scholarly journals Biatrial Remodeling in Patients with Cystic Fibrosis

2019 ◽  
Vol 8 (8) ◽  
pp. 1141 ◽  
Author(s):  
Aleksandar Dordevic ◽  
Martin Genger ◽  
Carsten Schwarz ◽  
Cesare Cuspidi ◽  
Elvis Tahirovic ◽  
...  

Background: Previous studies have focused on left and right ventricular remodeling in cystic fibrosis (CF), whereas atrial function has not been assessed in detail so far. We sought to investigate left and right atrial (LA and RA) function in patients with CF. Methods: This retrospective investigation included 82 CF patients (64 survivors and 18 non-survivors) who were referred to CF department over the period of four years, as well as 32 control subjects matched by age and gender. All participants underwent an echocardiographic examination including a strain analysis, which was performed offline and blinded for groups. Results: LA and RA volume indexes were significantly higher in CF patients than in controls and were particularly high in CF non-survivors. LA conduit and reservoir functions were significantly worse in CF survivors and non-survivors, compared with control subjects. RA phasic function was not different between controls, CF survivors and non-survivors. The parameters of lung function (forced vital capacity (FVC) and forced expiratory volume in the first second (FEV1)) and the LA and RA volume indexes were predictors of mortality in CF patients. However, in a multivariate analysis, only FVC was an independent predictor of mortality in CF patients. Conclusions: Our results suggest that both atria are enlarged, but only LA function is impaired in CF patients. LA reservoir and conduit function is particularly deteriorated in CF patients. Though statistical significance was not reached due to our limited sample size, there was a trend of deterioration of LA and RA function from controls across CF survivors to CF non-survivors. LA and RA enlargement represented predictors of mortality in CF patients.

2020 ◽  
Vol 24 (04) ◽  
pp. e434-e437
Author(s):  
Luciane Mazzini Steffen ◽  
Luise Sgarabotto Pezzin ◽  
Natassia Sulis ◽  
Nedio Steffen ◽  
Leonardo Araujo Pinto

Abstract Introduction Cystic fibrosis (CF) is a genetic disease that limits the quality of life mainly due to respiratory symptoms. The relationship between findings of the upper airways and CF lung disease is not yet completely understood. Objective The aim of the present study is to describe the most frequent nasal findings and pathogens in patients with CF and investigate the association between the findings of the upper respiratory tract and markers of lung disease progression. Methods Retrospective study in patients with CF from the Pediatric Pulmonology Department who underwent otorhinolaryngological evaluation between 2015 and 2017. Nasal endoscopy and nasal swab collection were part of the evaluation. The severity markers used were: percentage of predicted forced expiratory volume in the first second (FEV1%), body mass index (BMI) and the Shwachman-Kulczycki (SK) clinical score. Results A total of 48 patients with CF were included. The mean of the predicted percentage of FEV1% was 83.36 ± 30.04. The average 14 and SK score 89.11 ± 10.50. The bacteriology of the nasal swab was positive in 27 (54.1%) patients. Staphylococcus aureus was positive in 18 patients, Pseudomonas aeruginosa in 5, Pseudomonas cepacea in 3 and Stenotrophomonas maltophila in 1 patient. Nasal polyps were found in nine participants. Nasal polyps were found in nine participants and were associated with lower SK score. Conclusion The pathogens found in the upper airway were, in order: S. aureus, P. aeruginosa, P. cepacea e S. maltophila. The presence of polyps in the nasal cavity showed statistical significance and appears to have association with the prognostic factor measured by the SK score.


2021 ◽  
Vol 8 ◽  
Author(s):  
Yang-Yang Qu ◽  
Dominik Buckert ◽  
Gen-Shan Ma ◽  
Volker Rasche

Background: Left and right atrium (LA and RA) exert an essential and dynamic role in ventricular filling and hence affect heart performance. Strain quantification has been reported as a novel parameter to assess function. However, the assessment of bi-atrial strains with cardiovascular magnetic resonance (CMR) based techniques is still limited and gender- and age-specific normal values in a healthy population are missing.Methods: One hundred and fifty healthy volunteers (49.8 ± 17.3 years, 75 males) undergoing 1.5 Tesla CMR examination were retrospectively and consecutively recruited. LA and RA free wall (RAFW) radial and longitudinal strains (RS and LS) associated with atrial reservoir, conduit and booster pump functions were evaluated with CMR based tissue tracking (CMR-TT) technique.Results: The reservoir, conduit and pump LS resulted as 30.7 ± 10.2%, 19.5 ± 8.2%, 10.9 ± 3.7% for LA, and 52.2 ± 17.6%, 33.3 ± 14.2%, 19.1 ± 8.5% for RAFW, respectively. The amplitude of RA strains was significantly larger than that of LA strains, except for conduit RS. With the increase of age, the decrement of majority of reservoir and conduit strains were observed, while pump strains remained unaffected. Females presented with significantly larger RAFW strains compared with males, especially in the elderly. In addition to the positive correlation between atrial strains and emptying fraction, the negative correlation between atrial strains and volume index was also confirmed. Intra-observer reproducibility of LA strains was superior to RAFW strains (coefficient of variation: 10.12–17.04% vs. 10.80–27.36%, respectively), and the measurement of reservoir and conduit strains was more reproducible in comparison with pump strain.Conclusion: CMR-TT is a feasible and reproducible technique to quantify LA and RA strains and determine atrial phasic functions. The existence of age- and gender-related difference of strains suggests the necessity to establish specific normal values for individual populations.


