This paper reported a type 1 diabetes patient who had severe diabetic nephropathy, retinopathy, hypertension, and hypothyroidism before pregnancy. The patient's blood glucose control was poor before pregnancy, and the complications were not properly treated. This was an unintended pregnancy, with a pre-pregnancy glycated hemoglobin A1c of 7.8% and early pregnancy urine protein of 3.81-4.53 g/24 h. Considering the patient's poor blood glucose control before pregnancy and the lack of proper treatment for multiple complications including nephropathy, a multidisciplinary consultation at an external hospital recommended termination of the pregnancy. However, the patient was determined to continue the pregnancy and was referred to Peking University First Hospital. Through strict blood glucose control, monitoring and evaluation of complications, and comprehensive management, the patient's blood glucose and blood pressure were well controlled during pregnancy. Regular monitoring of urine protein, renal function, and ocular fundus was conducted. At 31 weeks and 4 days of gestation, the patient's 24-hour urine protein significantly increased. After promoting fetal lung maturity, a cesarean section was performed at 34 weeks and 1 day of gestation, resulting in a successful delivery with good maternal and neonatal outcomes. At the 42-day postpartum follow-up, the patient's blood glucose and blood pressure were stable, urine protein returned to pre-pregnancy levels, and the infant was in good general condition.

Objective To analyse phenotype and genetic variation of two congenital hypodysfibrinogenemia(Fg)caused by compound heterozygous variants and preliminary investigate their molecular pathogenic mechanisms.Metheds The proband A and B and their family members(a total of 19 members in 3 generations)who visited the First Hospital of Wenzhou Medical University on 4 May 2023 and 20 May 2023 for"parkinson's disease"and"pre-bilateral eyelid excision"were enrolled for the study.Prothrombin time(TT)and fibrinogen(Fg)activity were measured by coagulation assay and Fg antigen(Fg∶Ag)was measured by immunoturbidimetric assay for the two family members,and Fg aggregation assay was catalysed using human thrombin.FGG gene was amplified by PCR and se-quenced directly.The variant sites were analysed using Chromas software.Multiple sequence comparison was performed by ClustalX-2.1-win software.Pathogenicity analysis of the variant sites was performed using bioinformatics software.The analysis for FGG protein model was performed using PyMOL software.Results Phenotypic results showed TT of proband A and B extended to 27.5 s and 26.1 s,and plasma Fg activity reduced to 0.6 g/L and＜0.5 g/L,respectively.Genetic sequencing identified heterozygous c.1129+62_65delAATA on intron 8 of FGG gene in the both probands,resulting in the formation of aberrant amino acids at p.γGly377-Gly388 and an early ter-mination codon at p.γTyr389 site.A heterozygous missense variant c.103C＞A(p.AαArg35Ser)was found in exon 2 of the FGA gene of proband A,and a heterozygous missense variant c.569A＞G(p.BβAsn190Ser)was found in exon 4 of the FGB gene of proband B.Compared to the control group,the both probands showed significant decreases in peak and rate of Fg aggregation.Multiple sequence comparison analyses showed that all the three variant sites were conserved.Three bioinformatics software predicted both the missense variants were pathogenic.Protein modelling analysis showed that the number of hydrogen bonds in p.γGly377-Gly388 variant region was altered,resulting in steric hinderance.Conclusion All the two types of compound heterozygous variants,i.e.,c.1129+62_65delAATA and p.AαArg35Ser,c.1129+62_65delAATA and p.BβAsn190Ser,have been reported for the first time in Chi-na and worldwide to date,and the three variants may be related to the reduced Fg level and function in the two pedigree.

