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INTRODUCTION: The effectiveness of home high flow nasal cannula (HFNC) for the treatment of chronic respiratory failure in patients with chronic respiratory diseases (CRDs) has not been summarized. We aimed to conduct a systematic review of the effectiveness, adherence, and safety of HFNC in the long-term treatment of patients with chronic respiratory diseases and respiratory failure. METHODS: A systematic review was conducted. PubMed, Web of science, and SCOPUS were search up to August 2023. Long-term HFNC studies (≥4 weeks) reporting dyspnea; exacerbations, hospitalizations; peripheral oxygen saturation (SpO2), comfort; patient experience, health-related quality of life or partial pressure of carbon dioxide (paCO2) were included. RESULTS: Thirteen articles (701 patients) based on 10 studies were selected: randomized control trials (n=3), randomized crossover trials (n=2), crossover (n=3) and retrospective (n=2) studies. COPD (n=6), bronchiectasis (n=2), COPD/bronchiectasis (n=1) and ILD (n=1) were the underlined CRDs. HFNC reduced exacerbations when compared to usual care/home respiratory therapies (n=6). Quality of life outcomes were also in favor of HFNC in patients with COPD and bronchiectasis (n=6). HFNC had significant effects on hospitalizations, paCO2, and lung function. Adherence ranged from 5.2 to 8.6h/day (n=5). Three studies reported no events, 3 non-serious events and 2 no differences compared with other home respiratory therapies. CONCLUSIONS: HFNC seems more effective than usual care or other home respiratory therapies in reducing exacerbations and improving quality of life in patients with COPD and bronchiectasis, while presenting good adherence and being safe. Its apparently superior effectiveness needs to be better studied in future real-world pragmatic trials.
Subject(s)
Cannula , Oxygen Inhalation Therapy , Patient Compliance , Pulmonary Disease, Chronic Obstructive , Quality of Life , Respiratory Insufficiency , Humans , Respiratory Insufficiency/therapy , Oxygen Inhalation Therapy/instrumentation , Pulmonary Disease, Chronic Obstructive/therapy , Pulmonary Disease, Chronic Obstructive/complications , Chronic Disease , Home Care Services , Treatment Outcome , Bronchiectasis/therapy , Randomized Controlled Trials as Topic , Lung Diseases, Interstitial/therapy , Lung Diseases, Interstitial/complicationsABSTRACT
Acute respiratory failure due to COVID-19 pneumonia often requires a comprehensive approach that includes non-pharmacological strategies such as non-invasive support (including positive pressure modes, high flow therapy or awake proning) in addition to oxygen therapy, with the primary goal of avoiding endotracheal intubation. Clinical issues such as determining the optimal time to initiate non-invasive support, choosing the most appropriate modality (based not only on the acute clinical picture but also on comorbidities), establishing criteria for recognition of treatment failure and strategies to follow in this setting (including palliative care), or implementing de-escalation procedures when improvement occurs are of paramount importance in the ongoing management of severe COVID-19 cases. Organizational issues, such as the most appropriate setting for management and monitoring of the severe COVID-19 patient or protective measures to prevent virus spread to healthcare workers in the presence of aerosol-generating procedures, should also be considered. While many early clinical guidelines during the pandemic were based on previous experience with acute respiratory distress syndrome, the landscape has evolved since then. Today, we have a wealth of high-quality studies that support evidence-based recommendations to address these complex issues. This document, the result of a collaborative effort between four leading scientific societies (SEDAR, SEMES, SEMICYUC, SEPAR), draws on the experience of 25 experts in the field to synthesize knowledge to address pertinent clinical questions and refine the approach to patient care in the face of the challenges posed by severe COVID-19 infection.
