Genetic spectrum of CAKUT and risk factors for kidney failure: a pediatric multicenter cohort study.

BACKGROUND: Congenital anomalies of the kidney and urinary tracts (CAKUT) are the leading cause of kidney failure in children with phenotypic and genotypic heterogeneity. Our objective was to describe the genetic spectrum and identify the risk factors for kidney failure in children with CAKUT. METHODS: Clinical and genetic data were derived from a multicenter network (Chinese Children Genetic Kidney Disease Database, CCGKDD) and the Chigene database. A total of 925 children with CAKUT who underwent genetic testing from 2014 to 2020 across China were studied. Data for a total of 584 children wereobtained from the CCGKDD, including longitudinal data regarding kidney function. The risk factors for kidney failure were determined by the Kaplan-Meier method and Cox proportional hazards models. RESULTS: A genetic diagnosis was established in 96 out of 925 (10.3%) children, including 72 (8%) with monogenic variants, 20 (2%) with copy number variants (CNVs), and 4 (0.4%)with major chromosomal anomalies. Patients with skeletal abnormalities were more likely to have large CNVs or abnormal karyotypes than monogenic variants. Eighty-two patients from the CCGKDD progressed to kidney failure at a median age of 13.0 (95% confidence interval, 12.4-13.6) years, and twenty-four were genetically diagnosed with variants of PAX2, TNXB, EYA1, HNF1B and GATA3 or the 48, XXYY karyotype. The multivariate analysis indicated that solitary kidney, posterior urethral valves, bilateral hypodysplasia, the presence of certain variants and premature birth were independent prognostic factors. CONCLUSIONS: The genetic spectrum of CAKUT varies among different subphenotypes. The identified factors indicate areas that require special attention.

[Association of drug resistance of Mycoplasma pneumoniae with DNA load and genotypes in children with Mycoplasma pneumoniae pneumonia].

OBJECTIVE: To investigate the association of drug resistance of Mycoplasma pneumoniae (MP) with DNA load and genotypes in children with MP pneumonia. METHODS: A total of 230 children who were hospitalized and diagnosed with MP pneumonia between January 2012 and December 2016 were enrolled. Throat swabs were collected from the 230 children, and a rapid drug sensitivity assay was used to determine the sensitivity of clinical isolates of MP to nine commonly used antibacterial agents. Quantitative real-time PCR was used to measure MP-DNA load in throat swabs. PCR sequencing was used to determine the genotype of 2063 locus of the MP 23S rRNA V domain. RESULTS: Of the 230 children, 86 (37.4%) had genotype A in 2063 locus, 134 (58.3%) had genotype G, 8 (3.5%) had genotype C, and 2 (0.9%) had genotype T. Mutant strains (genotype G+C+T) had a significantly higher MP-DNA load than wild-type strains (genotype A) (P<0.05). The strains resistant to erythromycin, azithromycin, clarithromycin, and clindamycin had a significantly higher MP-DNA load than non-resistant strains (P<0.05). MP had a high drug resistance rate to macrolide antibiotics. More than 60% of the cases with resistance to macrolides were found to have A2063G mutations. MP was rarely resistant to quinolones (less than 2%). CONCLUSIONS: Mutations in 2063 locus of the MP 23S rRNA V domain may result in the resistance of MP to macrolides and the change in DNA load and can be used as a basis for selecting drugs for MP.

A Study on Clinical Screening of Neonatal Congenital Heart Disease in Jinjiang City.

