Objective:To observe the efficacy of eculizumab in children with atypical hemolytic uremic syndrome.Methods:It was a single-center observational study. The clinical data of children diagnosed with atypical hemolytic uremic syndrome and treated with eculizumab in Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2023 to May 2024 were retrospectively collected. Eculizumab was used at the conventional dose based on the children 's weight. Event-free survival (no death or end-stage renal disease) rate, complete remission rate and recurrence rate of thrombotic microangiopathy in children with atypical hemolytic uremic syndrome after eculizumab treatment were analyzed. The complete remission time of estimated glomerular filtration rate, hemoglobin, platelet, lactic dehydrogenase, urine routine and the adverse reactions during the treatment were observed. Whole exome sequencing was used to conduct genetic testing based on blood samples of the children and their parents.Results:There were 4 children enrolled in the study. Four children were all Han Chinese, including 3 males and 1 female. The median age of onset was 8 years (ranging from 7 to 10 years). Two patients had complement gene abnormalities, both of which were homozygous deletions of complement factor H-related 1 and complement factor H-related 3. All the patients were free of plasma exchange or perfusion after treatment with eculizumab, and the 6-month event-free survival rate and thrombotic microangiopathy complete remission rate were both 4/4. The complete remission time was 19 (14-28) days. The time for the complete recovery of platelets, lactate dehydrogenase, estimated glomerular filtration rate and hemoglobin in 4 children was 4 (1-5), 19 (14-28), 10 (5-14) and 29 (20-42) days, respectively. Except for 1 patient whose urine routine fluctuated between negative and weakly positive expression, the other 3 patients had normal urine routine. All the patients discontinued eculizumab. Two patients without gene mutations discontinued eculizumab after 7 doses, and there was no recurrence during the 1-year follow-up after drug withdrawal. Two patients with genetic abnormalities discontinued eculizumab after 26 weeks of treatment, and no recurrence was found during the 3-month follow-up after drug withdrawal. One patient developed rash approximately 7 days after receiving the third dose of eculizumab. The rash was relieved after anti-allergic treatment, and there was no recurrence after the continued use of eculizumab.Conclusion:Eculizumab is effective and safe in the treatment of children with atypical hemolytic uremic syndrome. Discontinuation of eculizumab can be considered in patients without gene mutations when their condition is stable, but close monitoring and follow-up are needed after drug withdrawal.

Objective:To observe the efficacy of eculizumab in children with atypical hemolytic uremic syndrome.Methods:It was a single-center observational study. The clinical data of children diagnosed with atypical hemolytic uremic syndrome and treated with eculizumab in Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2023 to May 2024 were retrospectively collected. Eculizumab was used at the conventional dose based on the children 's weight. Event-free survival (no death or end-stage renal disease) rate, complete remission rate and recurrence rate of thrombotic microangiopathy in children with atypical hemolytic uremic syndrome after eculizumab treatment were analyzed. The complete remission time of estimated glomerular filtration rate, hemoglobin, platelet, lactic dehydrogenase, urine routine and the adverse reactions during the treatment were observed. Whole exome sequencing was used to conduct genetic testing based on blood samples of the children and their parents.Results:There were 4 children enrolled in the study. Four children were all Han Chinese, including 3 males and 1 female. The median age of onset was 8 years (ranging from 7 to 10 years). Two patients had complement gene abnormalities, both of which were homozygous deletions of complement factor H-related 1 and complement factor H-related 3. All the patients were free of plasma exchange or perfusion after treatment with eculizumab, and the 6-month event-free survival rate and thrombotic microangiopathy complete remission rate were both 4/4. The complete remission time was 19 (14-28) days. The time for the complete recovery of platelets, lactate dehydrogenase, estimated glomerular filtration rate and hemoglobin in 4 children was 4 (1-5), 19 (14-28), 10 (5-14) and 29 (20-42) days, respectively. Except for 1 patient whose urine routine fluctuated between negative and weakly positive expression, the other 3 patients had normal urine routine. All the patients discontinued eculizumab. Two patients without gene mutations discontinued eculizumab after 7 doses, and there was no recurrence during the 1-year follow-up after drug withdrawal. Two patients with genetic abnormalities discontinued eculizumab after 26 weeks of treatment, and no recurrence was found during the 3-month follow-up after drug withdrawal. One patient developed rash approximately 7 days after receiving the third dose of eculizumab. The rash was relieved after anti-allergic treatment, and there was no recurrence after the continued use of eculizumab.Conclusion:Eculizumab is effective and safe in the treatment of children with atypical hemolytic uremic syndrome. Discontinuation of eculizumab can be considered in patients without gene mutations when their condition is stable, but close monitoring and follow-up are needed after drug withdrawal.