1988 ◽  
Vol 64 (5) ◽  
pp. 1810-1816 ◽  
Author(s):  
R. M. Buchdahl ◽  
M. Cox ◽  
C. Fulleylove ◽  
J. L. Marchant ◽  
A. M. Tomkins ◽  
...  

To explore the hypothesis that there is an increased metabolic rate in cystic fibrosis, resting energy expenditure was measured by indirect calorimetry in 23 subjects with cystic fibrosis in a stable clinical state and in 42 normal control subjects. Resting energy expenditure was found to be elevated by an average of 0.45 MJ/24 h [95% confidence interval (CI) = 0.26–0.64, t = 4.91, P less than 0.001] (108 kcal/24 h), or 9.2% above expected values derived from the regression relating resting energy expenditure to whole body weight and sex in control subjects. When related to lean body mass, values were still elevated by 0.36 MJ/24 h (95% CI = 0.18–0.53, t = 4.15, P less than 0.001) (86 kcal/24 h), or 7.2%. The increased values were found to be independent of age, sex, or body size. There were significant correlations between increased values and poor pulmonary function as measured by the ratio of the forced expiratory volume in 1 s to forced vital capacity (r = -0.44, P less than 0.05) and subclinical infection as indicated by the blood leukocyte count (r = 0.40, P less than 0.05). However, the correlations were low, suggesting that other factors may contribute to the increased resting energy expenditure, possibly including the putative metabolic defect in cystic fibrosis.


2016 ◽  
Vol 310 (11) ◽  
pp. H1479-H1485 ◽  
Author(s):  
Paula Rodriguez-Miguelez ◽  
Jeffrey Thomas ◽  
Nichole Seigler ◽  
Reva Crandall ◽  
Kathleen T. McKie ◽  
...  

Cystic fibrosis (CF) is a genetic, multisystemic disorder with broad clinical manifestations apart from the well-characterized pulmonary dysfunction. Recent findings have described impairment in conduit vessel function in patients with CF; however, whether microvascular function is affected in this population has yet to be elucidated. Using laser-Doppler imaging, we evaluated microvascular function through postocclusive reactive hyperemia (PORH), local thermal hyperemia (LTH), and iontophoresis with acetylcholine (ACh). PORH [518 ± 174% (CF) and 801 ± 125% (control), P = 0.039], LTH [1,338 ± 436% (CF) and 1,574 ± 620% (control), P = 0.045], and iontophoresis with ACh [416 ± 140% (CF) and 617 ± 143% (control), P = 0.032] were significantly lower in patients with CF than control subjects. In addition, the ratio of PORH to LTH was significantly ( P = 0.043) lower in patients with CF (55.3 ± 5.1%) than control subjects (68.8 ± 3.1%). Significant positive correlations between LTH and forced expiratory volume in 1 s (%predicted) ( r = 0.441, P = 0.013) and between the PORH-to-LTH ratio and exercise capacity ( r = 0.350, P = 0.049) were observed. These data provide evidence of microvascular dysfunction in patients with CF compared with control subjects. In addition, our data demonstrate a complex relationship between microvascular function and classical markers of disease severity (i.e., pulmonary function and exercise capacity) in CF.


Author(s):  
M Medvedev, M.V. Kubrina, O.S. Zarubina et all

Two cases of prenatal ultrasound diagnosis of left atrial isomerism in the second trimester of gestation is presented. These two cases were in combination with pulmonary atresia and right aortic arch. Left atrial isomerism was identify by the digit-like shape of the left and right atrial appendages. The pulmonary atresia was identified on the basis of reverse flow in small pulmonary artery. A right aortic was identified by “U”-shaped confluence of aorta and ductus arteriosus in view of three vessels and trachea. The trachea was located between the vessels. The pregnancies were terminated and prenatal diagnosis was conformed at autopsy


Medicina ◽  
2021 ◽  
Vol 57 (8) ◽  
pp. 774
Author(s):  
Christina Batsi ◽  
Evangelia Gkika ◽  
Loukas Astrakas ◽  
Athanassios Papadopoulos ◽  
Ioannis Iakovou ◽  
...  

