Lung cancer in a CF patient: combination of bad luck or is there more to say?

Patients with cystic fibrosis have increased risk for gastrointestinal cancer, lymphoid leukemia and testicular carcinomas. Chronic inflammation does not seem to be the only contributing factor. Mutations and epigenetic alterations in the CFTR gene may alter susceptibility to develop cancer. Lung cancer is up to now not frequently observed in CF patients. In lung cancer patients without CF low CFTR expression is significantly associated with advanced staging, lymph node metastasis. As the management and life expectancy of patients with cystic fibrosis have improved substantially in recent years, we expect an increased number of these patients diagnosed with lung cancer. In addition, it is possible that they, as a result of CFTR-dysfunction, will present with more aggressive lung tumors. Treating cancer in CF patients is a challenge because of multi-organ involvement and chronic colonization by resistant pathogens. The effectiveness and safety of immunotherapy in this population needs to be further evaluated.

Chronic oral corticosteroids use and persistent eosinophilia in severe asthmatics from the Belgian severe asthma registry.

BACKGROUND: Severe asthma (SA) may require frequent courses or chronic use of oral corticosteroids (OCS), inducing many known side effects and complications. Therefore, it is important to identify risk factors of chronic use of OCS in SA, considering the heterogeneity of clinical and inflammatory asthma phenotypes. Another aim of the present analysis is to characterize a subpopulation of severe asthmatics, in whom blood eosinophil counts (BEC) remain elevated despite chronic OCS treatment. METHODS: In a cross-sectional analysis of 982 SA patients enrolled in the Belgian Severe Asthma Registry (BSAR) between March 2009 and February 2019, we investigated the characteristics of the OCS treated patients with special attention to their inflammatory profile. RESULTS: At enrollment, 211 (21%) SA patients were taking maintenance OCS (median dose: 8 [IQR: 5-10]) mg prednisone equivalent). BEC was high (> 400/mm3) in 44% of the OCS treated population. Multivariable logistic regression analysis showed that risk factors for chronic use of OCS in SA were late-onset asthma (i.e. age of onset > 40 yr), frequent exacerbations (i.e. ≥2 exacerbations in the previous year) and non-atopic asthma. Late-onset asthma was also a predictor for persistently high BEC in OCS treated SA patients. CONCLUSION: These data showed a significant association between a persistently high BEC and late-onset asthma in OCS treated SA patients. Whether it is poor compliance to treatment or corticosteroid insensitivity the reasons for this association warrants further investigation.

Impact of a single 10 min education session on asthma control as measured by ACT.

BACKGROUND: Aim: Despite the availability of effective treatments for asthma, many patients still suffer from uncontrolled asthma. This study evaluates whether a single educational session could improve asthma control assessed by Asthma Control Test (ACT) score as well as knowledge of the inhaler device, knowledge of medication and inhalation technique. METHODS: This prospective single blinded randomized controlled trial of 160 adults with asthma, examined the effectiveness of a single standardized, educational intervention, performed by a respiratory nurse specialist. The education provided to the intervention group consisted of basic information about asthma treatment and instructions on inhalation technique for about 10min. This additional education was not offered to the control group. In both groups ACT scores, knowledge of medication, knowledge of inhaler device and inhalation technique were assessed at baseline and after three months. Asthma was considered well-controlled when the ACT score exceeded 19. RESULTS: At baseline there were no significant differences in patient demographics, degree of asthma control, knowledge of medication or device and inhalation technique between the intervention group and the control group. In the intervention group the educational session resulted in a significantly higher proportion of well-controlled asthma patients with an ACT>19 (43% versus 77%) (p < 0.001) after three months. In the control group this proportion remained similar (57% versus 67%) (p > 0.1). We also observed improvements in knowledge of medication (p < 0.001), knowledge of device (p < 0.001) and inhalation technique (p = 0.004) in the intervention group and not in the control group. CONCLUSION: A single 10 min, educational session provided by a respiratory nurse specialist can substantially improve asthma control determined by the ACT score after three months.

Whole-lung lavage: a successful treatment for restoring acinar ventilation distribution in primary acquired pulmonary alveolar proteinosis.

A 51-year-old active smoker with primary acquired pulmonary alveolar proteinosis (PAP) diagnosed by biopsy and anti-GM-CSF antibodies was treated safely with whole-lung lavage (WLL). This resulted in a rapid improvement of symptoms and arterial blood oxygenation, but not of standard lung function parameters. However, we also performed the multiple-breath nitrogen washout (MBW) test to determine the lung clearance index (LCI) as well as indices of acinar ventilation heterogeneity (S(acin)) and conductive ventilation heterogeneity (S(cond)). At baseline, a distinct abnormality was seen for S(acin) and LCI, while S(cond) was at the upper limit of normal for this subject. S(acin), in particular, was in excess of the S(acin) abnormality corresponding to a 20-pack-year smoking history. Immediately after WLL, S(acin) and S(cond) both fell to within a normal range while LCI also decreased but remained abnormal. The S(acin) decrease was much greater than the S(cond) decrease, which was to be expected after 1 week of smoking cessation at the hospital (smoking was resumed after release from hospital). A follow-up visit 7 weeks after WLL revealed a spectacular improvement on CT scan and improvements in standard lung function. Another follow-up visit 14 weeks after WLL showed further improvements in standard lung function, and both S(acin) and S(cond) remained well within the normal range, and LCI was above the upper limit of normal. We conclude that in this patient, removal of excess surfactant by WLL resulted in a restored ventilation distribution in most of the distal air spaces.

Primary tracheobronchial amyloidosis: a series of 3 cases.

Primary tracheobronchial amyloidosis is a form of localized pulmonary amyloidosis, characterised by the deposition of AL-amyloid in trachea and bronchi. It is a rare and slowly progressive disease, usually requiring repeated endoscopic treatment. In this case series we describe symptoms, diagnostic and therapeutic procedures, radiological findings and pulmonary function testing in 3 cases of different presentation and severity. Two patients were treated by endoscopic debulking and stent placement during rigid bronchoscopy, both with excellent clinical and functional results. In one of these patients regular endoscopic and clinical control exams were performed in the 5 years following the initial treatment, showing stable disease, requiring no further therapeutic intervention until today.

Antioxidants reverse the antiproliferative effect of high glucose and advanced glycosylation end products in cultured rat mesangial cells.

High glucose and elevated levels of advanced glycosylation end products (AGEs) exert an antiproliferative effect on cultured mesangial cells. In view of the role of oxygen free radicals in the pathogenesis of diabetic nephropathy, we tested whether two endogenous antioxidants, taurine and vitamin E, ameliorate the effects of an elevated ambient glucose and/or AGEs on mesangial cell growth in vitro. Regardless of whether cell proliferation was assayed by the incorporation of [3H]thymidine, direct cell counting or bromodeoxyuridine (BrdU) cell staining, both taurine and vitamin E reversed the inhibitory effect of high glucose and AGEs on mesangial cell growth. In conjunction with our previous studies indicating that taurine and vitamin E reduce collagen production in mesangial cells exposed to high glucose, these findings suggest that endogenous antioxidants attenuate diabetic glomerulosclerosis by interfering with the bioactivation of transforming growth factor-6.