This paper reports the diagnosis and treatment of a pregnant woman complicated with myotonic dystrophy (DM) type 1(DM1). The pregnant woman complained of occasional dull pain in the precardiac region accompanied by night sweats in the first trimester and a mild increase in myocardial enzymes was observed. Symptoms were improved after oral medication followed by no regular reexamination. While in the third trimester, abnormal myocardial enzyme, liver enzyme, and urinary protein were found and echocardiography and abdomen ultrasound suggested multiple organic lesions involving the heart and spleen. A girl was born through cesarean section at 36 +5 weeks of gestation due to maternal heart failure, with Apgar scores of 10 at 1 and 5 minutes. The patient developed acute respiratory failure, ventricular bigeminy, and diarrhea after surgery. Neurology consultation found incomplete buried eyelids in both eyes, weak bulging cheeks, and weak upper extremity muscle strength. Muscle biopsy confirmed the diagnosis of DM and genetic testing showed abnormal amplification of non-coding region [CTG] n in myotonic dystrophy protein kinase gene in chromosome 19q13.3, which indicated DM1. The mother recovered well at nine months postpartum. Further genetic analysis of pedigree and offspring is recommended.

Objective:To study the impact of preoperative serum HBV DNA levels on prognosis of hepatocellular carcinoma (HCC) patients undergoing hepatectomy with curative intent.Methods:The clinical data of patients with HCC treated by hepatectomy with curative intent at the Guangxi Medical University Cancer Hospital from January 2010 to December 2016 were retrospectively analyzed. According to the preoperative serum HBV DNA levels, patients were divided into three groups: the control group (HBV DNA negative), the low load group (<10 4 copy/ml) and the high load group (≥10 4 copy/ml). The clinical data of these patients were collected and long-term survival outcomes of these patients were followed-up. The Kaplan-Meier method was used to compare the overall survival (OS) and recurrence-free survival (RFS) rates among the three groups. Using the Barcelona clinic liver cancer classification (BCLC), patients with different serum HBV DNA levels were further divided into three subgroups: stage 0/A, stage B and stage C. The OS and RFS rates of patients in each of these subgroups were compared. Results:Of 1 180 patients who were enrolled in the study, there were 1 024 males and 156 females, aged (48.6±10.8) years. The 1-, 3- and 5-year OS rates for patients in the control group ( n=258) were 91.5%, 79.3% and 74.9%, respectively; while those in the low load group ( n=289) were 87.2%, 68.6% and 61.6%, respectively; and those in the high load group ( n=633) were 85.4%, 68.9% and 60.7%, respectively. The 1-, 3- and 5-year OS rates in the control group were significantly better than those in the low load group and the high load group ( P<0.05). The 1-, 2- and 3-year RFS rates in the control group were significantly higher than those in the high load group ( P<0.05). Subgroup analysis showed that in the BCLC 0/A subgroup ( n=786) the 1-, 3- and 5-year OS rates in the control group were significantly better than those in the high load group ( P<0.05). In the BCLC B subgroup ( n=181), the 1-, 2- and 3-year RFS rates in the control group were significantly higher than those in the high load group ( P<0.05). In the BCLC C subgroup ( n=214), there were no significant differences in the 1-, 3- and 5-year OS and 1-, 2- and 3-year RFS rates among the three groups ( P>0.05). Conclusion:For HCC patients undergoing hepatectomy with curative intent, the higher the preoperative serum HBV-DNA level, the worse the long-term survival outcomes.

