A novel presentation of diaphragmatic flutter in a patient with obstructive sleep apnea and recurrent cerebellar hemangioblastoma.

We report a unique case of diaphragmatic flutter in a patient with obstructive sleep apnea who had no respiratory symptoms related to flutter and a history of recurrent cerebellar hemangioblastoma. The flutter was detected during a routine follow-up monitoring through the built-in software of the positive airway pressure device; the flow and pressure curves showed abnormal and curious oscillations. The ultrasound confirmed the diagnosis and ruled out other causes of abnormal diaphragmatic movements. This case report contributes to the scientific literature by presenting a novel case of diaphragmatic flutter associated with recurrent cerebellar hemangioblastoma. It also emphasizes the need for more research on the pathophysiology and treatment of this rare condition. CITATION: Ciorba C, Espinoza Perez JA, Alfonso Imizcoz M, Errasti Viader J, Cebollero Rivas P, De Vito EL. A novel presentation of diaphragmatic flutter in a patient with obstructive sleep apnea and recurrent cerebellar hemangioblastoma. J Clin Sleep Med. 2024;20(2):313-317.

Epidemiology of Tracheostomized Adult Patients Admitted to Specialized Weaning Centers After Acute COVID-19.

BACKGROUND: Epidemiological data on patients with COVID-19 referred to specialized weaning centers (SWCs) are sparse, particularly in low- and middle-income countries. Our aim was to describe clinical features, epidemiology, and outcomes of subjects admitted to SWCs in Argentina. METHODS: We conducted a prospective, multi-center, observational study between July 2020-December 2021 in 12 SWCs. We collected demographic characteristics, laboratory results, pulmonary function, and dependence on mechanical ventilation at admission, decannulation, weaning from mechanical ventilation, and status at discharge. A multiple logistic model was built to predict home discharge. RESULTS: We enrolled 568 tracheostomized adult subjects after the acute COVID-19 phase who were transferred to SWCs. Age was 62 [52-71], males 70%, Charlson comorbidity index was 2 [0-3], and length of stay in ICU was 42 [32-56] d. Of the 315 ventilator-dependent subjects, 72.4% were weaned, 427 (75.2%) were decannulated, and 366 subjects (64.5%) were discharged home. The mortality rate was 6.0%. In multivariate analysis, age (odds ratio 0.30 [95% CI 0.16-0.56], P < .001), Charlson comorbidity index (odds ratio 0.43 [95% CI 0.22-0.84], P < .01), mechanical ventilation duration in ICU (odds ratio 0.80 [95% CI 0.72-0.89], P < .001), renal failure (odds ratio 0.40 [95% CI 0.22-0.73], P = .003), and expiratory muscle weakness (odds ratio 0.35 [95% CI 0.19-0.62], P < .001) were independently associated with home discharge. CONCLUSIONS: Most subjects with COVID-19 transferred to SWCs were weaned, achieved decannulation, and were discharged to home. Age, high-comorbidity burden, prolonged mechanical ventilation in ICU, renal failure at admission, and expiratory muscle weakness were inversely associated with home discharge.

Diagnosis of pulmonary embolism in patients with acute exacerbations of chronic obstructive pulmonary disease: A cross-sectional study.

INTRODUCTION: Pulmonary embolism (PE) remains a frequent complication in patients with chronic obstructive pulmonary disease (COPD). It is unclear that the extent to which the traditional risk stratifying scores for PE are accurate in this population. METHODS: Cross-sectional study of adult patients with COPD and suspected PE included in an Institutional Registry of Thromboembolic Disease at a tertiary teaching hospital in the city of Buenos Aires, Argentina. We estimated the area under the receiver operating characteristic curves (AU-ROC), sensitivity and specificity of the Wells and Geneva scores using a positive computed tomography angiography as the gold standard for PE. We also estimated the sensitivity and specificity for the presence of isolated worsening of dyspnea at presentation, without other cardinal symptoms of acute exacerbation of COPD. RESULTS: A total of 168 patients were included, of which 22% had confirmed PE. The AUC was 0.66 (95% CI 0.56-0.76) and 0.56 (95% CI 0.45-0.67) for the Wells and Geneva, respectively. Considering the most widely used cutoff points, the sensitivity and specificity were 24% and 90% for the Wells and 59% and 43% for the Geneva score, respectively. Isolated worsening of dyspnea on presentation had a sensitivity of 92% and specificity of 37%. CONCLUSIONS: Both Wells and Geneva scores exhibit poor diagnostic accuracy for the diagnosis of PE in patients with COPD. The presence of isolated worsening of dyspnea on presentation could be an easy to identify criteria for the initial triage in this population. Further validation of our findings remains warranted.

