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Girls and women with Turner syndrome (TS) suffer from increased risk of cardiovascular diseases. We hypothesized that left ventricular (LV) myocardial strain and aortic elasticity will be impaired in girls with TS. Cardiac MRI of 45 girls with TS and 14 healthy control girls was performed. Tissue tracking was used to assess LV Global Longitudinal (GLS), circumferential (GCS), radial short and long axes (GRS SAX and GRS LAX) in patients compared to controls. Maximal and minimal aortic areas were measured in ascending aorta, proximal descending, and aorta at diaphragm. Regional strain and distensibility were calculated using previously validated formulas. Comparisons were made between patients with and without bicuspid aortic valve (BAV). Inter-observer agreement was assessed for myocardial strain and aortic strain and distensibility. Results of the study showed GLS was significantly impaired in TS patients − 15.6 ± 1.8% compared to the control group − 17.2 ± 1%, p value = 0.013. No significant differences were observed in other strain parameters. Aortic diameter was similar in patients and control groups. Ascending aorta strain and distensibility were significantly lower in TS patients (33 ± 19% and 9.1 ± 5.5 mm Hg − 1 ) compared to control group (55 ± 17% and 13.9 ± 4.9 10 − 3 mm Hg − 1 ), p values 0.004 and 0.013. No significant differences were found in aortic strain and distensibility in the other 2 regions. No significant differences were observed between TS patients with and without BAV in myocardial strain and aortic strain and distensibility. Substantial to perfect inter-observer agreement was found for myocardial strain and aortic strain and distensibility. Cardiac MRI measurements of LV deformation and aortic function carry potential value as markers for early detection of myocardial and aortic disease in TS patients. Validation of the clinical impact and prognostic role require further longitudinal studies.

Renal disease is associated with increased arterial stiffness. The aim was to investigate the effect of renal disease on regional aortic strain and distensibility in children with chronic kidney disease (CKD) by cardiac magnetic resonance imaging (MRI). The study included 30 children with CKD on hemodialysis, and ten healthy control subjects. Using cardiac MRI, maximal and minimal aortic areas were measured in axial cine steady state free precision images at the ascending aorta, proximal descending, and aorta at diaphragm. Regional strain and distensibility were calculated using previously validated formulas. Second reader aortic areas measurements were used to assess inter-observer agreement. Ascending aorta strain was significantly reduced in patients (38.4 ± 17.4%) compared to the control group (56.1 ± 17%), p-value 0.011. Ascending Aorta distensibility was significantly reduced in patients (9.1 ± 4.4 [× 10 −3  mm Hg −1 ]) compared to the control group (13.9 ± 4.9 [× 10 −3  mm Hg −1 ]), p-value 0.006. Strain and distensibility were reduced in proximal descending aorta and aorta at diaphragm but did not reach statistical significance. Only ascending aorta strain and distensibility had significant correlations with clinical and cardiac MRI parameters. Inter-observer agreement for strain and distensibility was almost perfect or strong in the three aortic regions. Aortic strain and distensibility by cardiac MRI are important imaging biomarkers for initial clinical evaluation and follow up of children with CKD.

We present the imaging findings of thoracic systemic venous anomalies diagnosed by computed tomography and magnetic resonance imaging. Persistent left superior vena cava is the commonest anomaly of the thoracic systemic veins encountered either incidentally as an isolated finding or associated with congenital heart disease. Inferior vena cava (IVC) interruption with azygos continuation is the second most common anomaly, which may also be isolated or be associated with left isomerism syndrome. The article will also discuss other rarer systemic venous anomalies including retroaortic brachiocephalic vein and IVC drainage into the left atrium. Finally, the impact of pre-procedure reporting of thoracic systemic venous anomalies on the choice of intervention and patient outcome will be addressed.

Turner syndrome (TS) patients are at high risk of cardiometabolic disorders. Cardiometabolic risk factors are more commonly related to visceral rather than total body adiposity. Adipocytokines have been explored as a potential link between obesity and obesity-related cardiometabolic dysfunction. This study explored the validity of epicardial fat-thickness (EFT) and perihepatic fat-thickness (PHFT) measurement as cardiometabolic-risk predictors in TS-girls in relation to standard obesity-indices and metabolic syndrome (MetS) components. Forty-six TS girls and twenty-five controls (10-16 years) were subdivided into two age-groups (10 to less than 13 and 13-16). Participants were assessed for body mass index (BMI) Z-scores, waist circumference (WC), total-fat mass (FM) and trunk-FM by bioimpedance-technique, EFT and PHFT by cardiovascular magnetic resonance, lipid-profile, homeostasis model assessment of insulin resistance (HOMA-IR), and serum chemerin. MetS was defined according to International Diabetes Federation criteria. Overweight/obesity and MetS were detected in 45.7% and 37% of TS-girls respectively. BMI Z-score, WC, total-FM, trunk-FM, EFT and PHFT values were significantly higher in TS-age groups compared to age-matched control groups, being more pronounced in the older group when TS-girls had been exposed to estrogen. Dyslipidemia, higher HOMA-IR, chemerin, EFT and PHFT values were observed in lean-Turner compared to BMI-Z-matched controls. EFT and PHFT were significantly correlated with chemerin and several components of MetS. EFT at a cut-off-value of 6.20 mm (area under the curve=0.814) can predict MetS in TS-girls. TS-girls displayed an adverse cardiometabolic profile during late childhood and adolescence. EFT and PHFT are emerging cardiometabolic risk predictors in TS-patients. Excess EFT rather than total body adiposity may contribute to altered metabolic profile among lean-Turner patients.

