Chronic Hypoxemic Respiratory Failure Research Articles

Long COVID-19 Infections and Organizing Pneumonia in a Series of Patients with Indolent Lymphoma Treated with Rituximab

Background: Patients with hematologic malignancies have an increased risk of complications and mortality from COVID-19 (SARS-CoV-2). These patients are often more susceptible to protracted COVID-19 infections (e.g., long COVID-19). In addition, patients treated with the CD20-targeted immunotherapy rituximab in B-cell lymphomas are at greater risk for a protracted clinical course with COVID-19, including care in the ICU for respiratory failure. Hypogammaglobulinemia is an independent predictor of increased risk for long-COVID19. Immune depletion both leads to a persistent detection of SARS-CoV-2 and an inability to mount a sustained immune response. Other authors have reported on the appearance of a delayed organizing pneumonia (OP) after acute COVID-19 infection, but these patients were not immunocompromised by rituximab. In this case series, we report on three patients with indolent lymphoma who had the delayed presentation of long COVID-19 after an acute infection. All three were previously treated with rituximab, and each case was notable for prolonged OP that was refractory to steroid therapy. Methods: Summative data for the three patient cases was aggregated from the medical record including available physician notes, diagnostic body imaging, and laboratory data. Results: In total, we found three cases of long COVID-19 in patients with indolent lymphoma from our institution (Table 1). Patient 1 received just two doses of rituximab, Patient 2 received three doses of rituximab, and Patient 3 was treated with rituximab intermittently for nearly three years. All three patients experienced their first COVID-19 infections in August 2022 and had low IgG. This was followed by complications including acute hypoxemic respiratory failure, pulmonary embolism, and organizing pneumonia in these patients two to three months thereafter. These patients continue to have ongoing chronic hypoxemic respiratory failure requiring immune suppression and continuous oxygen support. Conclusions: Immune depletion with rituximab in patients with indolent lymphomas can put them at significant risk of long COVID-19, OP, and chronic hypoxemic respiratory failure after contracting SARS-Cov-2. Such patients may benefit from prolonged immune suppression, but the exact duration remains unclear. The mechanism of long COVID-19 and refractory OP is unclear and complex. Possible mechanisms may include inadequate clearance of virus or a dysfunctional immune response. Table 1: Lymphoma patients who received rituximab and then developed long COVID-19

Case of a 38-year-old man with NEMO deficiency syndrome complicated by marginal zone lymphoma

Here, we present a unique case of a 38-year-old man with history of Nuclear Factor-Kappa B Essential Modulator (NEMO) deficiency syndrome, whose course was complicated by marginal zone lymphoma. At 7 years of age, he was found to have MAI infection and throughout his life, developed further complications of MAI including osteomyelitis, intracranial lesions, adrenal abscesses, and cavitary pulmonary disease. Genetic testing revealed 1207 C-to-T mutation in NEMO, leading to R-for-C substitution at position 417 in the zinc finger domain. He has had a long history of recurrent viral and bacterial sinopulmonary infections since infancy continuing into adulthood, with over thirty lifetime episodes of serious pneumonia requiring hospitalizations, which has led to bronchiectasis.At 36 years of age, he presented to the hospital for five months of progressively worsening dyspnea and wheezing, hypoxemic respiratory failure, and a 15-pound weight loss. CT imaging demonstrated increased consolidation and nodularity, and sputum culture revealed methicillinsusceptible Staphylococcus aureus and Corynebacterium pneumonia treated with vancomycin. He was incidentally found to have a new 7.5 cm left periaortic lymph node conglomerate on CT abdomen/pelvis. Lymph node biopsy showed B-cell lymphoma with plasmacytic differentiation and with increased large cells, most concerning for marginal zone lymphoma. He received eight doses of rituximab as well as radiation therapy, with subsequent improvement on imaging. Following this diagnosis, lung biopsy was obtained, demonstrating nodular fibrosis in a primarily airwaycentered distribution with patchy, focal organizing pneumonia, most consistent with fibrosing chronic organizing pneumonia, treated with high dose prednisone. However, his course had since been complicated by increasingly frequent hospitalizations for acute on chronic hypoxemic respiratory failure corresponding with superimposed respiratory infectious as well as exacerbations of his organizing pneumonia.Hematopoietic stem cell transplantation was strongly considered as this would be a definitive treatment for his NEMO syndrome, as he has multiple severe complications of his NEMO deficiency syndrome. Unfortunately, after multidisciplinary discussions, he was ultimately considered to not be a candidate given his very poor pulmonary status. The patient was started on rituximab for his organizing pneumonia and continued on antibiotic prophylaxis with immunoglobulin replacement.

