ABSTRACT
Objective:To analyze the gene mutation, clinical manifestations and prognosis of children with steroid resistant nephrotic syndrome (SRNS), and to provide reference for the treatment of hereditary SRNS in children.Methods:The clinical data of 29 patients with SRNS and whole exon sequencing (WES) diagnosed in Xi′an Children′s Hospital from January 1, 2018 to December 31, 2020 were retrospectively analyzed.Results:In 29 cases of SRNS with genetic testing, 10 cases (34.5%) were gene mutations, including 2 cases of congenital nephrotic syndrome. The onset age of the patients with gene mutation ranged from 0.1 to 10.7(4.06±3.73)years, and the median age of onset was 3.3 years. The clinical type was mainly nephritis (8/10), and the pathological type was mainly focal segmental glomerulosclerosis (FSGS) (5/7). The main mutant genes were NPHS1 (2 cases), NPHS2 (2 cases), WT1 (2 cases), SMARCAL1 (1 case), COQ8B (1 case), TRPC6 (1 case) and COL4A3 gene (1 case). The main types of genetic variation were missense mutations, and 6 (60%) cases were new mutations that had never been reported in the database containing human pathogenic mutations before. Compared with the non-gene mutation group, 24 hour urinary protein was higher [(177.92±164.59)mg/(kg·24 h) vs (84.99±40.79)mg/(kg·24 h)] in gene mutation group, with statistically significant difference ( P<0.05). In the gene mutation group, there were 2 cases of complete remission, including 1 case of complete remission treated with coenzyme Q10, 1 case of partial remission, and 8 cases of immunosuppression treatment, with an effective rate of 2/8, while in the non-gene mutation group, the effective rate of immunosuppression treatment was 17/19, with statistically significant difference in prognosis between the two groups ( P<0.05). Conclusions:The pathological type of children with hereditary SRNS is mainly FSGS, which are often ineffective to immunosuppressive therapy, poor prognosis and easy to progress to end-stage renal disease. Gene detection is of great significance for etiological diagnosis, treatment and prognosis evaluation in children with SRNS.
ABSTRACT
0bjective To analyze the clinical characteristics, pathological types, treatment and prognosis in children with steroid resistant nephrotic syndrome (SRNS) in Northwest China, in order to provide reference for the treatment of SRNS. Methods:The clinical data, renal pathological results, treatment plan and efficacy of 102 children diagnosed with SRNS in the Department of Nephrology, Xi'an Children's Hospital of Shaanxi Province from January 1st, 2018 to December thirty-first, 2020 were analyzed retrospectively. All children were divided into groups according to age, clinical classification, pathological type, treatment scheme and treatment outcome, and the risk factors affecting the prognosis of children with SRNS were discussed. The measurement datas conforming to normal distribution were expressed as xˉ± s, and t test was used for comparison between groups. Measurement datas that did not conform to normal distribution were represented by M ( Q1, Q3), and Kruskall-Wallis test was used for comparison between groups.Enumeration datas were compared by χ 2 test. Risk factors were analyzed by multiple factor Logistic regression analysis. Results:The median age of onset of 102 children with SRNS was 3.0 years. Focal segmental glomerulosclerosis (FSGS) accounted for 36.3% (37/102), minimal lesions accounted for 33.3% (34/102), and mesangial proliferative glomerulonephritis accounted for 23.5% (24/102). The prevalence rates of hypertension (35.1% (13/37)), 24-h urine protein quantification (130.5 (91.5, 159.6) mg/(kg·24 h) and renal insufficiency (21.6% (8/37)) in FSGS group were higher than those in non-FSGS group (13.8% (9/65), 65.8 (51.2,85.5) mg/(kg·24 h), 4.6% (3/65)). The differences between the two groups were statistically significant (statistical values were χ 2=6.32, Z=5.90, χ 2=7.09; P values were 0.012, <0.001, 0.008). Logistic multivariate regression analysis showed that the hypertension ( OR=4.055, 95% CI 1.178-3.962) and 24 hour urinary protein ( OR=1.036, 95% CI 1.020-1.053) were associated with the increased risk of FSGS ( P values were 0.026 and <0.001). ROC curve ananlysis showed that the optimal critical value of 24 hour urinary protein was 85.65 mg/(kg·24 h) in FSGS. After treatment, complete remission was 61.8%(63/102), partial remission was 14.7%(15/102), and no remission was 23.5%(24/102). By the end of follow-up the treatment effective rate in the small lesion group (94.1%(32/34)) was higher than that in the FSGS Group (51.3%(19/37)), and the difference between the two groups was statistically significant (χ 2=16.02, P<0.001). In the initial immunosuppressive treatment, the complete remission rate of hormone combined with calcineurin inhibitor group (77.1%(37/48)) was higher than that of hormone combined with cyclophosphamide Group (11.1%(3/27)). There was significant difference between the two groups ( Z=32.28, P<0.001). Conclusion:The most common pathological type in children with SRNS was FSGS, and the age of onset was generally small. The prognosis of patients with pathological type FSGS was the worst, and the prognosis of small lesions was better. Hypertension and 24-hour urinary protein quantification were the risk factors of FSGS. Calcineurin inhibitors were the first choice for the second-line immunosuppressants of SRNS in children.
