A plasmapheresis protocol for refractory pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of proteinaceous material within the lungs. While typically managed with whole lung lavage (WLL), more recent PAP therapies aimed at reducing granulocyte-macrophage colony stimulating factor autoantibodies (anti-GM-CSF) have reduced symptoms and improved lung function. We present a patient with PAP refractory to WLL, exogenous GM-CSF and rituximab who underwent a novel plasmapheresis protocol as a therapeutic trial. While previously reported regimens have utilized plasmapheresis sessions distributed over months, our patient underwent five consecutive days of plasmapheresis, followed by rituximab. Anti-GM-CSF levels decreased from 24.8 to 2.7 mcg/mL post-plasmapheresis. This reduction of autoantibody correlated with reduction in WLL frequency, increase in diffusing capacity for carbon monoxide, and subjective improvement in dyspnea. Our case suggests that five consecutive days of plasmapharesis results in increased clearance of anti-GM-CSF and may be potentially efficacious in cases of refractory PAP.

A rare occurrence of pulmonary alveolar proteinosis after lung transplantation.

We present a case of pulmonary alveolar proteinosis (PAP) initially diagnosed 28 months after left single-lung transplantation for idiopathic pulmonary fibrosis. The diagnosis was based upon the presence of periodic acid-Schiff (PAS)-positive and surfactant immunostain-positive acellular lipoproteinaceous material within alveoli seen on transbronchial biopsy as well as in bronchoalveolar lavage fluid. The patient eventually also displayed a characteristic "crazy paving" pattern on radiographic imaging. Granulocyte macrophage-colony stimulating factor antibodies were negative, consistent with secondary PAP. PAP is a rare interstitial lung disease with only a few reported cases occurring after lung transplantation. The etiology is thought to be related to a defect in macrophage function caused by immunosuppression. Reduced immunosuppression has been associated with stabilization, but not reversal, of the condition in the case reported here. PAP is an exceptionally rare cause of dyspnea and radiographic infiltrates after lung transplantation and may be related to toxicity of immune-suppressive medications.

Noninvasive ventilation in difficult endotracheal intubation: systematic and review analysis.

Noninvasive ventilation has been widely used in the management of acute respiratory failure in appropriate clinical settings. In addition to known benefit of alleviating the need for invasive mechanical ventilation, recent literature suggested its beneficial use in the process of endotracheal intubation. Search of the PubMed database and manual review of selected articles investigating the methods and outcomes of endotracheal intubation in difficult airway due to hypoxemic respiratory failure and the role of noninvasive ventilation in this process. Large randomized controlled studies focused on alternative approaches to endotracheal intubation in severe hypoxemic respiratory failure are largely missing but there are several retrospective cohort analysis and reports describing the novel technique describing the application of noninvasive ventilation during endotracheal intubation. Noninvasive ventilation can be used as an adjunct intervention that may maintain oxygenation and ventilation, prevent significant hemodynamic instability and provide a pneumatic stent to maintain upper airway patency, thus reducing the risks of intubation-related complications.

A 26-Year-Old Woman With a Small Right Lung and a Right-Sided Heart.

A 26-year-old woman presented with abnormal findings on a chest radiograph. She had no significant history other than a fever 4 months prior to presentation that had resolved without a definite cause identified. She denied cough, shortness of breath, chest pain, history of smoking, environmental exposures, or prior pregnancies. She remained physically active.

A 34-Year-Old Woman With Recurrent Right-Sided Chest Pain and Dyspnea.

A 34-year-old woman presented with her third episode of acute-onset right-sided chest pain and dyspnea. She had two prior similar occurrences of right-sided sharp, pleuritic chest pain with radiation to the back and dyspnea. Chest radiographs during these presentations revealed a small apical right-sided pneumothorax that was managed conservatively with high-flow oxygen. All three presentations were associated with vigorous exercise and the first day of her menses. She denied cough, hemoptysis, fever, smoking history, airplane travel, scuba diving, or trauma during these presentations. The patient has been trying to conceive for the past year but has been unsuccessful because of uterine fibroids but no history of endometriosis.