High flow oxygen therapy with nasal cannula: use and applications in respiratory failure in internal medicine.

High-flow nasal cannula (HFNC) are an oxygen therapy device developed in the last years for the treatment of patients with acute or acute on chronic hypoxemic respiratory failure with different etiology and severity (including covid-19 pneumonia). HFNC combine the possibility of delivering high flows of gases, actively humidified and heated, with the use of a comfortable nasal interface, resulting generally well tolerated by most patients. In light of these characteristics, together with the simplicity of use and versatility, they have spread not only in intensive and semi-intensive care units but also in general medical ward in which they can play an important role in the treatment of elderly, frail patients with comorbidity where other more aggressive and invasive methods of ventilations are not indicated or not practicable.

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

Five-year experience in providing medical care to patients with bronchopulmonary pathology with chronic hypoxemic respiratory failure

Introduction. The article considers the management of medical care for patients with chronic hypoxemic respiratory failure (CHRF), including long-term oxygen therapy (LOT). To achieve this goal in 2013 there was established the St. Petersburg Municipal Pulmonary Center (MPC). The study aimed to assess the results of the MPC activity in 2014 - 2LC138727590CN018. Material and methods. The medical records of patients admitted to the MPC in 2014-2018 were studied. To process the information conventional statistical methods were used. Results. Over the past 5 years, MRC specialists have consulted 13,239 patients, mainly COPD cases. 1,054 patients with CHRF were hospitalized for an additional examination, and 435 cases were indicated to receive LOT. The necessary oxygen concentrator facilities were provided at the expense of the St. Petersburg city budget. Mortality rate accounted for 10.4% by the end of the first year of LOT, 15.1% by the end of the second year, reaching 27.0% and 38.3% by the end of the third and fourth years, respectively. In the group of COPD patients with CHRF (262 patients, 238 men, and 24 women, at the average age of 69 ± 3 years), LOT was accompanied by a decrease in the number of severe exacerbations of the disease (before the LOT - 3 (3; 4) severe exacerbations per year, after a year - 2 (1; 2); p <0.001), as well as cases of referral and duration of stay in the intensive care unit. This led to a decrease in the average cost of inpatient treatment from 264,912 ± 31,277 rubles per year to 134,826 ± 21,277; p <0.001. Conclusion. Early verification and correction of CHRF contribute not only to the decline in the number of exacerbations of respiratory disease but also to the reduction of financial costs necessary for the treatment of such patients.

0731 Obstructive Sleep Apnea with Predominant Respiratory Effort Related Arousals: Theta Power in C3- M2 And C4-M1 Derivation Comparison in Male And Female

