Patterns of democracy: Coalition governance and majoritarian modification in the United Kingdom, 2010–2015

The UK is often regarded as the archetype of Westminster democracy and as the empirical antithesis of the power-sharing coalitions of Western Europe. Yet, in recent years a different account has emerged that focuses on the subtler institutional dynamics which limit the executive. It is to this body of scholarship that this article responds, locating the recent chapter of coalition government within the wider context of the UK’s democratic evolution. To do so, the article draws Lijphart’s two-dimensional typology of democracies, developing a refined framework that enables systematic comparison over time. The article demonstrates that between over the course of the 2010-15 Parliament, the UK underwent another period of majoritarian modification, driven by factors including the long-term influence of the constitutional forces unleashed under Labour and the short-term impact of coalition management. The article makes several important contributions, salient in the UK and beyond. Theoretically, it offers a critical rejoinder to debates regarding the relationship between institutional design and democratic performance. Methodologically, it demonstrates that the tools of large-scale comparison can be effectively scaled-down to facilitate withincase analysis. Empirically, it provides a series of conclusions regarding the tenability of the UK’s extant democratic architecture under the weight of pressures to which it continues to be subject

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Severe hypertriglyceridemia in a newborn with monogenic lipoprotein lipase deficiency: an unconventional therapeutic approach with exchange transfusion.

Severe hypertriglyceridemia (sHTG) (plasma triglyceride level > 10 mmol/L) due to lipoprotein lipase (LPL) deficiency is a known risk factor for acute pancreatitis. A 23-day-old male with sHTG was admitted to the Neonatal Intensive Care Unit for plasmapheresis being at high risk for acute pancreatitis. Given the potential hazard of an extracorporeal technique in a very young infant, we decided to perform an exchange transfusion (ET), a procedure widely used by neonatologists and less invasive than plasmapheresis. ET led to a dramatic reduction in plasma triglyceride level, from 93.2 to 3.8 mmol/L at the end of the procedure, without adverse events. The subsequent administration of a special formula low in fat and high in medium-chain triglycerides was effective in keeping fasting plasma triglyceride level below 5.6 mmol/L during the first 5 months of life. The sequence of LPL gene revealed that the patient was apparently homozygous for a novel nucleotide deletion (c.840delG) in exon 6 leading to a premature termination codon (p.N281Mfs*23). However, family studies revealed that while the patient's mother was heterozygous for this mutation, the father was heterozygous for a novel deletion eliminating the whole LPL gene. The patient therefore turned out to be a compound heterozygous for two LPL gene mutations predicted to abolish LPL activity. This is the first case of sHTG treated with ET in a neonate reported in the literature. ET appears to be a safe procedure, alternative to plasmapheresis, to prevent acute pancreatitis in young infants with sHTG due to LPL deficiency