scholarly journals Risk factors for lung disease progression in children with cystic fibrosis

2018 ◽  
Vol 51 (6) ◽  
pp. 1702509 ◽  
Author(s):  
Marieke van Horck ◽  
Kim van de Kant ◽  
Bjorn Winkens ◽  
Geertjan Wesseling ◽  
Vincent Gulmans ◽  
...  

To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009–2014).Lung disease progression was expressed as a decline in lung function (forced expiratory volume in 1 s (FEV1) % pred) and pulmonary exacerbation rate. Potential risk factors at baseline included sex, age, best FEV1 % pred, best forced vital capacity % pred, genotype, body mass index z-score, pancreatic insufficiency, medication use (proton pump inhibitors (PPIs), prophylactic antibiotics and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis and colonisation with Pseudomonas aeruginosa.The data of 545 children were analysed. PPI use was associated with both annual decline of FEV1 % pred (p=0.017) and future pulmonary exacerbation rate (p=0.006). Moreover, lower FEV1 % pred at baseline (p=0.007), prophylactic inhaled antibiotic use (p=0.006) and pulmonary exacerbations in the baseline year (p=0.002) were related to pulmonary exacerbations in subsequent years.In a cohort of Dutch children with CF followed for 5 years, we were able to identify several risk factors for future exacerbations. In particular, the association between PPI use and lung disease progression definitely requires further investigation.

2020 ◽  
pp. 1357633X2097200
Author(s):  
Beste Ozsezen ◽  
Nagehan Emiralioglu ◽  
Dilber A Tural ◽  
Birce Sunman ◽  
Halime N Buyuksahin ◽  
...  

Introduction The global burden of the 2019 novel coronavirus disease pandemic on the healthcare system, as well as the high transmission risk of coronavirus disease has led to the use of alternative strategies for evaluation of children with chronic conditions. In this context, telemedicine has become the effective and affordable choice. In this study we aimed to evaluate the efficiency of telephone visits to determine pulmonary exacerbations and hospitalization rates of children with cystic fibrosis and interstitial lung disease. Methods A total of 119 children with cystic fibrosis or interstitial lung disease were enrolled and provided cases in which telephone visits were applied during the peak time of the coronavirus disease pandemic in our country. The recordings of respiratory, gastrointestinal and other symptoms, nutrition status, rate of acute pulmonary exacerbation, treatments initiated by telephone visits, referral to hospital and hospitalization were established from the electronic health reports of the patients. Results Thirteen patients (10.9%) were symptomatic, 12 of them (10%) were diagnosed with acute pulmonary exacerbation. One patient was diagnosed with peripheral facial paralysis. Nine patients were recalled to the hospital and seven patients (5.8%) were hospitalised. Discussion Using telemedicine the health status of patients can be defined, and patients can be guided on proper healthcare that they need, especially during the times of pandemics which we are facing. Communication with patients while minimising the risk of exposure to coronavirus disease is an important advantage of telemedicine. Telemedicine will have to be implemented on our daily medical practice in the near future.


2018 ◽  
Vol 11 (2) ◽  
pp. 527-533 ◽  
Author(s):  
Heidi Egloff ◽  
Kelley M. Kidwell ◽  
Anne Schott

Purpose: T-DM1 is an antibody drug conjugate with proven efficacy in metastatic breast cancer for progressive disease refractory to trastuzumab. Drug-induced pneumonitis is a rare serious potential adverse effect. The purpose of this review was to estimate the incidence of pulmonary toxicity at our institution. Methods: A retrospective analysis of electronic medical record data inclusive of all women and men aged 18 years and older treated with T-DM1 at out institution was undertaken. The records were reviewed for clinical symptoms and/or radiographic evidence concerning for pneumonitis. We identified variables of interest with regard to potential risk factors for toxicity. Results: A total of 50 patients were included, 6 (12%) of whom had radiographic and/or clinical symptoms concerning for T-DM1-induced pneumonitis. All 6 patients had metastatic or unresectable breast cancer. Of the 6 patients, 5 (83%) had suspected pulmonary metastases, 1 (17%) had a history of underlying lung disease, and 5 (83%) had a history of prior taxane therapy. Pulmonary metastases (p = 0.38), the median number of treatment cycles (p = 0.29), prior taxane therapy (p = 0.99), underlying lung disease (p = 0.99), and hormone receptor positivity (p = 0.66) did not have any statistical significance for an association with pneumonitis. Conclusion: Pneumonitis is a recognized toxic effect of T-DM1. While our sample size was small, the number of events was higher than described in the literature, which may be an artifact of referral bias. Future studies with a larger sample population may detect potential risk factors for toxicity.


Author(s):  
Marieke van Horck ◽  
Kim D. G. van de Kant ◽  
Bjorn Winkens ◽  
Geertjan Wesseling ◽  
Vincent Gulmans ◽  
...  

2018 ◽  
Vol 52 (4) ◽  
pp. 1801492
Author(s):  
Hafez Al Momani ◽  
Audrey Perry ◽  
Rhys Jones ◽  
Melissa McDonnell ◽  
Amaran Krishnan ◽  
...  

Author(s):  
Colin Rutherford ◽  
S Towell ◽  
I Lawrie ◽  
S Winward ◽  
P Ging ◽  
...  

2021 ◽  
Vol 20 ◽  
pp. S85
Author(s):  
A. Shakkottai ◽  
S. Irani ◽  
S. Nasr ◽  
L. O’Brien ◽  
R. Chervin

1990 ◽  
Vol 63 (01) ◽  
pp. 013-015 ◽  
Author(s):  
E J Johnson ◽  
C R M Prentice ◽  
L A Parapia

SummaryAntithrombin III (ATIII) deficiency is one of the few known abnormalities of the coagulation system known to predispose to venous thromboembolism but its relation to arterial disease is not established. We describe two related patients with this disorder, both of whom suffered arterial thrombotic events, at an early age. Both patients had other potential risk factors, though these would normally be considered unlikely to lead to such catastrophic events at such an age. Thrombosis due to ATIII deficiency is potentially preventable, and this diagnosis should be sought more frequently in patients with arterial thromboembolism, particularly if occurring at a young age. In addition, in patients with known ATIII deficiency, other risk factors for arterial disease should be eliminated, if possible. In particular, these patients should be counselled against smoking.


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