Objective To evaluate the clinical efficacy of Bushen Jianpi Formula(BSJPF)in treating type 2 diabetes mellitus(T2DM)complicated with non-alcoholic fatty liver disease(NAFLD)of spleen-kidney deficiency type.Methods Seventy-two patients with T2DM complicated with NAFLD of spleen-kidney deficiency type admitted to Clifford Hospital from June 2023 to September 2024 were randomized into treatment group(n=36,receiving lifestyle intervention+metformin+BSJPF)and control group(n=36,lifestyle intervention+metformin alone)for 12 weeks.TCM syndrome scores,liver function markers,ultrasonographic grading of fatty liver,glycolipid metabolic parameters were observed,and the clinical efficacy and safety were assessed.Results(1)Regarding dropouts,during the study,2 patients in the treatment group dropped out,while none occurred in the control group.Ultimately,34 patients in the treatment group and 36 in the control group completed efficacy evaluation.(2)In terms of clinical efficacy,after 12 weeks of treatment,the total effective rate was 88.24％(30/34)in the treatment group versus 66.67％(24/36)in the control group.Intergroup comparison(by chi-square test)showed significantly superior efficacy in the treatment group(P＜0.05).(3)For liver function indicators,after treatment,serum alanine aminotransferase(ALT),aspartate aminotransferase(AST),and γ-glutamyl transpeptidase(GGT)levels decreased significantly in both groups compared to those before treatment(P＜0.05),with substantially greater reduction in the treatment group(P＜0.05).(4)Regarding fatty liver ultrasound grading,both groups showed improvement after treatment(P＜0.05),with significantly greater enhancement in the treatment group(P＜0.05).(5)For glucose-lipid metabolism markers,both groups exhibited decreased fasting blood glucose(FBG),2-hour postprandial glucose(2hPG),glycated hemoglobin(HbA1c),fasting insulin(FINS),insulin resistance index(HOMA-IR),total cholesterol(TC),triglycerides(TG),and low-density lipoprotein cholesterol(LDL-C)levels(P＜0.05),with treatment group showing markedly greater reductions(P＜0.05);both groups demonstrated increased high-density lipoprotein cholesterol(HDL-C)levels(P＜0.05),with treatment group showing significantly greater elevation(P＜0.05).(6)In TCM syndrome score assessment,both groups showed reduced scores after treatment(P＜0.05),with treatment group demonstrating significantly greater improvement(P＜0.05).(7)Regarding safety,routine blood/urine/stool tests,renal function indicators,and electrocardiograms remained normal in both groups.The adverse reaction rate was 2.94％(1/34)in treatment group versus 5.56％(2/36)in control group,with no statistically significant difference between groups(P＞0.05).Conclusion BSJPF combined with metformin demonstrates superior efficacy to metformin alone for patients with T2DM complicated with NAFLD of spleen-kidney deficiency type.It is effective in relieving clinical symptoms,enhancing TCM syndrome efficacy,improving liver enzymes,fatty liver grading and insulin resistance,and regulating glycolipid metabolism.

Ribosome is an intracellular ribonucleoprotein particle that serves as the site of protein biosynthesis. Ribosomal dysfunction caused by mutations in genes encoding ribosomal proteins (RPs) and ribosome biogenesis factors (RBFs) can lead to a spectrum of diseases, collectively known as ribosomopathy. Phase separation is a thermodynamic process that produces multiple phases from a homogeneous mixture. The formation of membraneless organelles and intracellular structures, including ribosomes and nucleoli, cannot occur without the involvement of phase separation. Here, ribosome structure, biogenesis, and their relationship with ribosomopathy are systematically reviewed. The tissue specificity of ribosomopathy and the role of phase separation in ribosomopathy are particularly discussed, which may offer some clues for understanding the mechanisms of ribosomopathy. Then, some new ideas for the prevention, diagnosis, and treatment of ribosomopathy are provided.
Humans ; Ribosomes/physiology* ; Ribosomal Proteins/metabolism* ; Mutation ; Animals ; Cell Nucleolus/metabolism* ; Protein Biosynthesis ; Phase Separation

Meckel′s cartilage (MC) was first delineated in 1820 by the German anatomist Johann Friedrich Meckel the Younger through his examination of human embryos. During early development, MC functions as a supportive structure for the mandible and serves as a template for the subsequent formation of the jaw bones. Ultimately, MC transforms into either skeletal tissue, ligaments, or completely resorbs, integrating with the continuously developing osseous structures. This review elucidates the influence of MC development and degeneration on mandibular morphogenesis, delineating cellular processes and key factors that govern chondrogenic fate determination. The analysis reveals how the alterations of MC affect mandibular and craniofacial development. As a transient cartilaginous structure, investigation into MC may yield valuable insights for understanding oral and craniomaxillofacial pathologies.