Insuficiencia de la bomba ventilatoria: una perspectiva histórica / Ventilatory pump failure: historical perspective

En humanos, PaCO2 es controlada muy estrictamente. A diferencia de PaO2 y todas las pruebas funcionales respiratorias que cambian con la edad, PaCO2 permanece constante durante toda la vida. Por lo tanto, su desviación sostenida representa una alteración significativa de la homeostasis. La estructura responsable de mantener la PaCO2 dentro de límites muy estrechos es la bomba ventilatoria. Se compone de varias unidades anatómicas y funcionales que van desde la corteza cerebral hasta los músculos respiratorios. Varias condiciones clínicas que involucran estas estructuras pueden conducir a la insuficiencia de la bomba respiratoria, cuyo sello distintivo es la hipercapnia. La relevancia del concepto de bomba respiratoria ha sido reconocida a lo largo de las décadas. Unos pocos trabajos germinales abrieron la puerta a un notable número de proyectos básicos, aplicados y clínicos en torno a la insuficiencia de la bomba respiratoria y su relevancia clínica. Este artículo revisará algunos de estos estudios y narrará el camino hacia nuestro estado actual de conocimiento sobre el tema.

IIn humans, PaCO2 is very strictly controlled. Unlike PaO2 and all respiratory functional tests that change with age, PaCO2 remains constant throughout life. Therefore, its sustained deviation represents a significant alteration of homeostasis. The structure responsible for keeping PaCO2 within very narrow limits is the ventilatory pump. It consists of several anatomical and functional units that go from the cerebral cortex to the respiratory muscles. Several clinical conditions involving these structures can lead to failure of the respiratory pump, whose hallmark is hypercapnia. The relevance of the respiratory pump concept has been acknowledged for decades. A few initial works allowed for a remarkable number of basic, applied and clinical projects regarding the respiratory pump failure and its clinical relevance. This article reviews some of these studies and describes the process that lead to our current state of knowledge on the subject

Pulmonary restriction secondary to cutaneous sclerosis caused by chronic graft versus host disease: Case Report / [Restricción pulmonar secundaria a esclerosis cutánea por enfermedad injerto contra huésped crónica: Reporte de Caso.]

Chronic graft versus host disease (GVHD) is a major complication of the allogeneic stem cell transplant. One of most frequent manifestations of GVHD is the cutaneous compromise with the sclerodermatous variety being the most severe. We considered that the restrictive respiratory compromise and its evolution are not well characterized. We described the functional respiratory alterations of a patient with sclerodermatous chronic GVHD and considered differential diagnosis of pulmonary restriction in this type of patient. We reported the case of a 21-year-old woman with pulmonary restriction secondary to cutaneous sclerosis which was caused by chronic GVHD. This report illustrates the importance of utilizing both functional respiratory tests and diagnosis images to accurately characterize the cause of the respiratory compromise. We believe that the functional alterations described in this case could be caused by the cutaneous disorder found.

La enfermedad injerto contra huésped crónica (EICH) es una complicación mayor del trasplante de células progenitoras alogénico. Una de sus manifestaciones más frecuentes es el compromiso cutáneo, siendo su variedad esclerodermiforme la más severa. El compromiso respiratorio, predominantemente restrictivo y su evolución no han sido bien caracterizados. El caso describe las alteraciones funcionales respiratorias de una paciente con EICH cutánea crónica tipo esclerodermiforme en dos momentos de su evolución y considera los diagnósticos diferenciales que producen restricción en estos pacientes. Se presenta el caso de una mujer de 21 años con restricción pulmonar secundaria a esclerosis cutánea por EICH. Este caso ilustra la relevancia de la utilización conjunta de las pruebas funcionales respiratorias y de los métodos diagnósticos por imágenes para caracterizar adecuadamente el compromiso respiratorio de estos pacientes. A nuestro juicio las alteraciones funcionales respiratorias descriptas pueden ser explicadas por el trastorno cutáneo hallado.