Abstract Introduction Theta power in electroencephalography has been studied as a correlate to REM sleep. An increase in theta power during REM sleep has been observed in patients during recovery sleep after sleep deprivation. Emotional memories appear to be processed and consolidated during REM sleep.The role of hippocampal theta wave activity during REM sleep on emotional memory processing is limited. The importance of theta power has not been well characterized in patients with obstructive sleep apnea (OSA) with predominant respiratory Effort Related Arousals (RERAs). This report aims to study the theta power in patients with OSA with predominant Respiratory Effort Related Arousals (RERAs) with an apnea-hypopnea index (AHI) of < 5. Methods We have identified 38 patients with baseline polysomnograms performed from December 2019 to July 2019 with AHI < 5 and a Respiratory Disturbance Index (RDI) of at least 5 or greater. Patients with chronic hypoxemic respiratory failure, hypoventilation and predominant central sleep apnea were excluded from the study. Total power of frequency in bands was obtained for theta waves (4-8 Hz) and total waves (1-30 Hz). Relative theta power was calculated on the last REM sleep using C3-M2 and C4-M1 derivations. Paired two-tailed t-Test was performed on the theta power in C3-M2 and C4-M1 in both the sexes. Results Initial analysis was performed in 38 patients out of which 20 were male and 18 female. Among males, (Mean ± SEM) age was 52.3 (±2.9); Epworth Sleepiness Scale (ESS) of 6.6 (±1.1), AHI of 2.1 (±0.3), and RDI of 7.3 (±0.3).Whereas in female (Mean ± SEM) age was 46.8 (± 2.8), ESS of 7.7 (±1.4), AHI of 2.3 (±0.35), and RDI of 6.9 (±0.4). Statistically significant difference was noted in the theta power between the C3-M2 and C4-M1 derivations with P value of 0.03 and 0.04 in male and female respectively. However, no significant difference was found when C3-M2 and C4 -M1 was compared between male and female. Further, statistical analysis will be performed after gathering data from a larger sample size. Conclusion There was significant difference between C3-M2 and C4-M1;overall no difference was found between sexes. Support none

Диагностическое значение натрий-уретического пептида у больных хронической обструктивной болезнью легких с хронической гипоксемической дыхательной недостаточностью

Background: the progression of chronic obstructive pulmonary disease (COPD) is accompanied by pulmonary hypertension and right ventricular failure as well as left ventricular failure that can be associated with the elevated levels of N-terminal pro b-type natriuretic peptide (NT-proBNP). Aim: to evaluate the diagnostic importance of NT-proBNP in COPD with chronic hypoxemic respiratory failure. Patients and Methods: 60 patients with COPD GOLD stage 3 and 4 (group D, mixed phenotype: emphysematous bronchitic) during remission were examined. Mean age was 64.78±6.9 years. The patients were divided into three groups. Group 1 included 20 COPD patients with hypoxemia who received long-term oxygen therapy (LTOT). Group 2 included 20 COPD patients with hypoxemia who did not receive LTOT. Group 3 included 20 COPD patients with normoxemia. After the primary examination, group 1 patients were prescribed with in-home LTOT (16 hours daily). Group 2 and 3 patients were not prescribed with LTOT. All patients received basic treatment, i.e., a combined drug containing an inhaled corticosteroid and β2-agonist (budesonide/formoterol 400/12 μg twice daily) and a long-acting muscarinic antagonist (tiotropium bromide 2.5 μg, 2 inhalations once daily). The patients were reexamined after 12 months. Arterial blood gases and serum NT-proBNP concentration were measured, spirometry and Doppler echocardiography were performed. Results: NT-proBNP levels in COPD patients with hypoxemia were significantly elevated. The associations between NT-proBNP and partial oxygen arterial pressure (r= -0.71, p<0.05), NT-proBNP and pulmonary artery systolic pressure (PASP) (r=0.61, p<0.05) were revealed. ROC analysis of NT-proBNP demonstrated its sensitivity and specificity in COPD patients with the different severity of respiratory failure. LTOT for COPD with hypoxemia reduces PASP, improves left ventricular diastolic function, and decreases NT-proBNP levels. Conclusions: as chronic hypoxemic respiratory failure progresses in COPD, NT-proBNP concentration increases. ROC analysis demonstrates that NT-proBNP can be considered as a promising marker of hypoxemia and pulmonary hypertension. LTOT reduces NT-proBNP levels by improving pulmonary cardiac hemodynamics. KEYWORDS: COPD, hypoxemia, natriuretic peptide, long-term oxygen therapy, pulmonary hypertension, diastolic dysfunction, right ventricle. FOR CITATION: Kuzubova N.A., Titova O.N., Volchkov V.A., Sklyarova D.B. Diagnostic importance of natriuretic peptide in chronic obstructive pulmonary disease with chronic hypoxemic respiratory failure. Russian Medical Inquiry. 2020;4(4):174–179. DOI: 10.32364/2587-6821- 2020-4-4-174-179.

