Objective To analyze the evaluation value of the standard deviation of erythrocyte volume distribution width(RDW-SD),erythrocyte volume distribution width standard deviation and platelet ratio(RPR)and erythrocyte volume distribution width standard deviation and lymphocyte ratio(RLR)in the de-compensation stage of cirrhosis in primary biliary cholangitis(PBC).Methods The blood routine indexes of 68 patients with PBC admitted and treated in this hospital from January 2019 to June 2021 were retrospective-ly analyzed and divided into the compensation stage(n=36)and decompensation stage(n=32)according to the diagnostic standard.2 mL venous blood was extracted from the patient on an empty stomach in the early morning.The red blood cell(RBC),mean corpuscular volume(MCV),hemoglobin(Hb),hematocrit(HCT),mean erythrocyte hemoglobin content(MCHC),RDW-SD,white blood cell(WBC),neutrophil absolute value(N#),lymphocyte absolute value(L#),platelet count(PLT),mean platelet volume(MPV),platelet volume distribution width(PDW),etc.were detected.The platelet to lymphocyte ratio(PLR),RPR and RLR were calculated.The influencing factors of decompensation stage of PBC cirrhosis were analyzed by binary logistic regression,and the receiver operating characteristic(ROC)curve was used to analyze the diagnostic values of different indicators in the decompensation stage of PBC cirrhosis.Results There were statistically significant differences in age,RBC,Hb,HCT,RDW-SD,L #,PLT,RPR and RLR between the compensation group and decompensation group in PBC cirrhosis(P＜0.05).The binary logistic regression analysis showed that the age[odds ratios(OR)=1.087,95％confidence intervals(CI):1.015-1.165,P＜0.05],RDW-SD(OR=1.144,95％CI:1.030-1.270,P＜0.05)and RLR(OR=1.041,95％CI:1.007-1.075,P＜0.05)were the independent risk factors for progressing to the decompensation stage in the patients with PBC cirrhosis com-pensation stage.The ROC curve analysis showed that the areas under ROC curve(AUC)of RDW-SD,RPR and RLR for the diagnosis alone of decompensation stage of PBC cirrhosis were 0.726,0.778 and 0.798,re-spectively,and the differences were not statistically significant(P＞0.05).Conclusion Combined with the age factor,regular monitoring of RDW-SD,RPR and RLR levels has a high predictive value for the develop-ment of PBC cirrhosis compensation stage to decompensation stage.

Objective:To summarize the clinical and genetic characteristics, treatment and prognosis of four children with Steroid-resistant nephrotic syndrome (SRNS) due to variants of TRPC6 gene. Methods:Clinical data of four children with SRNS admitted to Children′s Hospital Affiliated to Zhengzhou University between May 2020 and August 2022 were collected. Peripheral blood samples were collected from the children and their parents, and whole exome sequencing was carried out. Sanger sequencing was used to verify the pathogenicity of the candidate variants among the children and their parents.Results:All of the four children were found to harbor heterozygous variants of the TRPC6 gene, including c. 523C>T (p.R175W), c. 1327T>A (p.F443I), c. 430G>C (p.E144Q) (unreported previously), and c. 523C>T (p.R175W), which were all missense variants. Two of the children have shown a simple type, whilst two have shown a nephritis type, none had extrarenal phenotype. Comprehensive renal pathology of three children revealed focal segmental glomerulosclerosis (FSGS). Two children were treated with steroids combined with calcineurin inhibitors (CNIs), among whom one showed significant improvement in symptoms. Conclusion:Discoveries of the novel c. 430G>C variant and the new SRNS phenotype of the c. 1327T>A variant have expanded the mutational and phenotypic spectrum of the TRPC6 gene, which has provided a reference for clinical diagnosis and genetic counseling for the families.

