Objective:To summarize and analyze the clinical phenotype and genotype characteristics of atypical hemolytic uremic syndrome (aHUS) in Chinese children.Methods:It was a retrospective study. The clinical data and genetic results of 6 children with aHUS admitted to Children's Hospital Affiliated to Capital Institute of Pediatrics from May 2016 to October 2022 were analyzed, and literature on Chinese aHUS children with genetic screening data by searching databases such as Wanfang, CNKI, and PubMed were reviewed and summarized. Through literature search, the children with aHUS were divided into genetic variation group and non-genetic variation group according to the results of genetic testing, and the differences of clinical phenotype, laboratory examination, follow-up and outcomes were compared between the two groups. Logistic regression method was used to analyze the risk difference of disease recurrence, end-stage kidney disease and death between genetic variation group and non-genetic variation group.Results:Among the 6 aHUS children in this center, there were 1 male and 5 females, with onset age of 7 months to 10 years old. Four patients had gene variations, including 1 patient of complement factor H ( CFH) gene variation, 1 patient of C3 gene variation, and 2 patients of CFHR1 combined with CFHR3 gene variation. Six children had gross hematuria, proteinuria, hypertension and decreased complement C3. The mean values of serum creatinine in 4 genetic variation and 2 non-genetic variation children were 153.9 μmol/L and 214.3 μmol/L, respectively; the mean values of estimated glomerular filtration rate were 26.4 ml·min -1·(1.73 m 2) -1 and 28.9 ml·min -1·(1.73 m 2) -1, respectively; the mean values of hemoglobin were 81 g/L and 57 g/L; the mean values of platelet were 46×10 9/L and 71×10 9/L; the mean values of lactic dehydrogenase were 2 408 U/L and 2 106 U/L, respectively; there were 1 and 2 cases of positive CFH antibody, and 1 and 1 case of nervous system complication, respectively. Ninety-seven aHUS children were retrieved including the reported 6 cases in this center, with 60 males and 37 females, and median onset age of 5 years old. The positive detection rate of genetic variation was 58.8% (57/97). The main type of genetic variation was CFHR gene variation (43.9%, 25/57), followed by CFH gene variation (33.3%, 19/57).There was no significant difference in onset age, sex distribution, proportions of gross hematuria, massive proteinuria, hypertension, complement C3 decline, positive CFH antibody and treatment method, platelet, and lactic dehydrogenase between genetic variation group and non-genetic variation group (all P>0.05). Compared with the genetic variation group, non-genetic variation group had higher serum creatinine ( Z=2.311, P=0.021) and lower hemoglobin ( Z=-2.636, P=0.008). The median follow-up time in genetic variation group and non-genetic variation group was 1 year and 2 years, respectively. The proportions of non-remission and recurrence in the genetic variation group were significantly higher than those in non-genetic variation group ( χ2=12.016, P=0.002; χ2=4.689, P=0.030). Logistic regression analysis showed that the recurrence risk of aHUS in children with genetic mutations was higher than that in children with non-genetic mutations ( OR=2.807, 95% CI 1.014-7.772). Conclusions:The main type of aHUS gene variation in Chinese children is CFHR gene variation, and the children with gene variation have poor prognosis and a higher risk of recurrence.

Hepatocellular carcinoma (HCC) is one of the most common malignant tumors, and its features of insidious onset, rapid development, and high recurrence bring great challenges to the long-term survival of HCC patients. Molecular-targeted drugs and immune checkpoint inhibitors have become the research hotspots in the treatment of HCC, and a large number of clinical trials have found that combined therapy has achieved a good clinical effect. This article mainly introduces the application of combined therapy with molecular-targeted drugs and immune checkpoint inhibitors and other combined therapies in HCC and points out that combined therapy related to systemic therapy or local therapy has become the latest research hotspot in HCC treatment.