PROGRESSION OF PULMONARY ALVEOLAR MICROLITHIASIS TO PULMONARY HYPERTENSION AND COR PULMONALE

SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Pulmonary alveolar microlithiasis (PAM) is a rare disease with less than 600 cases described in the literature. It is characterized by accumulation of calcium phosphate in the alveolar space. (1) Radiographic features are pathognomonic but clinical manifestations are non-specific. We present a case of PAM with progressive deterioration over 20 years leading to pulmonary hypertension (pHTN) and cor pulmonale. CASE PRESENTATION: A Caucasian female presented in her late 20s with pleurisy and based on imaging was diagnosed with sarcoidosis. She was relatively asymptomatic and went without treatment until her 40’s when she developed worsening dyspnea on exertion. PFTs showed mild restrictive lung disease (TLC 3.86L, 78% predicted) with a DLCO of 44%. CT Chest showed diffuse micronodular calcifications, consistent with PAM, not sarcoidosis. Her symptoms progressed over the next 10 years as lung function worsened, showing progressive restriction (TLC 2.97L, 57% predicted) with a DLCO of 4%. Her serial TTEs showed gradual development of right ventricular enlargement, right ventricular hypokinesis, and interventricular septum flattening. Patient underwent right heart catheterization, showing right atrial pressure 10 mmHg, pulmonary artery pressure 66/28 with mPAP 43 mmHg, LVEDP 13 mmHg and cardiac index 3.12 L/m/m2. A trial of tadalafil was attempted without improvement in symptoms and was discontinued. At age 68, the patient presented with acute on chronic hypoxemic respiratory failure with RV failure. CT Chest done demonstrated diffuse calcifications of the lung parenchyma, right ventricular septal bowing, and reflux of contrast into the hepatic veins. Despite aggressive diuresis and supportive care, the patient died from cardiopulmonary failure. DISCUSSION: This case illustrates the progression of PAM with clinical symptoms and radiographic features correlating with decline of pulmonary function, particularly restriction and diffusion capacity. Etiology of PAM remains unclear but has been linked to genetic mutations in SLC34A2 (1). Radiographic characteristics of fine sand-like micronodular calcifications, known as sandstorm, is pathognomonic. We captured the structural effect and strain on the right heart with development of pHTN, likely secondary to chronic hypoxia. Additionally, microliths can irritate alveolar walls, leading to destruction of alveolar capillaries and cause of pHTN. (2) PAM associated pHTN is rarely described as PAM is a rare entity and patients often die from respiratory failure prior to development of pHTN. Treatment is limited to case reports and is not defined (3). CONCLUSIONS: PAM associated with pHTN is infrequently described. Currently, there is still no effective treatment for PAM aside from lung transplant. The treatment of PAM associated pHTN is even more unclear and use of PAH-targeted therapy requires further studies. Reference #1: Lina M. Jönsson Å, Simonsen U, Hilberg O, Bendstrup E. Pulmonary alveolar microlithiasis: two case reports and review of the literature. European Respiratory Review. 2012;21(125):249. Reference #2: Ferreira Francisco FA, Pereira e Silva JL, Hochhegger B, Zanetti G, Marchiori E. Pulmonary alveolar microlithiasis. State-of-the-art review. Respiratory Medicine. 2013;107(1):1-9. Reference #3: Synetos A, Dilaveris P, Gialafos E, Giannopoulos G, Stefanadis C. Severe pulmonary hypertension due to pulmonary alveolar microlithiasis. International Journal of Cardiology. 2006;106(3):396-7. DISCLOSURES: No relevant relationships by Alexandra Haw, source=Web Response No relevant relationships by Ronak Shah, source=Web Response Speaker/Speaker's Bureau relationship with Bayer Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with United Therapeutics Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Gilead Please note: $1001 - $5000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Genentech Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Boehringer Ingelhein Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Honoraria Stock Ownership relationship with Pfizer Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=nothing, I purchased stock Speaker/Speaker's Bureau relationship with Portola Please note: $1001 - $5000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Honoraria Stock Ownership relationship with Portola Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=nothing, I purchased stock Stock Ownership relationship with La Jolla Pharmaceuticals Please note: $1001 - $5000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=nothing, I purchased stock Stock Ownership relationship with Seatle Genetics Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=nothing, I purchased stock PI relationship with United Therapeutics Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Grant/Research Support PI relationship with Actelion Please note: $5001 - $20000 Added 03/13/2019 by Paul Strachan, source=Web Response, value=Grant/Research Support

