Outcome after unilateral lung volume reduction surgery in patients with severe emphysema

Objective: Bilateral lung volume reduction surgery (LVRS) has emerged as a palliative treatment option in patients with severe pulmonary emphysema. However, it is not known if a sustained functional improvement can be obtained using an unilateral approach. Methods: We hypothesized that a palliative effect can also be obtained by unilateral LVRS and prospectively assessed lung function, walking distance, and dyspnea before and 3, 6, 12, 18, 24 and 36 months after unilateral LVRS. Results: Twenty-eight patients were operated by the use of video-assisted thoracoscopic surgery (VATS) with a mean follow-up of 16.5 months (range 3-36 months). Forced expiratory volume in 1 s (FEV1) was significantly improved up to 3 months (1007±432 compared to 1184±499 ml, P≪0.001), residual volume up to 24 months (4154±1126 compared to 3390±914 ml, P≪0.01), dyspnea up to 12 months (modified Borg dyspnea scale 6.6±1.8 compared to 3.9±1.8, P=0.01) and walking distance up to 24 months (343±107 compared to 467±77 m, P≪0.05) after unilateral LVRS compared to preoperative values. Overall, 25 of 28 patients reported a subjective benefit after unilateral LVRS. There was no 30-day mortality. Only two patients required surgery on the contralateral side after 4.5 and 6 months, respectively, both suffering from α-1-antitrypsin deficiency. Conclusions: Unilateral LVRS by the use of VATS results in a sustained beneficial effect, improving walking distance and dyspnea for up to 24 months in patients with severe emphysema. The preservation of the contralateral side for future intervention if required renders unilateral LVRS an attractive concept in this difficult palliative situatio

Anti-IgLON5 Disease: A New Bulbar-Onset Motor Neuron Mimic Syndrome

OBJECTIVE: To expand the spectrum of anti-IgLON5 disease by adding 5 novel anti-IgLON5-seropositive cases with bulbar motor neuron disease-like phenotype. METHODS: We characterized the clinical course, brain MRI and laboratory findings, and therapy response in these 5 patients. RESULTS: Patients were severely affected by bulbar impairment and its respiratory consequences. Sleep-related breathing disorders and parasomnias were common. All patients showed clinical or electrophysiologic signs of motor neuron disease without fulfilling the diagnostic criteria for amyotrophic lateral sclerosis. One patient regained autonomy in swallowing and eating, possibly related to immunotherapy. CONCLUSION: IgLON5 disease is an important differential diagnosis to evaluate in patients with bulbar motor neuron disease-like phenotype and sleep disorders. There is need for a deeper understanding of the underlying pathobiology to determine whether IgLON5 disease is an immunotherapy-responsive condition

Influence of bolus consistency and position on esophageal high-resolution manometry findings

BACKGROUND: Conventional esophageal manometry evaluating liquid swallows in the recumbent position measures pressure changes at a limited number of sites and does not assess motility during solid swallows in the physiologic upright position. AIM: To evaluate esophageal motility abnormalities during water and bread swallows in the upright and recumbent positions using high-resolution manometry (HRM). METHODS: Thirty-two-channel HRM testing was performed using water (10 ml each) and bread swallows in the upright and recumbent positions. The swallows were considered normal if the distal peristaltic segment >30 mmHg was >5 cm, ineffective if the 30-mmHg pressure band was 8 cm/s. Abnormal esophageal manometry was defined as the presence of > or =30% ineffective and/or > or =20% simultaneous contractions. RESULTS: The data from 96 patients (48 F; mean age 51 years, range 17-79) evaluated for dysphagia (56%), chest pain (22%), and gastroesophageal reflux disease (GERD) symptoms (22%) were reviewed. During recumbent water swallows, patients with dysphagia, chest pain, and GERD had a similar prevalence of motility abnormalities. During upright bread swallows, motility abnormalities were more frequent (p = 0.01) in patients with chest pain (71%) and GERD (67%) compared to patients with dysphagia (37%). CONCLUSIONS: Evaluating bread swallows in the upright position reveals differences in motility abnormalities overlooked by liquid swallows alone

Lung adenocarcinoma with BRAF G469L mutation refractory to vemurafenib.

BRAF V600E is an emerging drug target in lung cancer, but the clinical significance of non-V600 BRAF mutations in lung cancer and other malignancies is less clear. Here, we report the case of a patient with metastatic lung adenocarcinoma with BRAF G469L mutation refractory to vemurafenib. We calculated a structure model of this very rare type of mutated BRAF kinase to explain the molecular mechanism of drug resistance. This information may help to develop effective targeted therapies for cancers with non-V600 BRAF mutations