Objective:To analyze the influencing factors of low liver regeneration in patients with hilar cholangiocarcinoma (HCCA) after portal vein embolization (PVE).Method:Clinical data of 62 patients with HCCA undergoing PVE at Henan Provincial People's Hospital (People's Hospital of Zhengzhou University) from January 2019 to March 2024 were retrospectively analyzed, including 33 males and 29 females, aged (59.1±10.3) years. Patients were divided into two groups based on the median regeneration rate of remnant liver volume (28.6%) three weeks after PVE: low regeneration ( n=31, <28.6%) and high regeneration group ( n=31, ≥28.6%). The proportion of lymph node metastasis, history of alcohol consumption, liver fibrosis, biliary tract infection, alkaline phosphatase (ALP), and tumor necrosis factor-α (TNF-α) were compared between two groups. Multivariate logistic regression analysis was used to indentify the influencing factors of low liver regeneration in patients with HCCA after PVE surgery. Results:The proportion of lymph node metastasis, history of alcohol consumption, liver fibrosis, biliary tract infection, ALP, and level of TNF-α were higher in the low regeneration group than those in the high regeneration group (all P<0.05). Multivariate logistic regression analysis showed that patients with regional lymph node metastasis ( OR=2.561, 95% CI: 1.265-5.185), history of alcohol consumption ( OR=2.616, 95% CI: 1.321-5.181), liver fibrosis ( OR=2.351, 95% CI: 1.265-4.369), biliary tract infection ( OR=2.461, 95% CI: 1.226-4.940), elevated level of ALP ( OR=2.687, 95% CI: 1.351-5.344), and elevated level of TNF-α ( OR=2.781, 95% CI: 1.452-5.326) had an increased risk of low liver regeneration after PVE (all P<0.05). Conclusion:Regional lymph node metastasis, history of alcohol consumption, liver fibrosis, biliary tract infection, and elevated ALP and TNF-α are risk factors for low liver regeneration in patients with HCCA after PVE surgery, which should be noted in clinical practice.

The data of a patient with autosomal dominant optic atrophy (ADOA) and chronic renal insufficiency caused by SSBP1 gene mutation in the Children′s Hospital Affiliated to Zhengzhou University in July 2021 was analyzed retrospectively.Literature was reviewed.The patient was a 10-year-old girl, who visited the hospital due to " growth retardation for the past 3 years and elevated serum creatinine (Scr) for the past 2 years" . On admission, the patient′s height was 130 cm (<10 th percentile of the same sex of healthy age) and her weight was 22 kg (<3 rd precentile of the same sex of healthy age). Lab examination showed that the level of blood urea nitrogen (BUN) was 16.3 mmol/L, Scr was 115.4 μmol/L, and the estimated glomerular filtration rate was 41 mL/(min·1.73 m 2). The patient was complicated with metabolic acidosis and mild anemia.Imaging findings showed small volume of both kidneys, increased background parenchymal enhancement, scattered spot-like hyperechoes and unclear boundary between the cortex and medulla.Additionally, the patient had a history of optic atrophy.Both the father and mother of the patient had no related phenotypes.The genetic test of the patient showed that c. 320G>A (p.R107Q) was a heterozygous missense mutation, which was spontaneous.A total of 5 English papers were retrieved.There were 8 kinds of SSBP1 gene mutations reported, including 7 heterozygous missense mutations [c.320G>A (p.Arg107Gln), c.119G>T (p.Gly40Val), c.331G>C (p.Glu111Gln), c.184A>G (p.Asn62Asp), c.113G>A (p.Arg38Gln), c.422G>A (p.Ser141Asn), c.79G>A (p.Glu27Lys)] and 1 homozygous mutation [c.394A>G (p.Ile132Val)]. Studies have established that almost all patients carrying SSBP1 mutations have manifestations of eye involvement, and that some patients are complicated with progressive deterioration of renal function, sensorineural deafness, growth retardation, and hypothyroidism.It suggests that SSBP1 gene mutation can cause ADOA.For patients with optic atrophy, whether they are complicated with hearing loss and growth retardation, renal morphology and renal function evaluation are recommended.Early genetic examination is helpful for diagnosis and treatment.