Objective:To investigate the feasibility and safety of laparoscopic liver tumor resection in children.Methods:The clinical data of 6 children undergoing laparoscopic liver tumor resection from June 2018 to March 2020 in the Affiliated Tumor Hospital, Zhengzhou University were retrospectively analyzed.Results:There were 4 males and 2 females among the 6 cases, with the average age of (7±2)years.All the children were admitted to the hospital with a liver occupying examination due to physical discomfort.Preoperative diagnosis: 3 cases of hepatoblastoma, 1 case of primary liver cancer, 1 case of hepatic adenoma and 1 case of hepatic nodular hyperplasia.All the children successfully completed laparoscopic liver tumor resection without conversion to open surgery.Among them, 3 children obtained left hemihepatectomy, 1 child underwent hepatic left lobectomy, 1 child was given VI hepatectomy, and 1 child accepted hepatic caudate lobectomy.The operation time was(90±9)min, and the average intraoperative blood loss was (83±26) mL.All children had no blood transfusion during or after operation.There were no bleeding, bile leakage, infection and liver failure after operation.All children had no gastric tube before surgery and the fluid diet was given on the first day after operation, and the postoperative median hospital stay was 4(3-5) days.The pathology were consis-tent with the preoperative diagnosis, and the resections were all radical operation resections.The follow-up period was from 2 to 23 months, and all the children recovered well and no recurrence was observed.Conclusions:Laparoscopic liver tumor resection in children is safe and feasible, which can minimize trauma, reduce intraoperative bleeding and shorten the hospital stay without increasing the incidence of postoperative complications.Laparoscopic liver tumor resection in children has certain advantages.

Objective:To investigate the application value of a new internal cold circula-tion bipolar radio frequency device in open hepatectomy.Methods:The retrospective cohort study was conducted. The clinical data of 85 patients with hepatocellular carcinoma who underwent open hepatectomy at the Affiliated Tumor Hospital of Zhengzhou University from February 2017 to January 2020 were collected. There were 48 males and 37 females, aged from 32 to 74 years, with a median age of 52 years. Of 85 patients, 45 cases undergoing hepatectomy assisted by the new internal cold circulation bipolar radio frequency device were allocated into new internal cold circulation bipolar radio frequency device group, and 40 cases undergoing hepatectomy assisted by Habib-4X bipolar radio frequency device were allocated into Habib-4X group, respectively. Observation indicators: (1) intraoperative situations; (2) postoperative situations; (3) follow-up. Follow-up was conducted by outpatient, inpatient reexamination and telephone interview to detect the postoperative complications and death of patient within postoperative 30 days up to May 2020. Measurement data with normal distribution were represented as Mean± SD, and independent sample t test was used for comparison between groups. Measurement data with skewed distribution were expressed as M(range), and nonparametric Mann-Whitney U test was used for comparison between groups. Count data were represented as absolute numbers, and comparison between groups was analyzed using the chi-square test or Fisher exact probability. Repeated measurement data were anlyzed by the repeated ANOVA. Results:(1) Intraoperative situations: the time of crosscutting process for liver parenchyma, average area transection speed, coagulation tissue width, cases with needle bleeding rate and electrode needle charring were (55±16)minutes, (4.8±1.2)cm 2/minute, (1.4±0.2)cm, 6, 10 for the new internal cold circulation bipolar radio frequency device group, versus (64±15)minutes, (3.6±1.0)cm 2/minute, (1.8±0.2)cm, 14, 25 for the Habib-4X group, respectively, showing significant differences in the above indicators between the two groups ( t=2.665, 4.973, 9.204, χ2=5.525, 14.184, P<0.05). (2) Postoperative situations: for the new internal cold circulation bipolar radio frequency device group, the total bilirubin (TBil) was (20±12)μmol/L, (25±12)μmol/L, (20±14)μmol/L at postoperative 1, 3, 7 days, the prothrombin time (PT) was (15.4±2.2)seconds, (14.2±2.1)seconds, (12.7±0.8)seconds, the alanine aminotransferase (ALT) was (288±248)IU/L, (132±61)IU/L, (67±32)IU/L, the aspartate aminotransferase (AST) was (279±114)IU/L, (50±22)IU/L, (30±13)IU/L. For the Habib-4X group, the TBil was 1(22±15)μmol/L, (23±10)μmol/L, (19±8)μmol/L at postoperative 1, 3, 7 days, PT was (15.8±2.8)seconds, (14.3±2.0)seconds, (13.6±1.3)seconds, the ALT was (369±269)IU/L, (133±99)IU/L, (54±30)IU/L, the AST was (345±125)IU/L, (60±36)IU/L, (32±11)IU/L. There were significant differences in the time effect of PT, ALT, AST between the two groups ( F=18.364, 23.020, 93.786, P<0.05). There was no significant difference in the time effect, between-group effect, interaction effect of TBil or between-group effect, interaction effect of PT, ALT, AST between the two groups ( F=2.421, 1.424, 0.522, 1.593, 0.312, 0.121, 0.267, 1.027, 0.600, P>0.05). (3) Follow-up: 85 patients were followed up for 4 to 39 months, with a median follow-up time of 16 months. There were 5 patients in the new internal cooling circulation bipolar radiofrequency device group and 8 patients in the Habib-4X group with complications, respectively, showing no significant difference in overall complications between the two groups ( χ2=1.292, P>0.05). The number of deaths in the new internal cooling circulation bipolar radio frequency device group and Habib-4X group was 0 and 1, respectively, showing no significant difference between the two groups ( P>0.05). Conclusion:The new internal cold-circulating bipolar radio frequency device for open hepatectomy is safe and effective, which cuts the liver parenchyma faster, has lower carbonization rate at the tip of electrode needle, and has more accurate coagulation range.

