Objective:To evaluate the accuracy and feasibility of sentinel lymph node biopsy (SLNB) marked by 99Tcm-IT-Ritux-imab and to discuss the clinical value of the method in diagnosis and treatment of cutaneous melanoma. Methods:A total of 67 patients with cutaneous malignant melanoma received 99Tcm-IT-Rituximab-tagged SLNB from March 2008 to March 2012. Lymphoscintigra-phy was conducted 30 min to 1 h after intra-dermal injection of 99Tcm-IT-Rituximab. Subsequently, the surgery of SLNB was carried out using gamma probe. The detection and positive rates of SLNB were counted. The relationship between the status and the clinical features of the sentinel lymph node (SLN) was analyzed, such as the T stage, ulceration, age, gender, and location. The influence of SLN status on overall survival (OS) and disease-free survival (DFS) was evaluated. Results:SLNs were detected in all the 67 patients by SPECT and gamma detector, with detection rate of 100%. Fifteen patients had SLN metastasis, and the positive rate was 22.4%. Chi-square indicates that SLN metastasis is associated with age, T stage, and ulceration (P<0.05). A total of 63 patients were followed up for 24-69 months, and the median follow up time was 43 months. Kaplan-Meier survival analysis shows that both OS and DFS in the SLN-negative group are better than those in the SLN-positive group (OS:93.9%vs. 57.1%, P<0.01;DFS:79.6%versus 28.6%, P<0.01). Cox-regression multiple factors analysis suggests that both SLN status and T stage are independent factors that affect the DFS of malignant melanoma. Conclusion:SLNB assisted by 99Tcm-IT-Rituximab can well reflect the state of lymph node metastasis and is es-sential for accurate staging, prognosis judging, and treatment guiding. Its operation procedure is simple with high accuracy, and the im-aging status is stable. Therefore, it is convenient and feasible as a means of SLNB.

Objective:To explore the outcome of soft tissue sarcoma (STS) on patients with soft tissue metastasis. Methods:We ana-lyzed 25 STS patients with soft tissue metastasis primarily localized on extremity and trunk. The study was conducted from June 2010 to June 2016 by retrospective analysis of the clinical and pathological characteristics of the patients. The assessed endpoints were overall survival. Results:Six patients (24%) had synchronous soft tissue metastasis, and 19 patients (76%) had metachronous metasta-sis. The average time for primary tumor recession of metastatic lesions was 45.3 months. Metastases were most common in parts of the trunk in 18 patients (72%), followed by the head and neck in 5 patients (20%). Eleven patients (44%) with lung metastasis had poor prognosis. Conclusion:STS occurred more rarely in soft tissue metastasis than in pulmonary metastasis. Neoadjuvant chemotherapy and surgical treatment were the major therapies employed. Targeted therapy as a new treatment rendered good results.

Objective To analyze the normal reference values of the right ventricular volumes and ejection fraction of healthy adults measured by single-beat real-time three-dimensional echocardiography (sRT-3DE).Methods The subjects of this study included three hundred and thirty-four healthy adults,and were divided into 2 groups by gender and 6 groups by age.The sRT-3DE was chosen to measure their right ventricular volumes and ejection fraction.The parameters of right ventricular volumes were standardized by body surface area (BSA)and lean body mass (LBM)to calculate the ranges of their normal value.The correlation between these parameters and genders as well as the physical characteristics (height,weight, body mass index,BSA and LBM)was also clarified.Results Three hundred and thirty-four (96%)of the 348 subjects had been tested.Before standardizing,men had larger right ventricular volumes compared with women,and the differences of the right ventricular end diastolic volume (RVEDV),right ventricular end systolic volume (RVESV)and right ventricle stroke volume (RVSV)were statistically significant(P <0.001 ),but it had no statistical significance in the difference of the right ventricular ejection fraction (RVEF)(P >0.05 ).After standardizing by LBM,the RVEDV,RVESV and RVSV in male group were significantly lower than those in women group (P < 0.001 ).In different age groups,the difference of RVEDV,RVESV,RVSV ,but no RVEF were statistically significant (P < 0.05 ).Positive correlations between the RVEDV,RVESV,RVSV and the BSA,height,weight,body mass index and LBM had been detected,and it showed a negative relation between RVEDV,RVESV,RVSV with age.Noticeably,RVEF had no correlation with them.Conclusions sRT-3DE can provide a preliminary range for normal reference values of right ventricular volumes and RVEF in healthy adults according to gender and age and finds that LBM has certain impact on adult cardiac structure,which offers valuable evidence for the diagnosis of right heart diseases in clinical practices.

