Chronobiological analysis of blood pressure in a patient with atrial fibrillation at the development of heart failure and its therapeutic and surgical treatment

Dynamics of blood pressure (BP) and heart rate (HR) was traced by automatic monitoring every 30 min uninterruptedly along several months in a patient suffering from combined atrial fibrillation and heart failure during the development of disease and its therapeutic and surgical treatment (pacemaker implanting and atrioventricular ablation). Analyses of spectral components as well as signal's shape revealed instabilities in circadian and semicircadian parameters. A new approach for signal's form description without using cosine approximation is suggested. The meaning that referring a patient as dipper, night peaker, or nondipper might be useful at choosing tactics of his treatment is impugned, because all these "types" can transform themselves in the same person in few days. Optimization timing of treatment provides better results if not the "types" of daily profile would be taken to account but the real form of the BP-signal and timing its first and second derivatives. © 2013 Sergey Chibisov et al

Glycogen storage disease type II (Pompe disease) in children

The paper gives the data available in the literature, which reflect the manifestations, diagnosis, and current treatments of the rare (orphan) inherited disease glycogen storage disease type II or Pomp disease in children, as well as its classification. The infant form is shown to be most severe, resulting in death from cardiovascular or pulmonary failure generally within the first year of a child’s life. Emphasis is laid on major difficulties in the differential and true diagnosis of this severe disease. Much attention is given to the new pathogenetic treatment — genetically engineered enzyme replacement drug Myozyme®. The authors describe their clinical case of a child with the juvenile form of glycogen storage disease type II (late-onset Pompe disease). Particular emphasis is laid on the clinical symptoms of the disease and its diagnostic methods, among which the morphological analysis of a muscle biopsy specimen by light and electron microscopies, and enzyme and DNA diagnoses are of most importance. The proband was found to have significant lysosomal glycogen accumulation in the muscle biopsy specimen, reduced lymphocyte acid α-1,4-glucosidase activity to 4,2 nM/mg/h (normal value, 13,0—53,6 nM/mg/h), described in the HGMD missense mutation database from 1000 G>A p.Gly334er of the GAA in homozygous state, which verified the diagnosis of Pompe disease

The effect of health on labour supply in nine former Soviet Union countries

This paper examines for the first time the consequences of ill health on labour supply for a sample of nine countries from the former Soviet Union (FSU), using a unique multicountry household survey specifically designed for this region. We control for a wide range of individual, household, and community factors, using both standard regression techniques and instrumental variable estimation to address potential endogeneity. Specifically, we find in our baseline ordinary least squares specification that poor health is associated with a decrease in the probability of working of about 13 %. Controlling for community-level unobserved variables slightly increases the magnitude of this effect, to about 14 %. Controlling for endogeneity with the instrumental variable approach further supports this finding, with the magnitude of the effect ranging from 12 to 35 %. Taken together, our findings confirm the cost that the still considerable adult health burden in the FSU is imposing on its population, not only in terms of the disease burden itself, but also in terms of individuals' labour market participation, as well as potentially in terms of increased poverty risk. Other things being equal, this would increase the expected "return on investment" to be had from interventions aimed at improving health in this region