Recent studies have recognised the importance of pulmonary hypertension (PH) in sickle cell disease (SCD). The aim of this study was to determine the prevalence and prognostic impact of PH and its ...features in patients with SCD. 80 patients with SCD underwent baseline clinical evaluation, laboratory testing, 6-min walk tests (6MWTs) and echocardiography. Patients with a peak tricuspid regurgitant jet velocity (TRV) of ≥ 2.5 m·s(-1) were further evaluated through right heart catheterisation (RHC) to assure the diagnosis of PH. Our study evidenced a 40% prevalence of patients with elevated TRV at echocardiography. RHC (performed in 25 out of 32 patients) confirmed PH in 10% (95% CI 3.4-16.5%) of all patients, with a prevalence of post-capillary PH of 6.25% (95% CI 0.95-11.55%) and pre-capillary PH of 3.75% (95% CI -0.4-7.9%). Patients with PH were older, had worse performance in 6MWTs, and more pronounced anaemia, haemolysis and renal dysfunction. Survival was shorter in patients with PH. Our study reinforced the use of echocardiography as a screening tool for PH in SCD and the mandatory role of RHC for proper diagnosis. Our findings confirmed the prognostic significance of PH in SCD as its association to pronounced haemolytic profile.
The characterization of pulmonary arterial hypertension (PAH) relies mainly on right heart catheterization (RHC). Electrical impedance tomography (EIT) provides a non-invasive estimation of lung ...perfusion that could complement the hemodynamic information from RHC. To assess the association between impedance variation of lung perfusion (ΔZQ) and hemodynamic profile, severity, and prognosis, suspected of PAH or worsening PAH patients were submitted simultaneously to RHC and EIT. Measurements of ΔZQ were obtained. Based on the results of the RHC, 35 patients composed the PAH group, and eight patients, the normopressoric (NP) group. PAH patients showed a significantly reduced ΔZQ compared to the NP group. There was a significant correlation between ΔZQ and hemodynamic parameters, particularly with stroke volume (SV) (r = 0.76; P < 0.001). At 60 months, 15 patients died (43%) and 1 received lung transplantation; at baseline they had worse hemodynamics, and reduced ΔZQ when compared to survivors. Patients with low ΔZQ (≤154.6%.Kg) presented significantly worse survival (P = 0.033). ΔZQ is associated with hemodynamic status of PAH patients, with disease severity and survival, demonstrating EIT as a promising tool for monitoring patients with pulmonary vascular disease.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Improvement in quality of life together with better survival are the ultimate goals in the treatment of pulmonary arterial hypertension (PAH) patients. The objective of this study was to evaluate the ...health-related quality of life (HRQL) of pulmonary arterial hypertension (PAH) patients with the SF-36 generic questionnaire and to identify the prognostic implication of this assessment.
Fifty-four consecutive newly diagnosed PAH patients (WHO classification group I) in a single PAH reference center were included. Patients were evaluated at baseline for clinical and hemodynamic parameters, and they subsequently received first-line therapy with either an endothelin receptor antagonist or a phosphodiesterase-5 inhibitor. After 16 weeks of specific PAH therapy, all patients were re-evaluated using a 6MWT and a SF 36 questionnaire, and then they were followed up for at least 36 months.
After treatment, the patients demonstrated an improved 6MWT (414 ± 124 m vs. 440 ± 113 m, p = 0.001). Specific PAH therapy also improved the HRQL scores.Patients with a baseline Physical Component Score (PCS) higher than 32 had a better survival rate than those who had a score under 32 (p = 0.04). Similarly, patients with a PCS of at least a 38 after the 16 week therapy period had a better survival rate when compared with those who did not achieve this value (p = 0.016). Unlike the absolute PCS values, the post-treatment PCS variability was unable to predict better survival rates (p = 0.58).
