Pulmonary hypertension (PH), marked by sustained elevation of pulmonary artery pressure, imposes a heavy burden on the right ventricle and may culminate in right heart failure. Its pathogenesis is multifaceted, encompassing endothelial dysfunction, vascular smooth muscle proliferation, inflammation, thrombosis, and genetic factors. Animal models serve as core tools for exploring PH mechanisms and therapies, each with unique strengths and limitations. The single-dose monocrotaline (MCT) model is one of the most commonly used experimental animal models of PH and is widely applied in mechanistic studies, drug screening, and efficacy evaluation; it offers simplicity and cost-effectiveness, can induce PH within a short period, yet its pathophysiology differs to some extent from human idiopathic PH. In contrast, the Sugen5416 combined with chronic hypoxia model better mimics PH progression by placing animals under hypoxic conditions to induce pulmonary vasoconstriction and vascular remodeling, but it requires a longer modelling time, and the degree of hypoxia has a substantial impact on experimental outcomes. Beyond these two commonly used modeling approaches, a variety of emerging techniques have been applied in PH research; gene-editing technologies enable precise investigation of specific gene functions in PH. Additionally, induced pluripotent stem cell-based 3D organoid technology allows for individualized modelling while preserving patients' genetic information for precise clinical translation. Each model or technology can simulate different aspects of the pathological processes of human PH, and their findings provide key insights into the nature of the disease and serve as an important platform for the development of novel therapeutic targets. This paper comprehensively describes various animal models and emerging technologies used in PH research, analyzing their characteristics, applications, and limitations, with the aim of providing experimental and technical support for the development of new therapeutic strategies and drugs.

Objective To develop a standardized evaluation index system for assessing teaching quality in clinical teach-ing bases,providing a framework for self-assessment and continuous improvement.Methods An initial set of evaluation indica-tors was developed through a comprehensive literature review.Using the Delphi method,expert opinions were collected via struc-tured questionnaires from 22 specialists in the field.Indicator weights were determined through the fuzzy comprehensive evaluation method.The evaluation frequency and interpretive meaning of each indicator were subsequently established.Results A hierar-chical evaluation index system was constructed,consisting of 3 primary indicators,7 secondary indicators,and 17 tertiary indica-tors.The weight and significance of each indicator were defined.Based on expert consensus,it is recommended that evaluations be conducted once per academic semester.Conclusion The proposed evaluation index system aligns with both clinical and edu-cational principles,features comprehensive and practical content,and is well-suited for self-assessment by clinical teaching ba-ses.It provides a robust reference for enhancing the overall quality of clinical education.

Objective To develop a standardized evaluation index system for assessing teaching quality in clinical teach-ing bases,providing a framework for self-assessment and continuous improvement.Methods An initial set of evaluation indica-tors was developed through a comprehensive literature review.Using the Delphi method,expert opinions were collected via struc-tured questionnaires from 22 specialists in the field.Indicator weights were determined through the fuzzy comprehensive evaluation method.The evaluation frequency and interpretive meaning of each indicator were subsequently established.Results A hierar-chical evaluation index system was constructed,consisting of 3 primary indicators,7 secondary indicators,and 17 tertiary indica-tors.The weight and significance of each indicator were defined.Based on expert consensus,it is recommended that evaluations be conducted once per academic semester.Conclusion The proposed evaluation index system aligns with both clinical and edu-cational principles,features comprehensive and practical content,and is well-suited for self-assessment by clinical teaching ba-ses.It provides a robust reference for enhancing the overall quality of clinical education.