Objective:To investigate the clinical value of iodine density map and low keV virtual monoenergetic images (VMI) derived from dual-layer spectral detector CT (DLCT) for the pancreatic neuroendocrine tumors (pNETs) detection.Methods:From January to June 2019, data of 23 pathologically confirmed patients of pNETs were retrospectively analyzed. All of the patients underwent pancreas enhanced DLCT scanning within 1 week before surgery. The conventional polyenergetic images (PI), iodine density map and 40, 50, 60, 70 keV VMI were generated. One resident radiologist with 3 years’ experience and one senior radiologist with over 10 years’ experience interpreted the images for the lesion detection independently using the following image series: PI, VMI (40-70 keV), PI combined with iodine density map. Lesion detection rates were recorded and compared among different image series. The CT value and noise of lesion, normal pancreatic parenchyma, and abdominal subcutaneous fat were measured in PI and VMI in both arterial and portal vein phases. The contrast-to-noise ratio (CNR) of lesion was calculated. The CT value of lesion and normal pancreatic parenchyma, CNR of lesion, and image noise were compared using repeated one-way ANOVA test. Subjective image quality was assessed with a 5-point scale and compared with Friedman test.Results:A total of 26 lesions were confirmed from 23 patients. For resident radiologist and senior radiologist, the detection rates of pNETs lesion using PI were 76.9% (20/26) and 84.6% (22/26) respectively, and both improved to 92.3% (24/26) using image series of 40 and 50 keV VMI. For senior radiologist, the pNETs lesion detection rate was further improved to 96.2% (25/26) using image series of PI with iodine map. The CT value of lesion and normal pancreatic parenchyma, CNR, and image noise had statistical differences among PI and VMI (40-70 keV) in both arterial and portal vein phase ( P<0.001). The mean CT attenuation and CNR of lesion in VMI increased significantly as the energy level decreased.The CNR of lesion in all VMI (40-70 keV) was significantly higher than that in PI. The median of subjective scores of image quality in PI and VMI (40-70 keV) were 3, 3, 4, 4, and 5 respectively, and the difference was statistically significant (χ2=66.393, P<0.001). Conclusions:The low keV VMI derived from DLCT can increase the CT value and CNR of pNETs, and the lesion detection rate can be improved combined with iodine density map. The CNR of pNETs is the highest in 40 keV VMI, and image noise is still lower than that of PI, so 40 keV VMI is recommended for clinical application.

