ObjectiveTo investigate the influencing factors for recompensation in patients with decompensated liver cirrhosis， as well as the impact of recompensation on the prognosis of such patients， and to provide a basis for early identification of high-risk patients in clinical practice. MethodsA retrospective analysis was performed for the clinical data of patients who attended The Third People’s Hospital of Kunming from January 2016 to December 2022 and were diagnosed with decompensated liver cirrhosis due to hepatitis B， hepatitis C， alcoholic hepatitis， and autoimmune hepatitis， and they were divided into recompensation group and persistent decompensation group. To control for confounding factors， whether recompensation occurred was used as the rouping variable，and BMI， alcohol consumption history， HIV infection history， TG， CHOL， LDL， and HDL were used as covariates. The propensity score was calculated， and 1：1 nearest neighbor matching was performed with a caliper value of 0.1. After propensity score matching， the recompensation group and the persistent decompensation group with relatively balanced covariates were obtained. Univariate and multivariate Cox proportional-hazards regression model analyses were used to investigate the influencing factors for recompensation； the “rms” package was used to establish a nomogram； the receiver operating characteristic （ROC） curve was plotted to calculate the area under the ROC curve （AUC）； the Hosmer-Lemeshow test was used to assess the goodness of fit of the model； the “Calibration Curves” package was used to plot calibration curves for model assessment. The Kaplan-Meier method was used to plot survival curves， and the Log-rank test was used for comparison of survival curves. ResultsAmong the 863 patients with decompensated liver cirrhosis， 305 experienced recompensation， resulting in an incidence rate of 35.3%. After PSM， 610 cases were successfully matched， with 305 cases in each group. The univariate and multivariate Cox regression analyses showed that etiology （hepatitis C： hazard ratio［HR］=0.288， P=0.002）； male（HR=0.701， P=0.016）， age（HR=0.988， P=0.047）， hemoglobin （HGB）（HR=1.006， P=0.017）， and CD4 T cell（HR=1.001，P=0.047）， TIPS procedure （HR=1.808，P=0.042） were independent influencing factors for recompensation in patients with decompensated liver cirrhosis. During follow-up， 116 patients died of liver disease-related causes， with 27 patients （8.85%） in the recompensation group and 89 （15.95%） in the persistent decompensation group； 109 patients developed HCC， with 23 patients （7.54%） in the recompensation group and 86 （15.41%） in the persistent decompensation group. The Kaplan-Meier survival curves showed significant separation between the patients with different states of compensation in terms of liver disease-related mortality rate and the incidence rate of HCC， and the Log-rank test showed that there were significant differences between the two groups in liver disease-related mortality rate （χ²=9.023， P=0.003） and the incidence rate of HCC （χ²=10.526， P=0.001）. ConclusionEtiology，sex，age，TIPS，HGB，and CD4 T cell are independent influencing factors for recompensation in patients with decompensated liver cirrhosis. There is a significant difference in the incidence rate of recompensation between decompensated liver cirrhosis patients with different etiologies， and female patients and patients with a younger age，a history of TIPS， a higher HGB level， and a higher CD4 lymphocyte count are more likely to experience recompensation. Recompensation is the key to improving the long-term prognosis of patients and can significantly reduce long-term liver disease-related mortality rate and the incidence rate of HCC.