Hemorragia alveolar difusa: Diez años de experiencia / Diffuse Alveolar Hemorrhage. Ten Year Experience

Introducción: Las manifestaciones registradas de la hemorragia alveolar difusa (HAD) están influenciadas por las características de los pacientes y la experiencia de los centros de referencias donde son asistidos. Objetivos: Describir los hallazgos clínicos y de laboratorio, las etiologías y los factores de riesgo de mortalidad en pacientes con HAD. Métodos: Análisis retrospectivo de los informes de flbrobroncoscopias en pacientes con diagnóstico de HAD (período 2003-2013), según etiologías inmunológicas y no inmunológicas. Resultados: Se identiflcaron 20 pacientes que presentaron los signos clínicos y patológicos de HAD sobre un total de 6839 flbrobroncoscopias (0,3%). La edad mediana fue 57 años (rango 20-88). Los signos más frecuentes de presentación fueron disnea e inflltrados radiológicos pulmonares en el 100% de los casos y anemia en el 95%. La clásica tríada semiológica (inflltrados radiológicos pulmonares, anemia y hemoptisis) de la HAD se encontró solo en 4 casos, 20%. La etiologías no inmunológicas fueron las más frecuentes (15 casos, 75%), especialmente infecciosas, oncohematológicas y cardiovasculares. La mediana de estadía hospitalaria fue de 17.5 días (rango 2-90 días). Doce pacientes fueron tratados en la UTI. La letalidad fue 60% (12/20 pacientes). Los principales riesgos de muerte fueron pacientes inmunocomprometidos (OR 27.50; IC 95%: 1.99 - 378.00, p = 0.013), necesidad de asistencia respiratoria mecánica (OR 18.00; IC 95%: 1.49-216.00, p = 0.023) y estadía en UTI (OR 7.50; IC 95%: 0.92-61.00, p = 0.049). La mortalidad a los 14 días de internación en el grupo no inmunológico fue superior a la del grupo inmunológico (p = 0.007) pero la mortalidad global no fue diferente (p = 0.066). Conclusiones: En nuestra serie, los principales signos clínicos fueron disnea, anemia e inflltrados pulmonares; pero la tríada clásica fue de rara observación. En todos nuestros pacientes fue posible adscribir la HAD a una etiología deflnida. Las principales etiologías fueron no inmunológicas. La estadía en UTI y la necesidad de ARM se asociaron a mayor riesgo de mortalidad. La mortalidad a mediano plazo no fue diferente entre ambos grupos.

Introduction: The registered manifestations of Diffuse Alveolar Hemorrhage (DAH) are influenced by the characteristics of the patients and the experience of the referral centers where they were assisted. Objectives: To describe clinical and laboratory findings, etiologies and risk factors for mortality of patients with DAH. Methods: A retrospective analysis of fibrobronchoscopy reports in patients with DAH diagnosis was carried out for the period 2003-2013, according to to immunological and non-immunological etiologies. Results: Twenty patients with clinical and pathologic signs of DAH were identified, mean age 57 years old (range 20-88). The most common signs of presentation were dyspnea and radiologic pulmonary infiltrates (100%), and anemia (95%). The classical clinical semiology of three signs (radiologic pulmonary infiltrates, anemia and hemoptisis) was present only in 4 cases (20%). The most frequent etiologies were no immunological (75%), especially infectious, oncohematologic and cardiovascular etiologies. The median hospital stay was 17.5 days (range 2-90 days). Twelve patients were admitted into the Intensive Care Unit. Case fatality was 60% (12/20 patients). The main risk factors for death were immunocompromised patients (OR 27.50; IC 95%: 1.99-378.00, p = 0.013), need for prescription of mechanical ventilation (OR was 18.00; IC 95%: 1.49-216.00, p = 0.023) and intensive care unit admission (OR 7.50 (IC 95%: 0.92-61.00, p = 0.049). Mortality at 14 days in the group not immunological was lower (p=0.007) but the overall mortality was not different (p=0.066). Conclusion: The main clinical manifestation was dyspnea, anemia and pulmonary infiltrates, while the classic triad was infrequent. In all the cases it was possible to attribute the DAH to a defined etiology. The main etiologies were no immunological. The stay in the intensive care unit, the necessity for mechanical ventilation and immunological etiologies were associated with a higher risk of mortality. The medium-term mortality was not different between the two groups.