OBJECTIVE: This study explored the feasibility of congenital heart disease (CHD) screening by combining a percutaneous oxygen saturation (POX) test with cardiac auscultation method in neonates. METHODS: POX tests and cardiac auscultation were used concurrently to screen 8305 neonates born in Jinjiang City Hospital between January 2016 and December 2018 for CHD. The positive screening results (positive POX or positive cardiac auscultation) were confirmed with echocardiography, while any false negative results were identified through follow-up and parent feedback. Sensitivity, specificity, positive/negative predictive values, Youden's index, and the area under the receiver operator characteristic curve (AUC) of the single use and combined use of the two methods (a POX test and auscultation) were calculated, and the results were compared. RESULTS: Among 8305 neonates, 22 cases were positive for POX alone, of which 6 cases were diagnosed by echocardiography; 83 cases were positive for cardiac auscultation alone, of which 47 cases were diagnosed by echocardiography; and 8 cases were positive for both methods, all of which were confirmed by echocardiography. Four more cases were confirmed during follow-up. Sensitivity, specificity, and the positive and negative predictive values of combined screening were 93.85%, 99.37%, 53.98% and 99.95%, respectively, while Youden's index was 0.93, and the AUC was 0.966. Sixty-five cases of CHD were diagnosed, the total incidence being 7.82%, and a ventricular septal defect was found to be the most common type. CONCLUSION: The combination of POX test and cardiac auscultation as a screening method for neonatal CHD can reduce missed diagnoses and increase the detection rate of CHD in newborn infants.

[Application and Research Progress of Lineage Tracing in Differentiation Mechanism of Hematopoietic Stem Cells--Review].

Hematopoietic stem cells (HSCs) reside at the top of the hierarchy and have the ability to differentiate to variety of hematopoietic progenitor cells (HPCs) or mature hematopoietic cells in each system. At present, the procress of HSC and HPC differentiating to the complete hematopoietic system under physiological and stressed conditions is poorly understood. In vivo lineage tracing is a powerful technique that can mark the individual cells and identify the differentiation pathways of their daughter cells, it takes as a strong technical system to research HSC. Traditional lineage tracing studies mainly rely on imaging techniques with fluorescent dyes and nucleic acid analogs. Recently, newly cell tracing technologies have been invented, and the combination of clonal tracing and DNAsequencing technologies have provided a new perspective on cell state, cell fate, and lineage commitment at the single cell level. In this review, these new tracing methods were introduce and discuss, and their advantages over traditional methods in the study of hematopoiesis were summarized briefly.

rhTPO combined with chemotherapy and G-CSF for autologous peripheral blood stem cells in patients with refractory/relapsed non-Hodgkin's lymphoma.

OBJECTIVE: The mobilization and collection of sufficient autologous peripheral blood stem cells (APBSCs) are important for the fast and sustained reconstruction of hematopoietic function after autologous transplantation. This study aims to evaluate the mobilization effect and safety of thrombopoietin (TPO) combined with chemotherapy + G-CSF for APBSCs in patients with refractory/relapsed non-Hodgkin's lymphoma. METHODS: A total of 78 patients were included in the present study. After receiving mobilization chemotherapy, all patients were randomly divided into two groups: TPO group (n=40), patients were given subcutaneous injection of rhTPO + G-CSF, and control group (n=38), patients were given subcutaneous injection of G-CSF. The primary endpoint was the total number of obtained CD34+ cells. The secondary endpoints were the mononuclear cell count, the proportion of target and minimum mobilization, the engraftment time of neutrophils and platelets after APBSCT, the number of platelet and red blood cell infusions, the incidence of infectious fever and fever duration, and TPO-related side effects in patients. RESULTS: TPO participation significantly increased the total CD34+ cell count. A higher proportion of patients in the TPO group achieved the minimum and target CD34+ cells, when compared to the control group. TPO-related adverse events were not observed in either of these groups. In addition, there were no significant differences in engraftment time, the number of platelet and red blood cell transfusions, the incidence of infectious fever, and fever duration between these two groups. CONCLUSION: TPO combined with chemotherapy + G-CSF can safely and effectively enhance the mobilization effect for APBSCs in patients with refractory/relapsed non-Hodgkin's lymphoma.

[Effect of catechin microcapsule on the repair of DNA damage in glomerular mesangial cells induced by H2O2].

