Jacobsen Syndrome with Hypoplastic Left Heart Syndrome: Outcome after Cardiac Transplantation

Jacobsen syndrome (JS) is a rare syndrome caused by a deletion of chromosome 11q. We report a patient with JS and hypoplastic left heart syndrome (HLHS) who required cardiac transplantation. She had many of the recognized morphological features in addition to immunological (lymphopenia) and hematological (thrombocytopenia) issues. The patient underwent a Norwood procedure with a modified Blalock–Taussig shunt (MBTS) and subsequently a Glenn procedure at six months of age. She developed desaturation, with severe tricuspid regurgitation and right ventricular dysfunction, and underwent heart transplantation at 7 months of age. After the transplant, she was hospitalized several times for severe infections. The diagnosis of Jacobsen syndrome came 2 months after transplant. Now, 5 years post-transplant, she is in relatively good health—her heart is functioning normally, her hospitalization rate is getting lower, and her immunological profile is stable

Consensus Report of the Technical-Scientific Associations of Italian Dental Hygienists and the Academy of Advanced Technologies in Oral Hygiene Sciences on the Non-Surgical Treatment of Peri-Implant Disease

Background: The recent publication of the new classification of periodontal and peri-implant disease has given clear indications on the parameters to be taken into consideration to correctly diagnose the different phases of these diseases. To date, however, there are no equally clear indications on the treatments to be implemented to solve these diseases. The objective of this Consensus Report is to provide guidance for the non-surgical management of peri-implant mucositis and peri-implantitis. For the drafting of the consensus, the most recent scientific literature was analysed. Materials and Methods: A group of 15 expert Italian dental hygienists were selected by the Italian technical-scientific societies (AIDI, UNID and ATASIO) and, starting from the literature review, they formulated indications according to the GRADE method (Grading of Recommendations, Assessment, Development, and Evaluation, a tool for rating the quality of evidence, used to draw up systematic reviews and clinical guidelines) on the treatment of peri-implant mucositis, peri-implantitis and on management of the various implanting surfaces. Conclusions: in accordance with the international literature, non-surgical therapy alone can resolve peri-implant mucositis, but not peri-implantitis. Several adjunctive therapies have been considered and some appear to be helpful in managing inflammation

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS): third Paediatric (Paedi-EUROMACS) report

OBJECTIVES: A third paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS: Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (<19 years of age) performed from 1 January 2000 to 31 December 2020 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS: Twenty-five hospitals contributed 537 registered implants in 480 patients. The most frequent aetiology of heart failure was any form of cardiomyopathy (59%), followed by congenital heart disease and myocarditis (15% and 14%, respectively). Competing outcomes analysis revealed that a total of 86% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 21.9% died while on support. At 12 months, 45.1% received transplants, 7.5% were weaned from their device and 20.8% died. The 3-month adverse events rate was 1.59 per patient-year for device malfunction including pump exchange, 0.7 for major bleeding, 0.78 for major infection and 0.71 for neurological events. CONCLUSIONS: The overall survival rate was 79.2% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age; P = 0.01) and lower weight (<20 kg; P = 0.015). Transplant rates at 6 months continue to be low (33.2%)

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS):third Paediatric (Paedi-EUROMACS) report

OBJECTIVES A third paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients ( The lack of European registration on durable mechanical circulatory support (MCS) led to the foundation of European Registry for Patients with Mechanical Circulatory Support (EUROMACS) in 2009

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS): fourth Paediatric EUROMACS (Paedi-EUROMACS) report

