The Epidemiological Characteristics Of Children With Nephrotic Syndrome And Analysis Of Relapses And Frequent Relapses Related Factors

PartⅠ The epidemiological characteristics of childrenwith primary nephrotic syndromeObjectiveThis article analysis the relevant clinical information and laboratory parameters of children withPNS in our hospital and determine the epidemiological characteristics.MethodAll the research objects were come from Guangzhou Women and Children Medical Centermedical record system in January2002to December2011. All the children were under theage of16and diagnosed in our hospital. There are1374cases were included and recordthe onset of clinical data and laboratory test results. Count data using the chi-square test,measurement data using t test for analysis.Results1. There are1374patients were included in this study. The patients diagnosis and treat in our hospital were1039cases,826cases of male, female223cases, male:female ratio was3.66:1, with a median age of onset was2.8years(25%-75%:2.0-4.1years). In triglycerides, high density lipoprotein, apolipoprotein A andapolipoprotein B in the men’s and women’s difference was statistically significant (P<0.05).2. There are1138patients have clear time of onset, which showed seasonaldistribution features. Spring:334(29.35%) cases, summer:305(26.8%) cases, Fall:231(20.3%) cases, winter:268(23.55%) cases.3. In renal biopsy of69patients, MCD:56.52%(39/69), MsPGN:31.88%(22/69),FSGS:5.80%(4/69), MGA:2.90%(2/69) MN and Crescentic glomerulonephritis were1.45%(1/69). MCD and MsPGN, in the different group of cases the difference was notstatistically significant.ConclusionsThe incidence of male with PNS was significantly higher than female. The peak onsetwas2-4.1years. The incidence of children with PNS has seasonal characteristics of thedistribution. Part Ⅱ The analysis of relapses and frequent relapses related factors in childrenwith primary nephrotic syndromeBackground and objectivePrimary nephrotic syndrome (PNS) is a common pediatric urinary system disease,ranked first in the inpatient pediatric urinary system diseases. PNS was the common causeof progression to end-stage renal disease (ESRD), so the children with PNS is a majorthreat to public health and affect a child’s future quality of life. With the conduct ofpediatric renal biopsy, and the application of new immunosuppressive agents in thepediatric, the prognosis of children with PNS has a marked improvement. The commonclinical pathological is minimal change disease, it has a high relapse rate and duration ofthe disease related long. How to reasonable choice of drugs to maintain long-termremission of nephrotic syndrome in children, slowing the progress of renal function, hasbecome the most important issue of the treatment of children with PNS. This articleanalysis the relapses and frequent relapses related factors of children with PNS. Andprovide the practical method for prognosis prediction and reference for clinical treatment.Method1. All the research objects were come from Guangzhou Women and Children MedicalCenter medical record system in January2002to December2011. All the children wereunder the age of16and diagnosed in our hospital. Record the onset of clinical data andlaboratory test results.2. Collected a total of1374cases, exclusion of the self-withdrawal or self-less drugcause relapse cases. All the children followed up over1year. Altogether350cases werecollected,78cases without relapse,177cases of non-frequency relapse,95cases offrequency relapse. Count data using the chi-square test, measurement data using t test foranalysis. The risk factors of continuous variables significantly deemed to frequent relapsing by univariate analysis were further analyzed with the Receiver OperatingCharacteristic (ROC) curve to determine the cut-off value. The continuous variables wereconverted into categorical variables at the cut-off value. Risk factors that were deemed tocontribute significantly to FR after univariate analysis were further analyzed using themultivariate logistic regression model.Results1. In study PNS relapse of univariate analysis found that24-hour urinary proteinexcretion, serum albumin, serum total cholesterol, low density lipoprotein, erythrocytesedimentation rate and blood IgG in the relapse group and no-relapse group difference wasstatistically significant, variable binary logistic regression analysis found:24h urineprotein≥86mg/kg (OR: OR：2.592，95%CI：1.364~4.926；P=0.004), serum total cholesterol≥10mmol/L (OR：2.256，95%CI：1.131-4.499；P=0.021), IgG <1.5g/L (OR：3.533，95%CI：1.150-10.859；P=0.028) were children PNS independent risk factors for recurrence.2. In study PNS frequency of risk factors for recurrence in the univariate analysis wasfound that clinical classification, serum total protein, low-density lipoprotein, erythrocytesedimentation rate in the non-relapse group and the relapse of recurrence between the twogroups was statistically significant difference of multivariate. And binary LogisticRegression analysis found that TP <33g/L (OR：4.014，95%CI：1.684~9.570；P=0.002),LDLC≥6.7mmol/L (OR：4.240，95%CI：2.246~8.003；P=0.001) were the independentrisk factors for children PNS relapse frequency.Conclusions1. The24h urine protein≥86mg/kg, blood cholesterol≥10mmol/L, IgG <1.5g/Lare independent risk factors of PNS relapse in children.2. Total protein <33g/L, LDLC≥6.7mmol/L are independent risk factors of PNSfrequency relapse in children. PartⅢ The genetic diagnosis of refractory nephrotic syndromeThe genetic diagnosis of lipoprotein glomerulopathyObjectiveTo analyze the mutation of apolipoprotein E (apo E) aim to investigate the relativegene mutation.MehodsDetermine the patient’s blood, urine, hepatic function, renal function, and serumlipoprotein levels. Take3ml peripheral blood of the patient and her uncle, extracted fromperipheral blood leukocyte DNA; the patient and her uncle’s apoE coding region of exons3and4were PCR-amplified to determine the mutation sites.ResultsThe patient is carrying the heterozygous of apoE (Arg25Cys,) point mutation, whilethe patient’s uncle and five cases of normal control group did not carry any gene mutation.ConclusionThe case of lipoprotein glomerulopathy is carrying the apoE (Arg25Cys,) geneheterozygous mutation. The genetic diagnosis of Denys-Drash syndromeObjectiveThis article was analysis the WT1gene of one patient who diagnosed as Denys-Drashsyndrome aim to found the gene mutation.MehodsDetermine the patient’s blood, urine, hepatic function, renal function, serumlipoprotein levels and sex hormone levels. Take3ml peripheral blood of the patient and heruncle, extracted from peripheral blood leukocyte DNA. The patient WT1coding region ofexons1to10were PCR-amplified to determine the mutation sites.ResultsThe clinical manifestations of this Children with consistent with nephrotic syndrome, malepseudohermaphroditism, WT1gene mutations detected as His377Tyr.ConclusionThe patient of Denys-Drash syndrome was His377Tyr heterozygous mutation.