Background:Paediatric obesity is a worldwide health burden, with growing evidence linking obesity to myocardial function impairments. The study aims to evaluate left ventricular functions among prepubertal obese children to obesity-related clinical and metabolic parameters.Methods:Between June 2019 and March 2020, 40 prepubertal children with obesity were recruited and compared to 40 healthy controls. Patients were assessed for body mass index z scores, waist circumference, body adiposity by bioimpedance analysis, and obesity-related laboratory tests, for example, serum chemerin. Left ventricular functions were assessed using variable echocardiographic modalities, such as M-mode, tissue Doppler, and two-dimensional speckle tracking.Results:Mean patients’ age was 9.25 ± 1.05 years. Left ventricular mass index, E/E’, and myocardial performance index were significantly increased in obese children than controls. Although M-mode-derived ejection fraction was comparable in both groups, two-dimensional speckle tracking-derived ejection fraction, global longitudinal strain, and global circumferential strain were significantly lower in cases than controls. Left ventricular mass index displayed a positive correlation with body mass index z score (p = 0.003), fat mass index (p = 0.037), and trunk fat mass (p = 0.021). Global longitudinal strain was negatively correlated with body mass index z score (p = 0.015) and fat mass index (p = 0.016). Serum chemerin was positively correlated with myocardial performance index (p = 0.01).Conclusion:Alterations of left ventricular myocardial functions in prepubertal obese children could be detected using different echocardiographic modalities. Chemerin, body mass index z score, fat mass index, and trunk fat mass were correlated with subclinical left ventricular myocardial dysfunction parameters before puberty. Our results reinforce early and strict management of childhood obesity upon detection of changes in anthropometric and body adiposity indices.

PurposeTo evaluate whether extending the MRI scan to include the abdomen at the time of adnexal mass characterization could replace additional CT for peritoneal cancer index (PCI) assessment.MethodsAfter institutional review board approval for this prospective study, 36 consecutive females with ovarian and FT malignancies were included. All patients signed an informed consent. Patients underwent preoperative CT (32 patients) and MRI (36 patients). Images were interpreted by 2 independent observers. Surgical data were available in 27 patients. Region-by-region analysis was performed for detection rates of peritoneal carcinomatosis (PC). Inter-observer agreement for each region was evaluated by kappa statistics. Radiologic PCI was calculated by CT and MRI independently and inter-observer agreement for CT and MRI as well as agreement between radiologic and surgical PCI were evaluated by weighted-kappa statistics.ResultsOn region-by-region analysis, the highest detection rates of PC were noted at the central abdomen and pelvis. Detection rates were higher by MRI than CT, mainly in bowel serosal surface, pelvis, and right upper abdomen regions. Inter-observer agreement of MRI was higher than CT in most regions. The median PCI by CT was 5 and 4 for the first and second observers (range 0–21 for both observers), respectively. The median PCI by MRI was 6 (range 0–23 for both observers). The inter-observer agreement of PCI was excellent by both CT and MRI (k = 0.876 and k = 0.912, respectively). The agreement between CT and surgical PCI was 0.660 and 0.590 for the first and second observers, respectively. The agreement between MRI and surgical PCI was 0.797 and 0.798 for the first and second observers, respectively.ConclusionsExtending MRI scan to include the abdomen at the time of adnexal mass characterization allows accurate estimation of PC, with better results than CT, obviating the need for dedicated CT scan of abdomen and pelvis for imaging of PC.