ObjectiveTo construct and validate a predictive model for hepatic encephalopathy （HE） in patients with end-stage liver disease （ESLD） by combining key indicators such as cytokines and Model for End-Stage Liver Disease （MELD） score， and to provide evidence-based support for the early identification of high-risk populations and the optimization of intervention strategies in clinical practice. MethodsA retrospective analysis was performed for 2 167 patients with ESLD who were admitted to The Third People’s Hospital of Kunming from January 2022 to December 2024， the patients were divided into a training set with 1 517 patients and a validation set with 650 patients at a ratio of 7∶3 using the stratified random sampling method. A univariate analysis and a least absolute shrinkage and selection operator （LASSO） regression analysis were performed for the training set to identify potential influencing variables， and then a binary Logistic regression model was constructed to investigate the independent influencing factors for HE in ESLD patients. A nomogram prediction model was established based on this regression model， and the Hosmer-Lemeshow goodness-of-fit test was performed. The receiver operating characteristic （ROC） curve， calibration curves， decision curve analysis， and clinical impact curve were used to comprehensively evaluate the degree of fit， accuracy， consistency， and clinical practicability of the predictive model derived from the training set. The Mann-Whitney U test was used for comparison of non-normally distributed quantitative data between two groups. The chi-square test or Fisher exact test was used for comparison of categorical data between two groups. ResultsThe univariate analysis showed that there were significant differences between the study group and the control group in age， sex， blood ammonia， lymphocytes， hemoglobin， platelet count， prothrombin time， fibrinogen， international normalized ratio， total bilirubin， aspartate aminotransferase， total protein， albumin， prealbumin， alkaline phosphatase， cholinesterase， total bile acid， triglyceride， total cholesterol， high-density lipoprotein， low-density lipoprotein， blood glucose， CD3⁺ T cells， CD4⁺ T cells， CD8⁺ T cells， high-sensitivity C-reactive protein， carcinoembryonic antigen， triiodothyronine， thyroxine， free triiodothyronine， free thyroxine， interleukin-1β， interleukin-5， interleukin-6， interleukin-8， interleukin-12p70， interleukin-17， interferon-γ， and MELD score （all P<0.05）. The LASSO regression analysis identified nine variables of age， blood ammonia， albumin， prealbumin， cholinesterase， total cholesterol， thyroxine， interleukin-17， and MELD score， and the binary Logistic regression analysis showed that blood ammonia， interleukin-17， and MELD score were independent risk factors for HE in ESLD patients， while age and thyroxine were independent protective factors （all P<0.05）. A nomogram model was constructed based on these five variables. The Hosmer-Lemeshow goodness-of-fit test in the training set showed a good degree of fit （P=0.207）， with a McFadden’s pseudo-R² of 0.184， suggesting that the model had acceptable explanatory power； the Hosmer-Lemeshow goodness-of-fit test in the internal validation set further confirmed the calibration stability of the model （P=0.067）， suggesting that the model had a good degree of fit in independent data. The model had an area under the ROC curve of 0.784 in the validation set， with a sensitivity of 0.710 and a specificity of 0.752. The mean absolute error of the calibration curve was 0.067， and decision curve analysis and clinical impact curve showed that the nomogram model had positive net benefit and good clinical practicability. ConclusionThe nomogram model constructed based on age， blood ammonia， thyroxine， interleukin-17， and MELD score for predicting HE in patients with ESLD has a good degree of fit， high accuracy and consistency， and excellent clinical practicability.

ObjectiveTo investigate the predictive factors for HBsAg clearance in chronic hepatitis B （CHB） patients with a low level of hepatitis B surface antigen （HBsAg） treated with pegylated interferon α-2b （PEG-IFN-α-2b）， to establish a combined predictive model and a nomogram based on multiple factors， and to provide a reference for formulating individualized treatment regimens and predicting treatment outcome in clinical practice. MethodsA retrospective analysis was performed for 167 CHB patients with HBsAg <1 500 IU/mL who attended The Third People’s Hospital of Kunming from January 2022 to January 2024 and were treated with PEG-IFN-α-2b. According to whether clinical cure was achieved， the patients were divided into HBsAg clearance group and HBsAg non-clearance group. Related data were collected， including general information and serological/biochemical/virological indicators at different