Objective:To explore the image quality of 40 keV virtual monoenergetic images (VMI) derived from dual-layer spectral detector CT （DLCT） pancreas dynamic enhanced scanning and its optimal window setting.Methods:From January to July 2019, 28 patients who underwent pancreas enhanced DLCT scan within one week before surgery and pathologically confirmed of pancreatic neuroendocrine tumors (pNETs) were retrospectively enrolled. Conventional polyenergetic images (PI) and 40 keV virtual monoenergetic images （VMI 40 keV） were generated after scanning.CT value of normal pancreatic parenchyma, lesion, abdominal subcutaneous fat, abdominal aorta and portal vein were measured in PI and VMI 40 keV. The contrast-to-noise ratio (CNR) of the pNETs lesion was calculated. All these objective results were compared between VMI 40 keV and PI using paired ttest. Individual window settings （W-Ind, including window width and window level） of VMI 40 keVwere recorded. Calculated window settings （W-Calc） were mathematically calculated via regression analysis and optimized window settings （W-Opt） were obtained.Subjective image quality was assessed with a 5-point scale and compared among VMI 40 keV with different window settings (W-Std, W-Ind, W-Calc and W-Opt) using Friedman test, and compared PI with standard abdominal window setting (W-Std) and VMI 40keV with W-Opt settings using Wilcoxon test.The maximum diameter of lesion was measured and compared with one-way ANOVA analysis among PI and VMI 40 keV with different windows settings. Results:For VMI 40 keV in both arterial phase and portal vein phase, the CT attenuation [(464.0±136.7), (375.4±79.2) HU] of pNETs lesion were statistically significantly higher than those in PI [（168.8±38.0), (140.5±23.5) HU] （ t=-16.107,-22.225, P<0.001）, CNR （16.5±11.1, 10.9±6.1） were also statistically significantly higher than those in PI （4.5±2.9, 3.0±1.9） （ t=-7.838, -9.781， P<0.001）,while with lower image noise in VMI 40 keV[（11.8±1.5),(11.8±1.4) HU] than PI （13.1±1.5,12.9±1.3 HU）（ t=6.356,3.891， P<0.001）. The subjective score for PI with W-Std and VMI 40 keV with W-Std, W-Ind, W-Calc, W-Opt in arterial phase were 4（1）, 1（0）, 5（0）, 5（0.75）, 5（1）, and which in portal vein phase were 3.5（1）, 1（0）, 5（0）, 5（0）, 5（1）.The subjective score of VMI 40 keV with different window settings had statistical differences （χ 2=76.143,76.000， P<0.001）. Compared to the image quality of PI with W-Std settings, VMI 40 keV with W-Opt settings have higher objective score （ Z=4.685, 4.235， P<0.001）. The maximum diameter of lesion has no statistical difference among PI and VMI 40 keV with different window settings （ F=0.008, 0.004， P>0.999） in both arterial phase and portal vein phase. Conclusions:The VMI 40 keV in pancreas dynamic enhancement scanning derived from dual-layer spectral detector CT have higher image quality than PI. Due to changes of CT value of tissue in VMI 40 keV, it is recommended to optimize window settings （window width/window level， 880/230 HU for arterial phase and 840/260 HU for portal vein phase） to obtain the best image quality.

Hepatocellular carcinoma (HCC) is mostly treated by surgery combined with interventional therapy, chemotherapy, radiotherapy and local treatment. However, it often relapses after the operation. And the prognosis of advanced patients is poor, and the effective rate of chemotherapy and molecular targeted therapy is low. Recent studies indicated that the occurrence and progress of HCC are closely related to the immune function of the body. Immunotherapy, as a new type of tumor therapy, has drawn much attention. By improving the immunogenicity of tumor cells and the killing sensitivity of effector cells, it stimulates the immune function of the body, prolongs the progression-free survival time and the overall survival time of patients, and becomes an integral part of the comprehensive treatment of liver cancer. This article reviewd the progress in immunotherapy of HCC in the recent years.

Objective To explore the feasibility of the virtual non-contrast images derived from dual-layer spectral detector CT (SDCT) substitute for true non-contrast images.Methods From July 2017 to September 2017,40 patients under-went pre-and arterial-venous dual-phase post-contrast abdominal imagining on a SDCT in Shanghai Jiaotong University School of Medicine Affiliated Ruijin Hospital.The images were retrospectively analyzed.The arterial VNC images (VNC-a) and venous VNC images (VNC-v)were derived from spectral based image (SBI) datasets of arterial and portal venous phase using a dedicated software respectively.Then mean CT attenuation,mean image noise,signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were compared with one-way ANOVA analysis among TNC,VNC-a,VNC-v images in liver,spleen,pancreas,abdominal aorta,inferior vena cava,psoas muscle,L4 vertebra and perirenal fat.And Bland-Altman plots was used to analyze the CT attenuation equivalence between TNC and VNC.Subjective image quality was assessed with a 5-point scale and compared with Friedman H test.The dose length product (DLP) of pre-and post-contrast scans were recorded.Results The CT attenuation of abdominal aorta,perirenal fat,L4.vertebra among three kinds of images had significant differences (P＜0.05)which overestimated the CT attenuation of perirenal fat and underestimated CT attenuation of abdominal aorta and vertebra compared to TNC,VNC.Except for pancreas,the image noise of all other tissues among three kinds of images had significant differences,images noise of VNC images were lower than TNC images (P＜0.05).The SNR of liver,spleen,psoas muscle and inferior vena cava and CNR of pancreas,abdominal aorta,inferior vena cava,L4 vertebra and perirenal fat were statistically significant among the three images (P＜0.05).Except for vertebra and perirenal fat,good equivalence between VNC and TNC images were observed in all relevant tissues with Bland-Altman analysis.Image quality subjective scoring of TNC,VNC-a,and VNC-v were 5.00(1.00),5.00(0.75),5.00(1.00) respectively which had no significant differences (P=0.20).The DLP of TNC,arterial and venous phase scan were (255.2±62.0),(258.9±62.9),(252.0±61.2)mGy·cm,and the total DLP of dual-phase contrast enhanced scan was (766.2± 185.3) mGy· cm.The DLP of TNC accounted for 33.3％(255.2/766.2) of the total DLP,and effective dose of TNC was (3.83±0.90)mSv.Conclusions CT attenuation in VNC image which exhibited lower image noise was identical to TNC images in the majority of abdomen tissues except for vertebra and perirenal fat.and the VNC image.The VNC image which reduces radiation dosage derives from SDCT enhanced image might be used as a substitute for the TNC image.

