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Objective:To summarize the clinical features, genetic testing and treatment efficacy of 6 children with Dent disease.Methods:Six children diagnosed with Dent disease in Guangzhou Women and Children′s Medical Center from January 2014 to March 2019 were enrolled.Their medical history, clinical manifestations, laboratory results, genetic test results, and proteinuria level, calciuria level and renal function after medication were measured.Results:All patients were male, with the onset age ranged from 1 to 9 years old.They were followed up for 6 months to 4 years.All the children had low molecular weight proteinuria.Urine protein electrophoresis showed that the ratio of low molecular weight proteinuria in only 2 cases was more than 50%.Renal biopsy suggested that all cases were combined with glomerular lesions.Five cases had hypercalciuria.Under the microscope, there were 5 cases of hematuria.Two case had rickets, and there was no renal calcium deposition and hypophosphatemia.Five cases were detected with CLCN5 mutations, of which p. C160Yfs*49 and p. G523D were first reported.One case had an OCRL1 mutation.Patients were treated with Hydrochlorothiazide and angiotensin converting enzyme inhibitor (ACEI). The 24 h urinary calcium level after treatment was lower than that before treatment [0.40 (0.24, 0.43) mmol/kg vs.0.12 (0.11, 0.14) mmol/kg, U=2.00, P<0.01]. However, there was no significant decrease in the 24 h-urinary protein level before and after treatment [77.09 (62.41, 88.01) mg/kg vs.80.33 (66.03, 92.52) mg/kg, U=12.00, P>0.05]. Conclusions:Dent disease is mainly characterized by low molecular weight proteinuria, and some patients may not be associated with hypercalciuria.Gene tests help to identify the disease type.ACEI and Hydrochlorothiazide can reduce the urinary calcium level, but cannot improve the level of urinary protein.
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Objective To assess efficacy, infection rate and recurrence rate of tacrolimus prescribed in infants with steroid-resistant nephrotic syndrome (SRNS). Method From August 2011 to August 2014, 22 cases of SRNS infants (treatment group) received oral tacrolinms treatment, 0.1 to 0.15 mg/ kg per day and once every 12 hours were enrolled in this retrospective longitudinal study and were compared with 23 cases infant SRNS (control group) treated with high-dose methylprednisolone pulse therapy. Followed up for 1 year we analysed the data of proteinuria, lymphocyte count, proteinuria relapse and complication (infection, hyperglycemia) of the two groups’ patients at every point time. Results The pathology of the patients maintains of MCD, MsPGN, FSGS and IgM nephropathy so on. Follow-up to 6 months, the total remission rate 95.45% of treatment group was significantly higher than that in control group (60.87%). Follow-up to 6 months , 24 h urinary protein of the treatment group were respectively 67.88 mg/(kg·d) which were remarkably lower than base line [657.5 mg/(kg·d)], meanwhile which were obviously lower than the 6th month point of control group [305.55 mg/(kg·d)]. Lymphocyte counts had been done during the initial and the destination in the treatment group. Follow-up to 12 months, the CD4+ 795.16/uL, CD8+ 496.85/uL, CD19+ 358.23/uL had decreased observably than when at origin what was 2697.45/uL, 2265.63/uL, 1579.34/uL. Followed-up 1 year, the person-time of infection of treatment group existed superior to the control groups; The recurrence rate was 71.43% in treatment group, which compared with control groups (60.87%) without no significant difference. The treatment group with BG and CCr maintained stably. Conclusion Tacrolimus show its own advantages of reliable effect and less side-effect on the infant with steroid-resistant nephrotic symdrome associated with genes , but it could not lessen the relapse of the disease, and it′s long-term prognosis is still not very clear.
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Objective To explore the effect of urine neutrophil gelatinase-associated lipocalin(uNGAL) and urine interleukin-18(uIL-18) on the ill condition and prognosis in critically ill patients with acute kidney injury (AKI) at inception of continuous veno-venous hemofiltration (CVVH).Methods Children came from Department of Nephrology,PICU and health examination center in Guangzhou Women and Children's Medical Center were divided into 4 groups:critically ill patients with AKI receiving CVVH group(group A),critically ill patients with non-AKI receiving CVVH group(group B),critically ill patients with AKI didn't recevie CVVH group(group C),and healthy control group(group D).Serum creatinine(SCr),uNGAL and uIL-18 in all patients were analyzed.Results The uNGAL in group A and group C [(161.56 ± 71.44) μg/L,(153.69 ±51.33) μg/L] increased obviously when compared with group B and group D [(33.50 ± l 0.76) μg/L,(16.37 ± 6.20) μg/L] (all P ＜ 0.05).The uIL-18 in group A and group C[(4.16 ±1.13) μg/L,(3.81 ± 1.05) μg/L] was higher than that in group B and group D [(0.25 ± 0.04) μg/L,(0.19 ± 0.15) μg/L] (all P ＜ 0.05).There was no significance of uNGAL and uIL-18 between group B and group D(all P ＞ 0.05).The peak level of uNGAL[(241.76 ± 53.60) μg/L vs (196.32 ± 39.28) μg/L] and uIL-18[(5.15 ±0.78) μg/L vs (4.30 ±0.89) μg/L] in critically ill patients with AKI was higher in renal recoveries than in renal non-recoveries(P ＜0.05).The levels of uNGAL and uIL-18 critically ill patients at initiation of CVVH were higher in non-survivors when compared with survivors [(213.50 ± 104.78) μg/L vs (79.91 ± 55.81) μg/L,P ＜ 0.05],[(4.48 ± 2.32) μg/L vs (1.94 ± 1.88) μg/L,P ＜ 0.05].The levels of uNGAL and uIL-18 of critically ill patients with AKI at initiation of CVVH were higher in non-survivors than in survivors [(256.99 ± 49.33) μg/Lvs (127.11 ±38.99) μg/L,P＜0.05],[(5.48±0.67) μg/Lvs (3.65 ±0.98) μg/L,P＜0.05].The levels of uNGAL and uIL-18 at the first diagnosis time of AKI were higher in non-survivors than in survivors (P ＜ 0.05).Conclusions uNGAL and urine IL-18 at commencement of CVVH predicts short-term prognosis in critically ill patients with AKI.uNGAL and urine IL-18 can be as a prognostic value in the prediction of the need for renal replacement therapy initiation or mortality in critically ill patients with AKI.
