G402 The impact of a standardised transcranial doppler training programme in screening children with sickle cell disease: a european multi-centre perspective

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Project Financing for Generation Integrated Energy Storage: A U.K. Context

Selection and peer-review under responsibility of the scientific committee of CUE2019Large-scale of intermittent renewables such as wind and solar are used to reduce carbon emission. Energy storage is needed to provide controllability of power flows for the energy system. Specifically, Generation-integrated energy storage (GIES) systems store energy at an instance during the transformation between the primary energy (e.g., kinetic or thermal) form and electricity. The successful deployment of GIES systems requires a financing method to minimize investment risk. This includes identifying the role and interactions between stakeholders. A contract is a legally-binding agreement, which recognizes and governs the duties and rights of the contracting parties (e.g. stakeholders) to the agreement. Despite the importance of contracts, there is a limited amount of literature on project financing and the contractual models for renewable energy projects, in particular for energy storage and GIES systems. Project financing is the long-term financing of infrastructure using the projected cash as collateral. In this paper, the public private partnership (PPP) schemes for financing renewable energy projects are reviewed. This work presents a generic contractual model for the GIES system in the U.K. context. The challenges in contractual agreements between contracting parties are described.This work is sponsored by the EPSRC grant “GIES: Generation Integrated Energy Storage: A Paradigm Shift” (EP/P022049/1). This work is jointly sponsored by the Department of Finance and Education of Guangdong Province 2016 [202]: Key Discipline Construction Program, China; and the Education Department of Guangdong Province: New and Integrated Energy System Theory and Technology Research Group [Project Number 2016KCXTD022]. This work is supported by IEEE Standards Association P2814: Techno-economic Metrics Standard for Hybrid Energy and Storage Systems

Incidence of Shwachman-Diamond syndrome

No abstract availabl

Management of the Sickle Cell Trait: An Opinion by Expert Panel Members.

The number of individuals with the sickle cell trait exceeds 300 million worldwide, making sickle cell disease one of the most common monogenetic diseases globally. Because of the high frequency of sickle cell disease, reproductive counseling is of crucial importance. In addition, unlike other carrier states, Sickle Cell Trait (SCT) seems to be a risk factor for several clinical complications, such as extreme exertional injury, chronic kidney disease, and complications during pregnancy and surgery. This expert panel believes that increasing knowledge about these clinical manifestations and their prevention and management can be a useful tool for all healthcare providers involved in this issue

Absence of acquired copy number neutral loss of heterozygosity (CN-LOH) of chromosome 7 in a series of 10 patients with Shwachman-Diamond syndrome

We report that acquired copy number neutral loss of heterozygosity (CN-LOH) of chromosome 7 was not identified in a series of 10 patients with Shwachman\u2013Diamond syndrome (SDS)

Centralized cytogenetic analysis of pediatric acute leukemia: results of an italian collaborative experience

Background and Objective. Cytogenetic analysis of acute leukemia yields important information which has been demonstrated to be correlated to patient survival. A reference laboratory was created in order to perform karyotype analysis on all cases of acute leukemia enrolled in the AIEOP (Associazione Italiana Emato-Oncologia Pediatrica) protocols. Methods. From January 1990 to December 1995, 1115 samples of children with ALL or AML were sent in for cytogenetic analysis. The results of cell cultures were screened in the Reference Laboratory and then the fixed metaphases were sent to one of the six cytogenetic laboratories for analysis. Results. The leukemic karyotypes of 556 patients were successfully analyzed. An abnormal clone was detected in 49% of cases of ALL and in 66% of AML. In ALL the most frequent abnormality was 9p rearrangement. Other recurrent abnormalities were t(9;22), t(4;11) and t(1;19). In AML t(8;21), t(15;17) and 11q23 rearrangement were the most frequent structural abnormalities. These findings are similar to the results obtained in other multicenter studies using a similar approach. Interpretation and Conclusions. Our data confirm the feasibility of performing cytogenetic analysis in a centralized laboratory on mailed samples of bone marrow and/or peripheral blood; this is very important considering that cytogenetic analysis of neoplastic tissue requires a special laboratory and expert staff

Environmental Factors in Northern Italy and Sickle Cell Disease Acute Complications: A Multicentric Study

Background: Environmental factors seem to influence clinical manifestations of sickle cell disease (SCD), but few studies have shown consistent findings. We conducted a retrospective multicentric observational study to investigate the influence of environmental parameters on hospitalization for vaso-occlusive crises (VOC) or acute chest syndrome (ACS) in children with SCD. Methods: Hospital admissions were correlated with daily meteorological and air-quality data obtained from Environmental Regional Agencies in the period 2011–2015. The effect of different parameters was assessed on the day preceding the crisis up to ten days before. Statistical analysis was performed using a quasi-likelihood Poisson regression in a generalized linear model. Results: The risk of hospitalization was increased for low maximum temperature, low minimum relative humidity, and low atmospheric pressure and weakly for mean wind speed. The diurnal temperature range and temperature difference between two consecutive days were determined to be important causes of hospitalization. For air quality parameters, we found a correlation only for high levels of ozone and for low values at the tail corresponding to the lowest concentration of this pollutant. Conclusions: Temperature, atmospheric pressure, humidity and ozone levels influence acute complications of SCD. Patients’ education and the knowledge of the modes of actions of these factors could reduce hospitalizations

Acute chest syndrome in children with sickle cell disease: Data from a national AIEOP cohort identify priority areas of intervention in a hub-and-spoke system

: Acute chest syndrome (ACS) is a frequent cause of hospitalization in sickle cell disease (SCD). Despite advances in acute care, many settings still lack knowledge about ACS best practices. After the AIEOP Guidelines were published in 2012, suggesting standardized management in Italy, a retrospective study was performed to assess the diagnostic and therapeutic pathways of ACS in children. From 2013 to 2018, 208 ACS episodes were presented by 122/583 kids in 11 centres. 73 were male, mean age 10.9 years, 85% African, 92% HbSS or Sβ°. In our hub-and-spoke system, a good adherence to Guidelines was documented, but discrepancies between reference centres and general hospitals were noted. Improvement is needed for timely transfer to reference centres, use of incentive spirometry, oxygen therapy and pain management