To retrospectively analyze the clinical data of a case of shunt nephritis as the manifestation of systemic lupus erythematosus (SLE) admitted to the Department of Nephrology, Wuhan Children′s Hospital in November 2019, and to analyze its clinical characteristics and diagnosis and treatment through literature review.A 11-year-old female child was diagnosed as SLE for fatigue after exercise, onset of gross hematuria, auxiliary detection of anemia, hematuria albuminuria, hypocomplementemia, positive test for the antinuclear antibody, positive test for anti-human globulin, and hyperplastic lesions detected by renal pathology.However, immunosuppressive therapy was not effective.Considering the previous history of congenital hydrocephalus and ventricular and atrial shunt and through literature review, the patient was finally diagnosed was shunt nephritis.After active anti-infection and ventriculoatrial shunt to ventriculoperitoneal shunt, the symptoms were relieved.Shunt nephritis is a rare complication caused by ventricle shunt tract infection.Early recognition and treatment can avoid misdiagnosis and improve prognosis.

Objective:To explore the changes of serum neuron-specific enolase (NSE) and myelin basic protein (MBP) in children with cerebral palsy at high altitude during comprehensive rehabilitation and their clinical significance.Methods:A clinical randomized controlled study design was used to select 144 children with cerebral palsy who were diagnosed and treated in the Rehabilitation Center of Xining Traditional Chinese Medicine Hospital of Qinghai Province from June 2018 to October 2019, including 83 males and 61 females, aged 3-5 years old. According to the order of admission, the random number table was used to divide into a conventional treatment group ( n=72, 40 males and 32 females) and a comprehensive treatment group ( n=72, 43 males and 29 females). The conventional treatment group was treated with conventional rehabilitation. The comprehensive treatment group was treated with monosialotetrahexose ganglioside sodium on the basis of conventional rehabilitation. In addition, 30 healthy children aged 3-5 years, 16 males and 14 females, were selected as the control group during the physical examination of the Pediatrics Department of Xining Hospital of Traditional Chinese Medicine, Qinghai Province. The serum levels of NSE and MBP in each group were detected, and the children′s GMFM-88 scores were evaluated before and after treatment. The SPSS19.0 software was used for statistical analysis, the count data was tested by χ 2. Results:The serum NSE and MBP levels of the control group were (5.96±0.80), (0.71±0.15) μg/L. Before treatment, the serum NSE and MBP levels of children with severe, moderate, and mild cerebral palsy were [(21.63±1.92), (3.63±0.49) μg/L], [(17.86±1.43) μg/L, (2.21±0.07) μg/L] and [(15.14±0.95), (1.76±0.30) μg/L], respectively. After treatment, the serum NSE and MBP levels of the conventional treatment group and the comprehensive treatment group were [(13.54±2.41), (2.07±0.85) μg/L] and [(12.09±2.37), (1.81±0.69) μg/L], respectively, and the GMFM-88 score was (116.75±27.41) points and (125.94±24.93) points. The levels of NSE and MBP in the serum of children with cerebral palsy were significantly higher than those of normal children in the control group, and their levels increased with the degree of disease, and the corresponding gross motor function scores were lower. After treatment, the GMFM-88 scale assessment scores of the two groups of children were significantly improved ( t values were 310.97 and 70.86, P values were both<0.05), and serum NSE and MBP levels decreased to varying degrees compared with before treatment. The decline in the comprehensive treatment group was greater than that in the conventional treatment group. Conclusions:Serum NSE and MBP levels in children with cerebral palsy at high altitude are significantly higher than those in healthy children, and their levels are closely related to the degree of impairment and GMFM-88 scores in children with cerebral palsy. Dynamic monitoring of changes in NSE and MBP levels may be responsible for the condition and treatment effects of children with cerebral palsy judgments based.