Objective To analyse phenotype and genetic variation of two congenital hypodysfibrinogenemia(Fg)caused by compound heterozygous variants and preliminary investigate their molecular pathogenic mechanisms.Metheds The proband A and B and their family members(a total of 19 members in 3 generations)who visited the First Hospital of Wenzhou Medical University on 4 May 2023 and 20 May 2023 for"parkinson's disease"and"pre-bilateral eyelid excision"were enrolled for the study.Prothrombin time(TT)and fibrinogen(Fg)activity were measured by coagulation assay and Fg antigen(Fg∶Ag)was measured by immunoturbidimetric assay for the two family members,and Fg aggregation assay was catalysed using human thrombin.FGG gene was amplified by PCR and se-quenced directly.The variant sites were analysed using Chromas software.Multiple sequence comparison was performed by ClustalX-2.1-win software.Pathogenicity analysis of the variant sites was performed using bioinformatics software.The analysis for FGG protein model was performed using PyMOL software.Results Phenotypic results showed TT of proband A and B extended to 27.5 s and 26.1 s,and plasma Fg activity reduced to 0.6 g/L and＜0.5 g/L,respectively.Genetic sequencing identified heterozygous c.1129+62_65delAATA on intron 8 of FGG gene in the both probands,resulting in the formation of aberrant amino acids at p.γGly377-Gly388 and an early ter-mination codon at p.γTyr389 site.A heterozygous missense variant c.103C＞A(p.AαArg35Ser)was found in exon 2 of the FGA gene of proband A,and a heterozygous missense variant c.569A＞G(p.BβAsn190Ser)was found in exon 4 of the FGB gene of proband B.Compared to the control group,the both probands showed significant decreases in peak and rate of Fg aggregation.Multiple sequence comparison analyses showed that all the three variant sites were conserved.Three bioinformatics software predicted both the missense variants were pathogenic.Protein modelling analysis showed that the number of hydrogen bonds in p.γGly377-Gly388 variant region was altered,resulting in steric hinderance.Conclusion All the two types of compound heterozygous variants,i.e.,c.1129+62_65delAATA and p.AαArg35Ser,c.1129+62_65delAATA and p.BβAsn190Ser,have been reported for the first time in Chi-na and worldwide to date,and the three variants may be related to the reduced Fg level and function in the two pedigree.

This paper reported a type 1 diabetes patient who had severe diabetic nephropathy, retinopathy, hypertension, and hypothyroidism before pregnancy. The patient's blood glucose control was poor before pregnancy, and the complications were not properly treated. This was an unintended pregnancy, with a pre-pregnancy glycated hemoglobin A1c of 7.8% and early pregnancy urine protein of 3.81-4.53 g/24 h. Considering the patient's poor blood glucose control before pregnancy and the lack of proper treatment for multiple complications including nephropathy, a multidisciplinary consultation at an external hospital recommended termination of the pregnancy. However, the patient was determined to continue the pregnancy and was referred to Peking University First Hospital. Through strict blood glucose control, monitoring and evaluation of complications, and comprehensive management, the patient's blood glucose and blood pressure were well controlled during pregnancy. Regular monitoring of urine protein, renal function, and ocular fundus was conducted. At 31 weeks and 4 days of gestation, the patient's 24-hour urine protein significantly increased. After promoting fetal lung maturity, a cesarean section was performed at 34 weeks and 1 day of gestation, resulting in a successful delivery with good maternal and neonatal outcomes. At the 42-day postpartum follow-up, the patient's blood glucose and blood pressure were stable, urine protein returned to pre-pregnancy levels, and the infant was in good general condition.

Liver transplantation is the optimal treatment for end-stage liver disease and hepatocellular carcinoma, which can significantly improve clinical prognosis and quality of life of patients. However, multiple challenges, such as rejection, immune tolerance, shortage of donor liver, preservation of donor liver, ischemia-reperfusion injury and postoperative complications, etc., limit the efficacy of liver transplantation in clinical practice. Research teams in China have made significant contributions to the basic research related to liver transplantation by making continuous efforts and combining the development of emerging technologies, interdisciplinary integration and other emerging fields. In this article, the frontier progress in the basic research of liver transplantation in 2023 was reviewed, highlighting the progress made by Chinese research teams in the basic research of liver transplantation, aiming to provide reference for promoting the integration of Chinese characteristics into the research of liver transplantation, accelerate the integration of Chinese liver transplantation research with international community, and promote further advancement of liver transplantation in China.