Objective To study the correlation between serum prealbumin level before liver resection and prognosis of patients with primary hepatocellular carcinoma (HCC).Methods The clinical data of patients with HCC who underwent liver resection at the Affiliated Tumor Hospital of Guangxi Medical University from August 2007 to October 2016 were retrospectively analyzed.The previous albumin of 200 mg/L and the pre-albumin as predicted by the maximum selection rank statistic method were used as the bounding group,and reduced groups and the correlation between pre-operative serum pre-albumin levels and clinicopathological characteristics were analyzed.The Kaplan-Meier method was used to calculate the overall survival rate of patients with the different cutoff levels.The Cox proportional regression model was used to analyze,and cirrhosis,alpha-fetoprotein levels and Barcelona Clinic Liver Cancer staging were used to adjust the relationship between serum prealbumin and prognosis of liver resection for HCC patients.Analysis of stratified variables was performed and their interactions with serum prealbumin were analyzed.Results Of the 2 022 patients included in this study,there were 1 739 males and 283 females.Their age was 49.5 ± 11.2 years.The median follow-up was 37.4 months.The optimal cutoff value of prealbumin predicted by the maximum selection rank statistic method was 166 mg/L.Regardless of the cutoff values of previous albumin 200 mg/L or prealbumin 166 mg/L,multivariate analysis showed that preoperative serum prealbumin level was an independent prognostic risk factor for patients (P ＜0.05).The prognosis of patients with ＞200 mg/L (＞ 166 mg/L) serum prealbumin before surgery was significantly better than that of patients with ≤200 mg/L (≤166 mg/L) prealbumin,the differences were significant (all P ＜ 0.05).After adjusting for confounding factors,the prealbumin level correlated with prognosis of patients with HCC [cutoff value 200 mg/L:HR (95％ CI) was 1.59 (1.35-1.86),cutoff value 166 mg/L:HR (95％ CI) was 1.69 (1.44-1.98),all P ＜ 0.05].The results of stratified analysis showed that the relationship between prealbumin levels and the prognosis of HCC patients became more robust.Conclusions Preoperative serum prealbumin was an independent risk factor for prognosis of HCC patients,and it had predictive value on prognosis of HCC patients.

Objective: To investigate the impact of perioperative allogeneic red blood cells transfusion on the early recurrence of patients with hepatocellular carcinoma (HCC) after liver resection. Methods: Retrospective analysis was made on 999 patients who underwent surgical resection for HCC, and these patients were divided into two groups according to whether or not received perioperative allogeneic red blood cells transfusion. Differences between groups were balanced using propensity score matching (PSM). The Kaplan-Meier method was used for comparing the differences in early recurrence (within 2 years) between the two groups and the multivariate COX analysis regression was used to identify independent risk factors for early recurrence. Result: There were 100 patients in red cell transfusion group and 899 patients in non-red cell transfusion group. After PSM, 85 pairs of patients were successfully matched, and there was no significant difference in baseline data between groups. Before PSM, the early recurrence rate of the red blood cell group was significantly higher than that of the non-red blood cell group (P<0.05). However, there was no significant difference in early recurrence rates between the two groups after PSM (P=0.346). Multivariate analysis showed that perioperative allogeneic red blood cells transfusion was not an independent risk factor of early recurrence for patients with HCC after liver resection (P=0.153). Conclusion Perioperative allogeneic red blood cells transfusion has no impact on the early recurrence of patients with HCC after liver resection.

Objective: To compare the prognosis of radiofrequency ablation (RFA) for postoperative recurrent hepatocellular carcinoma and primary hepatocellular carcinoma(HCC). Methods: The clinical data of 179 patients with recurrent HCC (recurrent group) and primary HCC (primary group) treated by RFA from 2009 to 2015 were retrospectively analyzed. Overall survival rate (OS) and disease-free survival rate (DFS) were analyzed by Kaplan-meier log-rank test. The prognostic factors of RFA for recurrent HCC were analyzed by COX proportional hazard regression. Results: The 1, 3 and 5year′s OS of the recurrent group were 93%, 73%, 61%, respectively and 85%, 75%, 61% for the primary group(χ²=0.017, P=0.896). The corresponding 1, 3 and 5year′s DFS were 61%, 39%, 21% and 79%, 64%, 46% respectively (χ²=3.899, P=0.048). The independent risk factors affecting the OS of the recurrent group were the interval between hepatectomy to recurrence≤12 months (HR=0.264, 95% CI=0.077-0.901, P=0.033) and the Child-Pugh grading B before RFA (HR=4.501, 95% CI=1.426-14.208, P=0.01). Conclusions The DFS of patients with recurrent HCC were shorter than that with primary HCC treated by RFA. The interval between hepatectomy to recurrence and the Child-Pugh grading before RFA were independent risk factors for OS of the recurrent group.