Objective:To analyze the clinical prognostic factors of liposarcoma on the extremities and trunk, as well as to retrospectively analyze the effect of adjuvant radiotherapy and chemotherapy on liposarcoma of the extremities. Methods:Patients with liposarcoma of the extremities treated in our hospital from July 1, 2007 to December 31, 2012 were followed up. The relationship of clinical prognostic factors with gender, age, location, depth, and size of the tumors, as well as the histological grade and admission status, were statistically analyzed. The effects of adjuvant radiotherapy or chemotherapy on overall survival (OS) and disease free survival (DFS) were evaluated. Results:A total of 82 patients with extremity liposarcoma received surgery-based comprehensive treatment in our hospital. Of the total patients, 73 received a 24-month to 88-month satisfied follow-up;the median follow-up time was 47 months. The OS rate was 83.6%(61/73), and the DFS rate was 68.5%(50/73). Multivariate Cox regression analysis showed that the tumor location, histological grade, and admission status were the independent correlative factors influencing DFS, and the age and pathologic grading were the independent correlative factors influencing the OS. Kaplan-Meier survival analysis showed that radiation therapy can significantly improve the DFS and OS of the G2 and G3-grade liposarcoma (DFS:59.1 months vs. 28.4 months, P<0.01;OS:70.8 months vs. 55.1 months, P<0.05). Significant difference was not found in the effect of chemotherapy on OS and DFS. Conclusion:The prognosis of liposarcoma was significantly associated with the pathologic grades and subtypes. Auxiliary radiotherapy could improve the survival and prognosis of G2 and G3 liposarcoma of the extremities, but the role of chemotherapy in treating liposarcoma remained unclear.

The data of a child with early-onset epileptic encephalopathy in Qilu Children′s Hospital of Shandong University in February 2020 were analyzed retrospectively.The child was a 4-month-old girl, who was admitted to the hospital because of " repeated convulsions for 4 months and feeding difficulty for 1 month" at the age of 4 months.The patient suffered from epilepsy 1 day after birth, and the epilepsy type was tonic seizures.Severe developmental retardation was observed in the patient.Electroencephalogram showed multifocal discharge, which then turned to hypsarrhythmia.The cranial imaging was negative.Feeding difficulty occurred at the age of 3 months.The genetic testing revealed a de novo heterozygous missense mutation in the FGF12 gene (Arg114His). Various antiepileptic drugs and ketogenic diet were ineffective.There was no attack in 2 months after adding Phenytoin.The child could eat on her own after seizure control, but there was no progress in intellectual and motor development.Mutations in the FGF12 gene lead to poor prognosis of early-onset epileptic encephalopathy, and the seizures are difficult to control.Sodium ion channel blockers such as Phenytoin should be used as soon as possible.

