Pompe disease is an orphan hereditary accumulation disease associated with a deficiency of the lysosomal enzyme alglucosidase alpha. Manifestations of the disease are associated with pathological ...deposition of glycogen in body tissues as a result of GAA gene mutation and subsequent reduction in the activity of the enzyme alglucosidase alpha or acid maltase. The variety of phenotypic forms and varying degrees of damage to the skeletal and respiratory muscles, cardiomyocytes and internal organs greatly complicates the diagnosis and treatment of patients with Pompe»s disease. This article describes the clinical case of late-onset Pompe disease, which was followed by a course of enzyme replacement therapy, as well as an assessment of the condition before and after treatment and preliminary results.
In order to use shape memory Ni–Ti couplings repeatedly for the joining of pipelines, one should know how the multiple mandreling (expansion) affects the temperatures of the phase transformation in ...titanium nickelide and the quality of the inner surface of these couplings. This problem has been studied, using three couplings of commercial Ni–45wt.%Ti (TH1) alloy, which were designed to join the pipelines with an outer diameter of 11.9 mm. During expansion by mandreling the inner diameter of the couplings increased from 11.45 to 12.00 mm. It was observed that the required mandreling force decreased by a factor of 3–4 after a 10-fold mandreling. After each mandreling the coupling was heated, and the temperature of the initial and final stages of the reversible transformation was defined by the change in the inner diameter of the coupling. A decrease of the phase transformation temperatures by 8–20°C was registered between the 2nd and 10th mandreling cycle. In every cycle the average diameter of the coupling and the deviation from the ideal circular shape were measured before mandreling as well as after mandreling and heating and cooling back to room temperature. It was found that the deviation from the ideal circular shape increased with the number of mandreling cycles. This means that the quality of the couplings deteriorates with their repeated expansion by mandreling. This should be taken into account when using the couplings again for the joining of pipelines.
Psychosocial risk factors (RFs) play a major role in the development and progression of cardiovascular diseases (CVDs).
The COMET study aimed to obtain current data on psychosocial RFs in outpatients ...with arterial hypertension (AH) and/or coronary heart disease (CHD) seen in primary care facilities in 30 cities of Russia.
In 2016-2017, a multicenter cross-sectional study was carried out involving 325 physicians from community primary care facilities who enrolled 2,775 patients with AH and/or CHD ≥ 55 years of age. However, only 73 CHD patients (2.6%) were not hypertensive, therefore, these patients were excluded from the analyses. As a result, current paper is based on the comparison of AH patients (n=1687) vs. participants with both CHD and AH (AH+CHD; n=1015). We collected patients' socio-demographic data, clinical features, traditional and psychosocial RFs, such as anxiety and depression, stress level, type D personality, and treatment adherence.
The study population (women, 72%; mean age ± SD, 66.7 ± 7.9 years) had a significant prevalence of psychosocial RF. 43.8% of AH patients and 45.5 % of participants with AH+CHD rated their income as low or very low, a low educational level was reported in 21.6% and 26.0%, respectively (both p=n/s). Social isolation was uncommon, but it occurred more frequently in AH+CHD patients (8.3% vs. 5.2%, p<0.01). Nevertheless, 40.2% of AH patients and 39.4% of AH+CHD were not married, and 26.0% and 24.6% were living alone, respectively. Elevated stress level was prevalent in more than 60% of patients (67.9% in AH patients vs. 67.7% in AH+CHD patents, p=n/s), and 63.3% and 64.8% of patients, respectively, reported stressful life events in the preceding year (p=n/s). Type D personality was more common in AH+CHD patients (41.2% vs. 35.8%; p<0.01). Clinically significant anxiety symptoms were prevalent in 24.7% ofAH patients and in 27.4% ofAH+CHD patients (p=n/s), and clinically significant depressive symptoms were identified in 13.9% and 20.9%, respectively (p<0.001).
We obtained current data on psychosocial RFs prevalence in outpatients with the most common CVDs in primary care setting. At the moment, their prevalence remains significant.
Objective. To study medical awareness of cardiovascular risk factors (RFs) in different types of hospitalized patients (pts). Methods. A total of 150 pts from neurological, endocrinological and ...cardiac units one of Moscow city hospital were enrolled into the survey (50 pts in each unit). The pts were interviewed during the I-II days of the hospitalization. A special questionnaire was developed in‑ cluding socio-demographic and clinical indicators, open questions on the awareness of traditional cardiovascular RFs and their target values. Results. Pts of three units did not differ in gender and age. The range of diagnoses corresponded to the profile of the unit. The survey revealed an extremely low awareness of major cardiovascular RFs of pts in all 3 units: almost none of them pointed to elevated cholesterol (0%, 4% and 0%, respectively) and blood pressure (2%, 2% and 0%) respectively) as RF of cardiovascular diseases. The pts of the three units most often referred to stress (64%, 56% and 66%, respectively) and unhealthy diet (50%, 56% and 64%, respectively) as the main cardiovascular RFs. On average, pts in three units correctly indicated only 2 RFs. Conclusion. The survey revealed a low awareness of cardiovascular RFs in different types of medical pts, including cardiac pts, at time of hospital admission.
to assess the impact of preventive counseling with focus on diet modification on lipid and metabolic parameters in patients with high / very high cardiovascular (CV) risk who visited Health centers.