Background and Objectives: Vitamin D (Vit D) deficiency has been implicated in various conditions, including cardiovascular disease. The purpose of this retrospective study was to investigate the incidence of patients with myocardial ischemia in relation to their serum levels of vitamin D. Materials and Methods: A 64-month search (January 2016 to April 2021) in our database of the Nuclear Medicine Laboratory revealed 113 patients who had both myocardial perfusion imaging with single photon emission computed tomography (MPI SPECT) and Vit D measurements. MPI SPECT obtained myocardial images during both stress (summed stress score, SSS) and rest (summed rest score, SRS). Abnormal MPI SPECT was when the SSS was ≥4. Vit D was determined by radioimmunoassay (RIA). Patients with Vit D values <10 ng/mL, 10–29 ng/mL and ≥30 ng/mL were defined as having a deficiency, insufficiency and sufficiency, respectively. Results: Among patients, 46/113 (40.7%) were male and 67/113 (59.3%) were female. Abnormal MPI was found in 58/113 (51.3%) patients. Vit D deficiency was noted in 20/113 (17.7%) patients, insufficiency in 86/113 (76.1%) patients, and normal Vit D was noted in only 7/113 (6.2%) patients. Sixteen of the 20 patients (80%) with Vit D deficiency, and 38/86 (44.2%) with insufficiency had an abnormal MPI SPECT. In contrast, only 1/7 (14.3%) patients with sufficient Vit D levels had an abnormal MPI SPECT. The Mann-Whitney U-test showed that ischemia reduced the values of Vit D. Correlation analysis showed a negative association of Vit D levels with SSS (rho = −0.232, p = 0.014) and SRS (rho = −0.250, p = 0.008). Further evaluation with a Vit D cut off 20 ng/mL retrieved no statistical significance. Finally, Vit D and gender were independently associated with myocardial ischemia. Conclusions: Low Vit D levels may represent a risk factor for myocardial ischemia.


Author(s):  
Khalaf Kridin ◽  
Jennifer E. Hundt ◽  
Ralf J. Ludwig ◽  
Kyle T. Amber ◽  
Dana Tzur Bitan ◽  
...  

AbstractThe association between bullous pemphigoid (BP) and melanoma is yet to be investigated. We aimed to assess assess the bidirectional association between BP and melanoma and to delineate the epidemiological features of patients with both diagnoses. A population-based cohort study was performed comparing BP patients (n = 3924) with age-, sex- and ethnicity-matched control subjects (n = 19,280) with regard to incident cases of melanoma. A case–control design was additionally adopted to estimate the risk of BP in individuals with a preexisting diagnosis of melanoma. The prevalence of preexisting melanoma was higher in patients with BP than in control subjects (1.5% vs. 1.0%, respectively; P = 0.004). A history of melanoma confers a 50% increase in the risk of subsequent BP (OR 1.53; 95% CI 1.14–2.06). This risk was higher among males (OR 1.66; 95% CI 1.09–2.54) and individuals older than 80 years (OR 1.63; 95% CI 1.11–2.38), and persisted after adjustment for multiple putative confounders including PD-1/PDL-1 antagonists (adjusted OR 1.53; 95% CI 1.14–2.06). Conversely, the risk of melanoma among patients with BP was slightly elevated, but did not reach the level of statistical significance (adjusted HR 1.13; 95% CI 0.73–1.74). Patients with a dual diagnosis of BP and melanoma were older at the onset of BP and had lower body mass index. A history of melanoma is associated with a 50% increase in the incidence of subsequent BP. Physicians managing patients with both conditions should be aware of this association. Further research is warranted to reveal the underlying mechanism of these findings.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Grace Y. Lam ◽  
Sameer Desai ◽  
Joey Fu ◽  
Xun Yang Hu ◽  
Jiah Jang ◽  
...  

AbstractCystic fibrosis (CF) is a multi-system disease that is characterized by lung disease due to recurrent airway infection and inflammation. Endocrine complications, such as CF bone disease (CFBD), are increasingly identified as patients are living longer. The cause of CFBD is multifactorial with chronic systemic inflammation theorized to be a contributing factor. Thus, we attempted to identify inflammatory biomarkers that are associated with CFBD. We conducted a retrospective observational study of 56 adult patients with CF with an average percentage predictive forced expiratory volume in one second (ppFEV1) of 73.7% (standard deviation: 30.0) who underwent baseline serum analysis for osteoprotegerin (OPG) and pro-inflammatory biomarkers (IL-1β, IL-6, IL-8 and TNF-α), and had repeated dual-energy x-ray absorptiometry (DXA) scans separated by at least 2 years to examine correlations between serum biomarkers and bone mineral density (BMD) measurements. Univariate linear regression model analysis demonstrated that serum IL-1β and IL-8, but not other pro-inflammatory markers, were negatively correlated with baseline BMD results. However, after accounting for confounding variables, only the relationship between IL-8 and left femoral neck BMD remained statistically significant. Additionally, IL-8 level was associated with BMD decline over time. These results suggest that IL-8 might play a unique role in the pathophysiology of CFBD relative to other pro-inflammatory cytokines but further study is warranted before firm conclusions can be made.


