The clinical data of one child with metanephric stromal tumor (MST) and BRAF V600E gene mutation admitted to the First Affiliated Hospital of Zhengzhou University in June 2022 was analyzed retrospectively.Literature was reviewed.The patient, a 2-year-old girl, was diagnosed with a tumor in the left abdomen.The maximum diameter of the tumor was 10.5 cm.A radical nephrectomy was performed on the left kidney, and postoperative pathology revealed MST.Microscopically, the tumor had no envelope and exhibited expansive growth.The tumor cells were fusiform or stellate, and nuclear division was visible in the cell-rich region.Dysplastic blood vessels were seen inside the tumor.The tumor cells around the blood vessels and invaginated renal tubules were arranged like onion skin.CD34 was detected positive by immunohistochemical staining, and BRAF V600E mutation was also detected positive by fluorescent polymerase chain reaction.A total of 21 relevant case reports were retrieved, including 16 in English and 5 in Chinese.Fifty-eight MST patients, including the one in this report were analyzed.These patients were aged 2 days to 15 years, with a median age of 2 years.Except for 2 patients with unknown sex, the ratio of male to female was about 1.4∶1.0.Most MST patients were asymptomatic, with an average tumor size of 5.3 cm.The tumor cell CD34 showed positive expression in different degrees.Eight patients received the BRAF V600E mutation detection, and the results were all positive.Fifty-eight patients underwent nephrectomy and were followed up for 0-156 months, of which 7 patients were assisted with radiotherapy and chemotherapy.During the follow-up, 1 patient died, and 1 patient had a relapse.MST is a rare benign renal stromal tumor. BRAF V600E mutations are detected in a variety of malignancies.This paper is the first to report MST with BRAF V600E mutation in China and points out the importance of molecular detection of BRAF mutation for accurate diagnosis of MST.

Humans ; Factor XIII/genetics* ; Factor XIII Deficiency/genetics* ; Mutation ; Pedigree

Objective:To evaluate the efficacy and safety of mitoxantrone hydrochloride liposome injection in the treatment of peripheral T-cell lymphoma (PTCL) in a real-world setting.Methods:This was a real-world ambispective cohort study (MOMENT study) (Chinese clinical trial registry number: ChiCTR2200062067). Clinical data were collected from 198 patients who received mitoxantrone hydrochloride liposome injection as monotherapy or combination therapy at 37 hospitals from January 2022 to January 2023, including 166 patients in the retrospective cohort and 32 patients in the prospective cohort; 10 patients in the treatment-na?ve group and 188 patients in the relapsed/refractory group. Clinical characteristics, efficacy and adverse events were summarized, and the overall survival (OS) and progression-free survival (PFS) were analyzed.Results:All 198 patients were treated with mitoxantrone hydrochloride liposome injection for a median of 3 cycles (range 1-7 cycles); 28 cases were treated with mitoxantrone hydrochloride liposome injection as monotherapy, and 170 cases were treated with the combination regimen. Among 188 relapsed/refractory patients, 45 cases (23.9%) were in complete remission (CR), 82 cases (43.6%) were in partial remission (PR), and 28 cases (14.9%) were in disease stabilization (SD), and 33 cases (17.6%) were in disease progression (PD), with an objective remission rate (ORR) of 67.6% (127/188). Among 10 treatment-na?ve patients, 4 cases (40.0%) were in CR, 5 cases (50.0%) were in PR, and 1 case (10.0%) was in PD, with an ORR of 90.0% (9/10). The median follow-up time was 2.9 months (95% CI 2.4-3.7 months), and the median PFS and OS of patients in relapsed/refractory and treatment-na?ve groups were not reached. In relapsed/refractory patients, the difference in ORR between patients with different number of treatment lines of mitoxantrone hydrochloride liposome injection [ORR of the second-line, the third-line and ≥the forth-line treatment was 74.4% (67/90), 73.9% (34/46) and 50.0% (26/52)] was statistically significant ( P = 0.008). Of the 198 PTCL patients, 182 cases (91.9%) experienced at least 1 time of treatment-related adverse events, and the incidence rate of ≥grade 3 adverse events was 66.7% (132/198), which was mainly characterized by hematologic adverse events. The ≥ grade 3 hematologic adverse events mainly included decreased lymphocyte count, decreased neutrophil count, decreased white blood cell count, and anemia; non-hematologic adverse events were mostly grade 1-2, mainly including pigmentation disorders and upper respiratory tract infection. Conclusions:The use of mitoxantrone hydrochloride liposome injection-containing regimen in the treatment of PTCL has definite efficacy and is well tolerated, and it is a new therapeutic option for PTCL patients.