What does end stage in neuromuscular diseases mean? Key approach-based transitions.

PURPOSE OF REVIEW: To revise the definition of end stage in the setting of neuromuscular disease (NMD), to understand the implications for the patient, family and healthcare team, and to address the obstacles involved in the lack of definition. RECENT FINDINGS: Unlike several conditions such as cancer, kidney or liver disease, the literature reveals no clear definition or categorization for NMD. Many articles mention end stage without defining it. Many years ago an expert consensus panel defined it based on functional criteria (forced vital capacity values and hypercapnic events). Only for amyotrophic lateral sclerosis/motoneurone disease has a wider criteria been proposed. As a consequence, the management of this heterogeneous group of disorders is often fragmented compared with the well organized palliative care program for cancer patients. SUMMARY: Better end-stage NMD definitions should help to identify the goals of care, but a broad range in time and intensity of deterioration make a valid definition difficult for end-stage NMD. Respiratory care, life-prolonging therapies, and structured care planning should be seen as complementary rather than dichotomous. This article emphasized the relevance of an integrated approach through the whole trajectories of NMD patients considering key transitions.

Broncodilatadores agonistas b2 de acción larga durante las exacerbaciones de la EPOC / Agonist bronchodilators b2 long-acting during COPD exacerbations

La exacerbación aguda de la EPOC (EA-EPOC) constituye una causa frecuente de consulta en el ámbito de atención primaria y en los hospitales. Aproximadamente el 40% de los pacientes que acuden a un servicio de urgencias hospitalario debido a una exacerbación precisan ingreso al hospital1. Se define como EA-EPOC al cambio agudo en la situación clínica basal del paciente más allá de la variabilidad diaria, que cursa con aumento de la disnea, aumento de la expectoración, expectoración purulenta, o cualquier combinación de estos tres síntomas, y que requiere un cambio terapéutico1, 2. Su tratamiento consiste en diversas medidas farmacológicas (antibióticos, broncodilatadores, corticoides) y no farmacológicas (oxigenoterapia, kinesioterapia, asistencia ventilatoria), las cuales se encuentran pautadas tanto por ensayos clínicos como por consensos

Changing trends in the management of end-stage neuromuscular respiratory muscle failure: recommendations of an international consensus.

OBJECTIVE: Respiratory management of patients with end-stage respiratory muscle failure of neuromuscular disease has evolved from no treatment and inevitable respiratory failure to the use of up to continuous noninvasive intermittent positive pressure ventilatory support (CNVS) to avert respiratory failure and to permit the extubation of "unweanable" patients without tracheostomy. An international panel experienced in CNVS was charged by the 69th Congress of the Mexican Society of Pulmonologists and Thoracic Surgeons to analyze changing respiratory management trends and to make recommendations. DESIGN: Neuromuscular disease respiratory consensuses and reviews were identified from PubMed. Individual respiratory interventions were identified; their importance was established by assessing the quality of evidence-based literature for each one and their patterns of use over time. The panel then determined the evidence-based strength for the efficacy of each intervention and made recommendations for achieving prolonged survival by CNVS. RESULTS: Fifty publications since 1993 were identified. Continuous positive airway pressure, oxygen therapy, bilevel positive airway pressure used at both low and high spans, "air stacking," manually assisted coughing, low pressure (<35 cm H2O) and high pressure (≥40 cm H2O) mechanically assisted coughing, noninvasive positive pressure ventilation part time (<23 hrs per day) and full time (>23 hrs per day; CNVS), extubation and decannulation of ventilator-dependent patients to CNVS, and oximetry feedback for noninvasive positive pressure ventilation and mechanically assisted coughing were identified. All noted interventions are being used with increasing frequency and were unanimously recommended to achieve prolonged survival by CNVS, with the exception of supplemental oxygen and continuous positive airway pressure, which are being used less and were not recommended for this population. CONCLUSIONS: CNVS and extubation of unweanable patients to CNVS are increasingly being used to prolong life while avoiding invasive interfaces.