OBJECTIVES: The use of ventricular assist devices (VADs) in children is increasing. However, absolute numbers in individual centres and countries remain small. Collaborative efforts such as the Paedi-European Registry for Patients with Mechanical Circulatory Support (EUROMACS) are therefore essential for combining international experience with paediatric VADs. Our goal was to present the results from the fourth Paedi-EUROMACS report. METHODS: All paediatric (<19 years) patients from the EUROMACS database supported by a VAD were included. Patients were stratified into a congenital heart disease (CHD) group and a group with a non-congenital aetiology. End points included mortality, a transplant and recovery. Cox proportional hazard models were used to explore associated factors for mortality, cerebrovascular accident and pump thrombosis. RESULTS: A total of 590 primary implants were included. The congenital group was significantly younger (2.5 vs 8.0 years, respectively, P < 0.001) and was more commonly supported by a pulsatile flow device (73.5% vs 59.9%, P < 0.001). Mortality was significantly higher in the congenital group (30.8% vs 20.4%, P = 0.009) than in the non-congenital group. However, in multivariable analyses, CHD was not significantly associated with mortality [hazard ratio (HR) 1.285; confidence interval (CI) 0.8111-2.036, P = 0.740]. Pump thrombosis was the most frequently reported adverse event (377 events in 132 patients; 0.925 events per patient-year) and was significantly associated with body surface area (HR 0.524, CI 0.333-0.823, P = 0.005), CHD (HR 1.641, CI 1.054-2.555, P = 0.028) and pulsatile flow support (HR 2.345, CI 1.406-3.910, P = 0.001) in multivariable analyses. CONCLUSIONS: This fourth Paedi-EUROMACS report highlights the increasing use of paediatric VADs. The patient populations with congenital and non-congenital aetiologies exhibit distinct characteristics and clinical outcomes

The european registry for patients with mechanical circulatory support (EUROMACS): fourth paediatric EUROMACS (Paedi-EUROMACS) report.

OBJECTIVES The use of ventricular assist devices in children is increasing. However, absolute numbers in individual centers and countries remain small. Collaborative efforts such as the Paedi-EUROMACS are therefore essential in order to combine international experience with paediatric ventricular assist devices. In this paper, the results from the fourth Paedi-EUROMACS report are presented. METHODS All paediatric (<19 years) patients supported by a ventricular assist device from the EUROMACS database were included. Patients are stratified into a congenital heart disease group and a group with a non-congenital aetiology. Endpoints included mortality, transplantation and recovery. Cox proportional hazard models were used to explore associated factors for mortality, cerebrovascular accident, and pump thrombosis. RESULTS 590 primary implantations were included. The congenital group was significantly younger (2.5 versus 8.0 years respectively, p < 0.001) and were more commonly supported by a pulsatile flow device (73.5% vs 59.9%, p < 0.001). Mortality was significantly higher in the congenital group (30.8 vs 20.4%; p = 0.009) than in the non-congenital group. However, in multivariable analyses, congenital heart disease was not significantly associated with mortality (HR 1.285, CI 0.8111-2.036, p = 0.740). Pump thrombosis was the most frequently reported adverse event (377 events in 132 patients; 0.925 events per patient-year) and was significantly associated with BSA (HR 0.524 CI 0.333-0.823, p = 0.005), congenital heart disease (HR 1.641 CI 1.054-2.555, p = 0.028) and pulsatile flow support (HR 2.345 CI 1.406-3.910, p = 0.001) in multivariable analyses. CONCLUSIONS This fourth Paedi-EUROMACS report highlights the increasing use of paediatric ventricular assist devices. The patient populations with congenital and non-congenital aetiology exhibit distinct characteristics and clinical outcomes

The Italian phase of the EAQUATE measurement campaign

The international experiment EAQUATE (European AQUA Thermodynamic Experiment) was held in September 2004 in Italy and in the United Kingdom. The Italian phase, performed in the period 6-10 September 2004, was mainly devoted to assessment and validation of performances of new IR hyperspectral sensors and benefits from data and results of measurements of AQUA and in particular of AIRS. It is also connected with the preparatory actions of MetOp mission with particular attention to calibration and validation of IASI products (as water vapour and temperature profiles), characterization of semitransparent clouds and study of radiative balance, demonstrating the role of ground-based and airborne systems in validation operations.The Italian phase of the campaign was carried out within a cooperation between NASA Langley Research Center, University of Wisconsin, the Istituto di Metodologie per l'Analisi Ambientale (CNR-IMAA), the Mediterranean Agency for Remote Sensing (MARS) and the Universities of Basilicata, Bologna and Napoli. It involved the participation of the Scaled Composites Proteus aircraft (with NAST thermal infrared interferometer and microwave radiometer, the Scanning HIS infrared interferometer, the FIRSC far-IR interferometer), an Earth Observing System-Direct Readout Station and several ground based instruments: four lidar systems, a microwave radiometer, two infrared spectrometers, and a ceilometer. Radiosonde launches for measurements of PTU and wind velocity and direction were also performed as ancillary observations. Four flights were successfully completed with two different AQUA overpasses. The aircraft flew over the Napoli, Potenza and Tito Scalo ground stations several times allowing the collection of coincident aircraft and in- situ observations