Objective: To investigate the clinical characteristics and related factors of corticosteroid induced adrenal crisis (AC) in children with primary nephrotic syndrome (NS). Methods: Case control study. The case group included 7 children aged 1 to 18 years with NS combined with AC hospitalized in Peking University First Hospital from January 2016 to May 2021 (AC group). According to the ratio of case group: control group 1: 4, 28 children aged 1 to 18 years who were diagnosed with NS without AC during the same period were matched as controls (non-AC group). Clinical data were collected. The clinical characteristics of AC were described. The clinical parameters were compared between the 2 groups by t test, Mann-Whitney U test or Fisher's test. Receiver operating characteristic (ROC) curve was used to analyze the cutoff values of clinical parameters for prediction of AC. Results: The AC group included 4 boys and 3 girls aged 6.9 (4.6, 10.8) years. The non-AC group included 20 boys and 8 girls aged 5.2 (3.3, 8.4) years. All AC events occurred during the relapse of NS with infection. Seven children had gastrointestinal symptoms such as nausea, vomiting and abdominal pain. Six children had poor mental state or impaired consciousness. No significant differences in NS course, corticosteroid treatment course, corticosteroid type, steroid dosage, steroid medication interval, the proportion of gastroenteritis and fever existed between the two groups (all P>0.05). Compared with the non-AC group, the duration from the onset of the relapse of NS until hospitalization in the AC group was significantly shorter (0.2 (0.1, 0.6) vs. 1.0 (0.4, 5.0) month,U=25.50, P=0.005). The 24 h urinary total protein (UTP) level was significantly higher in the AC group (193 (135, 429) vs. 81 (17, 200) mg/kg, U=27.00,P=0.036) than the non-AC group. The serum albumin level in the AC group was significantly lower((13.1±2.1) vs. (24.5±8.7) g/L,t=-6.22,P<0.001) than the non-AC group. There were significantly higher total white blood cell counts ((26±9)×10⁹ vs. (11±5)×10⁹/L,t=4.26,P=0.004), percentage of neutrophils (0.71±0.08 vs. 0.60±0.19,t=2.56,P=0.017) and the proportion of children with C reactive protein level≥8 mg/L (3/7 vs. 0,P=0.005) in the AC group than in the non-AC group. ROC curve analysis showed that the cutoff value of 24 h UTP was 122 mg/(kg·d) with a sensitivity of 100.0% and specificity of 70.4%. The cutoff value of serum albumin was 17.0 g/L with a sensitivity of 100.0% and specificity of 82.1%. Conclusions: Gastrointestinal symptoms and poor mental state were prominent manifestations of AC in children with NS. High 24 h UTP level, low serum albumin level, high peripheral white blood cell counts, high neutrophils percentage, and high C-reactive protein level during the early stage of NS relapse may be related to the occurrence of AC in children with NS.
Nephrotic Syndrome/drug therapy* ; Humans ; Child ; Adolescent ; Male ; Female ; Gastrointestinal Diseases/diagnosis* ; Adrenal Cortex Hormones/therapeutic use* ; Nausea/chemically induced* ; Vomiting/chemically induced* ; Abdominal Pain/chemically induced* ; Mental Processes/drug effects* ; China