Management of dyspnea in advanced motor neuron diseases.

PURPOSE OF REVIEW: Patients with amyotrophic lateral sclerosis or motor neuron disease (ALS/MND) invariably develop respiratory muscle weakness and most die from pulmonary complications. Little evidence is available that identifies optimal management approaches for caring for the dying patient. This review discusses the state of the art on dyspnea in advanced ALS/MND and its treatment. RECENT FINDINGS: Multiple observational studies have demonstrated that noninvasive positive pressure ventilation is beneficial in ALS/MND. It is a relatively safe intervention in the late stages of disease with additional survival benefits when it is started relatively early and it can improve survival. Despite guidelines related to pulmonary function testing about the use of noninvasive positive pressure ventilation, the factors, which are most closely associated with noninvasive positive pressure ventilation utilization, are dyspnea and orthopnea. SUMMARY: In ALS/MND, loss of function relentlessly progresses, and subsequent death occurs mostly in a predictable manner. Therefore, the end of life care is heavily influenced by the type and quality of care provided from the earliest stages. Most patients with ALS/MND develop dyspnea, agitation, anxiety and air hunger in the final phase. Noninvasive positive pressure ventilation has become the standard of care for patients with ALS/MND and advanced respiratory insufficiency. A multidisciplinary approach is strongly recommended.

Nutritional assessment of patients with neuromuscular diseases.

OBJECTIVE: To study the nutritional status of patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis. DESIGN: A total of 34 Duchenne muscular dystrophy and seven amyotrophic lateral sclerosis patients were studied. Body mass index, patient's body weight for zero muscle mass as a percentage of the theoretical weight for zero muscle mass, and creatinine-height index were calculated. RESULTS: Substantial differences were found between body mass index and percentage of expected weight for zero muscle mass. No amyotrophic lateral sclerosis patients were classified as overweight by body mass index, whereas five were overweight by the percentage of expected weight for zero muscle mass method. Five Duchenne muscular dystrophy patients were classified as overweight by body mass index, and 30 were overweight by the percentage of expected weight for zero muscle mass. According to the creatinine-height index, no patient with amyotrophic lateral sclerosis or Duchenne muscular dystrophy showed normal body muscle mass. No correlation was found between creatinine-height index, percentage of expected weight for zero muscle mass, and body mass index. CONCLUSIONS: The body mass index should be used with caution for the evaluation of the nutritional status of patients with amyotrophic lateral sclerosis and Duchenne muscular dystrophy. Indices that incorporate the assessment of the compartmental distribution of muscle and fat are more sensitive.

Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease.

OBJECTIVE: To study the expiratory muscle force and the ability to cough estimated by the peak expiratory flow and peak cough flow in patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis. DESIGN: A total of 27 patients with amyotrophic lateral sclerosis and 52 patients with Duchenne muscular dystrophy were studied. From the group of 144 normal subjects of this laboratory, we selected 38 for comparison. RESULTS: The maximal inspiratory pressure in patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis was 64.5 +/- 24.7% and 37.8 +/- 21.8%, respectively, and maximal expiratory pressure was 64.2 +/- 32.5% and 37.7 +/- 21.6%, respectively. Patient groups showed a significant lower peak expiratory flow than normal subjects. Higher peak cough flow than peak expiratory flow was found in all groups. The peak cough flow-peak expiratory flow difference was 46 +/- 18% in normal subjects, 43 +/- 23% in patients with Duchenne muscular dystrophy, and 11 +/- 17% in patients with amyotrophic lateral sclerosis. The peak expiratory flow and peak cough flow were not different in bulbar onset amyotrophic lateral sclerosis. In patient groups, the dynamic and static behavior correlated positively. CONCLUSIONS: These results suggest that peak cough flow-peak expiratory flow is useful to monitor expiratory muscle weakness and bulbar involvement and to assess its evolution in these patients.