Author(s):  
Maria Theresa Costa Ramos de Oliveira Patrial ◽  
Rogério Hamerschmidt ◽  
Jorge Eduardo Fouto Matias ◽  
Evaldo Dacheux de Macedo Filho ◽  
Bettina Carvalho

Abstract Introduction The study of larynx lesions is of great importance. More than 50% of people with vocal complaints have benign vocal fold alterations and some require surgery. Objective To determine which factors are related to surgical recurrence of benign laryngeal lesions and to determine which videolaryngoscopic diagnoses are related to the risk or protection of surgical relapses over time. Methods Observational, analytical, cross-sectional study with retrospective data collection of 1,383 surgeries in 1,301 patients. Results The mean age at the first surgery was 39.2 years old. The predominant gender was female (65.6%).There were 396 cases of polyps (25.34%), 349 cases of cysts (22.33%), 261 cases of Reinke edema (16.7%), 175 cases of nodules (11.2%), 153 cases of minor structural alterations (MSAs) (9.79%), 94 cases of leukoplakia (6.01%), 77 cases of granulomas (4.93%) and 58 cases of pseudocysts (3.71%). The age presented statistical significance with the risk of surgical relapses (p = 0.016). Male gender was associated with the greatest chance of relapse.Diagnosis of granuloma (p < 0.001) and of leukoplakia (p < 0.001) were associated with a higher risk of surgical recurrence. Cyst diagnosis was associated with protection (p = 0.015) in relation to the chance of further surgeries. Patients with associated lesions, bilaterals or voice professionals did not present a statistically significant association with surgical recurrence. Conclusion Age and gender are statistically related to the increase of surgical relapses. Leukoplakia and granuloma are associated with increased surgical relapses. Patients submitted to cyst microsurgery appear to be protected against surgical recurrence.


2021 ◽  
Author(s):  
Alexandra S Mighiu ◽  
Alice Recalde ◽  
Klemen Ziberna ◽  
Ricardo Carnicer ◽  
Jakub Tomek ◽  
...  

Abstract Aims Gp91-containing NADPH oxidases (NOX2) are a significant source of myocardial superoxide production. An increase in NOX2 activity accompanies atrial fibrillation (AF) induction and electrical remodelling in animal models and predicts incident AF in humans; however, a direct causal role for NOX2 in AF has not been demonstrated. Accordingly, we investigated whether myocardial NOX2 overexpression in mice (NOX2-Tg) is sufficient to generate a favourable substrate for AF and further assessed the effects of atorvastatin, an inhibitor of NOX2, on atrial superoxide production and AF susceptibility. Methods and results NOX2-Tg mice showed a 2- to 2.5-fold higher atrial protein content of NOX2 compared with wild-type (WT) controls, which was associated with a significant (twofold) increase in NADPH-stimulated superoxide production (2-hydroxyethidium by HPLC) in left and right atrial tissue homogenates (P = 0.004 and P = 0.019, respectively). AF susceptibility assessed in vivo by transoesophageal atrial burst stimulation was modestly increased in NOX2-Tg compared with WT (probability of AF induction: 88% vs. 69%, respectively; P = 0.037), in the absence of significant alterations in AF duration, surface ECG parameters, and LV mass or function. Mechanistic studies did not support a role for NOX2 in promoting electrical or structural remodelling, as high-resolution optical mapping of atrial tissues showed no differences in action potential duration and conduction velocity between genotypes. In addition, we did not observe any genotype difference in markers of fibrosis and inflammation, including atrial collagen content and Col1a1, Il-1β, Il-6, and Mcp-1 mRNA. Similarly, NOX2 overexpression did not have consistent effects on RyR2 Ca2+ leak nor did it affect PKA or CaMKII-mediated RyR2 phosphorylation. Finally, treatment with atorvastatin significantly inhibited atrial superoxide production in NOX2-Tg but had no effect on AF induction in either genotype. Conclusion Together, these data indicate that while atrial NOX2 overexpression may contribute to atrial arrhythmogenesis, NOX2-derived superoxide production does not affect the electrical and structural properties of the atrial myocardium.


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