Introduction To evaluate effects of sarcopenia and visceral obesity on survival and postoperative outcomes in patients with ovarian cancer undergoing interval debulking surgery. Methods Preoperative abdominal CT of 61 patients was analyzed using dedicated software. Skeletal muscle index (SMI), skeletal muscle attenuation (SMA), visceral adipose tissue area (VAT), and ratio of visceral/ subcutaneous adipose tissue (VAT/SAT) were calculated. Patients were subgrouped according to previously published parameters thresholds. Overall and progression-free survival (OS and PFS) were estimated using Kaplan–Meier test. Cox regression was used for analysis of hazards ratios (HR) of death. Results Mean OS was significantly less in sarcopenic versus non-sarcopenic groups (31.5 ± 11.3, 67 ± 5.5 months, p  = 0.036). Mean PFS time was 26.5 ± 9.9 for sarcopenia and 45.4 ± 4.7 months for non-sarcopenia groups, p  = 0.174. Mean OS time was less in low SMA compared to high SMA groups, without statistical significance (50 ± 7.7, 69 ± 6.2 months, p  = 0.19). Mean PFS was 37.7 ± 6.8 for low SMA and 47 ± 5.9 months for high SMA groups, p  = 0.297. Mean OS was longer in obese and high VAT/SAT compared to nonobese and low VAT/SAT groups without statistical significance (66.4 ± 6.2 and 61.9 ± 6 vs. 51.4 ± 8.2 and 57.4 ± 8 months, p  = 0.395 and 0.409, respectively). Mean PFS time was 46.2 ± 5.6 and 42.3 ± 5.5 months for obese and high VAT/SAT compared to 37.8 ± 7.4 and 42.6 ± 6.64 months for nonobese and low VAT/SAT groups, p  = 0.394 and p  = 0.865, respectively. Multivariable analyses revealed that Clavien-Dindo complications score and sarcopenia were independent predictors of poorer OS (HR:1.719, p  = 0.019 and HR:6.507, p  = 0.015, respectively). Conclusion Sarcopenia is predictor of poor survival in ovarian cancer patients undergoing interval debulking surgery.

Children with steroid-dependent minimal change nephrotic syndrome are prone to serious steroid side effects. Alternative therapies, such as oral cyclophosphamide, may also have serious side effects. We conducted this novel prospective study to compare the long-term efficacies of levamisole and I.V. pulse cyclophosphamide as therapies with potentially fewer side effects. This study included 40 children with idiopathic steroid-dependent minimal change nephrotic syndrome (age 3-15 years; 31 boys and 9 girls). The patients were randomized into two equal groups. One group received levamisole 2.5 mg/kg on alternate days (levamisole group) while the other group received I.V. cyclophosphamide 500 mg/m2/month for six months (cyclophosphamide group). Prednisolone was gradually withdrawn. After stopping treatment, the number of patients that maintained remission was five (25%) in each group at six months, four (20%) versus two (10%) at one year and three (15%) versus one (5%) at two years in the levamisole and cyclophosphamide groups respectively, and one (5%) in each group at three and four years. The overall side effects were mild and both drugs were well tolerated. In view of the results, we recommend trial of levamisole before adopting other therapies with more serious side effects in such patients.

Background/Aims: In developing countries, kidney transplantation is greatly hindered by financial problems, especially due to costly newer immunosuppressive medications. Ketoconazole increases blood levels of tacrolimus and cyclosporine through inhibition of cytochrome P450 microsomal enzymes. We previously reported on the 6-month safety and the outstanding impact on treatment costs of the ketoconazole-tacrolimus combination in kidney transplant recipients. Data of this combination are still lacking in the literature. We hereby report on the 2-year results of our trial. Methods: This prospective, randomized study included 70 live-donor kidney transplant recipients receiving tacrolimus (age 16–45 years, 54 males and 16 females). Patients were randomized into two equal groups: group 1, where ketoconazole 100 mg/day was added, and group 2 (control group). Results: After 2 years, group 1 (ketoconazole) patients still showed a highly significant reduction of the tacrolimus dose (by 53.8%) and cost (by 52.9%) compared with the control group (p < 0.001) and a significant improvement in graft function in comparison to their own initial graft function (p = 0.002). Throughout the 2 years, no side effects of ketoconazole were noted. Conclusion: We conclude that the long-term ketoconazole-tacrolimus combination therapy in kidney transplant recipients during the 2 years is safe, has an outstanding impact on treatment costs and improves graft outcome.

In children with minimal change nephrotic syndrome (MCNS), the steroid dependent group constitutes an especially difficult case for management. Patients in this group are prone to serious steroid side effects. Additionally, alkylating agents commonly fail to maintain remission and expose patients to more side effects. Therapy with the immunostimulant drug levamisole may therefore be another option in the attempt to maintain remission with minimal side effects. We prospectively treated 20 of our steroid dependent primary MCNS patients with levamisole. All patients were children, with an age range of 3-15 years; 16 were boys and 4 were girls. Remission was firstly induced by steroids, then levamisole was added in a dose of 2.5 mg/kg body weight on alternate days for 6 months. During this period we attempted to withdraw steroids completely and maintain patients on levamisole alone. We followed up our patients for the occurrence of relapse and side effects during this period and for a further 6 months after stopping levamisole. In 11 out of 20 children (55%), we successfully stopped steroids for more than 2 weeks. At the end of the 6-month treatment period (i.e. after 4 months of steroid discontinuation), ten patients (50%) were maintaining remission on levamisole alone. At the end of the 12-month study period (i.e. after 6 months of levamisole discontinuation), five patients (25%) were still in remission without any treatment for the previous 6 months. No significant side effects were reported during levamisole therapy. None of the patients developed neutropenia, but the leukocyte count showed a significant reduction in those who responded to levamisole treatment. We concluded that levamisole therapy for 6 months is a safe and perhaps effective therapy in a subset of children with steroid dependent MCNS to enable an otherwise infeasible withdrawal of steroids. This may be worth a trial before other types of more hazardous adjunctive therapies are considered.