time points during treatment. The independent samples t-test was used for comparison of normally distributed continuous data， and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data； the chi-square test was used for comparison of categorical data. The multivariate logistic regression analysis was used to identify independent influencing factors. The receiver operating characteristic （ROC） curve was used to assess the value of indicators used alone or in combination in predicting clinical cure， and calibration curves were plotted to assess the risk prediction model. ResultsThe univariate analysis showed that there were significant differences between the two groups in age （t=-6.839， P<0.05）， history of nucleos（t）ide analogue treatment for over 1 year （χ²=59.339， P<0.05）， genotype （χ²=4.610， P<0.05）， nonalcoholic fatty liver disease （χ²=5.319， P<0.05）， hepatitis B virus DNA status before treatment （χ²=60.861， P <0.05）， compensated liver cirrhosis （χ²=10.960， P<0.05）， HBeAg status before treatment （χ²=19.060， P<0.05）， a history of interferon treatment （χ²=8.162， P<0.05）， presence of interferon antibodies after treatment （χ²=12.858， P<0.05）， HBsAg level before treatment （Z=-7.412， P<0.05）， alanine aminotransaminase （ALT） level at baseline （Z=-6.117， P<0.05）， ALT level at 12 weeks of treatment （Z=-7.171， P<0.05）， platelet count （PLT） at 24 weeks of treatment （Z=-3.622， P<0.05）， and thyroid stimulating hormone （TSH） level at 24 weeks of treatment （Z=-2.830， P<0.05）. The multivariate logistic regression analysis showed that age （odds ratio ［OR］=1.230， P=0.007）， history of nucleos（t）ide analogue treatment for over 1 year （OR=0.008， P=0.011）， HBeAg status before treatment （OR=0.003， P=0.012）， HBsAg level before treatment （OR=1.005， P=0.014）， ALT level at baseline （OR=0.949， P=0.014）， ALT level at 12 weeks of treatment （OR=0.969， P=0.016）， PLT at 24 weeks of treatment （OR=0.969， P=0.022）， and TSH level at 24 weeks of treatment （OR=3.608， P=0.045） were independent influencing factors for HBsAg clearance at 48 weeks of treatment in CHB patients with HBsAg <1 500 IU/mL. The Hosmer-Lemeshow goodness-of-fit test yielded χ²=1.398， P=0.994， indicating that the model had good fitting. The Bootstrap method was used to perform internal validation of the nomogram model， and there was a good degree of fitting between the calibration curve and the ideal curve， with a mean absolute error of 0.029. The ROC curve analysis showed that the combination of predictive factors had an area under the ROC curve of 0.982 （95% confidence interval： 0.961 — 0.999）， with a sensitivity of 94.10% and a specificity of 93.10%， suggesting that the nomogram model had a good discriminatory ability. For the CHB patients with HBsAg <1 500 IU/mL and different features， further analysis of HBsAg clearance rate at 48 weeks of treatment showed an HBsAg clearance rate of 69.60% for those with HBsAg ≤67.65 IU/mL before treatment， 58.30% for those with a baseline ALT level of ≥62.50 U/L， 68.30% for those with an ALT level of ≥92.50 U/L at 12 weeks of treatment， 42.40% for those with PLT ≥104×10⁹/L at 24 weeks of treatment， and 48.30% for those with a TSH level of ≤1.38 μIU/mL at 24 weeks of treatment， with significant differences between the two groups （all P<0.001）. ConclusionAge， history of nucleos（t）ide analogue treatment for over 1 year， HBeAg status before treatment， HBsAg level before treatment， baseline ALT level， ALT level at 12 weeks of treatment， PLT level at 24 weeks， and TSH level at 24 weeks of treatment are independent predictive factors. The combined prediction nomogram model constructed in this study has a relatively high value in predicting clinical cure at 48 weeks of PEG-IFN-α-2b treatment in CHB patients with HBsAg<1 500 IU/mL， thereby providing a reference for selecting suitable treatment population and predicting clinical cure.

ObjectiveTo investigate the influencing factors for recompensation in patients with decompensated hepatitis C cirrhosis， and to establish a predictive model. MethodsA total of 217 patients who were diagnosed with decompensated hepatitis C cirrhosis and were admitted to The Third People’s Hospital of Kunming l from January， 2019 to December， 2022 were enrolled， among whom 63 patients who were readmitted within at least 1 year and had no portal hypertension-related complications were enrolled as recompensation group， and 154 patients without recompensation were enrolled as control group. Related clinical data were collected， and univariate and multivariate analyses were performed for the factors that may affect the occurrence of recompensation. The independent-samples t test was used for comparison of normally distributed measurement data between two groups， and the Mann-Whitney U test was used for comparison of non-normally distributed measurement data between two groups； the