Objective To explore the clinical efficacy of internal fixation with dynamic anterior plate-screw system for quadrilateral area(DAPSQ) in the treatment of complex acetabular fractures involving the quadrilateral surface.Methods Between January 2005 and January 2017,a series of 143 patients with complex quadrilateral surface fracture of the acetabulum were treated at Department of Orthopaedics,General Hospital of Central Theater Command.They were 86 males 57 females,with a mean age 46.6 years (range,from 19 to 77 years).By the Letournel-Judet classification,there were 38 anterior column plus posterior hemitransverse fractures,26 T-shaped fractures and 79 double column fractures.They were all closed.The delay from injury to surgery averaged 9.6 days(range,from 3 to 21 days).All the cases were treated with DAPSQ internal fixation via the ilioinguinal approach.Their operation time,intraoperative bleeding volume,quality of fracture reduction,hip function and complications were recorded.Results The operation time for this series ranged from 2.5 to 5.0 hours,averaging 3.8 hours.The intraoperative blood bleeding ranged from 400 to 2,500 mL,with an average of 680 mL.By the Matta criteria for fracture reduction,76 cases were rated as excellent,51 as good and 16 as poor,giving an excellent and good rate of 88.8％.The 143 patients were followed up from 4 months to 10 years (mean,4.1 years).The clinical fracture union was achieved after 2 to 4 months (mean,3 months).By the modified Merle d'Aubigné & Postel criteria,the affected hips scored 9 to 18 points (mean,16.3 points) at the final follow-ups,with 64 excellent,52 good,19 fair and 8 poor cases,giving an excellent and good rate of 81.1％.The postoperative complications included lesion of lateral femoral cutaneous nerve in 7 cases,urinary tract infection in 15 ones,traumatic osteoarthritis in 20 ones 5 of whom had to receive total hip arthroplasty,and heterotopic ossification (Brooker Grade Ⅰ) in 5 ones.Conclusion Internal fixation with DAPSQ is safe and effective for the treatment of complex acetabular fractures involving the quadrilateral surface.

Objective: To investigate the safety, efficacy and the key points of ultrasound-guided percutaneous radiofrequency ablation (RFA) in treatment of caudate lobe liver tumor. Methods: The clinical data of 30 patients with caudate lobe liver tumor (57 lesions, 33 caudate lobe lesions) in the Affiliated Tumor Hospital of Zhengzhou University from January 2017 to December 2018 were retrospectively analyzed. All the patients were treated with ultrasound-guided percutaneous radiofrequency ablation. The puncture approach and single-needle or double-needle radiofrequency ablation were chosen according to the lesion location. The efficacy after treatment was evaluated according to the imaging examination. The deadline of follow-up was January 31, 2019, and the median follow-up time was 10 (1 to 20) months. After ablation, CT/MR was performed to evaluate whether the lesions were completely ablated. The effect of treatment and short-term complication were recorded. Results: Thirty patients were successfully treated with ultrasound-guided percutaneous radiofrequency ablation. Among the 33 caudate lobe lesions, complete ablation rate at one time was 81.8% (27/33); local tumor progression rate after treatment was 12.1% (4/33), and the median time was 2.5 months. The new lesion rate was 26.7% (8/30), and the median time was 3.0 months. Two patients had significant abdominal pain, 1 patient had fever, 1 patient had hypoxemia during the ablation, and total incidence of complication was 13.3% (4/30). There was no bile leakage, gastrointestinal tract injury, uncontrollable bleeding and other serious complications and related deaths. Conclusions For the caudate lobe liver tumor, ultrasound-guided percutaneous radiofrequency ablation is safe and effective. It is necessary to select the appropriate puncture approach, avoid the intrahepatic duct reasonably and master the key points of ablation.