Objective: To assess the correlation of glomerular C1q or IgA deposition with clinical and pathological features of primary membranous nephropathy (PMN) in children. Methods: The clinical and pathological manifestations including (phospholipase A2 receptor, PLA2R) and IgG subclasses staining in renal biopsies, serum anti-PLA2R antibody and therapeutic response of 33 children diagnosed with PMN in Peking University First Hospital from December 2012 to December 2020 were retrospectively summarized and analyzed. According to results of PLA2R test and findings renal pathological, the patients were divided into PLA2R-related group and non-PLA2R-related group, typical MN group and atypical MN group, C1q deposit group and non-C1q deposit group, as well as IgA deposit group and non-IgA deposit group respectively. T-test, Mann-Whitney U test and Fisher's exact probability test were used for comparison between the groups. Results: Among the 33 children with PMN, there were 20 males and 13 females, of that the age of onset was 11 (8, 13) years, and 32 patients had nephrotic level proteinuria. Renal biopsies were performed at 4.6 (2.1, 11.6) months after onset, and 28 patients (85%) received glucocorticoid or immunosuppressive therapy prior to renal biopsy. There were 20 cases (61%) with PLA2R-related MN and 13 cases (39%) with non-PLA2R-related MN. Compared with the non-PLA2R-related group, the PLA2R-related group had an older age of onset (12 (10, 13) vs. 7 (3, 12) years, Z=-2.52, P=0.011), a lower preceding infection rate (45% (9/20) vs. 11/13, P=0.032) and lower spontaneous remission rate (0 vs. 4/13, P=0.017). Renal PLA2R positivity was significantly associated with predominant or co-deposition of IgG4 (13/17 vs. 5/15, P=0.031) and low albumin levels at renal biopsy ((25±6) vs. (29±7) g/L, t=2.14, P=0.041). There were 12 patients with typical PMN and 21 patients with atypical PMN, and no significant difference in clinical and pathological manifestations was found between these 2 groups (all P>0.05). There were 10 cases (32.3%) with glomerular C1q deposition, and their disease course before renal biopsy was significantly shorter than those without C1q deposition (1.8 (0.8, 5.9) vs. 6.0 (2.5, 22.3) months, Z=-2.27, P=0.023). Twelve cases (36.4%) had glomerular IgA deposition, and their course of disease,clinical and pathological manifestations were not significantly different from those without IgA deposition (all P>0.05). Conclusion: Glomerular C1q or IgA deposition may not affect the clinical manifestations, glomerular PLA2R and IgG subclasses staining pattern, or the response to treatment of PMN in children.
Autoantibodies ; Child ; Complement C1q/metabolism* ; Female ; Glomerulonephritis, Membranous/drug therapy* ; Humans ; Immunoglobulin A/immunology* ; Immunoglobulin G ; Kidney Glomerulus ; Male ; Retrospective Studies

Objective: To assess the reliability of estimated urine protein to predict 24 h urine protein excretion in children with glomerular diseases. Methods: Four hundred and forty-three children with glomerular diseases, who were admitted to pediatric department of Peking University First Hospital from January 2001 to December 2021, were enrolled in the cross-sectional study. The 24 h estimated urine creatinine which calculated by 6 previously described equations, 24 h measured urine creatinine, measured urine protein-to-creatinine ratio(UPCR), 24 h urine protein (24 hUP) and urinary sediment analysis with microscopy were collected, estimated urine protein was computed as the product of measured UPCR and estimated or measured 24 h urine creatinine. Spearman correlation analysis, Bland-Altman analysis and linear regression analysis were used to compare the correlation, agreement and accuracy between estimated urine protein and 24 hUP, and the effect of urinary protein level and erythrocyte numbers on their relationship was analyzed. Results: Of 443 children with glomerular diseases (aged (11±4) years, 221 male, 222 female), there were 216 participants with nephrotic syndrome, 78 participants with IgA nephropathy, 47 participants with Alport syndrome, 42 participants with lupus nephritis, 58 participants with purpura nephropathy, and 2 participants with isolated proteinuria. Spearman correlation analysis showed a strong correlation between estimated urine protein and 24 hUP (r=0.90, P<0.05), and the correlation improved after multiplying the measured UPCR by 24 h measured urine creatinine (r=0.94, P<0.05). Improved correlation was also observed using the estimated urine creatinine which calculated by Hellerstein formula, Ghazali-Barratt formula, Ellam formula, Walser formula, Cockcroft-Gault formula, Ix formula (r=0.93, 0.94, 0.90, 0.90, 0.94, 0.93, all P<0.05).Bland-altman analysis showed that the difference between measured UPCR and 24 hUP was (-0.30±2.22) g, consistency limit was -4.65-4.04, and the consistency improved after 24 h measured urine creatinine correction (difference was (0.27±1.31) g, consistency limit -2.30-2.84). The consistency of estimated urine protein was further improved after correction by different formulas, and the Cockcroft-Gault formula showed the best consistency between estimated urine protein and 24 hUP (difference was (0.11±1.18)g, consistency limit was -2.20-2.42). Linear regression analysis showed that measured UPCR had poor accuracy in predicting 24 hUP (R²=0.55, α=0.48, β=0.60, P<0.05), and the accuracy improved after 24 h measured urine creatinine correction, the accuracy of estimated urine protein for predicting 24 hUP was further improved by using different formulas, and Cockcroft-Gault formula was the best (R²=0.81, α=0.18, β=0.96, P<0.05). With the increase of urinary protein level and the decrease of urinary erythrocyte numbers, the correlation, agreement and accuracy between estimated urine protein and measured UPCR and 24 hUP were improved(all P<0.05). Except Ellam and Ix formulas, estimated urine protein using the rest four formulas outperformed measured UPCR(all P<0.05). Conclusion: The 24 h urine creatinine excretion rate (obtained by the Cockcroft-Gault equation)-weighted urine protein-to-creatinine ratio more reliably predicts 24 hUP than measured UPCR alone in children with glomerular diseases.
Child ; Male ; Female ; Humans ; Creatinine/urine* ; Glomerular Filtration Rate ; Cross-Sectional Studies ; Reproducibility of Results ; Predictive Value of Tests