Subject(s)
COVID-19 , Noninvasive Ventilation , Humans , COVID-19/complications , COVID-19/therapy , Respiratory Insufficiency/therapy , Respiratory Insufficiency/etiology , Oxygen Inhalation Therapy , Consensus , SARS-CoV-2 , Pandemics , Interdisciplinary Communication , Positive-Pressure RespirationABSTRACT
High-flow nasal cannula (HFNC) is a respiratory support technique that delivers a controlled concentration of oxygen with high flow, heat, and humidity via the nasal pathway. As it has many physiological effects, its use has increased for a variety of clinical indications; however, there is limited guidance on using HFNC as a respiratory support tool during endoscopic procedures. We conducted a narrative review to evaluate the effect of HFNC as an adjuvant tool during fiberoptic bronchoscopy (FOB), upper gastrointestinal tract endoscopy, and surgical procedures in adults. A search of the PubMed and Cochrane databases were performed. Approximately 384 publications were retrieved, and 99 were selected (93 original works and 6 case reports with a literature review). In patients who underwent FOB, HFNC appears to be superior to conventional oxygen therapy (COT) in preventing hypoxaemia. In contrast, for gastrointestinal endoscopy, the current evidence is insufficient to recommend HFNC over COT in a cost-effective manner. Finally, in surgical procedures such as laryngeal microsurgery or thoracic surgery, HFNC has been shown to be a safe and effective alternative to orotracheal intubation. As the results are heterogeneous, we advocate for the need for more quality studies to understand the effectiveness of HFNC during endoscopic procedures.
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PURPOSE: Home non-invasive ventilation (NIV) is recommended in patients with COPD and hypercapnic chronic respiratory failure (HCRF). The mechanism by it can improve alveolar ventilation during spontaneous breathing is not yet completely explained. Our aim is to evaluate the impact of on diaphragm muscle function in a series of patients with HCRF. PATIENTS AND METHODS: Observational, longitudinal, prospective study of a series of patients with very severe chronic obstruction to airflow treated with home high imntensity NIV (HINIV). Patients underwent a baseline and after 12 months assessment including adherence to treatment, quality of life, respiratory function tests and diaphragmatic ultrasound. SPSS v.26 software was used for statistical analysis. RESULTS: We studied 30 patients, 63% male, the mean age was 60.8 (±6.4) years old. Patients had a severe obstructive ventilatory pattern [FEV1 21.8 (±6.1)%] and hypercapnia [pCO2 56.4 (±7.2) mmHg]. After 12 months of HINIV, we observed significant increases in FVC of 9.2% (p = 0.002), FEV1 of 3.5% (p = 0.04), MIP of 9.4% (p = 0.006), and 6-minute-walking test (6MWT) of 31.9 m (p = 0.001), as well as decreases in paCO2 of 12.5 mmHg (p = 0.001), HCO3 of 4.7 mmol/L (p = 0.001) and BODE index from 7 to 6. Diaphragmatic ultrasound demonstrated an increase in the thickening fraction of 14% (p = 0.002). Respiratory symptoms (p = 0.04), physical function (p = 0.03), and sleep (p = 0.04) also improved. CONCLUSION: In patients with HCRF due to very severe chronic obstruction to airflow, long-term HINIV can improve respiratory performance by improving the function of the diaphragmatic musculature. Larger multicenter clinical trials are needed to confirm the results suggested in this study.
Subject(s)
Noninvasive Ventilation , Pulmonary Disease, Chronic Obstructive , Respiratory Insufficiency , Diaphragm/diagnostic imaging , Female , Humans , Hypercapnia/diagnosis , Hypercapnia/etiology , Hypercapnia/therapy , Male , Middle Aged , Noninvasive Ventilation/adverse effects , Noninvasive Ventilation/methods , Prospective Studies , Pulmonary Disease, Chronic Obstructive/complications , Pulmonary Disease, Chronic Obstructive/diagnosis , Pulmonary Disease, Chronic Obstructive/therapy , Quality of Life , Respiratory Insufficiency/diagnosis , Respiratory Insufficiency/etiology , Respiratory Insufficiency/therapyABSTRACT
The intermediate respiratory care units (UCRI) are areas of monitoring and specialized care of patients with acute or chronic-exacerbated respiratory failure, whose severity does not require admission to an intensive care unit, but which due to their complexity cannot be treated in conventional hospitalization. Although the COVID-19 pandemic has proven its usefulness in the management of critical respiratory patients, the historical trajectory of the UCRI comes from many years ago, in which its cost-effectiveness has been demonstrated by far. This document presents a series of questions and answers on the history of the UCRI, in addition to the criteria for admission, infrastructure, human and technical resources, and the types of existing Units. Within the UCRI year 2021-2022 designated by the Spanish Society of Pneumology and Thoracic Surgery, any scientific dissemination linked to the in-depth knowledge of these units is timely, where multidisciplinarity and the work of professionals related to the care of critical respiratory patients converge.