ABSTRACT
Objective:To investigate the clinical features of children with kidney diseases who developed posterior reversible encephalopathy syndrome (PRES), explore the risk factors of PRES in these children, improve the understanding of the diseases, and help early diagnosis and effective treatment of the diseases.Methods:The clinical manifestations, laboratory inspection results, magnetic resonance imaging(MRI) material as well as the prognosis of 10 children with kidney diseases complicated by PRES who were admitted to the Department of Nephrology, Xi′an Children′s Hospital from November 2016 to August 2018 were analyzed retrospectively.Results:A total of 10 children were recruited, including 1 boy and 9 girls, with the onset age ranging from 4 years and 3 months to 13 years [(8.53±3.09) years]. The diagnosed kidney diseases in these patients were primary nephritic syndrome (6 cases), lupus nephritis (1 case), Hepatitis B-related nephritis (1 case), polyarteritis (1 case) and hemorrhagic fever with renal syndrome (1 case). Eight children received corticosteroids and 4 of them received other immunosuppressants simultaneously.Nine children suffered from the infections.All of them had acute onset, and the main symptoms were hypertension (10/10 cases, 100.0%), headache and dizziness (5/10 cases, 50.0%), nausea and vomiting (5/10 cases, 50.0%), visual disturbance (3/10 cases, 30.0%) and convulsions by the ways of seizures definitely (9/10 cases, 90.0%). There was nothing positive in the examinations of the nervous system and fundus.Computer tomography examinations of 9 cases showed nonspecific low-density foci.The cranial MRI scan showed abnormal signals on the cerebral cortex of frontal lobe, parietal lobe and occipital lobe in all these 10 cases.The hyperintensities were observed on the fluid-attenuated inversion recovery sequences of all the 10 cases.Slight hyperintensities on diffusion-weighted images of 4 cases indicated that PRES progressed from reversible angiogenic edema to irreversible cytotoxic edema, meaning a poor prognosis.After blood purification treatment and reducing intracranial pressure, these 4 cases recovered.After timely treatment upon PRES diagnosis, patients had no recurrence and showed a good outcome.Conclusions:Children with kidney diseases and PRES also suffer from hypertension, and the treatment with immunosuppressive agents may precede the occurrence of PRES.Cranial MRI is important for the diagnosis of PRES.
ABSTRACT
Objective:To explore the clinical effect of mycophenolate mofetil combined with glucocorticosteroid on the children with anaphylatic purpura nephritis.Methods:70 cases of children treated and diagnosed as anaphylatic purpura nephritis in our hospital from March,2012 to September,2015 were enrolled in this study.They were randomly divided into the observation group and the control group.Conventional therapy was applied to both groups,the observation group was treated by mycophenolate mofetil combined with prednisone,the control group was given cyclophosphamide combined with prednisone.The total effective rate,disappearence time of clinical symptoms,biochemical indicators,immune function as well as the incidence of adverse reactions were compared between the two groups.Results:The total effective rate of observation group was 94.29％,which was 85.17％ in the control group,no statistical difference was found between two groups(P＞0.05).After therapy,the disappearence time of albuminuria,hematuria in observation group showed no significant difference compared with those of the control group,the level of 24 h urine protein quantitation and CD19+ of both groups were significantly decreased,and the level of CD19+ of observation group was significantly lower than that of the control group (P ＜0.05),the level of albumin,total protein and CD3+,CD3+CD4+ of both groups were significantly increased,and the level of CD3+,CD3+CD4+of observation group were significantly higher than those of the control group (P＜0.05).The incidence of adverse reactions in observation group was significantly lower than that of the control group (P ＜0.05).Conclusion:Mycophenolate mofetil combined with glucocorticosteroid was more effective and safe on the children with anaphylatic purpura nephritis than that of cyclophosphamide combined with prednisone.
ABSTRACT
Objective To explore the clinical feature of Kimura disease complicated with nephrotic syndrome in children. Methods The clinical data from 4 children with Kimura disease complicated with nephrotic syndrome were retrospectively analyzed. Results In all of the 4 male children, level of serum IgE was increased but level of eosinophils was not increased in peripheral blood. The renal pathological manifestations were different among them. Two cases had acute tubular injury, one case had pathological changes in repeated renal biopsy, and one case had a little of eosinophils infiltration in renal interstitium. All of them were sensitive to oral hormone treatment. Except that one case had no relapse yet in short follow-up period, the other 3 cases had relapsed and all manifested as positive urinary protein without lymphadenectasis. The 3 cases with recurrence were treated by combined immunosuppressive agents, the palindromia of two cases were reduced which were combined with tacrolimus. Conclusion The Kimura disease complicated with nephrotic syndrome in children has a long course and different renal pathological manifestations. It is sensitive to hormone treatment but easy to relapse, and the treatment should be combined with immunosuppressive therapy. Tacrolimus may have the effect on reducing relapse and delaying the progress of renal pathology.