Objective:To explore the changes of serum neuron-specific enolase (NSE) and myelin basic protein (MBP) in children with cerebral palsy at high altitude during comprehensive rehabilitation and their clinical significance.Methods:A clinical randomized controlled study design was used to select 144 children with cerebral palsy who were diagnosed and treated in the Rehabilitation Center of Xining Traditional Chinese Medicine Hospital of Qinghai Province from June 2018 to October 2019, including 83 males and 61 females, aged 3-5 years old. According to the order of admission, the random number table was used to divide into a conventional treatment group ( n=72, 40 males and 32 females) and a comprehensive treatment group ( n=72, 43 males and 29 females). The conventional treatment group was treated with conventional rehabilitation. The comprehensive treatment group was treated with monosialotetrahexose ganglioside sodium on the basis of conventional rehabilitation. In addition, 30 healthy children aged 3-5 years, 16 males and 14 females, were selected as the control group during the physical examination of the Pediatrics Department of Xining Hospital of Traditional Chinese Medicine, Qinghai Province. The serum levels of NSE and MBP in each group were detected, and the children′s GMFM-88 scores were evaluated before and after treatment. The SPSS19.0 software was used for statistical analysis, the count data was tested by χ 2. Results:The serum NSE and MBP levels of the control group were (5.96±0.80), (0.71±0.15) μg/L. Before treatment, the serum NSE and MBP levels of children with severe, moderate, and mild cerebral palsy were [(21.63±1.92), (3.63±0.49) μg/L], [(17.86±1.43) μg/L, (2.21±0.07) μg/L] and [(15.14±0.95), (1.76±0.30) μg/L], respectively. After treatment, the serum NSE and MBP levels of the conventional treatment group and the comprehensive treatment group were [(13.54±2.41), (2.07±0.85) μg/L] and [(12.09±2.37), (1.81±0.69) μg/L], respectively, and the GMFM-88 score was (116.75±27.41) points and (125.94±24.93) points. The levels of NSE and MBP in the serum of children with cerebral palsy were significantly higher than those of normal children in the control group, and their levels increased with the degree of disease, and the corresponding gross motor function scores were lower. After treatment, the GMFM-88 scale assessment scores of the two groups of children were significantly improved ( t values were 310.97 and 70.86, P values were both<0.05), and serum NSE and MBP levels decreased to varying degrees compared with before treatment. The decline in the comprehensive treatment group was greater than that in the conventional treatment group. Conclusions:Serum NSE and MBP levels in children with cerebral palsy at high altitude are significantly higher than those in healthy children, and their levels are closely related to the degree of impairment and GMFM-88 scores in children with cerebral palsy. Dynamic monitoring of changes in NSE and MBP levels may be responsible for the condition and treatment effects of children with cerebral palsy judgments based.