What's hot that the other lot got

Nasal high-flow (NHF) therapy has been shown to be a beneficial therapy for patients with acute respiratory failure in hospital and might be beneficial in patients with chronic hypoxemic respiratory...

Long-term effects of oxygen-enriched high-flow nasal cannula treatment in COPD patients with chronic hypoxemic respiratory failure.

BackgroundThis study investigated the long-term effects of humidified high-flow nasal cannula (HFNC) in COPD patients with chronic hypoxemic respiratory failure treated with long-term oxygen therapy (LTOT).Patients and methodsA total of 200 patients were randomized into usual care ± HFNC. At inclusion, acute exacerbation of COPD (AECOPD) and hospital admissions 1 year before inclusion, modified Medical Research Council (mMRC) score, St George’s Respiratory Questionnaire (SGRQ), forced expiratory volume in 1 second (FEV1), 6-minute walk test (6MWT) and arterial carbon dioxide (PaCO2) were recorded. Patients completed phone interviews at 1, 3 and 9 months assessing mMRC score and AECOPD since the last contact. At on-site visits (6 and 12 months), mMRC, number of AECOPD since last contact and SGRQ were registered and FEV1, FEV1%, PaCO2 and, at 12 months, 6MWT were reassessed. Hospital admissions during the study period were obtained from hospital records. Hours of the use of HFNC were retrieved from the high-flow device.ResultsThe average daily use of HFNC was 6 hours/day. The HFNC group had a lower AECOPD rate (3.12 versus 4.95/patient/year, p<0.001). Modeled hospital admission rates were 0.79 versus 1.39/patient/year for 12- versus 1-month use of HFNC, respectively (p<0.001). The HFNC group had improved mMRC scores from 3 months onward (p<0.001) and improved SGRQ at 6 and 12 months (p=0.002, p=0.033) and PaCO2 (p=0.005) and 6MWT (p=0.005) at 12 months. There was no difference in all-cause mortality.ConclusionHFNC treatment reduced AECOPD, hospital admissions and symptoms in COPD patients with hypoxic failure.

Multiple Segmental Lung Lavages in the Treatment of Secondary Alveolar Proteinosis

