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Background Repeated ghrelin administration leads to improvements in symptoms, muscle wasting and exercise tolerance in cachectic patients with pulmonary disease. We investigated the optimal ghrelin dose for underweight patients with chronic respiratory failure. Methods In this multicenter, randomized, dose-comparison exploratory study, 44 cachectic patients with chronic respiratory failure were randomly assigned pulmonary rehabilitation with intravenous twice-daily administration of 1 or 2 μg/kg ghrelin for 3 weeks. The primary endpoint was improvement in 6-min walking distance (6MWD). The secondary endpoint was change in peak O.sub.2. Results Twenty-one patients were assigned to the 1 μg/kg ghrelin group and 23 to the 2 μg/kg ghrelin group. Change from baseline 6MWD after treatment was similar between groups(1 μg/kg: 53.9 m, 2 μg/kg: 53.9 m, p = 0.99). Mean change in peak O.sub.2 was significantly greater in the 2 μg/kg group (63.1 ml/min) than in the 1 μg/kg group (-63.8 ml/min, p = 0.048). Food intake and lean body mass significantly increased in both groups, and the St. George Respiratory Questionnaire score, body weight, and body mass index were remarkably improved in only the 2 μg/kg group, although there was no significant difference between groups. No treatment-related serious events were reported for either group. Conclusion Improvements in the oxygen uptake capacity were greater in patients receiving 2 μg/kg ghrelin twice daily for 3 weeks than in those receiving 1 μg/kg, although exercise tolerance was similar between groups at the end of the 3-week treatment period. Thus, a twice daily dose of 2 μg/kg ghrelin is recommended over 1 μg/kg ghrelin for patients with chronic respiratory failure and weight loss. Electronic supplementary material The online version of this article (doi:10.1007/s00408-015-9685-y) contains supplementary material, which is available to authorized users., Author(s): Nobuhiro Matsumoto[sup.1] , Keisuke Miki[sup.2] , Hironobu Tsubouchi[sup.1] , Akihiro Sakamoto[sup.1] , Yasuji Arimura[sup.1] , Shigehisa Yanagi[sup.1] , Hirotoshi Iiboshi[sup.1] , Makoto Yoshida[sup.3] , Ryosuke Souma[sup.4] , Hiroshi Ishimoto[sup.5] [...]

We report a rare case of desquamative interstitial pneumonia (DIP) with diffuse alveolar haemorrhage (DAH). A 56-year-old man diagnosed with DIP by surgical lung biopsy 2 years ago was admitted to our hospital because of severe acute respiratory failure. The DIP had progressed despite smoking cessation. On admission, the patient appeared extremely ill, and physical examination revealed respiratory distress. The patient required mechanical ventilation. High-resolution computed tomography showed diffuse g round glass opacity in both lungs. The bronchoalveolar lavage fluid was bloody, and numerous haemosiderin-laden alveolar macrophages were detected. Pulse steroid therapy followed by oral prednisolone immediately relieved the respiratory failure and improved the long-term control of the DIP. Paired sera tests confirmed the diagnosis of influenza A/H3N2 virus infection, which was the cause of the DAH. Chronically progressive DIP with acute respiratory failure due to DAH was successfully treated by steroid therapy. [ABSTRACT FROM AUTHOR]

Invasive mucinous adenocarcinoma (IMA) of the lung is a chemo-refractory type of lung cancer with frequent intrapulmonary dissemination. Patients with IMA of the lung often suffer from a productive cough and rapid deterioration of performance status (PS). There is currently no standard therapeutic strategy against this unrelenting condition. Here we report a patient with bilateral IMA of the lung with severe productive cough and dyspnea successfully controlled by palliative lung lobectomy. A 67-year-old Japanese man presented with a 3-month history of productive cough. Chest computed tomography (CT) revealed a mass lesion in the left lower lobe and a small nodule and multiple thin-walled cystic lesions in the right lung. He was diagnosed with stage IIB IMA of the lung. Over the next two weeks, his productive cough and dyspnea drastically worsened and his PS declined from 0 to 4. Chest CT showed increases in size of both the nodule and cystic lesions in the right lung and the mass lesion in the left lower lobe. He was re-diagnosed as stage IVA. Given the extreme heterogeneity of the tumor distribution, we decided to perform palliative resection of the left lower lobe. After the surgery, he experienced complete relief of respiratory symptoms, and his PS improved dramatically, enabling chemotherapy. Thirty-one months after surgery, he maintains good PS. In conclusion, our report suggests that aggressive introduction of palliative lung lobectomy played a substantial role for in the excellent outcome of our patient with relatively well confined, advanced-stage IMA. [ABSTRACT FROM AUTHOR]

Acute lung injury (ALI) is a critical syndrome consisting of acute respiratory failure associated with extensive pulmonary infiltrates. The pathological characterization of ALI includes injuries of alveolar epithelial cells (AECs), alveolar neutrophilic infiltration, and increases in proinflammatory cytokines, which cause destruction of the alveolar capillary barrier and subsequent devastating lung fibrosis. Rikkunshito (RKT), a traditional Japanese herbal medicine, is widely used for the treatment of patients with gastrointestinal symptoms and is known to stimulate ghrelin secretion. The therapeutic effects of RKT on organ inflammation and fibrosis remain unknown. We investigated the pharmacological potential of RKT in the treatment of ALI by using a bleomycin-induced ALI model in mice. RKT or distilled water (DW) was given to mice daily starting 12 h after bleomycin administration. The RKT-treated mice showed a definitively higher survival rate than the DW-treated mice after injury. They also had smaller reductions in body weight and food intake. The amelioration of neutrophil alveolar infiltration, pulmonary vascular permeability, induction of proinflammatory cytokines, activation of the NF-κB pathway, apoptosis of AECs, and subsequent lung fibrosis were notable in the RKT-treated mice. RKT administration increased the plasma ghrelin levels in wild-type mice, and it also mitigated the ALI response in both ghrelin-deficient mice and growth hormone secretagogue receptor-deficient mice after lung injury. Our results indicate that RKT administration exerts protective effects against ALI by protecting the AECs and regulating lung inflammation independently of the ghrelin system, and they highlight RKT as a promising therapeutic agent for the management of this intractable disease.

A 77-year-old woman presented with a 3-month history of right chest pain and a low-grade fever. Right pleural effusion had been detected at another hospital. Her chest CT scan revealed right pleural effusion, right pleural thickening, and bilateral multiple lung nodules. No specific findings were obtained from an examination of the pleural effusion. Thoracoscopic pleural and lung biopsies were conducted. Histologically, the tumor had an infiltrative growth pattern in the fibrously-thickened parietal pleura, visceral pleura, and lung parenchyma. The tumor was composed of epithelioid and spindle cells, and in some sections, the tumor cells had intracytoplasmic vacuoles, and had formed an immature vascular lumen. Proliferation in a papillary fashion in the alveolar spaces and vascular involvement of tumor were also seen. Immunohistochemically, the tumor cells were positive for factor VIII-related antigen, CD31, and CD34, and negative for calretinin and WT-1. The tumor was therefore diagnosed as pulmonary epithelioid hemangioendothelioma (PEH), which is a rare, low-to-moderate grade vascular tumor of the lung. This disease should be included in the differential diagnosis together with malignant pleural mesothelioma, in cases demonstrating unusual pleural thickening.