BACKGROUND Although it is known that Anderson-Fabry Disease (AFD) can mimic the morphologic manifestations of hypertrophic cardiomyopathy (HCM) on echocardiography, there is a lack of cardiovascular magnetic resonance (CMR) literature on this. There is limited information in the published literature on the distribution of myocardial fibrosis in patients with AFD, with scar reported principally ...

Background Lysosomal storage disease (LSD) is a rare inherited disease group. Consecutively there are few data on cardiac changes in mucopolysaccharidosis (MPS), Anderson Fabry disease (AFD), and other LSD (oLSD) including Pompe disease (PD) and Danon disease (DD), I-cell disease ICD and mucolipidosis III (ML III). Methods Between 1994 and 2011, we identified 39 patients with LSD: 25 with MPS...

This paper develops a modest proposal for introducing final outcome indicators in the IDA aid allocation formula. It starts with a review of the current formula and the rationale for it. It is argued that this formula, and in particular the Country Policy and Institutional Assessment (CPIA) part of it, implicitly relies too heavily on a uniform model of what works in development policy. Even if...

Anderson-Fabry disease (AFd) is a rare, inherited, x-linked disease characterized by the deficiency of the lysosomal enzymatic alpha-galactosidase A activity (alpha-Gal-A). The enzyme defect leads to progressive accumulation of glycosphingolipids (GL) in all kinds of cells, tissues, organs, and body fluids. The clinical manifestations are very protean, the residual activity of alpha-Gal-A and/o...

Recently, Boyd published a paper entitled ‘‘A proof that the discrete singular convolution (DSC)/ Lagrange-distributed approximating function (LDAF) method is inferior to high order finite differences’’ (J. Comput. Phys. 214 (2006) 538–549 [3]), which will be referred to as ‘‘Proof’’. This Letter analyzes the validity of central claims in ‘‘Proof’’. In its title and abstract, ‘‘Proof’’ refers t...

Homeostatic control of body fluid CO(2) is essential in animals but is poorly understood. C. elegans relies on diffusion for gas exchange and avoids environments with elevated CO(2). We show that C. elegans temperature, O(2), and salt-sensing neurons are also CO(2) sensors mediating CO(2) avoidance. AFD thermosensors respond to increasing CO(2) by a fall and then rise in Ca(2+) and show a Ca(2+...

This work presents the most detailed and extensive theoretical study to date of the structural configurations of Ruddlesden–Popper (RP) phases in antiferrodistortive (AFD) perovskites and formulates a program of study which can be pursued for RP phases of any AFD perovskite system. We systematically investigate the effects of oxygen octahedral rotations on the energies of RP phases in AFD perov...

OBJECTIVE With the use of a new cohort of adolescent subjects, predictors from the Semi-Structured Assessment for the Genetics of Alcoholism (SSAGA) interview and the Achenbach Youth Self Report (YSR) were combined to model age of first drink (AFD). METHODS Subjects consisted of 820 adolescents (ages 14-17) drawn from the current phase of the Collaborative Study on the Genetics of Alcoholism....

Neurons receive input from the outside world or from other neurons through neuronal receptive endings (NREs). Glia envelop NREs to create specialized microenvironments; however, glial functions at these sites are poorly understood. Here, we report a molecular mechanism by which glia control NRE shape and associated animal behavior. The C. elegans AMsh glial cell ensheathes the NREs of 12 neuron...