Introduction Holt & Oram (1960) linked various skeletal abnormalities with congenital heart disease in four generations of a family. In particular, malformations of the hands were associated with atrial septal defect (secundum type) and bizarre arrhythmias. In 1961, McKusick described a similar syndrome in a mother and daughter and suggested the term 'atrio-digital dysplasia'. Harris & Osborne ...

A clinical and genetic study of the Holt-Oram syndrome (HOS) has been carried out in the United Kingdom involving 55 cases designated Holt-Oram syndrome, together with their parents and sibs. Data from the clinical assessment of both familial and isolated cases were used to define the HOS phenotype and to outline the spectrum of abnormalities, especially factors affecting severity. Skeletal def...

Holt-Oram syndrome is an autosomal dominant disorder, characterised by skeletal abnormalities of the upper limb associated with congenital heart defect, mainly atrial and ventricular septal defects. Skeletal defects exclusively affect the upper limbs in the preaxial radial ray distribution and are bilateral and asymmetrical. They range from clinodactyly, absent or digitalised thumb, hypoplastic...

Holt-Oram syndrome is an autosomal dominant disorder characterised by radial ray and congenital heart defects. Recently, a gene for this disorder has been identified on chromosome 12q24.1, encoding a T box transcription factor. However, the functional role of the gene product is not completely understood. We present results of neurological, radiological, and muscle magnetic resonance imaging (M...

We generalize the correlation functions of the Clauser-Horne-Shimony-Holt (CHSH) inequality to arbitrarily high-dimensional systems. Based on this generalization, we construct the general CHSH inequality for bipartite quantum systems of arbitrarily high dimensionality, which takes the same simple form as CHSH inequality for two dimensions. This inequality is optimal in the same sense as the CHS...

We show that the no-disturbance principle imposes a tradeoff between locally contextual correlations violating the Klyachko-Can-Biniciogˇlu-Shumovski inequality and spatially separated correlations violating the Clauser-Horne-Shimony-Holt inequality. The violation of one inequality forbids the violation of the other. We also obtain the corresponding monogamy relation imposed by quantum theory f...

Photonic systems based on energy-time entanglement have been proposed to test local realism using the Bell inequality. A violation of this inequality normally also certifies security of device-independent quantum key distribution (QKD) so that an attacker cannot eavesdrop or control the system. We show how this security test can be circumvented in energy-time entangled systems when using standa...

We are the first to address the problem of efficient oblivious substring search over encrypted data supporting updates. Our two new protocols SA-ORAM and ST-ORAM obliviously search for substrings in an outsourced set of n encrypted strings. Both protocols are efficient, requiring communication complexity that is only poly-logarithmic in n. Compared to a straightforward solution for substring se...

We present Recursive Square Root ORAM (R-SQRT), a simple and flexible ORAM that can be instantiated for different client storage requirements. R-SQRT requires significantly less bandwidth than Ring and Partition ORAM, the previous two best practical constructions in their respective classes of ORAM according to client storage requirements. Specifically, R-SQRT is a 4x improvement in amortized b...

Holt-Oram syndrome is an autosomal-dominant condition characterized by congenital cardiac and forelimb anomalies. It is caused by mutations of the TBX5 gene, a member of the T-box family that encodes a transcription factor. Molecular studies have demonstrated that mutations predicted to create null alleles cause substantial abnormalities in both the limbs and heart, and that missense mutations ...