Objective:To investigate the protective effect of calycosin on microgravity-induced muscle atrophy by using real-time shear wave elastography (RT-SWE).Methods:The potential key active compound calycosin of anti-muscular atrophy in Astragali Radix was screened by systematic pharmacology. Eighteen healthy male Sprague-Dawley rats were divided into the control group [0.5% carboxymethyl cellulose-Na (CMC-Na) gavage], the hind limb unloading (HLU)+CMC-Na group (HLU+0.5% CMC-Na gavage), and the HLU+calycosin group (HLU+calycosin gavage) according to the method of random number table, with 6 rats in each group. After 28 d of continuous administration, the organ index, the toxicity of liver and kidney, the wet weight of soleus muscle and the ratio of muscle weight to body weight was measured, respectively. The non-invasive RT-SWE was used to evaluate the thickness and elastic modulus of rectus femoris in each group and the one-way analysis of variance was used to compare the differences among groups.Results:There was no significant difference in organ index, liver and kidney toxicity among different groups of rats (all P>0.05). There were significant differences in the weight of soleus muscle and the ratio of muscle weight to body weight among different groups of rats ( F=60.66, 56.44, both P<0.001). Compared with the HLU+CMC-Na group, the weight of soleus muscle and the ratio of muscle weight to body weight in the HLU+calycosin group increased, and the differences were significant (both P<0.01). The thickness and elastic modulus of rectus femoris of rats in different groups were significantly different ( F=35.47, 14.68, both P<0.001). Compared with the HLU+CMC-Na group, the muscle thickness and elastic modulus of rats in HLU+calycosin group were increased, and the differences were significant (both P<0.01). Conclusions:The treatment of calycosin has no side effects on rats. It can improve the thickness and elastic modulus of rectus femoris, and effectively prevent microgravity-induced muscle atrophy, which may provide a new candidate drug for astronaut muscular atrophy.

Objective:To investigate the protective effect of calycosin on microgravity-induced muscle atrophy by using real-time shear wave elastography (RT-SWE).Methods:The potential key active compound calycosin of anti-muscular atrophy in Astragali Radix was screened by systematic pharmacology. Eighteen healthy male Sprague-Dawley rats were divided into the control group [0.5% carboxymethyl cellulose-Na (CMC-Na) gavage], the hind limb unloading (HLU)+CMC-Na group (HLU+0.5% CMC-Na gavage), and the HLU+calycosin group (HLU+calycosin gavage) according to the method of random number table, with 6 rats in each group. After 28 d of continuous administration, the organ index, the toxicity of liver and kidney, the wet weight of soleus muscle and the ratio of muscle weight to body weight was measured, respectively. The non-invasive RT-SWE was used to evaluate the thickness and elastic modulus of rectus femoris in each group and the one-way analysis of variance was used to compare the differences among groups.Results:There was no significant difference in organ index, liver and kidney toxicity among different groups of rats (all P>0.05). There were significant differences in the weight of soleus muscle and the ratio of muscle weight to body weight among different groups of rats ( F=60.66, 56.44, both P<0.001). Compared with the HLU+CMC-Na group, the weight of soleus muscle and the ratio of muscle weight to body weight in the HLU+calycosin group increased, and the differences were significant (both P<0.01). The thickness and elastic modulus of rectus femoris of rats in different groups were significantly different ( F=35.47, 14.68, both P<0.001). Compared with the HLU+CMC-Na group, the muscle thickness and elastic modulus of rats in HLU+calycosin group were increased, and the differences were significant (both P<0.01). Conclusions:The treatment of calycosin has no side effects on rats. It can improve the thickness and elastic modulus of rectus femoris, and effectively prevent microgravity-induced muscle atrophy, which may provide a new candidate drug for astronaut muscular atrophy.

