1 Jervell A, Lange-Nielse F. Congenital deaf-mutism, functional heart disease with prolongation of the QT interval and sudden death. Am Hearty 1957;54:59-68. 2 Schwartz PJ. Idiopathic long QT syndrome: Progress and questions. Am Hearty 1985;109:399-41 1. 3 Gillette PC, Garson A. Ventricular arrhythmias. Pediatric arrhythmias: electrophysiology and pacing. Philadelphia: WB Saunders, 1990:468-72....

The tcc paradigm is a formalism for timed concurrent constraint programming. Several tcc languages differing in their way of expressing infinite behavior have been proposed in the literature. In this paper we study the expressive power of some of these languages. In particular, we show that: (1) recursive procedures with parameters can be encoded into parameterless recursive procedures with dyn...

F amilial hypertrophic cardiomyopathy (FHC) is typically characterised by left ventricular hypertrophy, diastolic dysfunction, and hypercontractility, and is often associated with disabling symptoms, arrhythmias, and sudden death. FHC shows both non-allelic and allelic genetic heterogeneity, and results from any one of more than 100 mutations in genes encoding sarcomeric proteins. Identified ge...

LQTS (long QT syndrome) is an important cause of cardiac sudden death. LQTS is characterized by a prolongation of the QT interval on an electrocardiogram. This prolongation predisposes the individual to torsade-de-pointes and subsequent sudden death by ventricular fibrillation. Mutations in a number of genes that encode ion channels have been implicated in LQTS. Hereditary mutations in the αand...

Continuous-time stochastic volatility models are becoming an increasingly popular way to describe moderate and high-frequency financial data. Recently, Barndorff-Nielsen and Shephard (2001a) proposed a class of models where the volatility behaves according to an Ornstein-Uhlenbeck process, driven by a positive Lévy process without Gaussian component. These models introduce discontinuities, or j...

We present a formulation for the lattice fermion derivative which is both -local and explicitly chiral. Provided that the continuum and infinite volume limits are taken properly the formulation avoids spectrum doubling, and gives a satisfactory weak coupling perturbation theory for the case of Yukawa-coupled scalars. For gauge theories we find that a sum over long string paths must be introduce...

Introduction: Literature regarding the different degrees of hearing loss in patients with Cornelia de Lange syndrome (CDLS) reports that half of the affected patients exhibit severe to profound sensorineural hearing loss. We present the first pre-school child with CDLS who underwent cochlear implantation for congenital profound sensorineural hearing loss.   Case Report: A 3-year-old boy with CD...

implications of Cornelia de Lange syndrome. Paediatr Anaesth 1998; 8: 159–161. 7 Lumb AB, Carli F. Respiratory arrest after a caudal injection of bupivacaine. Anaesthesia 1989; 44: 324–325. 8 Mizuno J, Ichiishi N, In-nami H et al. Anesthetic management in a patient with Cornelia de Lange syndrome. Masui 2004; 53: 921–924. 9 Papadimos TJ, Maro AP. Cornelia de Lange syndrome, hyperthermia and a d...

Congenital long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval at basal ECG and by a high risk of life-threatening arrhythmias. Disease prevalence is estimated at close to 1 in 2,500 live births. The two cardinal manifestations of LQTS are syncopal episodes, that may lead to cardiac arrest and sudden cardiac death, and electrocardiographic ...

Inherited forms of long QT syndrome (LQTS) are characterised by an extended QT interval and clinical manifestations that include syncope and sudden death. The known genes in which mutations give rise to LQTS all produce components of cardiac ion channels. The two genes mutated in the majority of cases are KCNQ1 or HERG. The proteins produced from these genes are subunits which form tetrameric t...