Objective:To investigate the clinical and genetic characteristics of a Henan Han family with pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL), aiming to enhance understanding of this disease.Methods:The proband was first admitted to the Department of Neurology, Henan Provincial People′s Hospital, Fuwai Central China Cardiovascular Hospital in December 2019 due to cerebral infarction and unilateral limb numbness and weakness. Detailed medical history collection, pedigree mapping, whole-exome sequencing screening, and Sanger sequencing validation were performed for the proband and family members. The patients′ clinical manifestations, imaging features, neuropsychological scale assessment results, and pathological changes were summarized, and genetic analysis was conducted on the gene variant site. Relevant literature was reviewed to summarize the characteristics of PADMAL.Results:The proband was a 47-year-old female, with 3 generations of family members affected, including 7 patients, 3 of whom had died. The clinical features of the patients were similar, with the first stroke occurring around the age of 40, without vascular risk factors such as hypertension or diabetes. The main clinical manifestation was unilateral limb numbness and weakness. The proband and her niece sought medical attention due to stroke symptoms. Brain magnetic resonance imaging revealed acute infarct lesions located in the pons, accompanied by multiple oval infarct foci (the "raisin bread sign") and white matter hyperintensity changes. Genetic testing showed that 4 patients carried a heterozygous c. *34GT mutation in the 3′-untranslated region (3′-UTR) of the COL4A1 gene, while the other 4 unaffected family members did not carry this variant, consistent with genotype- phenotype co-segregation in the family. Conclusions:PADMAL is an extremely rare monogenic cerebral small vessel disease caused by pathogenic variants in the 3′-UTR of the COL4A1 gene. The "raisin bread sign" in the pons is a relatively specific imaging feature that distinguishes it from other cerebral small vessel diseases. For patients with this sign, genetic testing for PADMAL should be considered.

Objective To explore the effects and mechanism of Jianpi Shuyi Decoction in improving pancreatic fibrosis in chronic pancreatitis(CP)based on network pharmacology and animal experiments.Methods TCMSP was used to screen the active components and targets of Jianpi Shuyi Decoction.GeneCards was used to obtain the disease targets of pancreatic fibrosis,and the intersection of drug and disease targets was used to construct the protein interaction network and the drug-component-target network,and the core target genes were screened out.GO and KEGG pathway enrichment analysis was performed on the intersecting targets.Caerulein was used to induce CP mouse model,and Jianpi Shuyi Decoction was given for gavage.HE and Sirius red staining were used to observe pancreatic tissue inflammation and collagen deposition,respectively.RT-qPCR was used to observe the mRNA expression levels of Acta2,COL1A1,PI3K and Akt1 in pancreatic tissue.Immunohistochemistry staining was used to observe the protein expression levels of α-SMA,COL-1,p-PI3K and p-Akt in pancreatic tissues.Results A total of 181 active components were screened from Jianpi Shuyi Decoction,corresponding to 284 targets,with 240 targets overlapping between drugs and disease and the core targets were PTGS2,HSP90AA1,etc.193 signaling pathways were obtained from KEGG pathway enrichment analysis,primarily involving lipids and atherosclerosis,chemical carcinogenic-receptor activation,PI3K-Akt signaling pathway and others.The results of animal experiments showed that,compared with the normal group,the model group showed a large number of inflammatory cell infiltration and collagen deposition in pancreatic tissue,the mRNA expression of Acta2,COL1A1,PI3K and Akt1 in pancreatic tissue significantly increased(P<0.01),and the protein expression of α-SMA,COL-1,p-PI3K,p-Akt significantly increased(P<0.01);Jianpi Shuyi Decoction significantly reduced the inflammation and collagen deposition in pancreas of mice,reduced the mRNA expression of Acta2,COL1A1,PI3K and Akt1(P<0.05),and attenuated the protein expression of α-SMA,COL-1,p-PI3K and p-Akt in pancreatic tissue(P<0.05).Conclusion Jianpi Shuyi Decoction may exert a therapeutic effect on CP pancreatic fibrosis by regulating the PI3K/Akt signaling pathway,attenuating inflammation and collagen deposition in the pancreas,and reducing the levels of α-SMA and COL-1.

Pasteurella is a genus of zoonotic pathogens that are widely distributed in both healthy and diseased ani-mals.Reports of human infections caused by wild animal bites are rare.This paper reports the first case of Pasteu-rella multocida infection caused by a snow leopard bite in China.The strains were identified using the DL-96 Ⅱ bac-terial identification system,BRUKER automatic microbial mass spectrometer,and Autof ms 1000 mass spectrome-ter.Antimicrobial susceptibility testing was performed using the DL-96 Ⅱ bacterial assay system.After wound de-bridement,surgical incision and drainage,and combined antimicrobial treatment,the patient recovered and was dis-charged from the hospital.This paper aims to improve clinical health care workers'understanding on this bacterium.

Meckel′s cartilage (MC) was first delineated in 1820 by the German anatomist Johann Friedrich Meckel the Younger through his examination of human embryos. During early development, MC functions as a supportive structure for the mandible and serves as a template for the subsequent formation of the jaw bones. Ultimately, MC transforms into either skeletal tissue, ligaments, or completely resorbs, integrating with the continuously developing osseous structures. This review elucidates the influence of MC development and degeneration on mandibular morphogenesis, delineating cellular processes and key factors that govern chondrogenic fate determination. The analysis reveals how the alterations of MC affect mandibular and craniofacial development. As a transient cartilaginous structure, investigation into MC may yield valuable insights for understanding oral and craniomaxillofacial pathologies.