OBJECTIVE: To explore the effect and possible mechanism of catechin microcapsulation on the repair of DNA damage in glumreular mesangial cells (GMCs) induced by H2O2. METHODS: According to H2O2 concentration, the experiment GMCs were divided into 6 groups: a control group, 50 micromol/L group, 100 micromol/L group, 150 micromol/L group, 200 micromol/L group and 250 micromol/L group. Each group was sub-divided into 3 groups: 6 h group, 12 h group and 24 h group, in order to determining the optimum dose and the best time of detecting the DNA damage in GMCs. The cultured cells were divided into 8 groups as follows: the NS control group, the H2O2 group, the catechin groups (the final concentrations were 10.0, 15.0, and 20.0 mg/L respectively) and the various catechin microcapsulation groups (the final concentrations were 10.0, 15.0, and 20.0 mg/L respectively). At the end of the experiment, hydroxy radical (OH), malonydialdehyde (MDA) and total superoxide dismutase (tSOD) concentration of supernadant in GMCs were determined by biochemistry assay, the repair of DNA damage in GMCs were detected by single cell gel electrophoresis assay. RESULTS: (1)At 6th h, H2O2 of 100 micromoL/L could cause the DNA damage of GMCs, and H2O2 of 150 micromol/L could result in DNA damage significantly. (2) No difference was found in the comet span of GMCs DNA in the catechin group and catechin microcapsulation group of different concentrations, while the DNA comet tail-long in the catechin microcapsulation group was shorter than that of the catechin group(all P(s)<0.05), and the fluorescence intensity of tail in the catechin microcapsulation group was lower than that of the catechin group(all P(s)<0.01). (3)When the concentration of catechin was 10.0 mg/L, no statistical significance was obtained in the concentration of dOH-, MDA and tSOD between the catechin microcapsulation group and the catechin group; while dOH- and MDA concentrations were lower, and the tSOD was higher in the catechin microcapsulation group than that in the catechin group when the concentration of catechin was 15.0 mg/L and 20.0 mg/L(all P(s)<0.05). CONCLUSION: Catechin microcapsulation can enhance the GMCs ability of repairing DNA damage,which may be due to elevating the capacity of its anti-oxidation by catechin microcapsulation.

[Pathologic features and prognosis of 21 children with isolated proteinuria].

OBJECTIVE: To discuss the pathologic features, treatment and prognosis of the children with isolated proteinuria (IP). METHODS: Twenty-one children with IP were enrolled according to their renal biopsy and were followed up for 0.5 to 10 years. RESULTS: Renal biopsy was performed in all children. Among them 13 were mesangial proliferation glomerulonephritis (MsPGN) (including 3 minor, 6 moderate, and 4 severe ones), 2 minimal change nephritis (MCN), 3 IgA nephropathy (IgAN) (1 in Grade I and 2 in Grade II), 2 focal segmemtal glomerulosclerosis (FSGS) and 1 endocapillary proliferative glomerulonephritis (EnPGN). Interstitial changes could be found in MsPGN and FSGS mostly, presenting interstitial fibrosis, infiltration of inflammatory cells, atrophy of renal tubule, and the vacuolar degeneration of epithelia. All children accepted the medical treatment except the EnPGN case. Fifteen children recovered with no relapse; proteinuria persisted in 3 severe MsPGN and FSGS cases; 2 got the impaired renal function accompanied by persistent proteinuria; and 1 had hypertension. CONCLUSION: The different degrees of renal damage can be found in all IP children who have persistent proteinuria. Most patients can get good outcome after aggressive therapies. However, the prognosis of those with severe MsPGN and FSGS was not so optimistic, and some reno-protective treatments should be given to postpone the deterioration of the renal function.

[Role of caspase-1 and cytokines activated by caspase-1 in brain injury of the developing rats following recurrent seizures].

