The effective doses of remimazolam besylate in the procedural sedation of endoscopic retrograde cholangiopancreatography.

This study aimed to determine the values of the half-effective dose (ED) and 95% effective dose (ED) of remimazolam besylate used in the procedural sedation of endoscopic retrograde cholangiopancreatography (ERCP). Sixty patients who fulfilled the inclusion and exclusion criteria of this study were selected. Sufentanil was administered intravenously and remimazolam besylate was administered 2 min later.

Uniparental disomy for chromosome 1 with splice-site variant causes muscle-eye-brain disease.

, encoding protein O-mannose beta-1,2-N-acetylglucosaminyltransferase 1, is one of the genes responsible for dystroglycanopathy (DGP), which includes multiple phenotypes such as muscle-eye-brain disease (MEB), congenital muscular dystrophy with intellectual disability, and limb-girdle muscular dystrophy Here, we report a case of MEB that is the result of a homozygous variant of that is revealed through uniparental disomy (UPD). An 8-month-old boy was admitted with mental and motor retardation, hypotonia, esotropia, early onset severe myopia, and structural brain abnormalities. A panel testing of genetic myopathy-related genes was used to identify a homozygous c.

Derivation of copper water quality criteria in the Bohai Sea of China considering the effects of multiple environmental factors on copper toxicity.

Copper has become one of the most important heavy metal pollutants in the environment because of its wide application and high toxicity, but research on water quality criteria (WQCs) on copper is limited, especially the derivation of seawater WQC. In addition, the toxicity of copper in the seawater system is affected by various environmental factors. Therefore, establishing a WQC that meets the characteristics of the regional environment is a top priority.

Establishment of a comprehensive method to derive seawater quality criteria of BDE-47 in China.

2,2',4,4'-tetrabromodiphenyl ether (BDE-47) is an emerging toxic organic pollutant widely detected in territorial waters. Accordingly, establishing the seawater quality criteria (SWQC) for BDE-47 is of significant importance to protect the marine ecosystems. In this study, published ecotoxicity data of BDE-47 to aquatic species in China were collected, and acute and chronic toxicity tests were carried out on saltwater aquatic organisms from 5 phyla and 8 families widely existed in the Chinese marine environment.

A new phenotype of syndromic retinitis pigmentosa with myopathy is caused by mutations in retinol dehydrogenase 11.

Retinol dehydrogenase 11 (RDH11) is an 11-cis-retinol dehydrogenase that has a well-characterized, albeit auxiliary role in the retinoid cycle. Diseases caused by mutations in the RDH11 gene are very rare, and only one affected family with eye and intelligence involvement has been reported. In the present study, we describe the clinical and genetic findings in a Chinese patient with retinitis pigmentosa (RP), juvenile cataracts, intellectual disability, and myopathy.

Phenotype and Genotype Study of Chinese -Related α-Dystroglycanopathy.

Objective: Alpha-dystroglycanopathy (α-DGP) is a subtype of muscular dystrophy caused by defects in the posttranslational glycosylation of α-dystroglycan (α-DG). Our study aimed to summarize the clinical and genetic features of -related α-DGP in a cohort of patients in China.

Methods: Pedigrees, clinical data, and laboratory tests of patients diagnosed with -related α-DGP were analyzed retrospectively.

Resolution and evaluation of 3-chlorophenyl-3-hydroxypropionylhydroxamic acid as antivirulence agent with excellent eradication efficacy in Helicobacter pylori infected mice.

The continuing emergence of drug-resistant Helicobacter pylori (HP) drives the ongoing need for the development of new and effective anti-HP drugs. Urease inhibitor has now gained strong interest as an alternative approach for HP infections. 3-Chlorophenyl-3-hydroxypropionylhydroxamic acid (CPH), a novel urease inhibitor identified in our group, shows impressive potency, which was optically separated for a further exploration.