Deterministic Concurrency: A Clock-Synchronised Shared Memory Approach

International audienceSynchronous Programming (SP) is a universal computational principle that provides deterministic concurrency. The same input sequence with the same timing always results in the same externally observable output sequence, even if the internal behaviour generates uncertainty in the scheduling of concurrent memory accesses. Consequently, SP languages have always been strongly founded on mathematical semantics that support formal program analysis. So far, however, communication has been constrained to a set of primitive clock-synchronised shared memory (csm) data types, such as data-flow registers, streams and signals with restricted read and write accesses that limit modularity and behavioural abstractions. This paper proposes an extension to the SP theory which retains the advantages of deterministic concurrency, but allows communication to occur at higher levels of abstraction than currently supported by SP data types. Our approach is as follows. To avoid data races, each csm type publishes a policy interface for specifying the admissibility and precedence of its access methods. Each instance of the csm type has to be policy-coherent, meaning it must behave deterministically under its own policy-a natural requirement if the goal is to build deterministic systems that use these types. In a policy-constructive system, all access methods can be scheduled in a policy-conformant way for all the types without deadlocking. In this paper, we show that a policy-constructive program exhibits deterministic concurrency in the sense that all policy-conformant interleavings produce the same input-output behaviour. Policies are conservative and support the csm types existing in current SP languages. Technically, we introduce a kernel SP language that uses arbitrary policy-driven csm types. A big-step fixed-point semantics for this language is developed for which we prove determinism and termination of constructive programs

First description of germline mosaicism in familial hypertrophic cardiomyopathy

Familial hypertrophic cardiomyopathy is a genetically and phenotypically heterogeneous disease caused by mutations in seven sarcomeric protein genes. It is known to be transmitted as an autosomal dominant trait with rare de novo mutations.
A French family in which two members are affected by hypertrophic cardiomyopathy was clinically screened with electrocardiography and echocardiography. Genetic analyses were performed on leucocyte DNA by haplotype analysis with microsatellite markers at the MYH7 locus and mutation screening by single strand conformation polymorphism analysis. Two subjects exhibited severe hypertrophic cardiomyopathy. A mutation in the MYH7 gene was found in exon 14 (Arg453Cys). The two affected patients were carriers of the mutation, which was not found in the circulating lymphocytes of their parents. Haplotype analysis at the MYH7 locus with two intragenic microsatellite markers (MYOI and MYOII) and the absence of the mutation in the father's sperm DNA suggested that the mutation had been inherited from the mother. However, it was not found in either her fibroblasts or hair.
This is the first description of germline mosaicism shown by molecular genetic analysis in an autosomal dominant disorder and more especially in hypertrophic cardiomyopathy. This mosaicism had been inherited from the mother but did not affect her somatic cells. Such a phenomenon might account for some de novo mutations in familial hypertrophic cardiomyopathy.


Keywords: hypertrophic cardiomyopathy; germline mosaicism; β myosin heavy chain; genetic

Genetic testing and genetic counselling in hypertrophic cardiomyopathy: the French experience

Methods and results: The main questions asked by patients and relatives concern presymptomatic diagnosis and prenatal counselling/diagnosis, while clinicians sometimes discuss diagnostic and prognostic testing. To take into account the complex medical and psychological implications of this new approach, we developed a specific, multidisciplinary, and multiple step procedure, including a cardiologist, a geneticist, and a psychologist. Seventy subjects were examined, including (1) 29 adults for presymptomatic diagnosis (of whom 10 left the procedure after the first visit and 19 continued, among whom six had a mutation and two experienced negative psychological impact, observed during follow up), (2) nine couples of parents for presymptomatic diagnosis in their children (the procedure was stopped after the first visit in eight and continued in one), (3) 22 couples for prenatal counselling (no prenatal genetic testing was asked for after the first visit), and (4) 10 subjects for diagnostic testing. We decided to perform no prognostic testing. Conclusion: Our preliminary experience confirms the complexity of the situation and suggests the necessity for a specific procedure to ensure good practice in genetic testing of HCM

Personality dimensions could be predictive of quality-of-life outcome after deep brain stimulation of the sub-thalamic nucleus in Parkinson's

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