Objective To compare the clinical features and surgical outcomes of hepatocellular carcinoma (HCC) combined with portal venous tumor thrombus (PVTT) and hepatic venous tumor thrombus (HVTT) or bile duct tumor thrombi (BDTT),and analyze the effects of different tumor thrombus (TT) types and different surgical methods for TT on prognosis.Methods The retrospective cross-sectional study was conducted.The clinical data of 220 HCC patients with lymphovascular invasion (LVI) who were admitted to the Affiliated Cancer Hospital of Guangxi Medical University between January 2004 and December 2014 were collected.Of 220 patients,140 were combined with PVTT,36 with HVTT and 44 with BDTT.According to patients' conditions,they underwent tumor and TT resection,and tumor resection + TT removal or single TT removal.Observation indicators:(1) comparisons of clinical features of HCC patients with PVTT or HVTT or BDTT;(2) surgical and postoperative situations;(3) follow-up and survival.Follow-up using outpatient examination and telephone interview was performed to detect postoperative survival up to December 2015.Measurement data with normal distribution were represented as (x)±s.Comparisons among 3 indicators were analyzed using the one-way ANOVA,and comparisons between 2 indicators were analyzed using the t test.Comparisons of count data were analyzed using the chi-square test.The survival curve and rate were respectively drawn and calculated by the Kaplan-Meier method,and the Log-rank test was used for survival analysis.Results (1) Comparisons of clinical features of HCC patients with PVTT or HVTT or BDTT:number of patients with Child-pugh A,Child-pugh B and peritoneal effusion,tumor diameter and cases with tumor capsule were respectively detected in 133,7,23,(10±4)cm,91 in HCC patients with PVTT and 35,1,4,(9±4)cm,27 in HCC patients with HVTT and 35,9,16,(6±4)cm,15 in HCC patients with BDTT,with statistically significant differences (x2 =12.693,10.408,F=11.300,x2 =17.188,P＜ 0.05).(2) Surgical and postoperative situations:of 140 HCC patients with PVTT,51 underwent tumor and PVTT resection,89 underwent tumor resection + PVTT removal through incising portal vein;68 received postoperative transcatheter arterial chemoembolization (TACE).Thirty-six HCC patients with HVTT underwent tumor and HVTT resection;24 received postoperative TACE.Of 44 HCC patients with BDTT,23 underwent tumor and BDTT resection,21 underwent tumor resection + BDTT removal through incising common bile duct;29 received postoperative TACE.(3) Follow-up and survival:① 220 patients were followed up for 1-73 months,with a median time of 12 months.The median survival time,1-,3-and 5-year survival rates were respectively 12 months,48.2％,25.0％,15.4％ in 140 HCC patients with PVTT and 28 months,77.1％,45.6％,24.5％ in 36 HCC patients with HVTT and 36 months,88.6％,48.3％,24.6％ in 44 HCC patients with BDTT,with a statistically significant difference in survival (x2 =13.316,P＜0.05).② Of 140 HCC patients with PVTT,49 were in type Ⅰ PVTT,and median survival time,1-,3-and 5-year survival rates were respectively 20 months,60.3％,32.6％ and 17.1％;70 were in type Ⅱ PVTT,and median survival time,1-,3-and 5-year survival rates were respectively 13 months,51.4％,26.0％ and 17.3％;21 were in type Ⅲ PVTT,and median survival time,1-,3-and 5-year survival rates were respectively 7 months,9.5％,4.8％ and 0,showing a statistically significant difference in survival (x2=18.102,P＜0.05).The median survival time,1-,3-and 5-year survival rates were respectively 21 months,72.5％,42.5％,26.2％ in 51 patients undergoing tumor and TT resection and 9 months,40％,14.4％,0 in 89 patients undergoing tumor resection + PVTT removal through incising portal vein,showing a statistically significant difference in survival (x2=24.098,P＜0.05).③ Of 36 HCC patients with HVTT,17 were detected in right HVTT,and median survival time,1-,3-and 5-year survival rates were respectively 14 months,64.7％,20.2％ and 0;10 were detected in left HVTT,and median survival time,1-,3-and 5-year survival rates were respectively 53 months,80.0％,70.0％ and 38.9％;9 were detected in middle HVTT,and median survival time,1-,3-and 5-year survival rates were respectively 40 months,88.9％,61.0％ and 30.5％;showing no statistically significant difference in survival (x2 =5.951,P＞0.05).④ Of 44 HCC patients with BDTT,24,6 and 14 were respectively detected in type Ⅰ,Ⅱ and Ⅲ BDTTs,and median survival time,1-,3-and 5-year survival rates were respectively 38 months,87.5％,60.4％,34.9％ in type Ⅰ BDTT patients and 26 months,83.3％,16.7％,0 in type Ⅱ BDTT patients and 35 months,78.6％,50.0％,21.4％ in type Ⅲ BDTT patients,showing no statistically significant difference in survival (x2 =5.312,P＞0.05).Of 44 patients,median survival time,1-,3-and 5-year survival rates were respectively 38 months,91.3％,59.5％,34.3％ in 23 patients undergoing tumor and TT resection and 26 months,85.7％,35.7％,15.3％ in 21 patients undergoing tumor resection + TT removal through incising common bile duct,showing no statistically significant difference in survival (x2 =2.071,P＞0.05).Conclusions HCC patients with PVTT have larger tumor diameter and worse liver dysfunction,and are prone to peritoneal effusion.HCC patients with different LVI undergo surgery.There is better prognosis in HCC patients with BDTT,and good prognosis in patients with HVTT,while poorer prognosis in patients with PVTT.The postoperative survival of HCC patients with PVTT is associated with TT type,and patients will have better prognosis after tumor resection + TT removal if TT type is confirmed earlier.The postoperative survival of HCC patients with BDTT is not associated with TT type,tumor resection + TT removal maybe prolong postoperative survival time.