Objective:To explore the impact of artificial intelligence compressed sensing technology (CS-AI) on image quality in three-dimensional proton density weighted imaging (3D PDWI) of the unilateral hip joint.Methods:High-resolution unilateral hip imaging was conducted on 67 healthy volunteers at West China Hospital of Sichuan University from January to July 2023. Imaging was performed by using CS-AI 3D PDWI sequence with acceleration factors (AF) of 4, 6, 8, and 10, respectively. According to the AF, all subjects were divided into 4 groups: CS-AI 4, CS-AI 6, CS-AI 8 and CS-AI 10, with CS-AI 4 serving as a reference. Recording the scan time, the signal and noise intensity of the femoral head, muscle, and subcutaneous fat were measured by a senior radiologist and the signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were then calculated. Additionally, two observers provided ratings for overall image quality and artifacts in the 4 groups, and statistical analysis was performed using the Friedman rank-sum test.Results:The acquisition times for CS-AI 4, CS-AI 6, CS-AI 8, and CS-AI 10 were 5 min 49 s, 3 min 54 s, 2 min 56 s and 2 min 22 s, respectively. Compared to CS-AI 4, the scanning time for CS-AI 6, CS-AI 8, CS-AI 10 were reduced by 32.95%, 50.14%, 59.31%, respectively. The objective evaluation revealed that the SNR and CNR of the femoral head and muscle in groups CS-AI 6, CS-AI 8, and CS-AI 10 were slightly lower than those in group CS-AI 4 ( P<0.05), and the differences were statistically significant. However, no statistically significant differences were found among the 3 groups ( P>0.05). The subjective evaluation indicated that the overall image quality scores of group CS-AI 8 [3 (3,4)] did not significantly differ from those of group CS-AI 4 and CS-AI 6( P>0.05); The mean scores of group CS-AI 4 and CS-AI 6 were 4 (4, 4); Scores of group CS-AI 10 was 3(3, 3), which statistically significant differ from those of the other groups ( P<0.05). The artifacts rating for groups CS-AI 4, CS-AI 6, CS-AI 8 and CS-AI 10 were 4 (4, 4), 4 (4, 4), 3 (3, 4), and 2 (2, 3) respectively. When AF was set to 10, the images exhibited the most severe artifacts ( P<0.05). For other AF values, artifact ratings did not differ significantly ( P>0.05). Conclusion:The CS-AI 3D-PDWI sequence with acceleration factor 8 can acquire high-resolution images of the unilateral hip joint that meet clinical diagnostic requirements while reducing scanning time.

Objective: To review the experience of using endoscopic retrograde cholangio-pancreatography (ERCP) in the clinical diagnosis and treatment of biliary complications during perioperative period of primary liver cancer at our hospital. Methods: A retrospective analysis was conducted on the clinical data of patients undergoing ERCP treatment for biliary complications during perioperative period of primary liver cancer at our hospital from October 2016 to June 2023. According to the indications of ERCP for biliary complications, the cases were divided into bile duct tumor thrombus group, bile duct stricture group, and bile leakage group. The baseline data, laboratory test results before and after ERCP treatment, treatment outcomes, and other datas of the patients with different types of biliary complication were statistically analyzed. Results: A total of 25 patients underwent 25 cases of ERCP, among which 23 patients were successfully cannulated, with a success rate of 92.0%. There were 14 cases of bile duct stenosis, mainly treated with biliary balloon dilation, probe dilation, stent implantation, and nasobiliary drainage; 6 cases of bile leakage, mainly treated with stent implantation and nasobiliary drainage; 5 cases of bile duct tumor thrombus, mainly treated with net basket and balloon thrombectomy, stent implantation, and nasobiliary drainage. After ERCP treatment, the patient's serum total bilirubin, serum direct bilirubin, and γ-glutamyl transferase significantly decreased compared to those before the treatment (P<0.05). The postoperative average amylase was (85.0±69.4)(21-306) U/L. No bleeding, gastrointestinal perforation, pancreatitis, or other ERCP-related complications occurred in all cases. Conclusions: Bile duct stricture is the most common biliary complication during the perioperative period of primary liver cancer. For these patients with biliary complications during perioperative period of primary liver cancer ERCP treatment is safe and effective.