OBJECTIVE: The expressions of caspase-1 and cytokines activated by caspase-1 are associated with the pathophysiology of many diseases for its proinflammatory and proapototic peculiarity. However its relationship to brain injury of developing rats following recurrent seizures has not yet been identified. This study aimed to investigate the role of caspase-1 and cytokines activated by caspase-1 in brain injury of developing rats following recurrent seizures. METHODS: A total of 96 postnatal 20 day Sprague-Dawley rats were randomly assigned into Control and Seizure groups. Seizures were induced in the Seizure group by flurothyl inhalation daily for six days. Brain tissues were sampled at 6 hrs, and at 1, 3, and 7 days after last seizure. The expressions of caspase-1, interleukin (IL)-18 and IL-1beta mRNA in the cerebral cortex were detected by RT-PCR. The water content of the brain and the pathological changes of cortex nerve cells were observed. Brain injury was evaluated using a semiquantitative neuropathological scoring system. RESULTS: The levels of caspase-1 and IL-18 mRNA in the cerebral cortex of the Seizure group were obviously higher than those in the Control group at 6 hrs, and at 1, 3, and 7 days after seizure (P < 0.05 or P < 0.01). The expression of IL-1beta mRNA in the Seizure group exhibited a biphasic pattern: increased significantly at 6 hrs, and at 1 and 7 days post-seizure (P < 0.01), but was not significantly different from the Control group at 3 days post-seizure. Edema, degeneration and necrosis of nerve cells in cerebral cortex, accompanying by inflammatory cell infiltration and apoptosis of nerve cells, were observed under a light microscope in the Seizure group after recurrent seizures. The water content of the brain in the Seizure group increased significantly compared with that in the Control group at 6 hrs, and at 1 and 3 days after recurrent seizures (P < 0.01). The Seizure group had significantly higher neuropathological scores than the Control group at each time point (P < 0.01). CONCLUSIONS: Caspase-1 and cytokines activated by caspase-1 play an important role in the developing brain injury after recurrent seizures.

[Rearrangement and altered expression of actin in macrophages induced by Mycobacterium tuberculosis].

OBJECTIVE: To investigate the effect of M. tuberculosis infection on actin in host-cells. METHODS: The form and distribution of fibrous actin and changes of actin expression were observed by confocal microscopy and Western blot analysis in macrophages infected with either M. tuberculosis H(37)R(a) or H(37)R(v) at 6 h, 12 h and 24 h after infection. Non-infected macrophages or macrophages treated with dead M. tuberculosis H(37)R(v) served as controls. RESULTS: F-actin aggregated and the actin expression was suppressed in macrophages infected with H(37)R(a) or H(37)R(v), and the effect appeared earlier in cells infected by the virulent H(37)R(v) strain than in cells infected by the avirulent H(37)R(a) strain. In macrophages treated with the dead H(37)R(v), actin was not affected. CONCLUSION: The results suggest that in the process of infection, M. tuberculosis evades the bactericidal mechanisms possibly by secretion of certain proteins or factors which affect the host-cell actin.

[The role of FDG-PET in staging of lymphoma and evaluation of therapeutic efficiency].

OBJECTIVE: To evaluate the application of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) to the staging and detecting residual masses of lymphoma. METHODS: The clinical data of 179 patients with lymphoma were analyzed retrospectively. The results of FDG-PET, computed tomography (CT) and bone marrow biopsy (BMB) were compared for detection of lymph node/extranodal lymphoid tissue and bone marrow infiltration. Therapeutic efficiency was assessed by International Workshop Criteria (IWC) and Revised Integrated International Workshop Criteria (IWC + PET). RESULTS: In the detection of 286 disease focuses in 98 patients before chemotherapy, the sensitivities of FDG-PET and CT were 73% and 70% (P < 0.01) in detecting nodal focus,and 87% and 45% (P < 0.01) in detecting extranodal lymphoma respectively. In detection of 104 lesions in 81 patients after chemotherapy,the sensitivities of FDG-PET and CT were 81% and 55% respectively (P < 0.01), and the specificities were 68% and 33%, respectively (P < 0.01) in detecting residual masses. According to IWC criteria, 33 patients achieved complete response/unconfirmed complete response (CR/CRu) , and 8 (24%) relapsed. Patients with PET-positive residual masses had a relapse rate of 40%, whereas only 21% of those with no such masses relapsed. Based on IWC + PET criteria, 25 patients achieved CR, with a relapse rate of 20%. Both FDG-PET and BMB produced positive results in 133/179 (74%) patients. Twenty-two patients with positive FDG-PET results were not detected by BMB. The sensitivities and specificities of FDG-PET for BM infiltration were 52% and 83%, respectively. CONCLUSIONS: FDG-PET is a high sensitive and specific technique in staging and detecting residual masses of lymphoma.