Objective: To explore the correlation between standard remnant liver volume(SRLV) and post-hepatectomy liver failure (PHLF)in patients with hepatocellular carcinoma(HCC)and cirrhotic livers.Methods:In total,181 patients who underwent hemihepa-tectomy in Affiliated Tumor Hospital of Guangxi Medical University from September 2013 to August 2016 were enrolled in the study. Total liver,tumor,remnant liver,and resected liver volumes were measured using the Myrian liver surgical planning system before sur-gery. Intraoperative resected liver volume (including resected normal liver and tumor volumes) were collected using the drainage method.The patients were divided into the PHLF(22 cases)and non-PHLF groups(159 cases)according to whether PHLF occurred based on the"50/50"criteria.The risk factors of PHLF were then explored.The cut-off of SRLV and efficiency of predicting PHLF were analyzed in the subgroup of patients with cirrhotic livers.The grade of liver cirrhosis was retrospectively analyzed using helical comput-ed tomography(CT).Results:Twenty-two of the 181 patients developed PHLF and one died of it.Preoperative total bilirubin levels and SRLV were identified as independent factors for predicting PHLF using a Logistic regression model.In total,102 patients with cirrhotic livers were selected in subgroup analysis based on postoperative cirrhotic pathology.Eighteen patients developed PHLF and one died of PHLF in the subgroup.Using receiver-operating characteristic(ROC)curve analysis,340 mL/m2was the cut-off of SRLV for patients with HCC and cirrhotic livers(area under the curve:0.861,P<0.01;sensitivity and specialty rates were 94.4% and 74.7%,respectively). Eighty-four cases were of grade Ⅰ or Ⅱ cirrhosis,18 cases were of grade Ⅲ cirrhosis,and there were no cases of grade Ⅳ cirrhosis based on retrospective analysis using helical CT.Conclusions:Patients with cirrhotic livers with an anticipated SRLV of≤340 Ml/m2after he-patic resection are at increased risk for PHLF after emihepatectomy.