Pasteurella is a genus of zoonotic pathogens that are widely distributed in both healthy and diseased ani-mals.Reports of human infections caused by wild animal bites are rare.This paper reports the first case of Pasteu-rella multocida infection caused by a snow leopard bite in China.The strains were identified using the DL-96 Ⅱ bac-terial identification system,BRUKER automatic microbial mass spectrometer,and Autof ms 1000 mass spectrome-ter.Antimicrobial susceptibility testing was performed using the DL-96 Ⅱ bacterial assay system.After wound de-bridement,surgical incision and drainage,and combined antimicrobial treatment,the patient recovered and was dis-charged from the hospital.This paper aims to improve clinical health care workers'understanding on this bacterium.

Objective To explore the effects and mechanism of Jianpi Shuyi Decoction in improving pancreatic fibrosis in chronic pancreatitis(CP)based on network pharmacology and animal experiments.Methods TCMSP was used to screen the active components and targets of Jianpi Shuyi Decoction.GeneCards was used to obtain the disease targets of pancreatic fibrosis,and the intersection of drug and disease targets was used to construct the protein interaction network and the drug-component-target network,and the core target genes were screened out.GO and KEGG pathway enrichment analysis was performed on the intersecting targets.Caerulein was used to induce CP mouse model,and Jianpi Shuyi Decoction was given for gavage.HE and Sirius red staining were used to observe pancreatic tissue inflammation and collagen deposition,respectively.RT-qPCR was used to observe the mRNA expression levels of Acta2,COL1A1,PI3K and Akt1 in pancreatic tissue.Immunohistochemistry staining was used to observe the protein expression levels of α-SMA,COL-1,p-PI3K and p-Akt in pancreatic tissues.Results A total of 181 active components were screened from Jianpi Shuyi Decoction,corresponding to 284 targets,with 240 targets overlapping between drugs and disease and the core targets were PTGS2,HSP90AA1,etc.193 signaling pathways were obtained from KEGG pathway enrichment analysis,primarily involving lipids and atherosclerosis,chemical carcinogenic-receptor activation,PI3K-Akt signaling pathway and others.The results of animal experiments showed that,compared with the normal group,the model group showed a large number of inflammatory cell infiltration and collagen deposition in pancreatic tissue,the mRNA expression of Acta2,COL1A1,PI3K and Akt1 in pancreatic tissue significantly increased(P<0.01),and the protein expression of α-SMA,COL-1,p-PI3K,p-Akt significantly increased(P<0.01);Jianpi Shuyi Decoction significantly reduced the inflammation and collagen deposition in pancreas of mice,reduced the mRNA expression of Acta2,COL1A1,PI3K and Akt1(P<0.05),and attenuated the protein expression of α-SMA,COL-1,p-PI3K and p-Akt in pancreatic tissue(P<0.05).Conclusion Jianpi Shuyi Decoction may exert a therapeutic effect on CP pancreatic fibrosis by regulating the PI3K/Akt signaling pathway,attenuating inflammation and collagen deposition in the pancreas,and reducing the levels of α-SMA and COL-1.

Objective:To investigate the clinical and genetic characteristics of a Henan Han family with pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL), aiming to enhance understanding of this disease.Methods:The proband was first admitted to the Department of Neurology, Henan Provincial People′s Hospital, Fuwai Central China Cardiovascular Hospital in December 2019 due to cerebral infarction and unilateral limb numbness and weakness. Detailed medical history collection, pedigree mapping, whole-exome sequencing screening, and Sanger sequencing validation were performed for the proband and family members. The patients′ clinical manifestations, imaging features, neuropsychological scale assessment results, and pathological changes were summarized, and genetic analysis was conducted on the gene variant site. Relevant literature was reviewed to summarize the characteristics of PADMAL.Results:The proband was a 47-year-old female, with 3 generations of family members affected, including 7 patients, 3 of whom had died. The clinical features of the patients were similar, with the first stroke occurring around the age of 40, without vascular risk factors such as hypertension or diabetes. The main clinical manifestation was unilateral limb numbness and weakness. The proband and her niece sought medical attention due to stroke symptoms. Brain magnetic resonance imaging revealed acute infarct lesions located in the pons, accompanied by multiple oval infarct foci (the "raisin bread sign") and white matter hyperintensity changes. Genetic testing showed that 4 patients carried a heterozygous c. *34GT mutation in the 3′-untranslated region (3′-UTR) of the COL4A1 gene, while the other 4 unaffected family members did not carry this variant, consistent with genotype- phenotype co-segregation in the family. Conclusions:PADMAL is an extremely rare monogenic cerebral small vessel disease caused by pathogenic variants in the 3′-UTR of the COL4A1 gene. The "raisin bread sign" in the pons is a relatively specific imaging feature that distinguishes it from other cerebral small vessel diseases. For patients with this sign, genetic testing for PADMAL should be considered.