Peritoneal seeding and subsequent progression of mantle cell lymphoma after splenectomy for debulking

Backgroun

Normosmic idiopathic hypogonadotropic hypogonadism due to a novel homozygous nonsense c.C969A (p.Y323X) mutation in the KISS1R gene in three unrelated families

PubMedID: 25262569Objective: The spectrum of genetic alterations in cases of hypogonadotropic hypogonadism continue to expand. However, KISS1R mutations remain rare. The aim of this study was to understand the molecular basis of normosmic idiopathic hypogonadotropic hypogonadism. Methods: Clinical characteristics, hormonal studies and genetic analyses of seven cases with idiopathic normosmic hypogonadotropic hypogonadism (nIHH) from three unrelated consanguineous families are presented. Results: One male presented with absence of pubertal onset and required surgery for severe penoscrotal hypospadias and cryptorchidism, while other two males had absence of pubertal onset. Two of four female cases required replacement therapy for pubertal onset and maintenance, whereas the other two had spontaneous pubertal onset but incomplete maturation. In sequence analysis, we identified a novel homozygous nonsense (p.Y323X) mutation (c.C969A) in the last exon of the KISS1R gene in all clinically affected cases. Conclusions: We identified a homozygous nonsense mutation in the KISS1R gene in three unrelated families with nIHH, which enabled us to observe the phenotypic consequences of this rare condition. Escape from nonsense-mediated decay, and thus production of abnormal proteins, may account for the variable severity of the phenotype. Although KISS1R mutations are extremely rare and can cause a heterogeneous phenotype, analysis of the KISS1R gene should be a part of genetic analysis of patients with nIHH, to allow better understanding of phenotype-genotype relationship of KISS1R mutations and the underlying genetic basis of patients with nIHH. © 2014 John Wiley & Sons Ltd

Cotton production in Australia

Australia is known worldwide for producing cotton of the finest quality. Cotton production has an important position in the economy of Australia, earning foreign exchange of two billion dollars annually. Australian cotton production is highly mechanized, and its production systems are quite specialized with emphasis on more crops per drop. However, several factors are making Australian cotton production challengeable, such as climate change, water scarcity, emergence of new insects, diseases and weeds, waterlogged soils and rising production cost. Australian cotton growers, as well as cotton scientists, put sincere efforts to cope up with these issues. New agronomic tools, for example, the skip‐row technique for water saving in the dry land area, timely planting, and use of glyphosate‐tolerant and Helicoverpa ‐resistant cultivars, were adopted to increase the yield potential. Synergy among weather forecasting, fertilizer, water, and pest management in the integrated crop management mode is being considered for high efficiency of cotton production systems. This book chapter explores current issues of Australian cotton production systems and opportunities to solve these issues

The evolution of cleavage voting in four Western countries: Structural, behavioural or political dealignment?

Since the heyday of cleavage voting in the 1960s and 1970s, the majority of studies presents evidence of a decline in cleavage voting – caused by either structural or behavioural dealignment. Structural dealignment denotes changes in group size responsible for a decrease in cleavage voting, whereas behavioural dealignment concerns weakening party–voter links over time. A third phenomenon posited in this article is the collective voting abstention of certain (social) groups, here referred to as ‘political dealignment’, which results in a new type of division of voting versus abstention. The purpose of this article is to examine the three underlying mechanisms for the decline in social class and religious cleavage voting across four Western countries (Great Britain, the Netherlands, Switzerland and the United States) over the last 40–60 years using longitudinal post‐election data. The results prove a strong presence of political dealignment and increasing turnout gaps regarding both the class and religious cleavage. Furthermore, whenever a decline in cleavage voting is present, it is mainly caused by changes in the social groups’ behaviour and less by changing social structures in a country