Congenitally corrected transposition of the great vessels (CCTGV) is a rare congenital heart defect associated with multiple cardiac morphological abnormalities and conduction defects. Complete ...atrioventricular (AV) block occurs in 30% of patients and it may be present at birth or develop later with a rate of 2% per year; moreover, a systemic right ventricle is frequently characterized by heart failure in adult life. We used a bifocal cardiac stimulation for a young woman affected by CCTGV and atrioventricular Mobitz 2 and 2:1 block, considering structural, anatomic condition, and the high rate of pacing she underwent. (PACE 2012; 35:e296–e298)
Strong evidence suggests that systemic inflammation and central adiposity contribute to and perpetuate metabolic syndrome. All of these alterations predispose individuals to type 2 diabetes mellitus ...(T2DM), cardiovascular disease, as well as Alzheimer's disease (AD), all characterized by chronic inflammatory status. On the other hand, extensive abnormalities in insulin and insulin-like growth factor I (IGF-I) and IGF-II signaling mechanisms in brains with AD have been demonstrated, suggesting that AD could be a third form of diabetes. The Src homology domain-containing inositol 5-phosphatase 2 (SHIP2) has an important role in the insulin pathway because its over-expression causes impairment of insulin/IGF-1 signaling. Because some single-nucleotide polymorphisms (SNP) of the gene encoding SHIP2 were significantly associated in T2DM patients with metabolic syndrome and some related conditions, we decided to conduct a case-control study on this gene, analyzing AD and T2DM subjects as cases and young, old, and centenarians as controls. Our results suggest a putative correlation between the the rs144989913 SNP and aging, both successful and unsuccessful, rather than age-related diseases. Because this SNP is an insertion/deletion of 28 bp, it might cause an alteration in SHIP2 expression. It is noteworthy that SHIP2 has been demonstrated to be a potent negative regulator of insulin signaling and insulin sensitivity. Many studies demonstrated the association of the insulin/IGF1 pathway with aging and longevity, so it is tempting to speculate that the found association with SHIP2 and aging might depend on its effect on the insulin/IGF-1 pathway.
Fragmented data exist on the emotional and psychological distress generated by hospital admission during the pandemic in specific populations of patients, and no data exists on patients scheduled for ...surgery. The aim of this multicentre nationwide prospective cross-sectional survey was to evaluate the impact of pandemic on emotional status and fear of SARS-CoV-2 contagion in a cohort of elective surgical patients in Italy, scheduled for surgery during the COVID-19 pandemic.
Twenty-nine Italian centres were involved in the study, for a total of 2376 patients surveyed (mean age of 58 years ± 16.61; 49.6% males). The survey consisted of 28 total closed questions, including four study outcome questions. More than half of patients had at least one chronic disease (54%), among which cardiovascular diseases were the commonest (58%). The most frequent type of surgery was abdominal (20%), under general anaesthesia (64%). Almost half of the patients (46%) declared to be frightened of going to the hospital for routine checkups; 55% to be afraid of getting SARS-CoV-2 infection during hospitalization and 62% were feared of being hospitalised without seeing family members. Having an oncological disease and other patient-related, centre-related or perioperative factors were independently associated with an increased risk of fear of SARS-CoV-2 infection during hospitalization and of being hospitalised without seeing family members. A previous infection due to SARS-COV-2 was associated with a reduced risk of worse emotional outcomes and fear of SARS-CoV-2 infection during hospitalization. Patients who showed the most emotionally vulnerable profile (e.g. use of sleep-inducing drugs, higher fear of surgery or anaesthesia) were at higher risk of worse emotional status towards the hospitalization during COVID-19 pandemic. Being operated in hospitals with lower surgical volume and with COVID-19 wards was associated with worse emotional status and fear of contagion.
Additional fear and worse emotional status may be frequent in patients scheduled for elective surgery during COVID-19 pandemic. More than half of the participants to the survey were worried about not being able to receive family visits. Psychological support may be considered for patients at higher risk of psychological distress to improve perioperative wellbeing during the pandemic.
Centenarians are the best example of extreme human longevity, and they represent a selected population in which the appearance of major age-related diseases, such as cancer, and cardiovascular ...diseases among others, has been consistently delayed or escaped. The study of the long-lived individual genetic profile has the purpose to possibly identify the genes and the allelic variations influencing extended life expectancy, hence considering them as biomarkers of age-related diseases onset and development. The present study shows no significant differences between allelic variations of ABO blood groups among a group of centenarians from Western Sicily.
Epidermolysis bullosa is a rare, inherited group of disorders characterized by blistering of the skin following friction or mechanical trauma. The aim of this study was to assess the family burden of ...epidermolysis bullosa in children aged 0-7 years. A postal survey was conducted. The perceived severity of the disease was evaluated by the caregivers, using the Patient Global Assessment 5-point scale. The caregiver received the Family Strain Questionnaire and the 12-item General Health Questionnaire to assess the probable presence of depression/anxiety. A single-item analysis was also performed for questions related to the burden of disease. Forty-two families were invited to participate. Data from 28 young patients and their caregivers were analysed (response rate 66.7%). The family burden increased with increasing caregiver's perceived disease severity, with increasing patient's body surface involved, and if parents had depression/anxiety, reaching statistical significance in several Family Strain Questionnaire scales. The family burden due to epidermolysis bullosa is very high independent of disease type/subtype.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Fabry disease is an X‐linked disorder resulting from a deficiency of the lysosomal enzyme alpha‐galactosidase leading to the accumulation of glycolipids in the cells. It has been demonstrated that ...polymorphisms of alfa‐galactosidase might influence the onset and development of inflammatory cardiovascular age related disease.
