Massive Pulmonary Thrombosis Following Haemoptysis in Type IV Ehlers–Danlos Syndrome / Trombosis pulmonar masiva tras hemoptisis en el Síndrome de Ehlers–Danlos tipo IV

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Schwannoma mediastínico gigante / Giant mediastinal schwannoma

El schwannoma es un tumor neurogénico que se presenta más frecuentemente en el ángulo costovertebral del mediastino posterior, pero también en otras localizaciones dentro del tórax. Habitualmente suele ser una masa única, encapsulada, bien definida, con un tamaño aproximado 2 a 10 cm. Presentamos el caso de un paciente de 66 años, derivado a nuestro hospital por una masa de 13 cm localizada en el hemitórax inferior izquierdo. Se realizó una biopsia, y la resonancia magnética corroboró el diagnóstico de un tumor mediastínico gigante de la vaina neural. Se procedió a su resección completa sin complicaciones.

Schwannomas are neurogenic tumors, commonly located in the costovertebral angle of the posterior mediastinum, but with many intrathoracic locations. They usually present as a solitary, well-circumscribed and encapsulated mass with a size between 2 and 10 cm. We report a case of a 66-year-old male, referred to our hospital for a mass located at the left lower hemithorax with 13 cm in size. A percutaneous biopsy was performed, and magnetic resonance imaging confirmed the diagnosis of a resectable giant mediastinal nerve sheath tumor. Surgery was performed without complications.

Randomised short-term trial of high-span versus low-span APAP for treating sleep apnoea.

PURPOSE: Auto-titrating continuous positive airway pressure (APAP) devices were developed to improve treatment efficacy and compliance in patients with obstructive sleep apnoea syndrome (OSAS). Since there are insufficient data on the optimal pressure range setting, we aimed to compare the adherence, efficacy and tolerability of treatment with high-span versus low-span APAP. METHODS: Seventy-six newly diagnosed OSAS patients fulfilling the treatment criteria were randomised to receive high-span (HS, range 4-15cmH2O, n = 38) or low-span (LS, range 8-12cmH2O, n = 38) APAP. Patients were assessed at 1 and 3 months. RESULTS: Median Epworth sleepiness scale (ESS) was 13 (IQR, 6-16) and median apnoea-hypopnoea index (AHI) was 35.9 (IQR, 27.6-56.3). There were no significant differences in baseline demographic and clinical characteristics between groups. Overall, no significant differences were found at the first month assessment. After 3 months of therapy, we found again no differences in residual AHI or ESS. However, the group HS proved less adherent than group LS, respectively, with median 87 % (IQR, 60.5-97.5) versus 94 % (IQR, 80.0-98.3) of the nights using ≥4 h (P = 0.014) and mean (±SD) usage 5.7 ± 1.6 versus 6.4 ± 1.2 h/night (P = 0.049). The group HS reported more frequently nasal congestion, excessive oronasal dryness and nocturnal awakenings of at least moderate intensity, the latter with statistical significance (P = 0.005). CONCLUSIONS: Both pressure ranges appear to be equally effective to correct AHI and to improve symptoms. Though, patients with high-span APAP were less compliant to treatment, raising issues about the tolerability of wide pressure range settings of these devices.

Aspergilosis pulmonar nodular, ¿todavía supone un desafío? / Pulmonary Aspergillus Nodule-Still Challenging?

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Effectiveness of a group education session on adherence with APAP in obstructive sleep apnea--a randomized controlled study.

PURPOSE: Auto-titrating continuous positive airway pressure (APAP) is an effective treatment for obstructive sleep apnea/hypopnea syndrome (OSAHS). We investigated whether a single group education session on APAP therapy is effective in promoting adherence among patients with OSAHS. METHODS: This prospective, randomized, controlled, parallel group study included patients newly diagnosed with OSAHS who met criteria for APAP therapy. Patients were randomized into a study group and a control group. All patients in the study group were assigned to a single group education session, 1 month after beginning APAP therapy. RESULTS: We evaluated 146 patients. The median percentage of APAP usage days was 88.3 %, with a median duration per day of use of 6.02 h; 59 % were classified as adherent. Overall, no significant difference in adherence was seen between the study and the control groups. Analyzing patient subgroups, the group session significantly improved APAP adherence among males and patients who were younger (<65 years old), obese (BMI ≥ 35 kg/m(2)), non-sleepy (Epworth sleepiness scale ≤ 11), smokers or past smokers, had hypertension or nocturia and those with non-severe OSAHS. CONCLUSION: To maximize the impact of group education sessions and, by that, saving resources, it may be important to select patients likely to benefit from these sessions.

Wegener's granulomatosis and alveolar hemorrhage - case report.

