Altered two-dimensional strain measures of the right ventricle in patients with Brugada syndrome and arrhythmogenic right ventricular dysplasia/cardiomyopathy

Aims: Brugada syndrome (BrS) is an inherited channelopathy that can be characterized by mild right ventricular (RV) abnormalities that are not detectable with conventional echocardiography. The aim of this study was to evaluate the presence of RV abnormalities in BrS patients when compared with controls and a group of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) using two-dimensional (2D) strain analysis. Methods and results: We enrolled 25 BrS, 15 ARVD/C patients, and 25 controls. Right and left ventricular dimension and systo-diastolic function were evaluated by conventional echocardiography. Longitudinal systolic strain (sS) peak, systolic and early diastolic strain rate of lateral RV segments were evaluated by 2D speckle tracking analysis. Left ventricle global and segmental strain measures were also evaluated. A reduced basal or mid-RV lateral sS were the parameters mostly associated with both BrS and ARVD/C. In BrS patients the minimum sS observed in these segments was significantly lower than that of controls (-28.9±3.2% vs. -32.3±3.2%, P: 0.002) but significantly greater than that evaluated in ARVD/C patients (-24.6 ±6.7%, P < 0.001 both vs. BrS and controls). No differences were found between the BrS and the control group when left ventricular strain measures were analysed. Conclusion: By 2D strain technique it is possible to observe mild abnormalities in RV systolic and diastolic function of BrS patients that are less pronounced than those observed in ARVD/C patients. These results help to better define the phenotypic characteristics of BrS patients and represent the basis for future studies aimed at testing their clinical usefulness in BrS patients

Focus on cardiologic findings in 30 children with PANS/PANDAS. an italian single-center observational study

Objective: Cardiac involvement in PANS has not been clarified relying on the scientific literature available until today. It is known that streptococcal infections play a role in the etiology of a great number of diseases including Sydenham chorea and rheumatic fever, among others. Based on the suspected pathogenesis of PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) reported in the medical literature, we decided to investigate the cardiologic involvement in children with a recent PANS/PANDAS diagnosis. Methods: The study population satisfies PANS (1) and PANDAS (2) criteria of diagnoses. Cardiologic assessment was performed through clinical examination, electrocardiography, and echocardiography. Results: In the selected pediatric population, a significant number of children presented mitral valve involvement, systolic murmurs and electrocardiographic abnormalities. High ASLOT levels did not seem to be associated to a cardiac involvement. Conclusions: Often PANS is difficult to diagnose because it is little known by physicians and most of the cardiologic findings described in this study are common among the healthy pediatric population. Also, ASLOT levels seems not to be predictive of cardiac involvement. Furthermore, the existence of PANDAS as a clinical entity is associated with a group of anti-neuronal autoantibodies found in Sydenham chorea is still controversial. We recommend a complete cardiologic evaluation in those children who meet the PANS/PANDAS diagnostic criteria

The fate of children with microdeletion 22q11.2 syndrome and congenital heart defect: clinical course and cardiac outcome

BACKGROUND: This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD). METHODS: A total of 49 consecutive children with 22q11.2 and CHD were retrospectively identified. The CHD consisted of tetralogy of Fallot and variances (n = 22), interrupted aortic arch (n = 10), ventricular septal defect (n = 8), truncus arteriosus (n = 6), and double aortic arch (n = 1). Extracardiac anomalies were present in 46 of 47 children. RESULTS: The median follow-up time was 8.5 years (range, 3 months to 23.5 years). Cardiac surgical repair was performed for 35 children, whereas 5 had palliative surgery, and 9 never underwent cardiac surgery. The median age at repair was 7.5 months (range, 2 days to 5 years). The mean hospital stay was 35 days (range, 7-204 days), and the intensive care unit stay was 15 days (range, 3-194 days). Significant postoperative complications occurred for 26 children (74%), and surgery for extracardiac malformations was required for 21 patients (43%). The overall mortality rate was 22% (11/49), with 1-year survival for 86% and 5-year survival for 80% of the patients. A total of 27 cardiac reinterventions were performed for 16 patients (46%) including 15 reoperations and 12 interventional catheterizations. Residual cardiac findings were present in 25 patients (71%) at the end of the follow-up period. CONCLUSIONS: Children with microdeletion 22q11.2 and CHD are at high risk for mortality and morbidity, as determined by both the severity of the cardiac lesions and the extracardiac anomalies associated with the microdeletion