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of periodic acid-Schiff (PAS)-positive material in the lungs. We present a case of multiple segmental lung lavages (MSLL) in the treatment of secondary PAP where whole-lung lavage (WLL) was precluded due to severe refractory hypoxemia. CASE PRESENTATION: A 34-year-old man with history of myelodysplastic syndrome (MDS) status post allogeneic bone marrow transplant was admitted to the intensive care unit (ICU) due to acute on chronic hypoxemic respiratory failure. He was afebrile, hemodynamically stable, his oxyhemoglobin saturation was 92% on 10L of oxygen via non-rebreather mask, and coarse breath sounds were present bilaterally. He had a history of Mycobacterium avium complex (MAC) pulmonary disease and a recent bone marrow biopsy showed MDS recurrence with positive familial GATA2 mutation. Computer tomography (CT) of the chest showed diffuse bilateral groundglass and interstitial opacities. Except for sputum culture positive for MAC, his infectious workup was negative. He was intubated and mechanically ventilated. A bronchoalveolar lavage demonstrated a milky fluid sample with proteinaceous sediment positive for PAS, suggesting PAP. MonoMac syndrome1 was suspected given his MAC pulmonary infections, MDS/GATA2 mutation, and PAP. With severe refractory hypoxemia, WLL was precluded and MSLL were considered as an alternative. Initially, the right lower lobe was lavaged with sequential instillations of saline 35mL-aliquots until the fluid became clear. Within 24 hours, FiO2 requirements decreased from ~0.8-1.0 to 0.65. Segmental lavages of the left lower lobe, and the right upper and middle lobes were completed within the next 48 hours. Unfortunately, the patient developed multiple organ system failure (MOSF) and his family opted for comfort cares. DISCUSSION: Secondary PAP can be found in association with MonoMac syndrome. This case was a diagnostic and therapeutic challenge due to the patient’s comorbidities, decreased functional status at baseline, and atypical findings for PAP on CT of the chest. MSLL resulted in decreased oxygen requirements; however, the impact of these interventions on the final outcome was lessened by the development of MOSF in this case. CONCLUSIONS: When severe hypoxemia among patient with PAP precludes WLL as a therapeutic intervention, multiple segmental lung lavages could be an alternative treatment option, or a temporizing method allowing clinical improvement prior to WLL. Reference #1: 1.Blood.2011;118:2656-2658 DISCLOSURE: The following authors have nothing to disclose: Gustavo Cortes Puentes, Darlene Nelson, James Utz No Product/Research Disclosure Information

Lung transplantation in IIP: A review.

The idiopathic interstitial pneumonias (IIP) encompass a large and diverse subtype of interstitial lung disease (ILD) with idiopathic pulmonary fibrosis (IPF) and non-specific interstitial pneumonia (NSIP) being the most common types. Although pharmacologic treatments are available for most types of IIP, many patients progress to advanced lung disease and require lung transplantation. Close monitoring with serial functional and radiographic tests for disease progression coupled with early referral for lung transplantation are of great importance in the management of patients with IIP. Both single and bilateral lung transplantation are acceptable procedures for IIP. Procedure selection is a complex decision influenced by multiple factors related to patient, donor and transplant centre. While single lung transplant may reduce waitlist time and mortality, the long-term outcomes after bilateral lung transplantation may be slightly superior. There are numerous complications following lung transplantation including primary graft dysfunction, chronic lung allograft dysfunction (CLAD), infections, gastroesophageal reflux disease (GERD) and airway disease that limit post-transplant longevity. The median survival after lung transplantation is 4.7 years in patients with ILD, which is less than in patients with other underlying lung diseases. Although long-term survival is limited, this intervention still conveys a survival benefit and improved quality of life in suitable IIP patients with advanced lung disease and chronic hypoxemic respiratory failure.

Ambulatory Home Oxygen: What Is the Evidence for Benefit, and Who Does It Help?

The beneficial effects of ambulatory home oxygen have been demonstrated since the 1950s, when Cotes and Gibson gave oxygen to ambulatory COPD patients from small portable high pressures cylinders in the United Kingdom. Over the ensuing 7 decades, oxygen has been prescribed to millions of COPD patients in the home setting. Additionally, it is common clinical practice to prescribe supplemental oxygen when chronic hypoxemic respiratory failure not due to COPD (eg, interstitial lung disease, pulmonary hypertension, kyphoscoliosis, and cystic fibrosis) is present or in patients with hypoxemia at hospital discharge following flares of their underlying chronic respiratory disorder, without any substantial evidence. Despite the importance of long-term oxygen therapy in clinical home management, there are many gaps in our current knowledge regarding its mechanisms of action, indications for prescription, and its effects on important patient outcomes. Research conducted in the 1970s and 1980s still provides the basis for clinical decision making and insurance coverage policies regarding long-term oxygen administration. Remarkably, little current research is being conducted to extend our knowledge regarding the indications, mechanisms, and benefits of long-term oxygen therapy. This review will focus on our current knowledge of the end points for supplemental oxygen at home, such as mortality, effects on functional performance, sensation of dyspnea, cognitive function, and quality of life, and highlight areas where future research is needed.

