tertiary pediatric hospital. Statistical analysis: PASW Statistics 18®. ResultsThirty-one children (61% female) belong to the LTMV-10 group. Median age at the beginning of ventilatory support was 3 years (birth to 13 years). Main indications for assisted ventilation were neuromuscular disease (n=12, 39%), metabolic disease (n=7, 23%) and central hypoventilation (n=6, 19%). Volume ventilation was used in 2 children, and positive pressure ventilation in the others, mainly bilevel positive airway pressure (n=25, 81%). Invasive ventilation via tracheostomy was used since the beginning in four cases, and subsequently in two other children. The mean time of ventilatory support was 146 months and the maximum was 219 months. Respiratory morbidity was the most frequent cause of hospitalization and the annual rate of such episodes was 0.17 per child. Global mortality rate was 19%. ConclusionsHMV programs provide necessary and safe assistance for children with severe chronic respiratory failure. As shown in our series, it is possible to be kept on this respiratory support modality for long periods with good compliance and a small number of hospitalizations.

Oxygénothérapie de longue durée en France, 2006–2011

The study aimed to describe the main characteristics of people treated with long-term oxygen therapy (LTOT) and the trends between 2006 and 2011 for prevalence and incidence. Data were provided from a sample comprising 1/97 of the permanent beneficiaries of the French health insurance. The sample contains anonymous socio-demographic characteristics and data on health care reimbursements. The analyses were carried out among adults aged 20 years and above who had received benefits on account of LTOT. In 2011, 2.6% of adults were treated with LTOT, corresponding to around 135,000 people. The crude incidence rate was 0.9‰. The prescription of LTOT included LTOT alone (70%), LTOT associated with assisted ventilation (23%) and LTOT associated with continuous positive airway pressure (7%). About 45% of the patients were supported financially in the long-term illness program of the French Social Security because of severe chronic respiratory failure. Between 2006 and 2011, the age-adjusted prevalence rates increased by 2.9% per year in men and by 6.7% per year in women. The age-adjusted incidence rates remained stable in men (+1.6% per year, P=0.45) but increased in women (+4.7% per year, P=0.04). The median survival was 27 months. This analysis of the data demonstrates an increasing burden associated with LTOT in France from 2006 to 2011.

Severe Measles Infection

France has recently witnessed a nationwide outbreak of measles. Data on severe forms of measles in adults are lacking. We sought to describe the epidemiologic, clinical, treatment, and prognostic aspects of the disease in adult patients who required admission to an intensive care unit (ICU). We performed a retrospective analysis of a cohort of 36 adults admitted to a total of 64 ICUs throughout France for complications of measles from January 1, 2009, to December 31, 2011. All cases of measles were confirmed by serologic testing and/or reverse transcription polymerase chain reaction.The cohort consisted of 21 male and 15 female patients, with a median age of 29.2 years (25th–75th interquartile range [IQR], 27.2–34.2 yr) and a median Simplified Acute Physiology Score (SAPS II) of 13 (IQR, 9–18). Among the 26 patients whose measles vaccination status was documented, none had received 2 injections. One patient had developed measles during childhood. Underlying comorbid conditions included chronic respiratory disease in 9 patients, immunosuppression in 7 patients, and obesity in 3 patients, while measles affected 5 pregnant women.Respiratory complications induced by measles infection led to ICU admission in 32 cases, and measles-related neurologic complications led to ICU admission in 2 cases. Two patients were admitted due to concurrent respiratory and neurologic complications.Bacterial superinfection of measles-related airway infection was suspected in 28 patients and was documented in 8. Four cases of community-acquired pneumonia, 6 cases of ventilator-associated pneumonia, 1 case of tracheobronchitis, and 2 cases of sinusitis were microbiologically substantiated.Of 11 patients who required mechanical ventilation, 9 developed acute respiratory distress syndrome (ARDS). Among the patients with ARDS, extraalveolar air leak complications occurred in 4 cases. Five patients died, all of whom were severely immunocompromised.On follow-up, 1 patient had severe chronic respiratory failure related to lung fibrosis, and 2 patients had mild lower limb paraparesis along with bladder dysfunction, both of which were ascribable to measles-induced encephalitis and myelitis. Among the 5 pregnant patients, the course of measles infection was uneventful, albeit 1 patient underwent emergent cesarean delivery because of fetal growth restriction.Measles is a disease with protean and potentially deceptive clinical manifestations, especially in the immunocompromised patient. Measles-associated pneumonitis and its complications, and less commonly postinfectious encephalomyelitis, are the main source of morbidity and mortality. In contrast with the usually benign course of the disease in immunocompetent patients, measles occurring in immunocompromised patients gives rise to lethal complications including ARDS, with or without bacterial superinfection. Other patients potentially at high risk for severe measles are young adults and pregnant women. Measles pneumonitis may predispose to air leak disease in patients using mechanical ventilation. To date, vaccination remains the most potent tool to control measles infection.

Choosing a ventilator for home mechanical ventilation

<h3>Educational aims</h3> To discuss the basic principles of ventilator function To help the reader to recognise the different features of a home care ventilator To outline the advantages and drawbacks of the different home care ventilators To provide some recommendations to facilitate the clinical management of patients on home care ventilator <h3>Summary</h3> Mechanical ventilation, applied either invasively through a tracheotomy tube or noninvasively <i>via</i> a mask, is increasingly used for long-term management of many forms of severe chronic respiratory failure in the home setting. In recent years, the quality of the ventilators for long-term home mechanical ventilation has improved considerably and, concomitantly, the number of machines available has also increased. This broader range of commercially available machines is clearly an advantage; however, it makes the choice of the optimal device for a specific patient more difficult. The aim of the present article is to provide useful information to help and guide the choice of device for long-term mechanical ventilation in the home setting.