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Objective To test the predicative roles of neutrophil gelatinase-associated lipocalin (NGAL) and kidney injury molecule-1( KIM-1 ) for acute kidney injury (AKI) in critically ill patients. Methods Children from pediatric intensive care unit, were randomly divided into four groups:critically ill patients with AKI (group 1 ), critically ill patients with non-AKI (group 2) , chronic kidney disease group (group 3), healthy control group (group 4). 1.5 ml venous blood and urine specimens were collected and kept under-70°C. Serum creatinine , urine NGAL and urine KIM-1 were analyzed. Results Compared with group 2, group 3 and group 4, the urine NGAL and urine KIM-1 increases obviously in group 1 (P0.05). The concentration of urine NGAL increased more than 10 times of base-line level 2 days before the diagnosis of AKI under the Acute Kidney Injury Network standard with area under curve (AUC) 0.955 (P<0.05) , and the concentration of urine KIM-1 increased more than 5 times of base-line level 1 day before AKI with AUC 0.878 (P<0.05). The AUC was 0.984 (P<0.01) when they were combined. There is negative correlation between the increased times of urinary KIM-1, urinary NGAL and vally value of creatin clearance rate. Conclusions The concentrations of urine NGAL and urine KIM-1 are useful early biomarkers for predicting AKI, especially when they were combined.
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Objective To investigate the early diagnostic value of serum Cystatin C in the diagnosis of acute kidney injury (AKI) in critically ill children.Methods Ninety-eight children of the severe case patients' rooms were divided into two groups:33 cases were AKI,65 cases were NAKI.Cystatin C and SCr in blood serum were detected by immunoturbidimetry and enzymic method every day.And compared serum Cystatin C,SCr concentrate and median diagnosis time between the two groups.Results Thirty-three cases in 98 cases of critically ill children occurred the AKI,65 patients without AKI.Comparing with the NAKI,the Cystatin C of AKI patients were significantly higher ((2.68 ±0.86) mg/L vs (0.76 ±0.15) mg/L) and SCr ((209.21 ± 100.53 ) μmol/L vs (77.46 ± 8.11) μmol/L),the differences were statistically significant (t =10.55,17.56,P ＜0.001) ; Cystatin C was in positive correlation with SCr (r =0.874,P ＜ 0.001) ; Median diagnosis time of AKI-Ⅰ,Ⅱ,and Ⅲ stage with Cystatin C were (3.0 ± 2.5) d,(5.0 ± 3.0) d and (9.0 ± 4.0) d,respectively,which was earlier than that of SCr ((5.0 ± 2.0) d,(7.0 ± 2.5) d and (10.0 ± 3.0) d,respectively; t =4.39,2.29,3.16,respectively ;P ＜ 0.01 or P ＜ 0.05).Conclusion In the process of AKI,the level of serum Cystatin C elevated significantly earlier than SCr,so it could as be one of the early dynamic criteria in the critically ill children.
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Primary nocturnal enuresis is a common childhood disorder.This review discusses the progress on the treatment of primary nocturnal enuresis in children.Alarm interventions for nocturnal enuresis and desmopressin are the first-line therapy of primary nocturnal enuresis.In therapy-resistant cases,anticholinergic treatment combined with desmopressin can be tried.In situations when all other treatments have failed,imipramine treatment is warranted,provided the cardiac risks are taken into account.More research is needed regarding both the above drugs and non-pharmacological therapies on children with therapy-resistant enuresis.
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Objective In order to know the influence of psychological nursing intervention on rehabilitation of children with primary nocturnal ensuresis. Methods Divided 112 children with primary nocturnal ensuresis into the research group (40 cases) and the control group (72 cases). Routine treatment and nursing cares were used in the control group, while the psychological nursing was used in the research group in addition. Compared the treatment effect betweent the two groups. Results The efficient rate in the research group was 90.0%, which higher than that of in the control group significantly. Conclusions Psychological nursing intervention can effective promote the rehabilitation for children with primary nocturnal ensuresis.