Safe Sr confinement in feldspar structures

Samples of synthetic Na-A zeolite, pre-exchanged in Sr form, were heat treated at various temperatures up to 1150◦C, in order to entrap the toxic cation in a non-exchanging ceramic matrix. It has been demonstrated that the Sr-loaded zeolite undergoes the following sequence of transformations: zeolite →amorphous phase (at 900◦C) → hexagonal strontium aluminosilicate (at 1000◦C)→ monoclinic strontium aluminosilicate (above 1100◦C). Strontium retention performances of the above Sr-based compounds were compared to each other, estimating cation leaching either by a back-exchange with NaCl solution or by a prolonged contact with distilled water at different pHs (availability test). Thermal treatments from 900◦C onwards were able to safely immobilize Sr2+. The immobilization level depended on the relevant phase formed and resulted the highest (only 0.14% of theinitial Sr concentration), when monoclinic Sr-aluminosilicate structure (Sr-feldspar) was obtained

Monoclinic Sr-celsian by thermal treatment of Sr-exchanged zeolite A, LTA-type framework

An unconventional method for preparing monoclinic Sr-feldspar starting from a synthetic Na-A zeolite is presented. Accordingly, samples of Sr2+-exchanged zeolite A (Sr-A), containing a residual 5% content of Na+, were thermally treated at temperatures up to 1150 °C for times up to 24 h. Sr-A zeolitic structure breakdown, occurred at about 900 °C and followed by the crystallization of the hexagonal formof Sr-feldspar (Sr-hexacelsian) at about 950 °C, resulted in the final conversion, in 1 h at 1150 °C, of the original zeolitic material into a monoclinic polymorph of Sr-feldspar. Increasing the duration of the thermal treatment up to about 5 h gave rise to the crystallization of monoclinic Sr-celsian at only 1000 °C

Monoclinic Sr-celsian by thermal treatment of Sr-exchanged zeolite A, LTA-type framework

An unconventional method for preparing monoclinic Sr-feldspar starting from a synthetic Na-A zeolite is presented. Accordingly, samples of Sr2+-exchanged zeolite A (Sr-A), containing a residual 5% content of Na+, were thermally treated at temperatures up to 1150 °C for times up to 24 h. Sr-A zeolitic structure breakdown, occurred at about 900 °C and followed by the crystallization of the hexagonal formof Sr-feldspar (Sr-hexacelsian) at about 950 °C, resulted in the final conversion, in 1 h at 1150 °C, of the original zeolitic material into a monoclinic polymorph of Sr-feldspar. Increasing the duration of the thermal treatment up to about 5 h gave rise to the crystallization of monoclinic Sr-celsian at only 1000 °C

The interpretation of a negative intradermal reaction to tuberculin in a case of tuberculous lymphadenitis

This work aims at describing a particular case of tuberculous lymphadenitis with negative tuberculin skin test in a five-year child who has afterwards developed Hodgkin disease. BK presence is initially demonstrated through the examination of a first bioptic sample of a lymph-node, then confirmed by the presence in the serum of mycobacterial antigens on the peripheral lymphocytes and monocytes. Nine months later the second bioptic sample puts in evidence the presence of Reed-Sternberg cells. The follow-up of our patient took place at the Pediatric Department of the University of Rome "La Sapienza" and lasted an overall period of twenty days. The child was hospitalized twice and underwent a large number of radiological and laboratory tests. The therapy consisted only of medical specific treatment. Our study results lead us to evaluate the presence of immunological suppressive mechanisms in some particular tuberculosis cases, justifying the tuberculin skin-test negativity. When Hodgkin's disease has turned up, it has been too difficult to outline a precise temporal sequence of events, we could only advance hypothesis