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Furthermore, a significant positive correlation with the dose of prednisolone and mycophenolate mofetil was found. Conclusions TTV levels show an association/correlation with the strength of IS. Further studies are needed in order to evaluate TTV measurement as a tool for IS monitoring for hard clinical outcomes such as presence of donor-specific antibodies, rejections or infections-common consequences of insufficient or too intense IS.Objective Dyspnea can be present as non-motor symptom in patients with Parkinson's disease (PD). Deep brain stimulation (DBS) of the subthalamic nucleus (STN) improves motor and non-motor symptoms in PD. GSK2606414 datasheet However, new-onset dyspnea has been reported after DBS surgery. We have studied respiratory characteristics of PD patients with bilateral STN-DBS to assess the impact of DBS on pulmonary function. Methods STN-DBS PD patients with dyspnea after surgery (cases) were matched with STN-DBS PD patients without dyspnea (controls). Motor and pulmonary function were assessed with stimulation and without medication (on stim/off med), and without stimulation and medication (off stim/off med). Pulmonary function was investigated with spirometry and dyspnea with the Medical Research Council Dyspnea Scale (MRCDS) and the Borg Scale (BS). Results Seven cases (five men, 58.30 ± 6.70 years of age) and seven controls (six men, 61.10 ± 6.30 years of age) were enrolled. MRCDS and BS revealed the presence of dyspnea in both groups. No significant changes in pulmonary function were found in both cases and controls in on stim/off med vs. off stim/off med condition (p less then 0.05), and in cases vs. controls in on stim/ off med condition (p less then 0.05). Conclusions No impact of STN-DBS on pulmonary function was found in cases. Impaired perception of dyspnea and spread of stimulation surrounding the STN might account for new-onset dyspnea after DBS surgery. Dyspnea was detected also in controls using ad hoc questionnaires. Our findings suggest further investigation of this non-motor symptom in PD patients.Objective To examine respiratory muscle function among late-onset Pompe disease (LOPD) patients in the Pompe Registry (NCT00231400/Sanofi Genzyme) during enzyme replacement therapy (ERT) with alglucosidase alfa by assessing the longitudinal course of forced vital capacity (FVC), prognostic factors for FVC, and impact of time from diagnosis to ERT initiation. Methods Longitudinal FVC data from LOPD (symptom onset > 12 months or ≤ 12 months without cardiomyopathy) patients were analyzed. Patients had to have baseline FVC (percent predicted upright) assessments at ERT start and ≥ 2 valid post-baseline assessments. Longitudinal analyses used linear mixed-regression models. Results Among 396 eligible patients, median baseline FVC was 66.9% (range 9.3-126.0). FVC remained stable during the 5-year follow-up (slope = - 0.17%, p = 0.21). Baseline FVC was lower among various subgroups, including patients who were male; older at ERT initiation; had a longer duration from symptom onset to ERT initiation; and had more advanced disease at baseline (based on respiratory support use, inability to ambulate, ambulation device use). Age at symptom onset was not associated with baseline degree of respiratory dysfunction. Differences between subgroups observed at baseline remained during follow-up. Shorter time from diagnosis to ERT initiation was associated with higher FVC after 5 years in all patients and the above subgroups using a cut-off of 1.7 years. Conclusion FVC stability over 5 years suggests that respiratory function is preserved during long-term ERT in real-world settings. Early initiation of alglucosidase alfa was associated with preservation of FVC in LOPD patients with better respiratory function at the time of treatment initiation.The article "Inflammation is a target of medical treatment for lower urinary tract symptoms associated with benign prostatic hyperplasia", written by Cosimo De Nunzio, Andrea Salonia, Mauro Gacci and Vincenzo Ficarra was originally published electronically on the publisher's internet portal on 14 February 2020 without open access.Chronic granulomatous disease (CGD) is a primary immunodeficiency of phagocyte function due to defective NADPH oxidase (phox). Compared with the common types of CYBB/gp91phox, NCF1/p47phox, and CYBA/p22phox deficiency, NCF4/p40phox deficiency is a mild and atypical form of CGD without invasive bacterial or fungal infections. It can be diagnosed using serum-opsonized E.coli as a stimulus in dihydrorhodamine (DHR) assay. Patients with CYBC1/Eros deficiency, a new and rare form of CGD, present as loss of respiratory burst and gp91phox expression in phagocytes. Neutrophils from patients with CGD are deficient in neutrophil extracellular traps (NETosis), autophagy, and apoptosis. The hyper-activation of NF-ĸB and inflammasome in CGD phagocytes also lead to long-lasting production of pro-inflammatory cytokines and inflammatory manifestations, such as granuloma formation and inflammatory bowel disease-like colitis. Patients with CGD and X-linked female carriers also have a higher incidence of autoimmune diseases. Th nucleases is a promising approach for patients with CGD in the future.Background Superior medullary velum cerebral cavernous malformations pose a challenge in terms of appropriate microsurgical approach. Safe access to this deep location as well as preservation of surrounding anatomical structures, in particular the superior cerebellar peduncle just lateral to the superior medullary velum and the dentate nuclei, is paramount to achieve a good functional outcome. Methods Cadaveric dissections provide useful knowledge of the normal anatomy while tractography allows a better understanding of the individual anatomy in the presence of a lesion. The medial-tonsillar telovelar approach provides a feasible corridor for accessing superior velum cerebral cavernous malformations without compromising the fibres contained in the superior cerebellar peduncle. The major cerebellar efferents-cerebello-rubral, cerebello-thalamic and cerebello-vestibular tracts-and afferents, anterior spinocerebellar, tectocerebellar and trigeminocerebellar tracts, within the superior cerebellar peduncle are preserved, and the dentate nuclei are not affected.