The authors present a clinical case of 55 years old female patient with limited form of Wegener's granulomatosis (WG), which first manifestation was non-erosive polyarthritis with rheumatoid factor positive that antedates one decade the pulmonary manifestations. She had acute episodes with purulent expectoration, fever and hemoptysis, with resolution in a week. The chest x-rays demonstrated migratory bilateral pulmonary infiltrates. Transthoracic lung biopsy was performed and revealed capilaritis and signs of old and recent hemorrhage. At that time, autoimmunity study was repeated and showed positive for rheumatoid factor, negative anti-cyclic citrullinated peptide antibodies (anti-CCP) and high sustained PR3 anti-neutrophil cytoplasmic antibodies. The diagnosis of WG was established and cyclophosphamide started. This patient had a less common presentation and a less common histological pattern compared to the typical necrotizing granulomatous inflammation. She was treated with immunosuppression therapy which could have contributed to a mild clinical expression and a lower diagnostic yield. In suspicious cases, repeat the autoimmunity study, when facing new findings, could confirm the correct diagnosis.

Indicaciones y cumplimiento con la insuflación-exuflación mecánica domiciliaria en pacientes con enfermedades neuromusculares / Indications and Compliance of Home Mechanical Insufflation-Exsufflation in Patients with Neuromuscular Diseases

IntroducciónCon frecuencia, los pacientes con enfermedades neuromusculares (ENM) presentan un deterioro del mecanismo de la tos. Se ha demostrado la eficacia de la insuflación-exuflación mecánica (IEM) en la mejora del aclaramiento de las vías respiratorias aunque no se dispone de datos relacionados con su utilización domiciliaria a largo plazo. El objetivo del presente estudio fue describir las indicaciones, tolerabilidad y cumplimiento con la IEM domiciliaria en pacientes con ENM.MétodosAnálisis observacional de 4 años de duración de 21 pacientes ENM tratados con IEM domiciliaria. El diagnóstico incluyó esclerosis lateral amiotrófica (ELA) bulbar y no bulbar y otras ENM. La edad mediana fue de 58 años. Solo se incluyeron pacientes cooperadores con valores de flujo pico de tos basal (FTM) no asistido <270l/min. Todos los pacientes estaban sometidos a ventilación mecánica continua (seis mediante traqueostomía). La función pulmonar previa al inicio de la IEM (mediana) era: FVC, 0,81 l, MIP, 28cmH2O, MEP=22cmH2O y PCF=60l/min. Cuidadores no profesionales adiestrados previamente con el apoyo permanente de un profesional sanitario experto efectuaron la IEM. En los pacientes se monitorizó la pulsioximetría y se aplicó IEM siempre que la SpO2 fue <95%. El seguimiento mediano fue de 12 meses (3–41 meses)(AU)

ResultadosUtilizaron diariamente IEM 10 pacientes (nueve con ELA). La utilizaron de forma intermitente 11 pacientes durante las exacerbaciones y en 8 la aplicación precoz de IEM (guiada por la información de la oximetría) evitó la hospitalización. Todos los pacientes traqueostomizados utilizaron la IEM a diario y un mayor número de veces al día que los pacientes sometidos a ventilación mecánica no invasiva (VNI). Requirieron ingreso hospitalario 4 pacientes (3 ELA bulbar) debido a la acumulación de secreciones. La IEM fue bien tolerada y no se asoció a complicaciones. En general, los cuidadores la consideraron eficaz. Durante este período, 4 pacientes fallecieron, en relación con la progresión de la enfermedad.ConclusionesLa IEM domiciliaria es bien tolerada, eficaz y segura cuando la utilizan cuidadores adiestrados apropiadamente. Debe considerarse un complemento de la ventilación mecánica(AU)

IntroductionNeuromuscular disease (NMD) patients frequently have impaired cough. Mechanical insufflation-exsufflation (MI-E) has proven efficacy in improving airway clearance, however data related to its long-term home use is lacking. The purpose of this study was to describe indications, safety and compliance of home MI-E in NMD patients.MethodsFour years observational analysis of 21 NMD patients on home MI-E. Diagnosis included bulbar and non-bulbar Amyotrophic Lateral Sclerosis (ALS) and other NMD. Median age was 58 years. Only cooperative patients with unassisted baseline Peak Cough Flow (PCF) <270L/min were included. All patients were under continuous mechanical ventilation (6 by tracheostomy). Pulmonary function before initiation of MI-E (median): FVC=0.81L, MIP=28cmH2O, MEP=22cmH2O and PCF=60L/min. MI-E was performed by previously trained non-professional caregivers, with an on-call support of a trained health care professional. Patients had pulse oximetry monitorization and applied MI-E whenever SpO2<95%. Median follow-up was 12 months (3–41 months).ResultsTen patients (9 ALS) used MI-E daily. Eleven patients used MI-E intermittently, during exacerbations, and in 8 patients early application of MI-E (guided by oximetry feed-back) avoided hospitalization. All tracheostomized patients used MI-E daily and more times a day than patients under NIV. Four patients (3 bulbar ALS), were hospitalized due to secretion encumbrance. MI-E was well-tolerated and there were no complications. In general, caregivers considered MI-E effective. During this period, 4 patients died, related to disease progression.ConclusionsHome MI-E is well tolerated, effective and safe if used by well trained caregivers. MI-E should be considered as a complement to mechanical ventilation(AU)

[Indications and compliance of home mechanical insufflation-exsufflation in patients with neuromuscular diseases]. / Indicaciones y cumplimiento con la insuflación-exuflación mecánica domiciliaria en pacientes con enfermedades neuromusculares.