Objective:To investigate the pathogenic distribution of benign infantile convulsions with mild gastroenteritis(BICE)and explore the relevance of serum cytokines and BICE.Methods:Eighty BICE infants admitted in Inner Mongolia Maternal and Child Health Hospital from January 2017 to December 2019 were selected as BICE group, and 80 mild gastroenteritis infants without convulsion attack were selected as control group during the same period.Fluorometric real-time PCR was used to detect the pathogen of enterovirus.Serum cytokines including interleukin(IL)-6, tumor necrosis factor(TNF)-α, IL-8 and soluble interleukin-2 receptor(SIL-2R)were detected by using chemiluminescence method.The relevance of frequency and duration of convulsion in infants with BICE and the indicators above were analyzed.Results:Rotavirus infection was the main cause of BICE during the study period in this region.Among 80 cases in the BICE group, rotavirus positive infants accounted for 38.8%(31/80) and norovirus positive infants accounted for 10.0%(8/80). The levels of IL-6, IL-8 and SIL-2R in the BICE group were prominently higher than those in the control group( P<0.05); the difference of TNF-α level between two groups had no statistical significance( P>0.05). The levels of IL-6, TNF -α, IL-8, SIL-2R in the group with convulsion attack times ≥ 2 and convulsion duration ≥5 min were higher than those in the group with convulsion attack times<2 and convulsion duration<5 min( P<0.05). The frequency and duration of convulsions were positively correlated with the levels of IL-6, TNF -α, IL-8 and SIL-2R( P<0.05). Conclusion:Rotavirus is the main pathogen of BICE in this region.There is immunologic imbalance in children with BICE, especially the changes of IL-6 and SIL-2R levels, which may provide effective cytological and experimental data evidence for judging progression and prognosis of the disease.

Objective To investigate the effectiveness and safety of balloon dilation technique when difficult ureteroscope accessing in ureteroscopic holmium laser lithotripsy in children.Methods Clinical data of 23 patients(25 sides)receiving ureteroscopic holmium laser lithotripsy with using balloon dilatation technique from March 2020 to October 2023 were retrospectively analyzed.When it was difficult to insert ureteroscope,the ureteral balloon catheter was retrogradely inserted through the ureteroscopic working channel to dilate the narrow part of the ureteral lumen.After dilation,the holmium laser fiber was inserted to powder the stones.Results Of the 23 patients(25 sides)with ureteral calculi,transcatheter ureteral balloon dilation was carried out in 26 places during the operation,including 23 places located in the inner part of the ureteral bladder wall and 3 places located in the lower part of the ureter.The extent of ureteral injury at dilated sites was grade Ⅰ in 23 places and grade Ⅱ in 3 places,respectively.After ureteral balloon dilatation,continued lithotripsy succeeded on 23 sides and failed on 2 sides.No complications related to ureteral dilated balloon catheter were found.The time for removing double-J ureteral stents was 26-45 d(mean,33.5±5.2 d),during which urinary tract infection occurred in 5 cases.Urinary system ultrasound examination was performed within 2 weeks after removal of double-J ureteral stents.The calculi were discharged in all the children,without hydronephrosis.No ureteral stenosis was observed in all the children during 6-24 months(mean,13.7 months)of postoperative follow-ups.Conclusion It is safe and effective to dilate the ureteral with balloon dilation technique through the working channel when difficult ureteroscope accessing.

OBJECTIVE: To explore the genetic cause for a child with growth retardation by next generation sequencing (NGS). METHODS: Clinical data of the patient was collected. Peripheral venous blood samples were taken from the neonate and his parents. Targeted capturing and NGS were carried out to detect mutations of genes associated with inborn errors of metabolism. Suspected mutations were validated by Sanger sequencing. RESULTS: The 15-month-old female patient was admitted to hospital for growth retardation for 4 months. Hypomyelination was found upon cranium MRI. Genetic testing revealed two novel insertional mutations in the GLB1 gene in the patient, namely c.2006-2007insT and c.475-476 insGGTCC. CONCLUSION The c.2006-2007insT and c.475-476 insGGTCC mutations of the GLB1 gene probably underlie the GM1 gangliosidosis resulting in the growth retardation in the child.
Female ; Gangliosidosis, GM1 ; genetics ; Humans ; Infant ; Infant, Newborn ; Mutation ; Pedigree ; beta-Galactosidase ; genetics