A 44-year-old man with bilateral pneumothorax.

A 44-year-old man presented with a 3-day history of persistent upper-back pain, chest discomfort, and dyspnea. He denied any precipitating events such as trauma or vigorous activity before the presentation of symptoms. His exercise capacity had been excellent. He is a lifetime nonsmoker and never had significant lung problems apart from intermittent asthma for which he had several ED visits in the past. Chest CT scan performed during an asthma exacerbation 2 years earlier demonstrated two left-side lung blebs. He had no prior surgical procedures.

A 65-year-old man with persistent cough and large nodular opacity.

A 65-year-old Asian man with a history of chronic hepatitis B infection presented to our pulmonary clinic for second opinion of his chronic, persistent, nonproductive cough. He was evaluated 10 months earlier with chest CT scan, which revealed a large lingular nodular opacity that was diagnosed as nodular cryptogenic organizing pneumonia by CT scan-guided percutaneous lung biopsy. Systemic corticosteroids were initiated and continued over the next 10 months. The dry cough persisted, and he developed intermittent left-sided pleuritic chest pain. He denied fevers, night sweats, hemoptysis, weight loss, or dyspnea. He was a lifelong nonsmoker and moved to the United States from China during childhood.

A 55-year-old man with a small right lung and a right-sided heart.

A healthy 55-year-old man without known medical problems presented for a routine physical examination and was found to have an abnormal ECG. He denied chest pain, dyspnea, palpitations, dizziness, or syncopal episodes. He also denied orthopnea, paroxysmal nocturnal dyspnea, and lower-extremity edema. His exercise capacity had been excellent. He was a lifelong nonsmoker and never had lung problems.

A 22-Year-Old Nonsmoker With Diffuse Cystic Lung Disease.

A 22-year-old previously healthy woman was evaluated in pulmonary clinic for shortness of breath and cough that had been slowly progressive over 3 months. She otherwise reported being fully functional and attended her college graduation a week prior to evaluation. She had no history of smoking, illicit drug use, connective tissue disease, or noxious exposures.

Hypocretin Deficiency Associated with Narcolepsy Type 1 and Central Hypoventilation Syndrome in Neurosarcoidosis of the Hypothalamus.

We report a case of a 53-year-old man presenting with depressed alertness and severe excessive sleepiness in the setting of neurosarcoidosis. Neuroimaging demonstrated hypothalamic destruction due to sarcoidosis with a CSF hypocretin level of 0 pg/mL. The patient also experienced respiratory depression that presumably resulted from hypocretin-mediated hypothalamic dysfunction as a result of extensive diencephalic injury. This is a novel case, demonstrating both hypocretin deficiency syndrome, as well as respiratory dysfunction from destruction of hypocretin neurons and extensive destruction of key diencephalic structures secondary to the underlying neurosarcoidosis.

The use of a home exercise program based on a computer system in patients with chronic obstructive pulmonary disease.

PURPOSE: To test the effectiveness of a home exercise program based on a user-friendly, computer system, the Nintendo Wii Fit. METHODS: In this longitudinal study, 25 clinically stable patients with chronic obstructive pulmonary disease began a 6-week nonintervention (baseline) period followed by 12 weeks of Wii exercise training at home. Patients were instructed to exercise 5 or more days per week. Exercise capacity, health status, and dyspnea were evaluated after home exercise training. RESULTS: Evaluable data were available in 20 patients after home exercise training; their force expiratory volume in 1 second was 45 ± 16%. Following 12 weeks of Wii exercise training, the Endurance Shuttle Walk Test increased by 131 ± 183 seconds over the baseline determination (P = .005). Significant improvements were also noted in arm-lift and sit-to-stand repetitions, the total score, and the emotion dimension of the Chronic Respiratory Questionnaire. Men had significantly greater increases in the Endurance Shuttle Walk Test than women, although their self-reported exercise durations were similar. There were no significant adverse outcomes. CONCLUSION: This study suggests that 12 weeks of regular, home exercise based on an interactive entertainment computer system can lead to positive short-term outcomes.