Fatty acid oxidation disorders and lipin-1 deficiency are the commonest genetic causes of rhabdomyolysis in children. We describe a lipin-1-deficient boy with recurrent, severe rhabdomyolytic episodes from the age of 4 years. Analysis of the LPIN1 gene that encodes lipin-1 revealed a novel homozygous frameshift mutation in exon 9, c.1381delC (p.Leu461SerfsX47), and complete uniparental isodisom...

New and advanced methodologies have been developed to characterize the nano and microstructure of cement paste and concrete exposed to aggressive environments. High resolution full-field soft X-ray imaging in the water window is providing new insight on the nano scale of the cement hydration process, which leads to a nano-optimization of cement-based systems. Hard X-ray microtomography images o...

Viridians streptococcal shock syndrome is a subtype of toxic shock syndrome. Frequently, the diagnosis is missed initially because the clinical features are nonspecific. However, it is a rapidly progressive disease, manifested by hypotension, rash, palmar desquamation, and acute respiratory distress syndrome within a short period. The disease course is generally fulminant and rarely presents in...

The presence of capillary pores and air voids influences concrete permeability to a large extent. Concrete density is inversely proportional to its porosity. The ingress of aggressive agents into the pore structure is responsible for various durability problems in concrete structures. Therefore, a durable concrete should have low permeability. Permeability of concrete increases with the increas...

Hyperleukocytosis (white blood cell count, >100×109/l), an uncommon presentation of leukemia, is associated with an increased risk of early mortality. It may present with a variety of symptoms secondary to leukostasis, a syndrome caused by the sludging of circulating leukemic blasts in the microvasculature. Adequate measures for managing this medical emergency include hydration, cytoreduction, ...

One of the most dangerous types cyclic effects, especially inherent in several regions world, is alternating impact wetting and drying on concrete reinforced structures. In current scientific literature practice, there not enough fundamental applied information about resistance to variotropic concretes obtained by centrifugal compaction methods. The purpose study was investigate effect various ...

Background Pancreatitis is the most common complication of endoscopic retrograde cholangiopancreatography (ERCP). Prophylactic rectal administration non-steroidal anti-inflammatory drugs (NSAIDs) considered as standard care to reduce risk post-ERCP pancreatitis. It has been suggested that aggressive hydration might further this risk. Guidelines already recommend in patients who are unable recei...

BACKGROUND Inadequate hydration in the elderly is associated with increased morbidity and mortality. However, few studies have addressed the knowledge of elderly individuals regarding hydration in health and disease. Gaps in health literacy have been identified as a critical component in health maintenance, and promoting health literacy should improve outcomes related to hydration associated il...

Supersulfated cement, with higher waste utilization and less cement clinker is the inevitable trend of the development of green material in the rapid developed industrial era. For the wide use of supersulfated cement in our country, the hydration heat is analyzed in this paper. The hydration kinetic parameters are obtained from the hydration heat and hydration heat rate curves measured by the i...