chi-square test or the Fisher’s exact test was used for comparison of categorical data between two groups. A binary Logistic regression analysis was used to investigate the influencing factors for recompensation in patients with decompensated hepatitis C cirrhosis， and the receiver operating characteristic （ROC） curve was used to assess the predictive performance of the model. ResultsAmong the 217 patients with decompensated hepatitis C cirrhosis， 63 （29.03%） had recompensation. There were significant differences between the recompensation group and the control group in HIV history （χ²=4.566， P=0.034）， history of partial splenic embolism （χ²=6.687， P=0.014）， Child-Pugh classification （χ²=11.978， P=0.003）， grade of ascites （χ²=14.229， P<0.001）， albumin （t=4.063， P<0.001）， prealbumin （Z=-3.077， P=0.002）， high-density lipoprotein （t=2.854， P=0.011）， high-sensitivity C-reactive protein （Z=-2.447， P=0.014）， prothrombin time （Z=-2.441， P=0.015）， carcinoembryonic antigen （Z=-2.113， P=0.035）， alpha-fetoprotein （AFP） （Z=-2.063， P=0.039）， CA125 （Z=-2.270， P=0.023）， TT3 （Z=-3.304， P<0.001）， TT4 （Z=-2.221， P=0.026）， CD45⁺ （Z=-2.278， P=0.023）， interleukin-5 （Z=-2.845， P=0.004）， tumor necrosis factor-α （Z=-2.176， P=0.030）， and portal vein width （Z=-5.283， P=0.005）. The multivariate analysis showed that history of partial splenic embolism （odds ratio ［OR］=3.064， P=0.049）， HIV history （OR=0.195， P=0.027）， a small amount of ascites （OR=3.390， P=0.017）， AFP （OR=1.003， P=0.004）， and portal vein width （OR=0.600， P<0.001） were independent influencing factors for the occurrence of recompensation in patients with decompensated hepatitis C cirrhosis. The ROC curve analysis showed that HIV history， grade of ascites， history of partial splenic embolism， AFP， portal vein width， and the combined predictive model of these indices had an area under the ROC curve of 0.556， 0.641， 0.560， 0.589， 0.745， and 0.817， respectively. ConclusionFor patients with decompensated hepatitis C cirrhosis， those with a history of partial splenic embolism， a small amount of ascites， and an increase in AFP level are more likely to experience recompensation， while those with a history of HIV and an increase in portal vein width are less likely to experience recompensation.

ObjectiveTo investigate the value of albumin-bilirubin （ALBI）， easy albumin-bilirubin （EZ-ALBI）， and platelet-albumin-bilirubin （PALBI） scores in predicting 2-year survival in patients with HCV-associated hepatocellular carcinoma （HCV-HCC）. MethodsA retrospective analysis was performed for the clinical data of 174 patients with HCV-HCC who were admitted to The Third People’s Hospital of Kunming from January 2020 to January 2022， and the patients were followed up till 2 years after admission. According to the follow-up results， the patients were divided into survival group with 95 patients and death group with 79 patients. The independent-samples t test or the Mann-Whitney U test was used for comparison of continuous data between two groups， and the chi-square test was used for comparison of categorical data between two groups. Univariate and multivariate Cox proportional-hazards regression model analyses were used to investigate the influencing factors for the survival of HCV-HCC patients. The Kaplan-Meier method was used to plot survival curves and analyze the 2-year survival rate of HCV-HCC patients with different EZ-ALBI grades， and the log-rank test was used for comparison between groups. ResultsThere were significant differences between the survival group and the death group in platelet count， aspartate aminotransferase （AST）， total bilirubin， albumin （Alb）， alpha-fetoprotein （AFP）， prealbumin， prothrombin time， international normalized ratio， PALBI score， ALBI score， EZ-ALBI score， Model for End-Stage Liver Disease （MELD） score， HCV genotype， peritoneal effusion， and vascular invasion （all P<0.05）. The univariate Cox regression analysis showed that AST， Alb， AFP， ALBI score， EZ-ALBI score， PALBI score， MELD score， Barcelona Clinic Liver Cancer Staging， and peritoneal effusion were influencing factors for the survival of patients （all P<0.05）， and the multivariate Cox regression analysis showed that EZ-ALBI score （hazard ratio ［HR］=1.850， 95% confidence interval ［CI］： 1.054 — 3.247， P=0.032） and peritoneal effusion （HR=1.993， 95%CI： 1.030 — 3.858， P=0.041） were independent risk factors for the survival of HCV-HCC patients. The survival curve analysis showed that the patients with EZ-ALBI grade 1/2/3 had a 2-year survival rate of 90.9%， 60.2%， and 32.2%， respectively， and there was a significant difference in cumulative survival rate between the patients with different EZ-ALBI grades （χ²=26.294， P<0.001）. ConclusionEZ-ALBI score and the presence or absence of peritoneal effusion can be used as predictors of the survival of HCV-HCC patients.