Objective:To observe the body fluid and cellular immune function of children with cerebral palsy (CP) in the plateau area, as well as the exchanges of these factors during the comprehensive rehabilitation treatment.Methods:A total number of 144 children admitted to Xining Hospital of Traditional Chinese Medicine from June 2018 to October 2019 were selected as the CP group for comprehensive rehabilitation treatment (consecutive courses). The peripheral blood immunoglobulin/complement (IgA, IgG, lgM, C3, C4) level, T lymphocyte subsets (CD3+, CD4+, CD8+, CD4+/CD8+) and neuron-specific enolase (NSE) content was examined in the clinical specimens before and after treatment by using the immunoturbidimetry, flow cytometry, electrochemiluminescence analysis according to the Gross Motor Function Classification System (GMFCS) and Gross Motor Function Test Scale (GMFM-88). Children were divided as the different degrees to evaluate the rehabilitation efficacy. A total number of 50 healthy children taken a health check/physical examinations during the same period were considered as the control group. For statistical Analysis, the χ2 test and independent sample t test were performed. Results:The levels of humoral immune IgG, IgA, IgM, C3 and C4 in CP Group [(6.42±1.05), (0.64±0.13), (0.89±0.13), (0.80±0.08), (0.17±0.03) g/L, respectively] in CP groups′ children were lower than those in the control group [(10.25±0.62), (1.04±0.06), (1.06±0.17), (1.04±0.04), (0.27±0.04) g/L, respectively]. The humoral immune IgG and IgA levels [severe (5.40±0.69) and (0.55±0.09)g/L, moderate (6.63±0.30) and (0.66±0.14)g/L, mild (7.57±0.63) and (0.74±0.09)g/L, P<0.05] were also related to the children with CP of different GMFCS grades. Moreover, the level of T lymphocyte subsets (CD3+,CD4+,CD8+,CD4+/CD8+) in the CP group were not statistically different to that in the control groups children. Receiving the rehabilitation treatment, the levels of serum humoral IgG and IgA in CP Group (7.69±1.14) and (0.79±0.17) g/L were significantly enhanced; whereas the serum NSE (12.82±2.49) μg/L was lower than that before treatment (18.57±3.08) μg/L, and the total score of GMFM-88 (121.35±26.51) was higher than that before treatment (101.04±27.62). The differences were statistically significant ( P<0.05). IgM, C3, C4 and T lymphocyte subsets (CD3+, CD4+, CD8+, CD4+/CD8+) had no significant difference compared with those before treatment ( P>0.05). Conclusions:Children with CP at high altitude have abnormal humoral immune function. IgG and IgA may be related to the severity of CP and neuronal damage. Comprehensive rehabilitation can not only improve the motor function of children with CP, relieve neuronal damage, but also enhance their humoral immunity status.

OBJECTIVE: To compare the image quality of three-dimensional time-of-flight (TOF) magnetic resonance angiography (MRA) with sparse undersampling and iterative reconstruction (sparse TOF) with that of conventional TOF MRA. MATERIALS AND METHODS: This study included 56 patients who had undergone sparse TOF MRA for intracranial artery evaluation on a 3T MR scanner. Conventional TOF MRA scans were also acquired from 29 patients with matched acquisition times and another 27 patients with matched scanning parameters. The image quality was scored using a five-point scale based on the delineation of arterial vessel segments, artifacts, overall vessel visualization, and overall image quality by two radiologists independently, and the data were analyzed using the non-parametric Wilcoxon signed-rank test. Contrast ratios (CRs) of vessels were compared using the paired t test. Interobserver agreement was calculated using the kappa test. RESULTS: Compared with conventional TOF at the same spatial resolution, sparse TOF with an acceleration factor of 3.5 could reduce acquisition time by 40% and showed comparable image quality. In addition, when compared with conventional TOF with the same acquisition time, sparse TOF with an acceleration factor of 5 could also achieve higher spatial resolution, better delineation of vessel segments, fewer artifacts, higher image quality, and a higher CR (p < 0.05). Good-to-excellent interobserver agreement (κ: 0.65–1.00) was obtained between the two radiologists. CONCLUSION: Compared with conventional TOF, sparse TOF can achieve equivalent image quality in a reduced duration. Furthermore, using the same acquisition time, sparse TOF could improve the delineation of vessels and decrease image artifacts.
Acceleration ; Arteries ; Artifacts ; Humans ; Magnetic Resonance Angiography