Our findings suggest that HRQL is associated with prognosis in PAH. Furthermore, achieving pre-determined PCS scores might represent a specific goal to be reached in treatment-to-target strategies.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) may be one of the most prevalent forms of pulmonary arterial hypertension (PAH) worldwide. However, the clinical and ...hemodynamical response to specific PAH therapy in Sch-PAH is not known. Methods We retrospectively analyzed the charts of all patients with Sch-PAH who initiated specific PAH treatment between June 2003 and June 2010 in a single PAH reference center in São Paulo, Brazil. Clinical and hemodynamical data were retrospectively collected and evaluated in two periods: baseline and posttreatment. Results The study population consisted of 12 patients with Sch-PAH. They were treated with phosphodiseterase-5 inhibitors (seven patients), endothelin receptor antagonists (four patients), or combination therapy (one patient). Mean treatment period was 34.9 ± 15.5 months. Patients with Sch-PAH presented significant improvements in terms of functional class, 6-min walk test distance (439 ± 85 to 492 ± 79 m, P = .032), cardiac index (2.66 ± 0.59 to 3.08 ± 0.68 L/min/m2 , P = .028), and indexed pulmonary vascular resistance (20.7 ± 11.6 to 15.9 ± 9 W/m2 , P = .038) with the introduction of specific PAH treatment. Conclusions We conclude that specific PAH therapy may be of benefit to patients with Sch-PAH, considering clinical, functional, and hemodynamic parameters.
Triple combination therapy is suggested in current pulmonary arterial hypertension guidelines in case of unsatisfactory treatment with oral double combination therapy. However, there is a lack of ...evidence concerning some of the drug combinations currently employed. We demonstrate the clinical and hemodynamical benefits of inhaled iloprost as third add-on therapy in idiopathic pulmonary arterial hypertension.
Background and purpose
Spinocerebellar ataxia type 10 is a neurodegenerative disorder that is due to an expanded ATTCT repeat tract in the ATXN10 gene. Our aim was to describe clinical ...characteristics and intragenic haplotypes of patients with spinocerebellar ataxia type 10 from Brazil and Peru.
Methods
Expanded alleles were detected by repeat‐primed polymerase chain reaction. Disease progression was measured by the Scale for the Assessment and Rating of Ataxia, and the Neurological Examination Score for Spinocerebellar Ataxias when possible. Haplotypes were constructed based on polymorphic markers within and outside the gene.
Results
Thirteen new families were diagnosed (three from Peru). Patients from three Brazilian families diagnosed previously were also reassessed. In total, 25 individuals (16 families) were evaluated. Mean (± SD) age at onset and disease duration were 34.8 ± 10.2 and 12 ± 8 years, respectively. Common findings were ataxia, dysarthria/dysphagia, nystagmus, pyramidal signs, ophthalmoparesis and seizures. No associations were found between clinical findings and geographical origins. Twelve patients living in remote regions were examined only once. In the remaining individuals, the Scale for the Assessment and Rating of Ataxia score, and Neurological Examination Score for Spinocerebellar Ataxias worsened by 0.444 (95% CI, −0.088 to 0.800) and 0.287 (95% CI, −0.061 to 0.635) points/year, respectively. A common haplotype, 19CGGC14, was found in 11/13 of Brazilian and in 1/3 of Peruvian families.
Conclusions
The progression rate was slower than in other spinocerebellar ataxias. A consistently recurrent intragenic haplotype was found, suggesting a common ancestry for most, if not all, patients.
OBJECTIVE:To evaluate the behavior of central blood pressure (BP) variables in male athletes before and during a very long distance walking - 310Km route in five days.
DESIGN AND METHOD:Longitudinal ...study with 25 participants. This walking nominated as ’Ecological Walk’ happens in Brazil every july since 1991. Its main goals are environmental preservation awareness, health lifestyle incitement as well as exercise practice incitement. The participants traveled the 310 Km during five days alternating walking and light running, a 72 Km/day average. First data collection occurred one month before the walking (V0) and the others, during the second (V2), third (V3) and fourth walking day (V4); just after the athletes finished the daily route. Mobil O Graph was the device used to get central BP variablespulse wave velocity (PWV), augmentation index (AIx), peripheral vascular resistance (PVR), central pulse pressure (PPc) and amplified pulse pressure (PPA).