Objective To analyze the influencing factors of chronic liver failure in patients with primary hepatic carcinoma(PHC)before intervention,and to establish and evaluate a nomogram risk prediction model.Methods A retrospective analysis was conducted by collecting general data and clinical test data within 24 hours of admission for PHC patients.Univariate analysis and Lasso regression were used for variable selection,followed by multivariate logistic regression analysis to identify independent influencing factors for CLF before PHC intervention,leading to the establishment of a nomogram risk prediction model.The model was evaluated using the Hosmer-Lemeshow test,receiver operating characteristic(ROC)curve,calibration curve,clinical decision curve,and clinical impact curve.Result A total of 353 cases of PHC patients were collected,including 153 cases in the liver failure group and 200 cases in the non-liver failure group,with a prevalence rate of 43.3％.Variables selected by Lasso regression included gastrointestinal bleeding,prothrombin time(PT),albumin(ALB),total bilirubin(TBIL),and gamma glutamyl transferase(GGT).Multivariate logistic regression analysis showed that gastrointestinal bleeding(OR=13.549,95％CI:2.899～63.322,P=0.001),PT(OR=1.599,95％CI:1.282～1.995,P＜0.001),TBIL(OR=1.016,95％CI:1.006～1.025,P=0.002),and GGT(OR=1.002,95％CI:1.000～1.003,P=0.028)were independent risk factors for chronic liver failure prior to PHC intervention,leading to the establishment of a nomogram risk prediction model.The Hosmer Lemeshow test showed that the model had a good fit(x2=6.152,P＞0.05);the area under ROC was 0.902(0.869-0.934),with a sensitivity of 80.4％and a specificity of 87.5％.The calibration curve indicated that the model predicts chronic liver failure prior to PHC intervention with good consistency.Clinical decision curve analysis and clinical impact curve analysis showed that the model has good clinical utility within a certain threshold range.Conclusion Gastrointestinal bleeding,PT ≥16.05s,TBIL≥37.80 mmol/L,and GGT≥ 99.00 U/L are independent risk factors for the occurrence of chronic liver failure before PHC intervention.The established nomogram risk prediction model has certain clinical application value in predicting the risk of chronic liver failure before PHC intervention.

Objective To compare the efficacy of Tenofovir Alafenamide(TMF)and Tenofovir Disoproxil Fumarate(TDF)in terms of liver function restoration,virus clearance,immune regulation,anti liver fibrosis,lipid metabolism,bone and renal safety,and adverse reactions.Methods A retrospective analysis was conducted on 110 patients with chronic hepatitis B(CHB)admitted to Kunming Third People's Hospital from January 2022 to December 2022.Patients were divided into the TMF treatment group(n=55)and the TDF treatment group(n=55)based on their treatment regimen.We compared the levels of transaminase levels,antiviral efficacy,T cell subsets,renal function electrolytes,lipid metabolism,four liver fibrosis-related indicators,and changes in liver stiffness grading before and after treatment in two groups of patients.The incidence of adverse reactions post-treatment was also compared.Results After 48 weeks of treatment,the levels of TBIL,ALT,AST,GGT,and GLOB in both groups of patients were significantly lower than pre-treatment levels(P<0.05).The decrease in AST levels in the TMF group was lower than that in the TDF group(P<0.05).After 48 weeks of treatment,the HBV-DNA seroconversion rate in the TMF group(90.90%)was higher than that in the TDF group(83.64%).The serological HBsAg clearance rate in the TMF group(7.3%)was lower than that in the TDF group(9.1%),while the HBeAg clearance rate in the TMF group(38.2%)was significantly higher than that in the TDF group(18.2%),with statistical significance(P<0.05).After 48 weeks of treatment,levels of CD3+,CD4+,and CD8+in both groups were significantly elevated compared to pre-treatment levels(P<0.05);notably,the TMF group had higher post-treatment levels of CD3+,CD4+,and CD8+than the TDF group.After 48 weeks,the average values of HA,IV-C,and LN among the TMF group for liver fibrosis indicators were significantly lower than those in the TDF group(P<0.05).The proportions of F0 and F2 in both groups significantly increased post-treatment,while the proportions of F3 and F4 significantly decreased(P to be supplemented);furthermore,the proportions of F0 and F2 in the TMF group were significantly higher than those in the TDF group,and the proportions of F3 and F4 in the TMF group were significantly lower than those in the TDF group(P<0.05).After 48 weeks,HDL-C levels in the TMF group increased compared to pre-treatment(P<0.05).There were no significant differences in TG,TC,HDL-C,or LDL-C levels in the TDF group compared to pre-treatment(P>0.05).After 48 weeks of treatment,there was no difference in the levels of BUN、Cr、P+,and Ca+in the TMF group compared to pre-treatment(P>0.05);however,BUN and Cr levels in the TDF group were significantly higher than pre-treatment levels,while P+and Ca+levels were significantly lower(P<0.05).The incidence of elevated uric acid and bone pain was significantly higher in the TMF group compared to the TDF group(P<0.05);the incidence of diarrhea and abdominal pain was slightly higher in the TMF group compared to the TDF group(P>0.05).Conclusion Compared to TDF,TMF demonstrates a higher rate of liver function recovery,a greater virological response,enhanced anti fibrotic efficacy,and improved drug safety,making it worthy of clinical application in the future.