...This was a prospective randomized controlled study of patients aged 40 to 65 years with high/very high CV risk (≥5% according to the Systematic Coronary Risk Evaluation scale SCORE) and any 2 criteria for metabolic syndrome. Patients were 1:1 randomized into 2 groups. The intervention group (n=50) received comprehensive preventive counseling with focus on diet modification followed by remote preventive counseling by phone every two weeks for the first 3 months after enrollment (a total of 6 sessions). The control group (n=50) received usual care in Health centers which also included basic preventive counseling.
A total of 100 patients (women 82%, age 59.74±4.66 years) were randomized. At baseline 81% of patients had high and 19% - very high CV risk. The groups were well balanced according to demographic and clinical features. At 1 year of follow-up patients from the intervention group experienced significant improvement of metabolic parameters compared with controls: their diastolic blood pressure (BP) decreased by 5.62±7.7 mm Hg, total and low-density lipoprotein cholesterol (TC and LDL-C) - by 0.5±0.83 and 0.46±0.62 mmol/l, respectively. Both groups experienced statistically and clinically significant decreases in systolic BP (intervention, - 17.76±16.2 mm Hg, control, - 13.44±15.6 mm Hg; both groups p.
Purpose:
to assess efficacy of correction of anxiety states by anxiolytic drug fabomotizole in ambulatory patients with arterial hypertension (AH) and / or ischemic heart disease (IHD).
Materials and ...methods
. In the framework of multicenter cross-sectional study with participation of patients aged ≥55 years with verified AH / IHD we conducted the therapeutic part of the COMETA program in which we included patients with comorbid anxiety state (≥11 points on the Hospital Anxiety and Depression Scale Anxiety HADS-A and clinically expressed anxiety state) without clinically expressed depressive symptoms (<11 points on the HADS-Depression). Participants were randomized into main and control groups. Patients in the main group in addition to therapy prescribed because of AH / IHD were given a recommendation to take fabomotizole (10 mg thrice a day), patients of control group received standard therapy. Efficacy of therapy was evaluated by HADS and visual analog scale after 6 and 12 weeks of observation.
Results
. We included 182 and 104 in the main and control groups, respectively. Most patients in main and control groups had AH (97.3 and 95.2 %, respectively, about one third had IHD (36.8 and 30.8 %, respectively). Social-demographic, clinical characteristics, and recommended for AH / IHD treatment of participants of both groups were similar. Portion of patients with complete reduction of anxiety symptoms (<8 points on HADS-A) was significantly higher already after 6 weeks of fabomotizole therapy (37.9 and 19.2 %, respectively, p<0.001). Analogous picture was noted by the end of observation (66.9 and 32 %, respectively; p<0.001). Mean estimate of chronic psychoemotional stress in the main group decreased by 25 % after 6 weeks (from 6.45±2.20 to 5.05±1.96 points; р<0.001) and by 40 % after 12 weeks (from 6.45±2.20 to 3.98±1.99 points; р<0.001). In the control group it also decreased but degree of lowering was 2 times less than in the main group (11.1 % vs. 25 % after 6 weeks, р=0.016; and 20 % vs. 40 % after 12 weeks, р<0.001, respectively).
Conclusion
. The use of fabomotizole by patients with AH / IHD provided improvement of psychological status (reduction of anxiety symptoms and lowering of the level of chronic psychoemotional stress).
Patients with coronary heart disease (CHD) and abdominal obesity (AO) are a priority group for the most active implementation of secondary prevention efforts. The paper focuses on most challenging ...issues of cardiovascular risk factors (RFs) correction via comprehensive cardiac rehabilitation (CR) programs in patients with CHD and AO. Based on large randomized clinical trials results, intensive behavioral interventions in the form of counselling are beneficial for such patients especially during the long-term support stage. They produce small but important changes in health behaviors (which translate into weight reduction, more healthy nutrition and higher physical activity) and improve selected intermediate clinical endpoints.
Background.
Psychosocial (PS) risk factors (RF) make a substantial contribution in populational burden of cardio-vascular diseases (CVD) and their complications.
Purpose.
The KOMETA (Comet) study was ...directed to obtaining actual information on PSRF among ambulatory patients with arterial hypertension (AH) and / or ischemic heart disease (IHD) in 30 cities of Russian Federation.
Materials and methods.
This multicenter cross-sectional study was conducted in 2016–2017. Doctors participating in the study (n=325) recruited in state polyclinics 2775 patients aged ≥55 years with AH and / or IHD. Information collected from these patients comprised social-demographic and clinical characteristics, data on RF, adherence to therapy. Assessment of PSRF was carried out with consideration of levels of anxiety, depression and stress, presence of personality type D. Results. Population of patients studied (72 % women) was characterized by considerable prevalence of PSRFs. Low levels of education and income were found in 24.5 and 44.2 % of patients, respectively; 25.2 % of patients reported living alone, 6.3 % – felt social isolation. Elevated, extremely high levels of stress, type D personality were detected in 67.8, 10, and 37.6 % of patients, respectively; clinically significant symptoms of anxiety and depression were found in 25.5 and 16.3 %, respectively. Most RFs were significantly more often detected in women, and older people. One third of patients (33.1 %) during a year preceding inclusion took some psychotropic drugs mainly herbal or barbiturate-containing (27.1 %). Moreover, 30 % of patients had lowering of cognitive functioning.
Conclusion.
In this large-scale study we revealed high prevalence of PSRFs among ambulatory patients with AH and / or IHD in Russia. Despite positive dynamics of prevalence of states of anxiety and depression relative to earlier studies in this country their negative impact on prognosis of CVD and quality of life of affected patients requires optimization of efforts for organization of adequate care and directed to timely diagnosis and correction of these states.
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