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Coronavirus Disease 2019 (COVID-19) pandemic has implacably stricken on the wellness of many countries and their health-care systems. The aim of the present study is to analyze the clinical characteristics of the initial wave of patients with COVID-19 attended in our center, and to identify the key variables predicting the development of respiratory failure. Prospective design study with concurrent data retrieval from automated medical records of all hospitalized adult patients who tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) rRT-PCR assay performed on respiratory samples from March 2nd to 18th, 2020. Patients were followed up to May 1st, 2020 or death. Respiratory failure was defined as a PaO2/FiO2 ratio ≤ 200 mm Hg or the need for mechanical ventilation (either non-invasive positive pressure ventilation or invasive mechanical ventilation). We included 521 patients of whom 416 (81%) had abnormal Chest X-ray on admission. Median age was 64.6 ± 18.2 years. One hundred eighty-one (34.7%) developed respiratory failure after a median time from onset of symptoms of 9 days (IQR 6-11). In-hospital mortality was 23.8% (124/521). The modeling process concluded into a logistic regression multivariable analysis and a predictive score at admission. Age, peripheral pulse oximetry, lymphocyte count, lactate dehydrogenase and C-reactive protein were the selected variables. The model has a good discriminative capacity with an area under the ROC curve of 0.85 (0.82-0.88). The application of a simple and reliable score at admission seems to be a useful tool to predict respiratory failure in hospitalized COVID-19 patients.
Subject(s)
COVID-19 , Respiratory Insufficiency , Adult , Aged , Aged, 80 and over , COVID-19/complications , Humans , Middle Aged , Pandemics , Prospective Studies , Respiratory Insufficiency/epidemiology , SARS-CoV-2ABSTRACT
BACKGROUND: Recessive mutations in the thymidine kinase 2 (TK2) gene cause a rare mitochondrial myopathy, frequently with severe respiratory involvement. Deoxynucleoside therapy is currently under investigation. RESEARCH QUESTION: What is the impact of nucleosides in respiratory function in patients with TK2-deficient myopathy? STUDY DESIGN AND METHODS: Retrospective observational study of patients treated with deoxycytidine and deoxythymidine. Evaluations were performed every 3 to 4 months after treatment during approximately 30 months. Forced vital capacity (FVC), maximuminspiratory and expiratory pressures (MIP/MEP), sniff nasal inspiratory pressure (SNIP), cough peak flow (CPF), arterial blood gas and nocturnal pulse oximeter (SpO2) were collected. RESULTS: We studied six patients, five of which were women, with a median age at onset of symptoms was 35.8 (range 5 to 60) years old. Patients presented a restrictive ventilatory pattern (median FVC of 50 (26 to 71)%) and severe neuromuscular respiratory weakness (MIP 38 (12 to 47)% and SNIP 14 (8 to 19) cmH2O). Four patients required ventilatory support before starting the treatment. FVC improved by 6%, proportion of sleep time with SpO2 <90% diminished from 14% to 0%, CPF increased by 23%, MEP increased by 73%, production and management of bronchial secretions improved and respiratory infections diminished. INTERPRETATION: Early detection of respiratory involvement requires an active search, even in asymptomatic patients. The nucleosides therapy may improve respiratory function, and stabilise the loss of respiratory capacity.