ABSTRACT
Objective To screen and identify the main pro-angiogenic compounds of Shexiang Baoxin pill ( SBP) presenting in the plasma.Methods The pro-angiogenic effects of SBP and its compounds absorbed into blood were measured by the cell prolif-eration and cell migration assays by xCELLigence .And the cell tube formation and rat aortic ring models were established to evaluate their pro-angiogenic effect.Results SBP(10 -4 ~10 -2 μg/ml), ginsenoside Rg3(1 ~10 μmol/L) and ginsenoside Rh2(1 ~10μmol/L)significantly stimulated human umbilical vein endothelial cells (HUVECs) proliferation, migration and tube-like structures formation at different concentrations (P<0.05).In addition, compared to the control group, only the high concentration group of SBP (10 -2 μg/ml), Rg3(10 μmol/L) and Rh2(10 μmol/L)could induce endothelial cell sprouting from the aortic ring(P<0.05) .Conclusion SBP, ginsenosideRg3 and Rh2 exhibited significantly pro-angiogenic effect in vitro.
ABSTRACT
Objective To build hypoxia/reoxygenation injury model in cultured neonatal rat cardiomyocyte and screen active components from Shexiang Baoxin Pill ( SBP) absorbed in blood against hypoxia/reoxygenation injury .Methods Cardiomyocytes were isolated and purified from hearts of neonatal Sprague Dawley rats (1~3 days old) and were used to build hypoxia/reoxygenation injury model.The components of SBP absorbed in blood were screened by methyl thiazolil tetracolium (MTT) colorimetic method.Results SBP showed significant protective effect against cardiomyocytes hypoxia /reoxygenation injury atthe concentration of 50 μg/ml.Ginsen-oside Rb1, Rb2, bufalin and muscone of twenty components from SBP absorbed in blood also possessed significant protective effect a -gainst cardiomyocytes hypoxia/reoxygenation injury .Conclusion SBP have the protective activity against cardiomyocytes hypoxia /reoxygenation injury , and ginsenoside Rb1, Rb2, bufalin, muscone are the main active components of SBP .This experiment offered basis for further pharmacodynamics and mechanism study of SBP .
ABSTRACT
Objective To explore the effect of evidence-based nursing on ward rounds on nursing teaching.Methods Ten nursing students in the oncology department conducted their internship of word rounds on nursing teaching by evidence-based nursing. After the internship,the effect of internship was investigated.Results One hundred percent of the nursing students agreed that the evidence-based nursing could help them to master the methods for evidence-based nursing,promote them to actively participate in internship,learn how to get information and improve their ability in critical thinking ability. Besides,93.3% of them believed that it could arouse their interests in nursing scientific research.Conclusion Evidence-based nursing applied in ward rounds on nursing teaching in oncology department can improve the quality of care,explore the new mode of teaching ward rounds and improve the comprehensive ability of interns.
ABSTRACT
<p><b>OBJECTIVE</b>To investigate the role of ATP-binding cassette transporter G1 (ABCG1) in endothelial dysfunction induced by high glucose.</p><p><b>METHODS</b>Human aortic endothelial cells (HAECs) were incubated in the presence of 5.6 or 30 mmol/L glucose for 24-72 h with or without a 2-h pretreatment with the LXR agonist 22(R)-hydroxycholesterol. Real-time PCR and Western blotting were used to measure the mRNA and protein expressions of ABCG1; the intracellular cholesterol efflux and endothelial nitric oxide synthase (eNOS) activity were measured by scintillation counting.</p><p><b>RESULTS</b>High glucose time-dependently suppressed ABCG1 expression and cholesterol efflux to HDL in HAECs. High glucose also decreased eNOS activity. ABCG1 down-regulation induced by high glucose, along with decreased cholesterol efflux and eNOS activity, was abolished by treatment of the cells with the LXR agonist.</p><p><b>CONCLUSION</b>Endothelial dysfunction induced by high glucose is associated with decreased ABCG1 expression.</p>
Subject(s)
Humans , ATP Binding Cassette Transporter, Subfamily G, Member 1 , ATP-Binding Cassette Transporters , Genetics , Metabolism , Aorta , Cell Biology , Cell Line , Down-Regulation , Endothelial Cells , Cell Biology , Metabolism , Physiology , Glucose , PharmacologyABSTRACT
Objective To study the changed status of mortality from urinary and male genital diseases in Shanghai Hongkou district in recent years. Methods Mortality from diseases in Shanghai Hongkou district population from 1991 to 1998 was studied and analyzed. Results There were 1130 persons died of urinary and male genital diseases,749 being male and 381 female.It accounted for 2.08% of the total death,being 2.58% in the male and 1.51% in the female.The 5 major urinary and male genital diseases were in turn nephritis and nephropathy,bladder cancer,benign prostatic diseases,prostate cancer and renal carcinoma.Mortality from bladder cancer,renal cancer,prostate cancer and other prostatic diseases has been rising as compared to those in the 80s especially protatic cancer and other prostatic diseases whereas mortality from nephritis and nephropathy,pyelonephritis and renal failure was decreasing. Conclusions Mortality from prostate cancer and other prostatic diseases in population is remarkably rising.Attention is called to the study,prophylaxis and treatment of prostatic diseases.