Success in Pulmonary Rehabilitation in Patients with Chronic Obstructive Pulmonary Disease

Pulmonary rehabilitation (PR) is beneficial for some, but not all, patients with chronic lung disease. To determine the success rate of a comprehensive PR program for patients with chronic obstructive pulmonary disease (COPD) and to characterize the differences between responders and nonresponders. A chart review was performed on patients with a clinical diagnosis of COPD who were referred for PR. Success was defined according to clinically important changes in St George's Respiratory Questionnaire scores and⁄or 6 min walk test distance. The majority of subjects were men (58%) with a mean (± SD) age of 69±10 years (n=177). Sixty-two per cent of participants had a successful outcome with PR, with proportionally more responders noting subjective improvement than objective improvement on a 6 min walk test (73% versus 51%). Subjects with poor baseline St George's Respiratory Questionnaire scores tended to improve the most (P=0.011 [ANOVA]). Successful participants had a greater forced expired volume in 1 s (1.1 L versus 0.9 L; P<0.05) and a lower BODE index (body mass index, airflow obstruction, dyspnea, and exercise capacity index) at baseline (9.6 versus 10.3; P<0.05). Success of PR was not correlated with age, sex, chronic hypoxemic respiratory failure or other chronic conditions. Successful participants were more likely to be compliant and to experience fewer adverse events (P≤0.001). Our study reinforced the belief that the majority of participants with COPD benefit from PR. Few baseline characteristics were predictive of success. Subjectively measured improvement occurred more frequently than objectively measured improvement and was greatest in those with the poorest baseline values.

Transtracheal High-Flow Insufflation Supports Spontaneous Respiration in Chronic Respiratory Failure

Transtracheal insufflation of oxygen-enriched air at a high flow rate has been proposed to support ventilation. The purpose of this study was to investigate the physiologic effects of high-flow insufflation unobtrusively with a respiratory inductive plethysmograph in patients with chronic respiratory failure. Using a respiratory inductive plethysmograph also permitted monitoring of end-expiratory lung volume, and respiratory variables could be quantified independently of the tracheal bias flow. Prospective randomized comparison of low-flow vs high-flow transtracheal insufflation. Pulmonary division of a tertiary teaching hospital. Fourteen spontaneously breathing outpatients with chronic hypoxemic respiratory failure carrying a transtracheal catheter for long-term oxygen therapy. Oxygen-enriched air (fraction of inspired oxygen, 0.37) at 15 L/min and oxygen at 1.5 L/min were transtracheally administered for 1 h each. The breathing pattern and the end-expiratory lung volume were monitored by inductive plethysmography along with pulse oximetry and transcutaneous PCO2. Arterial blood gases were also analyzed at the end of the hour of both low-flow and high-flow insufflation. High-flow insufflation decreased the mean (+/- SEM) minute ventilation (Ve) by 20% from 8.37 +/- 0.49 to 6.66 +/- 0.57 L/min, the mean respiratory rate from 19.2 +/- 0.9 to 15.7 +/- 1.0 breaths/min, while mean expiratory time increased from 2.0 +/- 0.1 to 2.8 +/- 0.2 s, and end-expiratory lung volume decreased by 0.55 +/- 0.15 L compared to low-flow oxygen insufflation (p < 0.05 for all comparisons). Mean arterial and transcutaneous PCO2 decreased from 45 +/- 1 to 43 +/- 1 mm Hg and from 54 +/- 2 to 53 +/- 2 mm Hg, respectively (p < 0.05 in both instances), while arterial PaO2 and oxygen saturation did not change. High-flow transtracheal insufflation of oxygen-enriched air assists ventilation by reducing Ve without compromising gas exchange and by reducing end-expiratory lung volume, possibly through the reversal of dynamic hyperinflation.