Subject(s)
Deoxycytidine , Mitochondrial Myopathies , Respiratory Muscles , Thymidine , Adolescent , Adult , Child , Child, Preschool , Deoxycytidine/therapeutic use , Female , Humans , Middle Aged , Mitochondrial Myopathies/diagnosis , Mitochondrial Myopathies/drug therapy , Mitochondrial Myopathies/genetics , Thymidine/therapeutic use , Thymidine Kinase/genetics , Vital Capacity , Young AdultABSTRACT
OBJECTIVE: To report a case of a patient infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) who acutely developed a hypokinetic-rigid syndrome. METHODS: Patient data were obtained from medical records from the Hospital Universitario 12 de Octubre in Madrid, Spain. [123I]-ioflupane dopamine transporter (DaT) SPECT images were acquired 4 hours after a single dose of 185 MBq of 123I-FP-CIT. Quantitative analysis was performed with DaTQUANT software providing the specific binding ratio and z score values of the striatum. RESULTS: We report a previously healthy 58-year-old man who developed hyposmia, generalized myoclonus, fluctuating and transient changes in level of consciousness, opsoclonus, and an asymmetric hypokinetic-rigid syndrome with ocular abnormalities after a severe SARS-CoV-2 infection. DaT-SPECT confirmed a bilateral decrease in presynaptic dopamine uptake asymmetrically involving both putamina. Significant improvement in the parkinsonian symptoms was observed without any specific treatment. CONCLUSION: This case study provides clinical and functional neuroimaging evidence to support that SARS-CoV-2 can gain access to the CNS, affecting midbrain structures and leading to neurologic signs and symptoms.
Subject(s)
Coronavirus Infections/physiopathology , Parkinson Disease, Postencephalitic/physiopathology , Pneumonia, Viral/physiopathology , Putamen/diagnostic imaging , Betacoronavirus , Brain/diagnostic imaging , Brain/metabolism , COVID-19 , Consciousness Disorders , Coronavirus Infections/complications , Coronavirus Infections/diagnostic imaging , Disease Progression , Dopamine Plasma Membrane Transport Proteins/metabolism , Electroencephalography , Humans , Hypokinesia/diagnostic imaging , Hypokinesia/etiology , Hypokinesia/physiopathology , Magnetic Resonance Imaging , Male , Middle Aged , Muscle Rigidity/diagnostic imaging , Muscle Rigidity/etiology , Muscle Rigidity/physiopathology , Nortropanes , Ocular Motility Disorders , Pandemics , Parkinson Disease, Postencephalitic/diagnostic imaging , Parkinson Disease, Postencephalitic/etiology , Pneumonia, Viral/complications , Pneumonia, Viral/diagnostic imaging , Putamen/metabolism , SARS-CoV-2 , Tomography, Emission-Computed, Single-PhotonABSTRACT
No disponible
Subject(s)
Humans , Diaphragm/diagnostic imaging , Ultrasonography/methods , Sensitivity and Specificity , Diaphragm/physiopathologyABSTRACT
INTRODUCTION AND OBJECTIVES: Humidification and non-invasive ventilation are frequently used together, despite the lack of precise recommendations regarding this practice. We aimed to analyse the impact of active external and built-in humidifiers on the performance of home ventilators, focusing on their pressurization efficacy and their behaviour under different inspiratory efforts. METHODS: We designed a bench study of a lung simulator programmed to emulate mechanical conditions similar to those experienced by real respiratory patients and to simulate three different levels of inspiratory effort: five different commonly used home NIV devices and active humidifiers attached to the latter (internal or "built-in") or to the circuit (external). To test ventilator pressurization under different humidification and effort settings, pressure-time products in the first 300ms and 500ms of the respiratory cycle were calculated in the 45 situations simulated. Inferential statistical analysis was performed. RESULTS: A significant reduction of PTP 300 and PTP 500 was observed with the external humidifier in three of the devices. The same pattern was noted for another device with an internal humidifier, and only one device showed no significant changes. This impact on pressurization was commonly higher under high inspiratory effort. CONCLUSIONS: These results indicate the need to monitor pressure changes in the use of external humidification devices in some home NIV ventilators.