INTRODUCTION: Neuromuscular disease (NMD) patients frequently have impaired cough. Mechanical insufflation-exsufflation (MI-E) has proven efficacy in improving airway clearance, however data related to its long-term home use is lacking. The purpose of this study was to describe indications, safety and compliance of home MI-E in NMD patients. METHODS: Four years observational analysis of 21 NMD patients on home MI-E. Diagnosis included bulbar and non-bulbar Amyotrophic Lateral Sclerosis (ALS) and other NMD. Median age was 58 years. Only cooperative patients with unassisted baseline Peak Cough Flow (PCF) <270 L/min were included. All patients were under continuous mechanical ventilation (6 by tracheostomy). Pulmonary function before initiation of MI-E (median): FVC=0.81 L, MIP=28cmH(2)O, MEP=22 cmH(2)O and PCF=60 L/min. MI-E was performed by previously trained non-professional caregivers, with an on-call support of a trained health care professional. Patients had pulse oximetry monitorization and applied MI-E whenever SpO(2)<95%. Median follow-up was 12 months (3-41 months). RESULTS: Ten patients (9 ALS) used MI-E daily. Eleven patients used MI-E intermittently, during exacerbations, and in 8 patients early application of MI-E (guided by oximetry feed-back) avoided hospitalization. All tracheostomized patients used MI-E daily and more times a day than patients under NIV. Four patients (3 bulbar ALS), were hospitalized due to secretion encumbrance. MI-E was well-tolerated and there were no complications. In general, caregivers considered MI-E effective. During this period, 4 patients died, related to disease progression. CONCLUSIONS: Home MI-E is well tolerated, effective and safe if used by well trained caregivers. MI-E should be considered as a complement to mechanical ventilation.

Prevalence and characteristics of sleep apnoea in patients with stable heart failure: Results from a heart failure clinic.

BACKGROUND: Heart failure (HF) and sleep apnoea (SA) association has been recognized but whether it results from confounding factors (hypertension, ischaemia, obesity) remains unclear.We aimed to determine the prevalence of SA in HF and to identify potential risk factors for SA in HF population. METHODS: We prospectively evaluated 103 patients with stable HF on optimized therapy. In-laboratory polysomnography was performed. Type and severity of SA were defined according international criteria. Demographic, anthropometric and clinical characteristics were collected. Continuous data are expressed as median and interquartile range. RESULTS: SA was found in 72.8%, moderate to severe in a significant proportion (apnoea-hypopnoea index > or = 15- 44.7% of all patients) and predominantly obstructive (60.0% of patients with SA). Most patients were non-sleepy (Epworth < 10- 66%). SA patients were predominantly men (85.3 vs 60.7%, p-0.015), had larger neck (38.0 (35.0-42.0) vs 35.0 (33.2-38.0) cm, p-0.003), severe systolic dysfunction, (63.9 vs 33.3%, p-0.018), left ventricle (LV) hypertrophy (16.2 vs 0.0%, p-0.03), LV and left atria (LA) dilatation (49.0 (44.0-52.0) vs 42.0 (38.0-48.0) mm, p < 0.001; 60.0 (54.0-65.0) vs 56.0 (52.0-59.0) mm, p-0.01). However, only LA diameter was an independent predictor of SA. Higher body-mass index (BMI) was associated with moderate to severe SA. Patients with obstructive SA had larger neck and a trend for higher BMI, snoring and sleepiness. Hypocapnia was not associated with central SA. CONCLUSIONS: In our HF population, SA was prevalent, frequently asymptomatic and without characteristic risk factors. Unlike previously reported, obstructive SA was the predominant type. These results suggest that SA is underdiagnosed in HF and there is a possible correlation between them, independent of confounding factors. Recent advances in HF therapy might influence prevalence and type of SA in this population.

A spontaneous pneumothorax in a "buffalo chest".

Spontaneous contralateral pneumothorax after pneumonectomy is a rare condition. A high index of suspicion is required for a correct diagnosis. Management can be challenging, as surgical intervention, if necessary, is a very high risk procedure. Chemical pleurodesis can be a simple and effective measure in cases with no major air leak. We describe the case of a 21-year-old female with spontaneous pneumothorax in a single lung, which posed several diagnostic and therapeutic problems. The presence of a "buffalo chest" made the initial chest x-ray interpretation difficult. The patient had an important air leak without complete pulmonary expansion and thoracic surgery was considered of risk. Accordingly, instillation of talc slurry through one of the thoracic drains was undertaken with eventual resolution. We are convinced that bronchopleural fistula resolution was facilitated by slurry talc, despite complete lung expansion never having been achieved.