Objective To investigate the value of albumin-bilirubin(ALBI),easy albumin-bilirubin(EZ-ALBI),and platelet-albumin-bilirubin(PALBI)scores in predicting 2-year survival in patients with HCV-associated hepatocellular carcinoma(HCV-HCC).Methods A retrospective analysis was performed for the clinical data of 174 patients with HCV-HCC who were admitted to The Third People's Hospital of Kunming from January 2020 to January 2022,and the patients were followed up till 2 years after admission.According to the follow-up results,the patients were divided into survival group with 95 patients and death group with 79 patients.The independent-samples t test or the Mann-Whitney U test was used for comparison of continuous data between two groups,and the chi-square test was used for comparison of categorical data between two groups.Univariate and multivariate Cox proportional-hazards regression model analyses were used to investigate the influencing factors for the survival of HCV-HCC patients.The Kaplan-Meier method was used to plot survival curves and analyze the 2-year survival rate of HCV-HCC patients with different EZ-ALBI grades,and the log-rank test was used for comparison between groups.Results There were significant differences between the survival group and the death group in platelet count,aspartate aminotransferase(AST),total bilirubin,albumin(Alb),alpha-fetoprotein(AFP),prealbumin,prothrombin time,international normalized ratio,PALBI score,ALBI score,EZ-ALBI score,Model for End-Stage Liver Disease(MELD)score,HCV genotype,peritoneal effusion,and vascular invasion(all P<0.05).The univariate Cox regression analysis showed that AST,Alb,AFP,ALBI score,EZ-ALBI score,PALBI score,MELD score,Barcelona Clinic Liver Cancer Staging,and peritoneal effusion were influencing factors for the survival of patients(all P<0.05),and the multivariate Cox regression analysis showed that EZ-ALBI score(hazard ratio[HR]=1.850,95%confidence interval[CI]:1.054-3.247,P=0.032)and peritoneal effusion(HR=1.993,95%CI:1.030-3.858,P=0.041)were independent risk factors for the survival of HCV-HCC patients.The survival curve analysis showed that the patients with EZ-ALBI grade 1/2/3 had a 2-year survival rate of 90.9%,60.2%,and 32.2%,respectively,and there was a significant difference in cumulative survival rate between the patients with different EZ-ALBI grades(χ2=26.294,P<0.001).Conclusion EZ-ALBI score and the presence or absence of peritoneal effusion can be used as predictors of the survival of HCV-HCC patients.