Objective: To investigate the physical and intellectual development and mutation characteristics of the phenylalanine hydroxylase (PAH) gene among 53 newborns with phenylketonuria (PKU), so as to provide insights into the management and genetic counseling of PKU Methods: The medical records of 54 children with definitive diagnosis of PKU and standardized therapy until 2 years at the Center for Neonatal Disease Screening of Shanxi Children' s Hospital from 2018 to 2021 were collected. Newborns' body weight and height developments were evaluated using the World Health Organization growth chart (2006 version), and the intellectual development was assessed using the national criteria of Development Behavior Assessment Scale among Children at Ages of 0 to 6 Years (WS/T 580-2017). The gene mutations were detected among neonates and their children, and the physical, intellectual developments and genetic characteristics of neonates with PKU were descriptively analyzed. Results: The 53 PKU cases included 29 male children and 24 female children, 36 cases with classic PKU and 17 cases with mild PKU, and 30 cases from rural areas and 23 cases from urban areas. The study subjects had a median age of 30 (10) d at initial therapy, and a mean blood phenylalanine concentration of (1 507±685) μmol/L at definitive diagnosis. There were 52 cases with normal height developments (98.11%), and all cases had normal weight and intellectual developments. The mean developmental functional quotient (DFQ) was significantly greater among urban children with PKU than among rural children [(94.92±8.57) vs. (87.65±6.57); t=-3.498, P=0.001], and the mean DFQ was significantly higher among children with mild PKU than among those with classic PKU [(95.55±8.76) vs. (88.57±7.11); t=-3.095, P=0.003]. There were 37 mutations detected in the PAH gene, which were mainly distributed in exons 3, 6, 7, 11, 12 and intron 4. Three high-frequency mutation sites were detected, including c.728G>A, c.611A>G and c.1197A>T, including three novel mutations (c.674C>G, c.1316-2A>C and c.1069T>C). Conclusions Following standardized treatment, the children with PKU have comparable physical and intellectual developments as compared to normal children. c.728G>A, c.611A>G and c.1197A>T were predominant mutations in the PAH gene among these 53 children with PKU, and three novel mutations were identified, including c.674C>G, c.1316-2A>C and c.1069T>C.

Objective:To investigate the distribution of clustered regularly interspaced short palindromic repeats (CRISPR) in group B Streptococcus (GBS) in the genital tract of women during the third trimester and in infants with invasive infection and its relationship with multilocus sequence typing (MLST) and drug-resistance genes. Methods:This study retrospectively collected 84 GBS strains isolated from pregnant women with GBS colonization and infants with invasive GBS infection who were admitted to Children's Hospital Affiliated to Shanxi Medical University from January 2017 to January 2022. CRISPR, MLST, and drug-resistance phenotype and genes were detected and analyzed using χ 2 test or Fisher exact probability method. MEGA11 was used to construct a dendrogram. Results:There were ten sequence typing in the 84 GBS strains and ST10 was the dominant one (46.4%). GBS was sensitive to penicillin, and its resistance rates to erythromycin (75.0%) and clindamycin (73.8%) were high. Among the 17 invasive GBS strains, ST10 had 100% resistance to erythromycin, clindamycin, and levofloxacin. CRISPR1 gene was amplified in 62 strains (73.8%). CRISPR1-positive strains had a significantly higher proportion of ST10 [56.5%(35/62) vs 18.2%(4/22), χ 2=9.56, P=0.002] and ermB, gyrA, parC [54.8%(34/62) vs 22.7%(5/22), 67.7%(42/62) vs 36.4%(8/22), 71.0%(44/62) vs 36.4%(8/22); χ 2=6.73, 6.64, and 8.25, all P<0.05], and a lower proportion of ermA [6.5%(4/62) vs 31.8%(7/22), χ 2=7.09, P=0.008] than CRISPR1-negative strains. Conclusions:ST10 is the main GBS genotype among the colonized microbiota the genital tract of pregnant women and in infants with invasive GBS infection, which is also a dominant type in CRISPR1-positive strains. GBS is sensitive to penicillin and CRISPR1 gene is linked to the spread of some drug-resistance genes.

Objective:To construct a bowel preparation assessment scale for patients undergoing enteroscopy based on Delphi method and provide clinical nurses with a scientific and convenient bowel preparation assessment tool.Method:The bowel preparation assessment scale for patients undergoing enteroscopy was constructed through literature review, Delphi expert correspondence and analytic hierarchy process. Using the convenient sampling method, a total of 1 988 patients who needed bowel cleansing due to enteroscopy in the Affiliated Hospital of Qingdao University from June 2020 to January 2022 were selected as the research objects. The patients who underwent enteroscopy from June 2020 to April 2021 were set up as the pre-application group ( n=980) and patients who underwent enteroscopy from April 2021 to January 2022 were set up as the post-application group ( n=1 008) . The qualified rate of bowel preparation in patients undergoing enteroscopy was compared between the two groups. Results:A total of 16 experts were invited for 2 rounds of correspondence. The effective recovery rates of the two rounds of questionnaires were respectively 100.00% and 93.75%, the expert authority coefficients were respectively 0.837 and 0.800, and the Kendall's harmony coefficients were respectively 0.207 and 0.280 (all P<0.01) . The final constructed bowel preparation assessment scale for patients undergoing enteroscopy included 4 first-level indicators, 18 second-level indicators and 47 third-level indicators. After the assessment scale was applied to the clinic, the bowel preparation qualified rate (82.24%) of patients in the post-application group was higher than that in the pre-application group (66.43%) , and the difference was statistically significant ( P<0.01) . Conclusions:The bowel preparation assessment scale for patients undergoing enteroscopy constructed based on the Delphi method is reliable, scientific and practical, which can effectively guide clinical nursing work.