Objective:To investigate the efficacy and safety of plasma exchange(PE) in the treatment of autoimmune hemolytic anemia in children.Methods:The data from 8 hospitals in China during November 2014 to April 2017 were collected, and the clinical characteristics of PE in children with AHA were analyzed retrospectively.Results:A total of 21 children with AHA were included in the study, including 17 cases from PICU and 4 cases from pediatric kidney ward, with 11 boys and 10 girls, and the median age was 3.64(0.25, 11.10)years old, and median hospital stay was 12(4, 45)days.There were 15 cases(71.4%) with infection, 2 cases(9.5%)with autoimmune diseases, 4 cases(19.0%) with unknown.Consciousness disturbance occurred in 4 patients before replacement and recovered to normal after PE.The volume of blood decreased in two cases(9.5%) and completely relieved.There were 20 cases of anemia (95.2%), 15 cases were normal after PE, and 5 cases were improved.Jaundice occurred in 18 cases (85.7%), 12 cases were normal after PE, 6 cases were improved.Hepatosplenomegaly was found in 11 cases, 10 cases were normal after PE, 1 case was improved.After PE, the hemoglobin and red blood cell count increased, while the total bilirubin, indirect bilirubin, urea nitrogen and lactate dehydrogenase decreased, there were significant differences between pre-and post-replacement ( P<0.05). Only 1 case had allergic reaction, which was improved after symptomatic treatment, and PE was continued.After PE, 2 cases (9.5%) had complete remission, 16 cases (76.2%) had partial remission and 3 cases (14.3%) had been discharged. Conclusion:PE therapy can obviously improve the clinical symptoms and laboratory indexes of children with AHA who have failed to respond to conservative treatment.It can be used as a treatment measure for children with severe AHA and has a good safety.

Objective:To explore the association between left-behind status, screen time (ST) and behavior of autism in rural preschool children.Methods:Cross section study was used in this study.A sample of 3 636 rural preschool children aged from 3 to 6 years old in 26 kindergartens were selected from four counties in Anhui province of China.The contents of the questionnaire include: basic information questionnaire, self-made left behind status questionnaire, self-made screen time questionnaire, Clancy autism behavior scale.EpiData 3.2 and SPSS 23.0 software were used for data entry and statistical analysis.Chi-square test was used to analyze the difference of positive rate of autism behavior.Logistic regression analysis was used to further explore the relationship between left-behind status, screen time and autism behavior of rural preschool children.Results:Compared with non-left-behind children (NLBC), the risk of autism behavior for left-behind children (LBC) increased 36%.The risk of autism behavior increased by 40% for 1 h/d 2 h/d when compared with ST ≤ 1 h/d.While comparing to NLBC with ST ≤ 1 h/d, the risk of autism behavior increased by 97% in LBC with 1 h/d 2 h/d.Conclusions:There is an additive effect on the risk of autism behavior when left-behind experience and excessive ST combined together.The daily ST should be strictly controlled within 2 h/d for NLBC, and within 1 h/d for LBC in order to reduce the risk of autism behavior in preschool children of rural areas.

Objective:To customize the individualized 3D printed head film for patients with head tumors undergoing radiotherapy, and to evaluate the physical properties of the material and the precision of this technology compared with the thermoplastic head film.Methods:The 3D printed head film and thermoplastic head film were placed on the solid water surface, and the depth and surface dose were measured at 5 cm by ionization chamber and film, respectively. Thirty patients with head tumors receiving radiotherapy were randomly divided into the control and experimental groups. The patients were fixed with thermoplastic head film and 3D printed head film. The translational and rotational errors in the x, y and z direction were obtained by CBCT.Results:The radiation attenuation rate of two materials at the depth of 5 cm was less than 1%. The dose of thermoplastic head film in the surface position was increased by 27%, and increased by 18% in the 3D printed head film. In two groups, 116 sets of setup errors were collected. The average translational setup errors in the control and experimental groups were 1.29 mm and 1.16 mm, 1.42 mm and 1.24 mm, 1.38 mm and 1.16 mm, respectively, and the average rotational setup errors were 1.29°and 1.08°, 1.02°and 0.96°, 1.01°and 1.00°, respectively. The translational setup errors in the y and z direction and rotational setup errors in the x direction significantly differed between the control and experimental groups (all P<0.05), but no statistical significance was found in the other direction (all P>0.05). Conclusion:The 3D printed head film fixation meets the precise setup requirements of modern radiotherapy, which deserves further application in clinical trials.