[Clinical and mutational study of a Chinese infant with isovaleric acidemia].

OBJECTIVES: Isovaleric acidemia (IVA) is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD) resulting in the accumulation of derivatives of isovaleryl-CoA. IVA is considered to be a severe, potentially life-threatening disorder that manifests with acute neonatal encephalopathy in approximately half of affected individuals, and recurrent episodes of vomiting, lethargy, coma and varying degrees of developmental delay in the other half of patients. This study was conducted to investigate the clinical features and IVD gene mutations of a Chinese patient with IVA. METHODS: The clinical features, routine laboratory data, blood amino acid and acylcarnitine profiles and urinary organic acid profiles of a patient with IVA were reviewed. Whole coding exons of IVD gene were PCR-amplified for DNA sequencing. The novel mutation c.466G > C (G127A) was confirmed by RFLP with restriction endonuclease Hph I. RESULTS: The patient was a 2 year and 7 month-old boy. At 3 days of age, he began to show severe vomiting and acidosis. He was treated with pyloromyotomy at 10 days of age. His recurrent vomiting was not ameliorated until beginning transition to a diet that included more carbohydrate from 4 months. He had 3 recurrent severe vomiting and acidosis later and showed obvious psychomotor retardation. Blood spot acylcarnitine profiles by MS-MS demonstrated an elevation of C5-carnitine with a peak concentration of 12.89 micromol/L (< 0.5 micromol/L). Organic acid analysis of urine by GC-MS revealed a relatively high level of isovaleric glycine. Mutational analysis of the patient's IVD gene revealed heteroallelic mutations of c.149G > A (R21H) and c.466G > C (G127A) which is a novel missense mutation. G127A mutation was not detected in any of 50 normal controls. CONCLUSIONS: From the clinical course, obvious elevation of blood C5-carnitine and urine isovaleric glycine, this patient's disorder should be classified as "metabolically severe" type of IVA which suggest that c.466G > C (G127A) mutation could severely damage the function of the IVD protein. To our knowledge, this is the first characterization of IVD gene mutations in the mainland of China.

[Clinical significance of dynamic monitoring of cell chimerism following allogeneic hematopoietic stem cell transplantation].

OBJECTIVE: To evaluate the relationship of chimerism status of cell subsets with engraftment, occurrence of acute graft versus host disease (aGVHD), graft rejection and disease relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT). METHODS: Chimerism status in peripheral blood (PB) and bone marrow (BM) of 65 patients received allo-HSCT were monitored at regular intervals post-transplant. Fluorescence-activated cell sorter (FACS) was used to sort CD3(+)T lymphocytes in 65 cases, CD3(-)CD56(+)CD16(+)NK cells in 52 cases, CD15(+) granulocytes in 32 cases and CD19(+)B lymphocytes in 20 cases post transplants. The chimerism status of different lineage cells was analyzed by polymerase chain reaction amplification of short tandem repeats (PCR-STR). RESULTS: On day +7, NK-cells donor chimerism (DC 55.5%) was higher than other cell subsets. T lymphocyte was the latest one to reach complete donor chimerism (CDC) with a median on day +21. Patients whose T lymphocytes donor chimerism was more than 70% on day +7 and more than 95% on day +14 had a high risk for acute aGVHD. In all cases except those with ALL, the decreased DC of T lymphocytes were observed before molecular or hematological relapse occurred. CONCLUSION: Serial and quantitative T cell chimerism analysis provides a reliable and rapid screening method for the early detection of engraftment, graft rejection, disease relapse and occurrence of aGVHD, therefore, is a prognostic tool to identify patients at high risk of aGVHD and disease relapse following allo-HSCT.

[Prognostic factor analysis of 77 old patients with acute myelogenous leukemia].