Compared to conventional cancer treatment, combination therapy based on well-designed nanoscale platforms may offer an opportunity to eliminate tumors and reduce recurrence and metastasis. In this study, we prepared multifunctional microspheres loading I-labeled hollow copper sulfide nanoparticles and paclitaxel (I-HCuSNPs-MS-PTX) for imaging and therapeutics of W256/B breast tumors in rats. F-fluordeoxyglucose (F-FDG) positron emission tomography/computed tomography (PET/CT) imaging detected that the expansion of the tumor volume was delayed (<0.05) following intra-tumoral (i.t.) injection with I-HCuSNPs-MS-PTX plus near-infrared (NIR) irradiation. The immunohistochemical analysis further confirmed the anti-tumor effect. The single photon emission computed tomography (SPECT)/photoacoustic imaging mediated by I-HCuSNPs-MS-PTX demonstrated that microspheres were mainly distributed in the tumors with a relatively low distribution in other organs. Our results revealed that I-HCuSNPs-MS-PTX offered combined photothermal, chemo- and radio-therapies, eliminating tumors at a relatively low dose, as well as allowing SPECT/CT and photoacoustic imaging monitoring of distribution of the injected agents non-invasively. The copper sulfide-loaded microspheres, I-HCuSNPs-MS-PTX, can serve as a versatile theranostic agent in an orthotopic breast cancer model.

Objective To investigate the value of arterial spin labeling (ASL) MRI in evaluation of renal cortex perfusion in patients with type 2 diabetes.Methods Fifty patients with type 2 diabetes were enrolled and divided into simple diabetes (SD) group (n=25) and diabetes kidney disease (DKD) group (n=25) according to suffering from DKD or not.Based on estimated glomerular filtration rate (eGFR),DKD group were further divided into mild disease subgroup (n=11,eGFR≥ 60 ml/[min · 1.73m2]) and moderate-severe disease subgroup (n=14,eGFR＜60 ml/[min · 1.73m2]).Twenty-five healthy volunteers were recruited as control group at the same time.ASL MRI were performed on all participants.The cortical renal blood flow (RBF) of bilateral kidneys were measured by 2 radiologists.The consistency between 2 radiologists was analyzed.Statistical analysis were conducted to analysis the differences in cortical RBF among different groups.Correlation analysis were performed to evaluate the relationship between RBF and eGFR in type 2 diabetes patients.Results Cortical RBF values measured by two radiologists showed high consistency (all ICC＞0.90).There was significant difference in cortical RBF among control group ([269.71±33.28]ml/[100 g · min]),SD group ([258.52±42.30]ml/[100 g · min]),mild disease group ([242.86±56.86]ml/[100 g · min]) and moderate-severe disease group ([173.39±27.16]ml/ [100 g· min];F=20.66,P＜0.01).Moreover,the RBF in moderate-severe disease group was significantly lower than those in other groups (all P＜0.01).And no significant differences of RBF was found among the remainder groups (P=0.064,0.320).RBF in type 2 diabetes patients was positively correlated to eGFR (r=0.646,P＜0.001).Conclusion ASL MRI is a valuable tool to quantitatively assess the renal perfusion in patients with type 2 diabetes mellitus,which can provide potential imaging indicator as RBF for the functional evaluation of kidney.

Objective To design a speech command module to replace manual command during X-ray examination.Methods The module was composed of the parts for speech,pushbutton actuator,speaker,miniature voice box,power source and etc.The electrical conductors were used to connect the above parts,and the speech command was formed by special software.Results The module had the speech command triggered by the pushbutton,and the speech command was pellucid and clear so that the workload could be decreased.Conclusion The module facilitates the speech command during X-ray examination,and thus is worthy promoting practically.