Objective:To explore the phenotypes and genotypes of molybdenum cofactor deficiency type B (MoCD-B) manifested as Leigh-like syndrome.Methods:The clinical data, laboratory tests, neuroimaging and gene results of one patient diagnosed as MoCD-B at Beijing Children′s Hospital and Hebei Children′s Hospital in December 2018 were collected. Related literature was searched and reviewed at Wanfang Data Knowledge Service Platform, China National Knowledge Infrastructure and PubMed (up to September 2020) by using terms "MOCS2" "molybdenum cofactor deficiency" "Leigh-like syndrome,MOCS2" "molybdenum cofactor deficiency, Leigh-like syndrome". The phenotypes and genotypes of MoCD-B were summarized.Results:A 7 months and 14 days old boy with the chief complaint of "cough for 6 days, abnormal posture for 4 days and fever for 2 days" was admitted to Hebei Children′ Hospital on December 2018. His abnormal posture presented as opisthotonos accompanied with dysphagia, without seizures. His previous psychomotor development was described as normal. He was born at term after an uneventful pregnancy to non-consanguineous parents. Blood test showed a slightly increased lactic acid and a significantly decreased uric acid. Urine metabolism test showed an obviously increased xanthine and hypoxanthine. Brain magnetic resonance imaging showed hyperintense signal on T2 weighted image and fluid attenuated inversion recovery in bilateral globus pallidus and pedunculus cerebri. The patient was diagnosed with Leigh-like syndrome. No obvious improvement was achieved after cocktail therapy and symptomatic treatment. The whole exome sequencing showed that the patient carried a homozygous variant of MOCS2 gene, c.19G>T(p.Val7Phe), which was a previously reported pathogenic site in the literature and could cause MoCD-B. His parents carried a heterozygous variant respectively. A total of 41 MoCD-B cases with MOCS2 gene variants were collected through literature review and our study, among which 30 cases had full medical records. The onset ages of 23 (77%) cases were in neonate, manifesting with severe encephalopathy, including neonatal-onset intractable seizures, developmental delay, laboratory abnormalities included very low levels of serum and urinary uric acid, increased urinary levels of xanthine and hypoxanthine. Cranial imaging showed cerebral atrophy, cystic encephalomalacia, etc. The onset ages of 7 patients varied from 5 months to 23 years. Four cases had normal psychomotor development before disease onset. Neurological disorders appeared acutely or exacerbated after external triggers and all of them had basal ganglia involvement. Among the 30 cases, 3 cases had a relatively milder phenotype with the ability of brief communication and walking without or with support.Conclusions:Molybdenum cofactor deficiency is a rare disease. Most cases had severe phenotypes and poor outcomes, but some cases may have mild phenotype. MoCD-B caused by MOCS2 gene variants may manifest as Leigh-like syndrome with a normal psychomotor development before the trigger of infection strike. Hypouricemia, xanthinuria and hypoxanthinuria can be indicators of the disease. The presence of MOCS2 gene variants would confirm a final diagnosis.

Sensory modalities are important for survival but the molecular mechanisms remain challenging due to the polymodal functionality of sensory neurons. Here, we report the C. elegans outer labial lateral (OLL) sensilla sensory neurons respond to touch and cold. Mechanosensation of OLL neurons resulted in cell-autonomous mechanically-evoked Ca