Objective:To explore the efficacy of pedicled bladder muscle flap in the repair of urinary tract obstruction.Methods:The data of 26 patients with urinary tract obstruction admitted to Shanghai Sixth People's Hospital Affiliated to Shanghai Jiaotong University School of Medicine from March 2016 to June 2021 were retrospectively reviewed. There were 14 males and 12 females, with the age ranged from 2 to 75 years old. Refractory bladder neck obstruction after prostatic hyperplasia surgery in 12 cases, with the age of (70.0±3.5) years old.They all experienced at least 2 times of transurethral stenosis incisionor resection. Transpubic cystostomy tube was placed in 9 patients. Posttraumatic pelvic fractures lead to bladder neck atresia and urethral injury in 6 girls, with the age of (10.5±2.1) years old. The bladder neck atresia and urethral obliteration length was 1-2 cm determined by urethrography. Eight cases suffered ureteral strictures after gynecological myomectomy or ureteroscopy holmium laser lithotripsy(4 cases of each type), including two males and six females, with the age of (55.0±3.2) years old. The length of ureteral stricture or defect was 5-6 cm determined by intravenous urography(IVU) or CT urography(CTU). The patients with bladder neck obstruction underwent the following surgery: The "Y" incision of the bladder and stenosis of the prostate urethra was performed and the pedicled bladder muscle flap was inserted into the normal urethral mucosa to complete the Y-V plasty. In the 6 girl patients, pedicled bladder muscle flap(2-4 cm) augmented reconstruction were performed. All above 18 patients, whose urethral catheter was indwelled for 3-4 weeks, urinary flow rate and urethroscopy examination were performed to evaluate the effect of surgery 4 weeks and 3 months after the operation. As the 8 cases with ureteral strictures, the pedicled bladder muscle flap (7-8 cm) ureteroplasty was performed and the ureteral stent was retained for 4 weeks. Ultrasonography and IVU/CTU were performed 4 weeks and 3 months postoperatively. The patency of the ureteral lumen and whether it is accompanied by hydronephrosis, lower back pain, and urinary tract infection were assessed.Results:All patients underwent pedicled bladder muscle flap reconstructive surgery successfully and no serious complications occurred postoperatively. The patients were followed up for (8.2± 2.2) months. As urethral catheters were removed, 10 patients with refractory neck obstruction could return to normal urination with the urinary flow Q max (17.2±2.8)ml/s, while 2 patient had dysuria and were treated with regular urethral dilatation. The catheter was removed 4 weeks after the reconstructive surgery in 6 girls with bladder neck atresia and urethral injury after posttraumatic pelvic fracture. Five could successfully urinate with the urinary flow Q max of (16.7±1.1)ml/s, and one girl had urinary incontinence, waiting for further operation.The ureteral stent was removed after ureteroplasty in 8 patients. CTU and IVU examination showed no ureters with obstruction.No one had low back pain, discomfort, or urinary tract infection. Conclusions:The reconstruction using the pedicled bladder muscle flap was a convenient, minimally invasive and effective technique for the management of adjacent lower ureters, bladder neck, and proximal urethra.