Subject(s)
Humidifiers/standards , Noninvasive Ventilation/instrumentation , Pressure/adverse effects , Pulmonary Disease, Chronic Obstructive/therapy , Ventilators, Mechanical/standards , Computer Simulation , Equipment Design/methods , Humans , Humidifiers/statistics & numerical data , Inhalation/physiology , Monitoring, Physiologic/standards , Noninvasive Ventilation/methods , Respiratory Mechanics/physiology , Ventilators, Mechanical/trendsABSTRACT
BACKGROUND: Gastrointestinal complications after lung transplatation are associated with an increased risk of morbidity and mortality. This study aims to describe severe gastrointestinal complications (SGC) after lung transplantation. METHODS: We performed a prospective, observational study that included 136 lung transplant patients during a seven year period in a tertiary care universitary hospital. SGC were defined as any diagnosis related to the gastrointestinal or biliary tract leading to lower survival rates or an invasive therapeutic procedure. Early and late complications were defined as those occurring < 30 days and ≥ 30 days post-transplant. The survival function was calculated through the Kaplan-Meier estimator. Variables were analyzed using univariate and multivariate analysis. Statistical significance was defined as p < 0.05. RESULTS: There were 17 (12.5%) SGC in 17 patients. Five were defined as early. Twelve patients (70.6%) required surgical treatment. Mortality was 52.9% (n = 9). Patients with SGC had a lower overall survival rate compared to those who did not (14 vs 28 months, p = 0.0099). The development of arrhythmias in the first 48 h of transplantation was a risk factor for gastrointestinal complications (p = 0.0326). CONCLUSIONS: SGC are common after lung transplantation and are associated with a considerable increase in morbidity-mortality. Early recognition is necessary to avoid delays in treatment, since a clear predictor has not been found in order to forecast this relevant comorbidity.
Subject(s)
Gastrointestinal Diseases/mortality , Lung Transplantation/adverse effects , Lung Transplantation/mortality , Postoperative Complications/mortality , Adult , Aged , Comorbidity , Female , Gastrointestinal Diseases/etiology , Humans , Kaplan-Meier Estimate , Lung Diseases/complications , Lung Diseases/surgery , Male , Middle Aged , Morbidity , Multivariate Analysis , Postoperative Complications/epidemiology , Prospective Studies , Risk Factors , Survival Analysis , Survival RateABSTRACT
BACKGROUND: TK2 gene encodes for mitochondrial thymidine kinase, which phosphorylates the pyrimidine nucleosides thymidine and deoxycytidine. Recessive mutations in the TK2 gene are responsible for the 'myopathic form' of the mitochondrial depletion/multiple deletions syndrome, with a wide spectrum of severity. METHODS: We describe 18 patients with mitochondrial myopathy due to mutations in the TK2 gene with absence of clinical symptoms until the age of 12. RESULTS: The mean age of onset was 31 years. The first symptom was muscle limb weakness in 10/18, eyelid ptosis in 6/18, and respiratory insufficiency in 2/18. All patients developed variable muscle weakness during the evolution of the disease. Half of patients presented difficulty in swallowing. All patients showed evidence of respiratory muscle weakness, with need for non-invasive Mechanical Ventilation in 12/18. Four patients had deceased, all of them due to respiratory insufficiency. We identified common radiological features in muscle magnetic resonance, where the most severely affected muscles were the gluteus maximus, semitendinosus and sartorius. On muscle biopsies typical signs of mitochondrial dysfunction were associated with dystrophic changes. All mutations identified were previously reported, being the most frequent the in-frame deletion p.Lys202del. All cases showed multiple mtDNA deletions but mtDNA depletion was present only in two patients. CONCLUSIONS: The late-onset is the less frequent form of presentation of the TK2 deficiency and its natural history is not well known. Patients with late onset TK2 deficiency have a consistent and recognizable clinical phenotype and a poor prognosis, due to the high risk of early and progressive respiratory insufficiency.