Objective:To investigate effects of bone-resorptive lesion on stress distribution of femoral head and on progression in patients with osteonecrosis of the femoral head (ONFH).Methods:From April 2014 to September 2018, a total of 155 femoral heads from 94 patients diagnosed with ARCO stage II and III ONFH were retrospectively reviewed, including 77 males and 17 females with aged 39.90±10.45 years old (ranged from 18-64 years). The hips were divided into two groups according to whether there were bone-resorptive lesions. Further, we compared whether there was statistical difference between the two groups in staging. Then, a case of ARCO II hip joint without bone-resorptive lesion was selected from the included patients. Six femoral head with different diameters of spherical bone-resorptive lesion of 5 mm, 7 mm, 10 mm, 14 mm, 18 mm, and 23 mm were simulated. The influence of bone-resorptive lesion on the stress distribution of necrotic area and a spherical shell extending 1 mm radially around the bone-resorptive lesion was investigated by finite element method in slow walking conditions.Results:Of the 155 ONFH hips, 67 hips are complicated by bone-resorptive lesions, of which 17 were ARCO II, 50 were ARCO III. A total of 88 hips did not contain bone-resorptive lesions, of which 58 were ARCO II, ARCO III 30 cases. The proportion of ARCO stage II in the group with bone-resorptive lesions was significantly higher than that in the group without bone-resorptive lesions (χ 2=25.03, P=0.000). The finite element stress distribution cloud diagram showed that there was a stress concentration area around the bone-resorptive lesions. The maximum von Mises stress around bone-resorptive lesions in the models that contained a synthetic bone-resorptive lesions were significantly higher than those reported in the matched, non-synthetic bone-resorptive lesions finite element models ( t=3.139, P=0.026). The values for maximum von Mises stress around bone-resorptive lesions were 6.94±1.78 MPa and 5.01±0.35 MPa for the group with synthetic bone-resorptive lesions and the group non-synthetic bone-resorptive lesions, respectively. There was a positive correlation between the diameter of bone-resorptive lesions and the maximum and mean von Mises stress of necrotic areas as well as the maximum von Mises stress around bone-resorptive lesions. Conclusion:Bone-resorptive lesions can increase the maximum stress and average stress in the necrotic area. The larger the bone-resorptive lesion, the more the stress increases. There is a stress concentration area around the bone-resorptive lesions, which may accelerate the collapse of the femoral head.

Objective: To investigate the effect of Utrecht applicator on dosimetrics of targets and OARs in brachytherapy for advanced cervical cancer. Methods: Data of patients with locally advanced cervical cancer who received brachytherapy with Utrecht Source Applicator from 2017 to 2018 in the Department of Radiotherapy, Cangzhou Hospital of Integrated Traditional Chinese and Western Medicine, Hebei province, were collected. The original plan was intracavitary /interstitial brachytherapy (IC/IS-BT). With the original contour and prescription dose unchanged, the implantation was removed and new intracavitary brachytherapy (IC) plan was designed. The dosimetric parameters of IC/IS plan and IC plan were compared and analyzed, and the effect of IS implantation on dose was evaluated. Results: The D₉₀ of HR-CTV was (88.68±1.84) Gy in IC/IS group and (85.54±0.54) Gy in IC group, with significant difference(t=6.200, P<0.05). In addition, conformity index of the IC/IS plan was significantly higher than that of the IC plan (IC/IS: 0.58±0.08, IC: 0.43±0.05; t=1.010, P<0.05). In terms of OAR, there was no significant difference in dosage between the two groups (P>0.05). Conclusions For the brachytherapy of locally advanced cervical cancer, the use of Utrecht Source Applicator can satisfy the prescription dose coverage. Adding IS insertion needle can improve the optimization space of the plan. Without exceeding the dose limit of OAR, the radiation dose to HR-CTV can be significantly increased, and the uniformity of dose distribution can be improved as well.

Objective: To compare the clinical effects between laparoscopic cholecystectomy and choledochotomy versus traditional open cholecystectomy plus choledochotomy. Methods: One hundred and sixty-eight elderly patients with gallbladder and choledocholithiasis were divided into a laparoscopy group(n=75, receiving laparoscopic cholecystectomy and choledochotomy)and an open abdominal group(n=93, undergoing traditional open cholecystectomy and common bile duct exploration). The surgical incision length, operation time, intraoperative blood loss, anal exhaust time, hospital stay and postoperative complications were compared between the two groups. Results: The surgical incision length, operation time, intraoperative blood loss, anal exhaust time, hospital stay were lower in the laparoscopic group than in the open abdominal group(P<0.05). The incidence of postoperative complications was lower in the laparoscopic group than in the open group(P<0.05). Conclusions Laparoscopic cholecystectomy and choledochotomy can obviously improve the clinical efficacy and reduce complications in elderly patients with gallbladder and choledocholithiasis, and it is worthy of clinical application.