Objective To evaluate the efficacy of pegylated interferon ( PegIFN ) α-2a plus ribavirin ( RBV) therapy for chronic hepatitis C ( CHC) in non-responders, and to investigate the related influencing factors.Methods A prospective, open, multicenter and randomized study was conducted.A total of 81 CHC non-responders were recruited from 10 clinical centers during February 2009 to November 2011.Patients were randomly assigned into two groups:group A (n=37) was given PegIFNα-2a plus RBV treatment for 72 weeks and group B (n=44) was given PegIFNα-2a plus RBV treatment for 96 weeks.Both groups were followed up for 24 weeks after treatment.Virological responses in two groups were observed, and treatment efficacies among patients with different genotypes, and among those with different previous treatment were compared.SAS software was used for statistical analysis.Results Fifty-two patients ( 28 from group A and 24 from group B) completed the study in total.The rates of rapid virological response ( RVR) , complete early virological response ( cEVR ) , end of treatment viral response ( ETVR ) and sustained virological response (SVR) in group A were 25.0% (7/28), 60.7% (17/28), 67.9%(19/28) and 60.7%(17/28), respectively; while those in the group B were 41.7% (10/24), 70.8%(17/24), 70.8%(17/24) and 70.8% (17/24), respectively; and there were no significant differences between two groups (P>0.05).SVR was observed in 82.9%(29/35) of patients with CC genotype of IL-28B, which was higher than that in patients with other genotypes ( 3/13 ) , and the difference was of statistical significance (P<0.01).There was no significant difference in viral responses between patients previously treated with IFN plus RBV and those treated by IFN only (P>0.05).The rates of RVR, cEVR, ETVR and SVR in patients who were previously treated with IFN were 36.4%(12/33), 81.8%(27/33), 81.8%(27/33) and 75.8%(25/33), and the rates of cEVR, ETVR and SVR were higher than those in patients who were previously treated with PegIFN (P<0.05), but no significant difference was observed in RVR (P>0.05).Adverse events occurred in 38 patients (46.9%), but no severe ones were observed. Conclusion The efficacy of PegIFNα-2a plus RBV therapy for CHC in non-responders is satisfactory, which may influenced by IL-28B genotypes and previous treatment.

Objective:To retrospectively analyze the incidence, mortality, clinical characteristics and related factors of transient and persistent acute kidney injury in patients with sepsis in emergency department.Methods:Patients with sepsis ≥ 18 years old hospitalized in the Emergency Department of Guangdong Provincial Hospital of Chinese Medicine from June 2018 to May 2020 were selected as the research objects. According to the renal function injury at admission and the renal function recovery at 48 h after admission, patients with sepsis were divided into three groups: non-S-AKI group, transient S-AKI group, and persistent S-AKI group.Results:A total of 197 patients with sepsis were included, including 95 patients with non-S-AKI, 39 patients with transient S-AKI and 63 patients with persistent S-AKI. The mortality in the persistent S-AKI group was significantly higher than that in the non-S-AKI group (36.5% vs. 17.9%, P<0.05), but there was no significant difference between the transient S-AKI group and non-S-AKI group (20.5% vs. 17.9%, P>0.05). Multiple categorical disordered Logistic regression analysis showed that urea was associated with transient S-AKI ( OR=1.440, 95% CI: 1.235-1.680), and age ( OR=1.049, 95% CI: 1.008-1.092), urea ( OR=1.440, 95% CI: 1.277-1.733), and infection site in non-urinary tract ( OR=0.149, 95% CI: 0.050-0.448) were associated with persistent S-AKI. Conclusions:The incidence of persistent S-AKI is higher than that of transient S-AKI. Persistent S-AKI is related to the prognosis of patients with sepsis, and the mortality rate is higher, while transient S-AKI has no significant relationship. The urea of patients with S-AKI would increase significantly, and advanced age and urinary tract infection are related factors for the occurrence of persistent S-AKI. For patients with sepsis with such factors, attention should be paid to early protection of renal function to prevent persistent S-AKI.