Subject(s)
Mitochondrial Myopathies/enzymology , Thymidine Kinase/deficiency , Adolescent , Adult , Child , DNA, Mitochondrial/genetics , Female , Humans , Late Onset Disorders/enzymology , Late Onset Disorders/metabolism , Late Onset Disorders/pathology , Male , Middle Aged , Mitochondrial Myopathies/genetics , Muscle, Skeletal/enzymology , Muscle, Skeletal/metabolism , Muscle, Skeletal/pathology , Muscular Diseases/enzymology , Muscular Diseases/genetics , Mutation/genetics , Retrospective Studies , Thymidine Kinase/genetics , Young AdultABSTRACT
Introducción y objetivo: Aunque los trastornos respiratorios durante el sueño son frecuentes en pacientes con insuficiencia respiratoria crónica grave (IRCG), hay poca información sobre su prevalencia. Nuestro objetivo ha sido describir la prevalencia y características del síndrome de apnea-hipopnea del sueño (SAHS) en estos pacientes. Material y métodos: Estudio prospectivo observacional de pacientes con IRCG incluidos en lista de espera de trasplante pulmonar a los que se les realizó una polisomnografía estándar. Resultados: Ciento cinco pacientes fueron valorados. Ochenta y cinco cumplieron los criterios de inclusión. La prevalencia de SAHS fue del 24,7%, y un 19% de ellos eran graves. La enfermedad respiratoria más frecuente fue la EPOC (62%). El SAHS se relacionó con el sexo masculino (p=0,002), el peso (p=0,013), el IMC (p=0,034) y la circunferencia cervical (p=0,01). Aunque la mayoría de los pacientes presentaron algún síntoma indicativo de SAHS, la media de puntuación en la Escala de Somnolencia de Epworth fue baja. Conclusiones: Hemos observado una alta prevalencia de SAHS en pacientes con IRCG, sin datos clínicos que puedan evidenciar su existencia, por lo que consideramos adecuado realizar estudios de sueño dada la baja sospecha clínica pretest (AU)
Introduction and objective: Although sleep disordered breathing is common in patients with a severe chronic respiratory insufficiency (SCRI), there is few information on its prevalence. Our aim was to describe the prevalence and characteristics of the obstructive sleep apnea-hypopnea syndrome (OSAHS) in these patients. Material and methods: Prospective and observational study carried out on patients with a SCRI included in a waiting list for a lung transplantation and who had undergone a standard polysomnography. Results: A total of 105 patients were examined, of which 85 met the study's inclusion criteria. The prevalence of the OSAHS was 24.7%, with 19% of cases being severe. The most common underlying respiratory condition was COPD (62%). The OSAHS was linked to the male gender (P=.002), weight (P=.013), BMI (P=.034) and neck circumference (P=.01). Although most patients experienced symptoms suggestive of an OSAHS, the average score obtained in the Epworth Sleepiness Scale was low. Conclusions: We observed a high prevalence of OSAHS in patients with a SCRI but without clinical data suggestive of its diagnosis; hence, we believe that sleep studies should be carried out in these patients given the low pre-test clinical suspicion of the disease (AU)
Subject(s)
Humans , Respiratory Insufficiency/complications , Sleep Apnea, Obstructive/epidemiology , Hypoxia/epidemiology , Lung Transplantation , Chronic Disease , Catastrophic Illness , Pulmonary Disease, Chronic Obstructive/complications , Prospective Studies , Risk Factors , Disorders of Excessive Somnolence/epidemiology , PolysomnographyABSTRACT
INTRODUCTION AND OBJECTIVE: Although sleep disordered breathing is common in patients with a severe chronic respiratory insufficiency (SCRI), there is few information on its prevalence. Our aim was to describe the prevalence and characteristics of the obstructive sleep apnea-hypopnea syndrome (OSAHS) in these patients. MATERIAL AND METHODS: Prospective and observational study carried out on patients with a SCRI included in a waiting list for a lung transplantation and who had undergone a standard polysomnography. RESULTS: A total of 105 patients were examined, of which 85 met the study's inclusion criteria. The prevalence of the OSAHS was 24.7%, with 19% of cases being severe. The most common underlying respiratory condition was COPD (62%). The OSAHS was linked to the male gender (P=.002), weight (P=.013), BMI (P=.034) and neck circumference (P=.01). Although most patients experienced symptoms suggestive of an OSAHS, the average score obtained in the Epworth Sleepiness Scale was low. CONCLUSIONS: We observed a high prevalence of OSAHS in patients with a SCRI but without clinical data suggestive of its diagnosis; hence, we believe that sleep studies should be carried out in these patients given the low pre-test clinical suspicion of the disease.