OBJECTIVE: To compare the effects of electroacupuncture (EA) at "Zusanli" (ST 36) versus "Yanglingquan" (GB 34) in the pregnant rats on perinatal nicotineexposureinduced lung function and morphology of newborn rats and explore the rule of acupoint effect in EA for the prevention from lung dysplasia in newborn rats. METHODS: A total of 24 female SD rats were randomized into a normal saline group (S group), a nicotine group (N group), a nicotineST 36 group (N + ST 36 group) and a nicotineGB 34 group (N+GB 34 group), 6 rats in each one. Starting at the 6th day of pregnancy, 0.9% sodium chloride solution was injected subcutaneously in the S group, 1 mg/kg; and in the rest 3 groups, nicotine of the same dose was injected through to the 21st postnatal day to establish the perinatal nicotineexposure model. Simultaneously, during model preparation, EA was applied at "Zusanli" (ST 36) and "Yanglingquan" (GB 34) in the N+ST 36 group and the N+GB 34 group respectively, once a day, through to the 21st postnatal day. The lung function analytic system for small animal was adopted to observe the changes in lung function indicators in newborn rats, such as peak inspiratory flow (PIF), peak expiratory flow (PEF), expiratory resistance (RE), inspiratory resistance (RI) and dynamic compliance (Cdyn). HE staining was used to observe the morphological changes of lung, such as alveolar fusion and rupture. RESULTS: Compared with the S group, PEF and Cdyn were lower and PIF, RI and RE higher in the N group (all <0.01), additionally, alveoli were fused and ruptured, alveolar wall thickened, the numbers of alveoli reduced, the interspace of alveoli enlarged and the diameter increased (<0.01). Compared with the N group, in the N+ST 36 group, PEF and Cdyn were increased, PIF, RI and RE reduced (<0.05, <0.01), the alveolar fusion and rupture relieved, the numbers of alveoli increased, alveolar wall thinner, the interpsace of alveoli became normal and the diameter was reduced significantly (<0.01). In the N+GB 34 group, the changes of lung function and morphological indicators were not significant (>0.05). CONCLUSION Electroacupuncture at "Zusanli" (ST 36) in the pregnant rats significantly improves the perinatal nicotineexposureinduced lung function and morphology of newborn rats than electroacupuncture at "Yanglingquan" (GB 34).
Acupuncture Points ; Animals ; Animals, Newborn ; Electroacupuncture ; Female ; Lung ; drug effects ; physiopathology ; Nicotine ; toxicity ; Pregnancy ; Random Allocation ; Rats ; Rats, Sprague-Dawley

Objective:To investigate the clinical features and side effects,with regard to glucocorticoid-induced ocular hypertension,glaucoma or cataract in children with primary nephrotic syndrome.Methods:Clinical data were collected and analyzed from 71 cases of primary nephrotic syndrome with glucocorticoid-induced ocular hypertension,glaucoma or cataract from Jun.2014 to Jun.2016.These children were hospitalized in Peking University First Hospital.Results:Totally 1 580 children with primary nephrotic syndrome were collected,glucocorticoid-induced complications in eyes were found in 71 cases,and the incidence was 4.5％.There were 66 cases with ocular hypertension,2 cases with glucocorticoid glaucoma,2 cases with glucocorticoid glaucoma combined with cataract,1 case with high intraocular pressure combined with cataract.There were 41 boys and 30 girls with eye-related side effects caused by glucocorticoid.The average age of onset of glucocorticoid-induced eye adverse reactions in children with primary nephrotic syndrome in our research were 8 (2,16) years.The average duration or interval time from glucocorticoid medication use to eye adverse effects was 157 (6,420) days.No statistical significance was found in intraocular pressure between different genders,types of glucocorticoid,different route of glucocorticoid and whether methylprednisolone pulse treatment (P ＞ 0.05).There was no significant correlation between age,body mass index,blood pressure,cumulative dosage,duration time of glucocorticoid,mean daily dosage and glucocorticoid-induced ocular hypertension (P ＞ 0.05).The ocular hypertension was controlled after treatment.Conclusion:Children with nephrotic syndrome after treatment of glucocorticoid are susceptible to ocular complications,and the occurrence of ocular hypertension is closely related to glucocorticoid susceptibility of the nephrotic children.Regular eye monitor is indispensable for the children suffering from primary nephrotic syndrome.