Objective:To analyze the clinical efficacy and safety of carglumic acid in the treatment of neonatal hyperammonemia caused by organic acidemia.Methods:A case summary was made.Six cases of neonatal hyperammonemia caused by organic acidemia treated at the Neonatal Intensive Care Unit of Henan Children′s Hospital from March to September in 2024 were included.They received comprehensive ammonia-lowering treatment in combination with oral carglumic acid dispersible tablets.The clinical data of the children were collected and analyzed retrospectively.Changes in blood ammonia levels, blood gas parameters, and complete blood count before and after treatment with carglumic acid were analyzed using the Wilcoxon test.The incidence of adverse reactions and clinical regression during the treatment with carglumic acid was observed.Results:There were 2 females and 4 males in the 6 patients included.Four children suffered from isolated methylmalonic acidemia caused by MUT gene mutations, and the other 2 had propionic acidemia.The clinical manifestations were poor breastfeeding in 6 cases, vomiting in 2 cases, poor response in 6 cases, weight loss in 6 cases, and convulsions in 3 cases.Acute metabolic decompensation abnormalities were presented in all children, such as metabolic acidosis, hyperammonemia, leukopenia and thrombocytopenia.The first dose of carglumic acid was 62-255 mg/kg, the second dose was 75-172 mg/kg.The blood ammonia level decreased from 411.7 (339.7, 623.8) μmol/L before treatment to 108.1 (35.5, 229.1) μmol/L after 48 hours of treatment, showing a statistically significant reduction ( Z=2.20, P<0.05).Three cases with a blood ammonia level higher than 400 μmol/L, it was effectively reduced after treatment with carglumic acid.Two cases did not undergo hemodialysis or peritoneal dialysis.One case underwent hemodialysis but died after withdrawing the treatment.After administration of carglumic acid, metabolic acidosis was corrected in all children, and 2 patients ultimately died after discontinuing the treatment.No causal relationship was identified between adverse events and carglumic acid treatment.The examinations at discharge and during the follow-up period (2-7 months) showed that most laboratory abnormalities (including leukopenia, anemia, thrombocytopenia, hyperlactatemia, hyponatremia, hyperkalemia, elevated myocardial enzymes, and hyperbilirubinemia) returned to normal. Conclusions:Carglumic acid can effectively reduce neonatal hyperammonemia caused by organic academia, improve metabolic disorders, and reduce the need for blood purification or peritoneal dialysis, with good safety.

Objective To investigate the influencing factors for recompensation in patients with decompensated hepatitis B cirrhosis,and to establish a predictive model.Methods A total of 517 patients who attended The Third People's Hospital of Kunming and were diagnosed with decompensated hepatitis B cirrhosis from January 1,2016 to December 31,2022 were enrolled.The clinical data of the patients were reviewed,and the 207 patients with no portal hypertension-related complications within at least 1 year were enrolled as recompensation group,while the 310 patients without recompensation were enrolled as persistent decompensation group.Related clinical data were collected,and the univariate and multivariate Cox regression analyses were performed for the factors that might affect the occurrence of recompensation.The independent-samples t test was used for comparison of normally distributed continuous data between two groups,and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups;the chi-square test or the Fisher's exact test was used for comparison of categorical data between two groups.The"rms"package was used to establish a nomogram;the receiver operating characteristic(ROC)curve was plotted,and the area under the ROC curve(AUC)was calculated;the Hosmer-Lemeshow test was used to evaluate the degree of fitting of the model;the"Calibration Curves"package was used to plot the calibration curve for model assessment.Results Among the patients with decompensated hepatitis B cirrhosis,207(40.03%)had recompensation.The univariate Cox regression analysis showed that there were significant differences between the recompensation group and the persistent decompensation group in TIPS history,genotyping,portal vein thrombosis,complicated infection,Child-Pugh class,age,hemoglobin(Hb),platelet count,total protein,albumin(Alb),alanine aminotransferase,triglyceride,cholesterol,creatinine,Na,interleukin-6,high-sensitivity C-reactive protein,portal vein width,and portal vein velocity(all P<0.05).The multivariate regression analysis showed that TIPS history(hazard ratio[HR]=2.491,95%confidence interval[CI]:1.325-4.681,P=0.005),portal vein thrombosis(HR=0.345,95%CI:0.152-0.783,P=0.001),Hb(HR=1.007,95%CI:1.000-1.013,P=0.028),Alb(HR=1.048,95%CI:1.017-1.080,P=0.002),and portal vein width(HR=0.899,95%CI:0.835-0.967,P=0.004)were independent influencing factors for recompensation in patients with decompensated hepatitis B cirrhosis.A nomogram model was established based on the above five influencing factors,and the Hosmers-Lemeshow test showed that this model had a good degree of fitting(χ2=3.202,P=0.921).The nomogram model had an AUC of 0.728,a sensitivity of 50.3%,and a specificity of 85.0%,and the calibration curve showed good consistency between the actual value of this model in predicting the occurrence of recompensation and the predicted value in patients with decompensated hepatitis B cirrhosis.Conclusion Patients with decompensated hepatitis B cirrhosis who have a history of TIPS and high levels of Alb and Hb are more likely to have recompensation,and it is relatively difficult for patients with portal vein thrombosis and an increase in portal vein width to achieve recompensation.