HEART UK statement on the management of homozygous familial hypercholesterolaemia in the United Kingdom

This consensus statement addresses the current three main modalities of treatment of homozygous familial hypercholesterolaemia (HoFH): pharmacotherapy, lipoprotein (Lp) apheresis and liver transplantation. HoFH may cause very premature atheromatous arterial disease and death, despite treatment with Lp apheresis combined with statin, ezetimibe and bile acid sequestrants. Two new classes of drug, effective in lowering cholesterol in HoFH, are now licensed in the United Kingdom. Lomitapide is restricted to use in HoFH but, may cause fatty liver and is very expensive. PCSK9 inhibitors are quite effective in receptor defective HoFH, are safe and are less expensive. Lower treatment targets for lipid lowering in HoFH, in line with those for the general FH population, have been proposed to improve cardiovascular outcomes. HEART UK presents a strategy combining Lp apheresis with pharmacological treatment to achieve these targets in the United Kingdom (UK). Improved provision of Lp apheresis by use of existing infrastructure for extracorporeal treatments such as renal dialysis is promoted. The clinical management of adults and children with HoFH including advice on pregnancy and contraception are addressed. A premise of the HEART UK strategy is that the risk of early use of drug treatments beyond their licensed age restriction may be balanced against risks of liver transplantation or ineffective treatment in severely affected patients. This may be of interest beyond the UK

Classification Ensembles for Beach Cast and Drifting Vegetation Mapping with Sentinel-2 and PlanetScope

Along the Baltic coastline of Germany, drifting vegetation and beach cast create overlays at the otherwise sandy or stony beaches. These overlays influence the morphodynamics and structures of the beaches. To better understand the influence of these patchy habitats on coastal environments, regular monitoring is necessary. Most studies, however, have been conducted on spatially larger and temporally more stable occurrences of aquatic vegetation such as floating fields of Sargassum. Nevertheless, drifting vegetation and beach cast pose a particular challenge, as they exhibit high temporal dynamics and sometimes small spatial extent. Regular surveys and mappings are the traditional methods to record their habitats, but they are time-consuming and cost-intensive. Spaceborne remote sensing can provide frequent recordings of the coastal zone at lower cost. Our study therefore aims at the monitoring of drifting vegetation and beach cast on spatial scales between 3 and 10 m. We developed an automated coastline masking algorithm and tested six supervised classification methods and various classification ensembles for their suitability to detect small-scale assemblages of drifting vegetation and beach cast in a study area at the coastline of the Western Baltic Sea using multispectral data of the sensors Sentinel-2 MSI and PlanetScope. The shoreline masking algorithm shows high accuracies in masking the land area while preserving the sand-covered shoreline. We could achieve best classification results using PlanetScope data with an ensemble of a random forest classifier, cart classifier, support vector machine classifier, naïve bayes classifier and stochastic gradient boosting classifier. This ensemble accomplished a combined f1-score of 0.95. The accuracy of the Sentinel-2 classifications was lower but still achieved a combined f1-score of 0.86 for the same ensemble. The results of this study can be considered as a starting point for the development of time series analysis of the vegetation dynamics along Baltic beache