Objective To retrospectively study the prognostic impact of paraneoplastic erythrocytosis (PE) on patients with hepatocellular carcinoma (HCC) after liver resection.Methods 713 patients with HCC who underwent partial hepatic resection in The Affiliated Cancer Hospital of Guangxi Medical University were divided into two groups:the PE group (n =81) and the non-PE group (n =632).The overall survival between the two groups were compared after reducing confounding bias by using propensity score matching (PSM).Independent prognostic predictors were determined by the Cox proportional hazards model.Results 80 pairs of patients were matched using PSM.In the matched cohort,the PE group exhibited significantly longer overall survival (OS) compared to the NPE group of patients without erythrocytosis.The 1-,3-,and 5-year overall survival rates were 88.6％,74.2％,69.0％ in the PE group,and 91.0％,60.1％,41.6％ in the non-PE group,respectively (P ＜ 0.05).Using the log-rank test,tumor size ≥ 10cm,macrovascular invasion,Barcelona Clinic Liver Cancer (BCLC) stage C,PE and complete tumor encapsulation were significantly associated with OS in patients with HCC after liver resection.The Cox regression analysis indicated that tumor size ≥ 10 cm,and Barcelona Clinic Liver Cancer (BCLC) stage C were independent prognostic factors of poor prognosis,while complete tumor encapsulation and paraneoplastic erythrocytosis were independent predictors of good prognosis.Conclusions For patients with HCC who underwent surgical resection,patients with PE had better prognosis than those without PE under the condition of similar tumor burden.PE was an independent predictor of good prognosis.

Objective To evaluate the prognostic significance of preoperative serum gamma glutamyl transpeptidase (GGT) level in patients with hepatocellular carcinoma (HCC) after liver resection.Methods A total of 432 patients undergoing hepatectomy for HCC were divided into normal GGT group (175 patients with GGT ≤ 50 U/L) and high GGT group (257 patients with GGT ＞ 50 U/L).After balancing baseline characteristics by propensity score analysis,disease-free survival (DFS) and overall survival (OS) were compared between the two groups.Independent risk factors influencing DFS and OS were identified by Cox multivariate analyses.Results Propensity score analysis identified 124 matched pairs of patients from each group.In the propensity-matched cohort,DFS at 1,3,and 5 years in normal GGT group (69.3％,36.1％,12.8％) was significantly higher than that in high GGT group (60.6％,18.7％,7.5％;P=0.039).OSat1,3,and5 years innommlGGTgroup (90.7％,73.7％,66.1％) was also significantly higher than that in high GGT group (89.2％,63.6％,43.3％;P =0.024).COX multivariate analyses revealed that alpha-fetoprotein ≥400 ng/ml,GGT ＞ 50 U/L,macrovascular invasion,tumor size ≥ 10 cm,and tumor number ≥3 were independent risk factors for DFS in patients with HCC after liver resection.Albumin ＜ 35 g/L,GGT ＞ 50 U/L,macrovascular invasion,tumor size ≥ 10 cm,and tumor number ≥ 3 were identified as independent risk factors for OS.Conclusions Preoperative serum GGT level is an independent factor predicting tumor recurrence and long-term survival in HCC patients after liver resection.

Objective To explore the association between ratio index of gamma glutamyl transpeptidase/platelet (GPRI) and the prognosis of patients with hepatitis B virus (HBV) related hepatocellular carcinoma (HCC) before liver resection. Methods A total of 368 patients underwent liver resection for HBV-related HCC were retrospectively analyzed in this study. Patients were divided into high GPRI group (n=184, GPRI≥0.38) and low GPRI group (n=184, GPRI<0.38). Clinicopathologic characteristics including overall survival (OS) and disease-free survival (DFS) were compared between the two groups. Independent risk factors influencing DFS and OS were determined by Cox multivariate analysis. Results Compared to low GPRI group, there were higher levels of serum total bilirubin and alanine aminotransferase, higher proportions of tumor diameter larger than 10 cm, amount of tumou more than 3, and patients with macrovascular invasion and intermediate or advanced HCC in high GPRI group (all P<0.05). Values of DFS at 1, 3, and 5 years were significantly lower in high GPRI group (50.8%, 16.9%and 5.7%) than those in low GPRI group (69.0%, 33.3%, 10.7%;P=0.001). Values of OS at 1, 3, and 5 years were also significantly lower in high GPRI group (75.0%, 51.8%and 36.0%) than those in low GPRI group (89.8%, 72.8%and 63.2%;P<0.05). Cox multivariate analysis also demonstrated that GPRI ≥0.38 was an independent risk factor for DFS and OS in patients with HBV-related HCC after liver resection. Conclusion Preoperative GPRI can predict tumor recurrence and long-term survival in patients with HBV-related HCC after liver resection.