RESULTS:Sample composition was 25 males, regular physical activity practitioners, mean age 45,3 ± 9,1 years old. Brachial systolic (SBP) and diastolic blood pressure (DBP) had the same response during the route, reduction from V0 to V2, and a slight increase from V2 to V3 and V4. PPc decreased from V0 (33,5mmHg) to V2 (28,7mmHg); p = 0,05 and increased from V2 (28,7mmHg) to V4 (32,9mmHg); p = 0,01. Brachial PP decreased from V0 (49,2mmHg) compared with all the others evaluated days (38,2mmHg; 42,7mmHg; 41,2mmHg), respectively. PPA increased from V0 to V2, and decreased from V0 to V3 and from V0 to V4. PVR increased from V3 (1,07s*mmHg/ml) to V4 (1,13 s*mmHg/ml); p = 0,046. PWV decreased from V0 (7,0m/sec) to V2 (6,6m/sec) and from V0 (7,0m/sec) to V3 (6,5m/sec). Pulse wave velocity showed strong correlation with age during all the measurements.
CONCLUSIONS:These datas indicate that central blood pressure changes are sharpest in the first days, after this it seems that an exercise physiological accommodation occurred. There was a strong correlation between age and pulse wave velocity in this sample.
Compounding of silicone rubbers with TiO2 was carried out in a two-roll mill from a mixture of TiO2 and poly(dimethylsiloxane) (PDMS) with several other additives. The silicone matrix was crosslinked ...by a platinum-catalyzed hydrosilylation reaction. Characterization of the rubbers was performed by thermogravimetric and dynamic mechanical analyses, swelling measurements in toluene and in a salicylic acid/water solution, UV–vis spectroscopy and field emission scanning electron microscopy. The photocatalytic activity of the rubbers was evaluated with respect to the photodegradation of salicylic acid in aqueous solution under UV radiation, by measuring total organic carbon. The thermal stability of the PDMS matrix was enhanced by the addition of 10phr (parts per hundred parts of rubber, by weight) of TiO2, but a further increase in the quantity of TiO2 favored thermal degradation of the matrix. The stiffening effect promoted by the incorporation of TiO2 into the matrix was more pronounced at higher TiO2 amount. The increase in the quantity of TiO2 also promoted a decrease in the crosslinking density of the matrix, favoring the swelling of the rubbers. The rubber with 78phr of TiO2 degraded 56% of the salicylic acid from an aqueous solution.
The objective of this study was to determine the subacute blood pressure behavior of pharmacologically-treated elderly hypertensive patients after a session of resistance exercise. This was a ...controlled clinical trial carried out on 30 elderly hypertensive women. The study procedures took place over three days with an interval of 48 hours, and included a test session, an experimental protocol (EP) and a control protocol (CP).
A 10RM test was carried out to define the EP load for the following exercises: leg press, knee extension, pull-up, biceps curl and machine bench press. In the EP, three sets of exercises with 8 to 10 repetitions were carried out at 2-minute intervals. In the CP all procedures were the same as in the EP, but without the exercises. BP was measured before, immediately after and every ten minutes up to 60 minutes after the EP and CP.
There was no significant variation in systolic blood pressure (SBP) or diastolic blood pressure (DBP) in either the EP or the CP immediately after the exercises. In the period up to sixty minutes after the exercises, there was a significant difference in BP, with a decrease in SBP and DBP for both EP and CP.
The data from the present study offer a good indication that resistance exercises may be prescribed safely to this group of patients. The pressure reduction appears to be influenced by the rest that occurred after the protocols and not by exercise.
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