Pediatric Patients with Sitosterolemia: Next-Generation Sequencing and Biochemical Examination in Clinical Practice.

Here, we report the pediatric cases of sitosterolemia, a rare autosomal-recessive genetic disorder, characterized by high concentrations of plant sterols in blood and heterogeneity manifestations. All three patients (two girls aged 2 and 6 years old, and one boy aged 14 years old) were initially diagnosed with hypercholesterinemia. Next-generation sequencing (NGS) revealed homozygous (p.

Nanostructured Temperature Indicator for Cold Chain Logistics.

Food, chemicals, agricultural products, drugs, and vaccines should be transported and stored within an appropriate low-temperature range, following cold chain logistics. Violations of the required temperature regime are generally reported by time-temperature indicators; however, current sensors do not cover a sufficiently broad low-temperature range and may lack thermal and photostability. Here, we report a nanostructured solvatochromic temperature indicator formed from cellulose nanocrystals decorated with carbon dots (C-dots).

Hybrid Materials Based on Carbon Nanotubes and Nanofibers for Environmental Applications.

With the advances in material science, hybrid nanomaterials with unique mechanical, electrical, thermal and optical characteristics have been developed. Among them, hybrids based on filamentous forms of carbon, such as carbon nanotubes and carbon nanofibers, in combination with inorganic nanoparticles attract particular attention. Due to the structure and morphology, charge and energy transfer processes lead to synergistic effects that allow the use of less material with higher productivity.

Determination of carvedilol by its quenching effect on the luminescence of terbium complex in dosage form.

A new, simple, sensitive luminescence methods for the determination of carvedilol have been developed and validated. Carvedilol was remarkably quenching the luminescence intensity of the Tb(III) ion in the new terbium complex with 1-butyl-4-hydroxy-2-oxo-1,2-dihydroquinoline-3-carboxylic acid-(4-methyl-pyridin-2-yl)-amide (R) in aqueous solutions containing urotropine buffer (pH 7.5) at lambda(ex) = 317 nm and lambda(em) = 545 nm.

Luminescence method for the determination of lorazepam in tablets.

New terbium complex of 7-chloro-5-(2-chlorophenyl)-3-hydroxy-1,2-dihydro-3H- 1,4-benzdiazepin-2-one (lorazepam, L), which is highly luminescent and do not require luminescence enhancers, is reported. The luminescence intensity of the Tb-L complex was enhanced by the addition of Tergitol 7 in water solution. The Tb-L-Tergitol 7 complex with a components ratio 1:1:2 was proposed to be used as the analytical form for the luminescence determination of drug--lorazepam.

Indirect spectrofluorimetric determination of mosapride citrate in pharmaceutical formulations.

New europium complexes of 3-oxo-1-hydroxy-quinoline-2-carboxylic acid amide derivatives (L1-L3), which are highly luminescent and do not require luminescence enhancers are reported. The luminescence intensity of the Eu-L1-3 complexes was enhanced by the addition of citrate ions in water solution. A sensitive luminescence enhancement system was developed for the determination of citrate ions on the base of Eu-9-fluoro-1-hydroxy-5-methyl-3-oxo-6,7-dihydro-3H,5H-pyrido[3,2,1-ij]quinoline-2-carboxylic acid (2-piperazin-1-yl-ethyl)-amide (L1) complex.

Sensitive terbium probes for luminescent determination of both alkaline phosphatase and codeine phosphate.

New terbium complexes of 2-oxo-4-hydroxy-quinoline-3-carboxylic acid derivatives are reported for determination of alkaline phosphatase (aPase) and codeine phosphate (CP). Complexation with terbium occurs by way of the oxygen atoms of the 4-hydroxyl group and the carbonyl group of the 3-carboxy function, respectively. The complexes are highly luminescent and do not require luminescence enhancers.