Familiar Mediterranean fever (FMF) is a autosomal recessive inflammatory based disease, characterized by recurrent fever, peritonitis, arthritis, pleuritis, and cardiac problems. MEFV encodes the pyrin protein it has been suggested to play a role in the innate immunity regulating the inflammatory response. Several data suggest a role of FMF polymorphisms in worsening recurrent inflammatory diseases. The aim of this study is to analyze the interaction between polymorphisms of this two diseases in order to find possible association. We have analyzed 46 patients with cardiac and neurological diseases holding Fabry's disease polymorphisms. Surprisingly, we have found that out of the 46 patients 9 individuals were bearing also Pyrin allelic mutations (more that 19.6%). Since the percentage of Pyrin mutation in a healthy population is more or less 5%, we can conclude that the presence of these mutations might load of further inflammatory burden the status on Fabry's patients.
Background & Aims
The Pre‐TACE‐Predict model was devised to assess prognosis of patients treated with trans‐arterial chemoembolization (TACE) for hepatocellular carcinoma (HCC). However, before ...entering clinical practice, a model should demonstrate that it performs a useful role.
Methods
We performed an independent external validation of the Pre‐TACE model in a cohort that differs in setting and time period from the one that generated the original model. Data from 826 patients treated with TACE for naïve HCC (2008‐2018) were used to assess calibration and discrimination of the Pre‐TACE‐Predict model.
Results
The four risk‐categories identified by the Pre‐TACE‐Predict model had gradient monotonicity, with median survivals of 52.0, 36.2, 29.9, and 14.1 months respectively. However, predicted survivals systematically underestimated observed survivals (R2: 0.667). A recalibration was adopted maintaining fixed the prognostic index and modifying the baseline survival function. This resulted in an almost perfect calibration (R2: 0.995) in all the four risk categories. Cox regressions showed that aetiology and macrovascular invasion, included in the Pre‐TACE‐Predict model, had no prognostic impact in the present study population, and that coefficients for tumour size and multiplicity were overestimated. The c‐index was similar to that of the m‐HAP‐III, but higher than those of HAP, m‐HAP‐II and the six‐and‐twelve models.
Conclusions
The recalibration of Pre‐TACE‐Predict model improved the estimation of survival probabilities of HCC patients treated with TACE. The highest discriminatory ability of the Pre‐TACE‐model in comparison to other available models, together with risk stratification and recalibration, makes it the best prognostic tool currently available for these patients.
The use of autologous platelet gel in orthopedics is effective in accelerating the healing process of osteochondral, muscle, tendon and ligament lesions. The aim of our study was to verify whether ...the variability in response to infiltration with platelet gel was dependent on the underlying disease treated, sex and age of the patients. During four years, 140 patients have been treated for musculoskeletal injuries by infiltration of gel platelet and lysate platelet obtained from autologous thrombin, with echo-ultrasound guided. The response to treatment was assessed at different time points T0, T1, T2 with respect to pain estimation (VAS), joint mobility (ROM scale) and echo-ultrasound evaluation. This data collection has allowed classifying the response to treated lesions in three categories: NR (no response), PR (partial response), CR (complete response).
The data here reported showed that the ability to physical recovery response is evident in tendon injuries, while the large joints injuries gave a poor response. Almost all patients showed a significant pain relief after the first infiltration, but in terms of echo-ultrasound evaluation and tissue repair, only the muscle and tendon injuries showed hyperechoic areas, signs or evidences of repair. Concerning the correlation between response to infiltration with platelet gel and gender/age of the patients, the clinical results appear not influenced by the age and the gender of the patient.
Our data indicate that, pain relief and ability to physical recovery of muscles, tendons and ligaments depend on tissue repair clearly visible by echo ultrasound evaluation. On the other hand tissue repair seems not occur in the large joints (hip and knee) where arthritis and /or corrosion of articular cartilage cannot be repaired and the only relief is exclusively linked to the reduction of periarticular inflammation (reduction of the inflammatory leakage and signs).
The mechanical properties of hydrogel scaffolds based on hyaluronic acid (HA) that were chemically crosslinked with
α,β
-poly(
N
-2-hydroxyethyl)(2-aminoethylcarbamate)-
D,L
-aspartamide (PHEA-EDA) ...were investigated. Variation of these properties as a function of three different PHEA-EDA amounts used to crosslink HA has been related to the reaction efficiency evaluated using a colorimetric assay. Moreover, the amount of unreacted amino groups that was still present in the hydrogels was related to the attachment behavior of human dermal fibroblasts to the hydrogel surface. The mechanical data and biological results suggest the suitability of the investigated hydrogels as scaffolds for the regeneration of soft tissues such as skin.
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