Subject(s)
Respiratory Insufficiency/complications , Sleep Apnea, Obstructive/etiology , Adult , Aged , Chronic Disease , Female , Humans , Male , Middle Aged , Polysomnography , Prevalence , Prospective Studies , Risk Factors , Severity of Illness Index , Sleep Apnea, Obstructive/diagnosis , Sleep Apnea, Obstructive/epidemiologyABSTRACT
INTRODUCTION: Congenital Central Hypoventilation Syndrome (CCHS) is a very rare genetic disease. In 2012 the European Central Hypoventilation Syndrome (EuCHS) Consortium created an online patient registry in order to improve care. AIM: To determine the characteristics and outcomes of Spanish patients with CCHS, and detect clinical areas for improvement. MATERIALS AND METHOD: An assessment was made on the data from Spanish patients in the European Registry, updated on December 2015. RESULTS: The Registry contained 38 patients, born between 1987 and 2013, in 18 hospitals. Thirteen (34.2%) were older than 18 years. Three patients had died. Genetic analysis identified PHOX2B mutations in 32 (86.5%) out of 37 patients assessed. The 20/25, 20/26 and 20/27 polyalanine repeat mutations (PARMs) represented 84.3% of all mutations. Longer PARMs had more, as well as more severe, autonomic dysfunctions. Eye diseases were present in 47%, with 16% having Hirschsprung disease, 13% with hypoglycaemia, and 5% with tumours. Thirty patients (79%) required ventilation from the neonatal period onwards, and 8 (21%) later on in life (late onset/presentation). Eight children (21%) were using mask ventilation at the first home discharge. Five of them were infants with neonatal onset, two of them, both having a severe mutation, were switched to tracheostomy after cardiorespiratory arrest at home. Approximately one-third (34.3%) of patients were de-cannulated and switched to mask ventilation at a mean age of 13.7 years. Educational reinforcement was required in 29.4% of children attending school. CONCLUSION: The implementation of the EuCHS Registry in Spain has identified some relevant issues for optimising healthcare, such as the importance of genetic study for diagnosis and assessment of severity, the high frequency of eye disease and educational reinforcement, as well as some limitations in ventilatory techniques.
Subject(s)
Hypoventilation/congenital , Sleep Apnea, Central , Adolescent , Adult , Child , Child, Preschool , Cross-Sectional Studies , Europe , Female , Humans , Hypoventilation/diagnosis , Hypoventilation/epidemiology , Hypoventilation/therapy , Infant , Male , Registries , Sleep Apnea, Central/diagnosis , Sleep Apnea, Central/epidemiology , Sleep Apnea, Central/therapy , Spain , Young AdultABSTRACT
BACKGROUND: Noninvasive ventilation (NIV) titration may be difficult when dynamic airway obstruction episodes persist, even with high expiratory positive airway pressure (EPAP). We aimed to determine the usefulness of videolaryngoscopy during NIV for identifying mechanisms and sites of obstruction and for providing a guide for their resolution in difficult-to-titrate subjects. METHODS: When obstructions during NIV were present in the built-in software, EPAP was raised to 12 cm H2O. If obstructions persisted, a polygraphy during NIV was performed; if the events occurred with effort, a videolaryngoscopy with nasal and oronasal masks in awake subjects was performed. RESULTS: In a population of 208 subjects in whom NIV was initiated, 13 were identified as difficult to titrate with persistent obstructions during NIV despite an EPAP of 12 cm H2O. Videolaryngoscopy during NIV was able to identify the mechanism and the site of obstruction in all cases. The obstruction under oronasal mask ventilation was due to soft-palate (velum) collapse in 4 subjects, to epiglottic backward movement in 5 other subjects, and to tongue-base obstruction reducing the retroglossal space in 3 more. Videolaryngoscopy during NIV demonstrated improvement in 9 subjects (69%) upon changing to nasal mask and suggested a possible surgical approach in 2 (15%); in one of these 2 subjects, a successful uvulopalatopharyngoplasty was performed. CONCLUSIONS: The use of videolaryngoscopy during NIV in difficult-to-titrate patients may help to identify the sites and mechanisms of obstruction and in some cases may improve quality of ventilation.