Objective:To explore the expression level and clinicopathological characteristics of 14-3-3θ protein in distal cholangiocarcinoma tissues, and further analyze the long-term prognosis of patients with different expression levels.Methods:A retrospective cohort study was conducted to collect and analyze the clinical data of 135 patients with distal cholangiocarcinoma who underwent surgical resection at the Department of Hepatobiliary Surgery, Beijing Chaoyang Hospital Affiliated to Capital Medical University from January 2015 to December 2021, including 86 males and 49 females.(65.1±10.1) years old. Immunohistochemistry was used to detect the expression level of 14-3-3θ protein in cholangiocarcinoma tissue. The postoperative pathological sections of patients were evaluated based on the immune response score(IRS). The optimal cutoff value was determined through the receiver operating characteristic(ROC) curve was 3.5. Currently, the area under the curve was 0.741, the sensitivity was 73.5%, and the specificity was 71.3%. The patients were divided into two groups: IRS<4 was the 14-3-3θ low expression group( n=81), IRS≥4 was the 14-3-3θ high expression group( n=54). After surgery, the patient′s survival status was followed up through a combination of outpatient review and telephone follow-up. Normally distributed measurement data were expressed as mean ± standard deviation( ± s), and comparisons between groups were made using the t test; non-normally distributed measurement data were expressed as M( Q1, Q3), and comparisons between groups were made using the rank sum test. The chi-square test was used to compare enumeration data between groups. Results:The preoperative CA19-9 and lymph node metastasis in the 14-3-3θ low expression group were 44.3(20.8, 132.2) U/mL and 28 cases respectively. The preoperative CA19-9 and lymph node metastasis in the 14-3-3θ high expression group were 82.3(43.4, 396.9) U/mL and 32 cases respectively. The difference between the two groups was statistically significant( P<0.05). All patients had regular postoperative telephone calls or return to the hospital for follow-up. The median postoperative survival time of the 14-3-3θ low-expression group was 36 months. The 1-, 2-, and 3-year survival rates after surgery were 88.9%, 66.5%, and 66.5%, respectively. 49.4%, the median survival time after surgery in the 14-3-3θ high expression group was 13 months, and the 1, 2, and 3-year survival rates after surgery were 53.7%, 23.3%, and 13.3% respectively. The difference between the two groups was statistically significant. significance( P<0.01). Cox proportional hazard model performed multivariate analysis, CA19-9>37 U/mL ( RR=1.970, 95% CI: 1.186-3.272, P=0.009), lymph node metastasis( RR=1.681, 95% CI: 1.035-2.729, P=0.036) and 14-3-3θ staining intensity≥4 ( RR=2.438, 95% CI: 1.546-3.845, P<0.001) have worse long-term prognosis. Conclusions:The expression level of 14-3-3θ protein is related to CA19-9 and lymph node metastasis in distal cholangiocarcinoma. A high expression of 14-3-3θ protein indicates poor long-term prognosis in patients with extrahepatic cholangiocarcinoma.

Objective To investigate the correlation between TCM syndrome types and solar term of onset in patients with gastric cancer.Methods A total of 359 patients with gastric cancer admitted to the Cancer Center of the First Affiliated Hospital of Guangzhou University of Chinese Medicine from January 2020 to August 2023 were included in this study,and the frequency distribution method was used to analyze their information such as sex,age,solar term of onset,differentiation,metastasis and TCM syndrome type.The solar term of onset corresponding to the onset date was calculated,and then the association between solar term of onset and TCM syndrome type in gastric cancer was analyzed.The circular distribution method was used to explore the peak of solar term of onset and TCM syndrome type.Results Among the 359 patients with gastric cancer included in this study,male patients were more than female patients(1.69∶1).The age of onset was mainly between 60 and 70 years old(117 cases,32.6%),and male patients(85 cases,72.6%)were more than female patients(32 cases,27.3%)in this age group.The most common TCM syndromes were qi and blood deficiency syndrome(160 cases,44.6%)and phlegm damp condensation syndrome(94 cases,26.2%).The onset of the disease mainly occurred in winter(132 cases,36.8%),and the peaks were light snow(31 cases,23.5%),major cold(25 cases,18.9%),heavy snow(23 cases,17.4%)and the start of winter(22 cases,16.7%).Qi and blood deficiency syndrome and phlegm damp condensation syndrome in patients with gastric cancer were correlated with solar terms and seasons(P<0.05).The 285 patients with gastric cancer were mainly poorly differentiated gastric cancer(175 cases,61.4%),mainly concentrated in winter(66 cases,37.7%).The main route of gastric cancer metastasis is lymph node metastasis,followed by liver and abdominal cavity metastasis.Conclusion Qi and blood deficiency syndrome and phlegm dampness condensation syndrome are common in patients with gastric cancer.The onset time of gastric cancer is mostly in winter,and the syndrome type is significantly correlated with the onset solar term and differentiation degree.