Objective To set up a three-dimensional(3D) craniofacial cone-beam CT(CBCT)superimposition method based on voxel registration,and evaluate the accuracy and reliability of different reference superimposition areas.Methods CBCT scans were taken on a dry skull for three times with a time-interval of two weeks,and CBCT scans were taken on ten adult volunteers twice with a time-interval of one month.The 3D superimposition based on voxel registration was set up by means of Analyze 12.0.The bilateral-zygomatic bone and anterior cranial base were selected as different reference areas for voxel overlapping.The 3D overlapping images were output into Geomagic Qualify 2013,and the surface distances on different craniofacial regions were measured.Three operators repeated three times of these superimposition and measurement.SPSS 19.0 were used to analyze the accuracy and reliability.Results When bilateral-zygomatic bone used as superimposition reference,the mean surface distance range of dry skull were from 0.035 to 0.064 mm,and volunteers from 0.099 to 0.182 mm.When the anterior cranial base used as superimposition reference,the mean surface distance range of dry skull were from 0.038 to0.071 mm,and volunteers from 0.127 to 0.218 mm.All these mean values were less than the CBCT voxel size0.25 mm(P＜0.05),and got sub-pixel precision.No significant difference was found between the different operators at different times(P＞0.05).Conclusions This study showed the high accuracy and reliability of 3D CBCT superimposition based on voxel registration,either the anterior cranial base or the bilateral-zygomatic bone as reference.This method could be used for evaluating 3D craniofacial treatment effects.

BACKGROUNDDual sensor (DS) for rate adaption was supposed to be more physiological. To evaluate its superiority, the DS (accelerometer [ACC] and minute ventilation [MV]) and normal sinus rate response were compared in a self-controlled way during exercise treadmill testing.

METHODSThis self-controlled study was performed in atrioventricular block patients with normal sinus function who met the indications of pacemaker implant. Twenty-one patients came to the 1-month follow-up visit. Patients performed a treadmill test 1-month post implant while programmed in DDDR and sensor passive mode. For these patients, sensor response factors were left at default settings (ACC = 8, MV = 3) and sensor indicated rates (SIRs) for DS, ACC and MV sensor were retrieved from the pacemaker memories, along with measured sinus node (SN) rates from the beginning to 1-minute after the end of the treadmill test, and compared among study groups. Repeated measures analysis of variance and profile analysis, as well as variance analysis of randomized block designs, were used for statistical analysis.

RESULTSFifteen patients (15/21) were determined to be chronotropically competent. The mean differences between DS SIRs and intrinsic sinus rates during treadmill testing were smaller than those for ACC and MV sensor (mean difference between SIR and SN rate: ACC vs. SN, MV vs. SN, DS vs. SN, respectively, 34.84, 17.60, 16.15 beats/min), though no sensors could mimic sinus rates under the default settings for sensor response factor (ACC vs. SN P-adjusted < 0.001; MV vs. SN P-adjusted = 0.002; DS vs. SN P-adjusted = 0.005). However, both in the range of 1 st minute and first 3 minutes of exercise, only the DS SIR profile did not differ from sinus rates (P-adjusted = 0.09, 0.90, respectively).

CONCLUSIONSThe DS under default settings provides more physiological rate response during physical activity than the corresponding single sensors (ACC or MV sensor). Further study is needed to determine if individual optimization would further improve adaptive performance of the DS.

Adult ; Aged ; Cardiac Pacing, Artificial ; methods ; Exercise ; physiology ; Exercise Test ; Female ; Humans ; Male ; Middle Aged ; Pacemaker, Artificial