Subject(s)
Airway Obstruction/diagnostic imaging , Airway Obstruction/therapy , Laryngoscopy/methods , Noninvasive Ventilation/instrumentation , Aged , Aged, 80 and over , Airway Obstruction/etiology , Epiglottis/physiopathology , Female , Humans , Male , Masks/adverse effects , Middle Aged , Palate, Soft/physiopathology , Pressure , Tongue/physiopathologyABSTRACT
Objetivo: Determinar la prevalencia de trombosis de muñón arterial (TMA) en cirugías de resección pulmonar por carcinoma broncogénico, y describir su evolución radiológica y tratamiento. Material y métodos: Estudio observacional y retrospectivo de casos de TMA detectados mediante angiotomografías con contraste intravenoso (TAC). La comparación de variables clínicas, radiológicas, y el análisis de supervivencia mediante curvas de Kaplan-Meier, se realizó planteando 3 grupos: pacientes con TMA, pacientes con tromboembolismo pulmonar (TEP) y pacientes sin TMA ni TEP. Resultados: Se detectaron 9 TMA en 473 cirugías (1,9%), 6 de ellas en el lado derecho (67% de las TMA), con una mediana de tiempo de detección desde la cirugía de 11,3 meses (rango intercuartílico 2,7-42,2 meses). Salvo el número de TAC en pacientes con TMA comparados con el grupo sin TEP ni TMA, y la recidiva tumoral en pacientes con TEP en comparación con los restantes 2 grupos, no se encontraron diferencias estadísticamente significativas en las características basales ni en las oncológicas. Igualmente no se encontraron diferencias en el análisis de supervivencia. Conclusiones: En nuestra serie, la TMA fue una patología infrecuente que tendió a localizarse en las cirugías del lado derecho, y cuya detección a lo largo del tiempo fue variable. No se asoció a factores de riesgo previos a la cirugía ni tuvo predisposición en relación con la estirpe histológica, estadificación oncológica o recidiva tumoral
Objective: To determine the prevalence of arterial stump thrombosis (AST) after pulmonary resection surgery for lung cancer and to describe subsequent radiological follow-up and treatment. Material and methods: Observational, descriptive study of AST detected by computerized tomography angiography (CT) using intravenous contrast. Clinical and radiological variables were compared and a survival analysis using Kaplan-Meier curves was performed after dividing patients into 3 groups: patients with AST, patients with pulmonary embolism (PE), and patients without AST or PE. Results: Nine cases of AST were detected after a total of 473 surgeries (1.9%), 6 of them in right-sided surgeries (67% of AST cases). Median time to detection after surgery was 11.3 months (interquartile range 2.7-42.2 months), and range 67.5 months (1.4-69.0 months). Statistically significant differences were found only in the number of CTs performed in AST patients compared to those without AST or PE, and in tumor recurrence in PE patients compared to the other 2 groups. No differences were found in baseline or oncological characteristics, nor in the survival analysis. Conclusions: In this series, AST prevalence was low and tended to occur in right-sided surgeries. Detection over time was variable, and unrelated to risk factors previous to surgery, histopathology, and tumor stage or recurrence. AST had no impact on patient survival
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Arterial Occlusive Diseases/diagnostic imaging , Thrombosis/diagnostic imaging , Postoperative Complications/diagnostic imaging , Pneumonectomy , Risk Factors , Retrospective Studies , Follow-Up Studies , Pulmonary Embolism , Prevalence , Platelet Aggregation Inhibitors/therapeutic use , Lung Neoplasms , Computed Tomography Angiography , Kaplan-Meier EstimateABSTRACT
OBJECTIVE: To determine the prevalence of arterial stump thrombosis (AST) after pulmonary resection surgery for lung cancer and to describe subsequent radiological follow-up and treatment. MATERIAL AND METHODS: Observational, descriptive study of AST detected by computerized tomography angiography (CT) using intravenous contrast. Clinical and radiological variables were compared and a survival analysis using Kaplan-Meier curves was performed after dividing patients into 3 groups: patients with AST, patients with pulmonary embolism (PE), and patients without AST or PE. RESULTS: Nine cases of AST were detected after a total of 473 surgeries (1.9%), 6 of them in right-sided surgeries (67% of AST cases). Median time to detection after surgery was 11.3 months (interquartile range 2.7-42.2 months), and range 67.5 months (1.4-69.0 months). Statistically significant differences were found only in the number of CTs performed in AST patients compared to those without AST or PE, and in tumor recurrence in PE patients compared to the other 2 groups. No differences were found in baseline or oncological characteristics, nor in the survival analysis. CONCLUSIONS: In this series, AST prevalence was low and tended to occur in right-sided surgeries. Detection over time was variable, and unrelated to risk factors previous to surgery, histopathology, and tumor stage or recurrence. AST had no impact on patient survival.