BACKGROUND & OBJECTIVE: The manifestations of old acute myelogenous (AML) patients have their special biological and clinical characteristics, with lower response rate to therapy and shorter survival time. This study was to investigate the prognostic factors of elderly patients with AML retrospectively. METHODS: 77 patients aged> or =60 years with AML from 1994 to 2005 were admitted to our study and all the possible prognostic factors were analyzed with Kaplan-Meier survival analysis. The significant factors were further analyzed by Cox proportional hazard model analysis. RESULTS: Seventy-two patients were evaluated. The patients aged 60-70 (median survival time was 350 days) had significantly longer survival time than those aged more than 70 (median survival time is 60 days)(P<0.001), which their CR ratios were 71.4% and 29.4% (P=0.001). The patients with performance status 0 or 1 (median survival time was 402 days) had significantly longer survival time than those with performance status 2, 3 or 4 (median survival time was 31 days)(P<0.001), which their CR ratios were 75% and 15% (P<0.001). The patients with primary AML (median survival time was 98 days) had significantly longer survival time than those with secondary AML (median survival time was 32 days)(P=0.007), which their CR ratios were 50% and 0% (P=0.023). The patients treated with sub-standard dosage of anthracycline (median survival time was 293 days) had significantly longer survival time than those treated with reduced dosage of anthracycline (median survival time was 35 days)(P=0.006), which their CR ratios were 63.6% and 33.3% (P=0.02). The patients with bone marrow blast cell ratio< or =50% (median survival time was 98 days ) had significantly longer survival time than those with bone marrow blast cell ratio >50% (median survival time was 55 days)(P=0.006). The patients with favorable karyotype (median survival time was 293 days) had significantly longer survival time than those with unfavorable or normal karyotype (median survival time was 31 days)(P=0.005). The patients without CD34 expression (median survival time was 201 days) had significantly longer survival time than those with CD34 expression(median survival time was 36 days)(P<0.001). The patients with the peripheral blood white blood cell count (PBWBC)>10x10(9)/L (50%) had significantly higher CR ratio than those with PBWBC< or =10x10(9)/L (25%)(P=0.043). The patients received chemotherapy (50%) had significantly higher CR ratio than those received supportive therapy (0%)(P=0.001). In the stepwise COX proportional hazard regression model, all the seven factors related to OS remained independent and significant. CONCLUSIONS: Factors, including age >70, PS 2 to 4, percentage of blasts in bone marrow >50%, secondary AML, unfavorable karyotype, expression of CD34, lower dosage.

[Effect of glucocorticoid on glucocorticoid-resistant children with primary nephrotic syndrome].

OBJECTIVE: Glucocorticoid (GC) is the first therapeutic choice of primary nephrotic syndrome (PNS). The response to GC treatment is an important indicator for the outcome of PNS children. Children with GC-resistant PNS present with incomplete or no response to GC, and may herald the progression to end-stage renal failure. However, the detailed mechanism of GC-resistance or GC-sensitive effect in these PNS children has not been clearly elucidated. The previous study by the authors indicated that there was increased expression of GR beta in PBMCs in GC-resistant children with PNS, and the over expression of GR beta resulted in GC resistance via influencing the ability of GR alpha nuclear translocation. To elucidate the relationship between GR beta expression in renal and in PBMCs and the effect of glucocorticoid on glucocorticoid-resistance children with PNS, the expression of GR alpha and GR beta in renal tissue and in PBMCs were detected by immunohistochemistry. METHODS: Forty children with PNS were divided into two groups, GC-resistant group(20) and GC-sensitive group(20), the expression of GR alpha and GR beta in renal intrinsic cells and in PBMCs were measured with the immunohistochemistry technique. A semiquantitative score was used to evaluate the injury degree of the glomeruli and tubulointerstitium. RESULTS: Compared with GC-sensitive group, the glomerular pathologic scores (6.91 +/- 1.98) and renal tubular pathologic scores (7.12 +/- 1.62) in GC- resistant group were significantly different (P < 0.01, respectively). GR alpha expressions of renal tissue and PBMCs were higher in the control group (58.3 +/- 2.6, 59.1 +/- 7.2) than those in the GC-sensitive group (40.2 +/- 7.2 and 36.6 +/- 5.1, P < 0.01, respectively) and GC-resistant group (35.0 +/- 8.2 and 36.4 +/- 6.6, P < 0.01, respectively). GR beta expressions of renal tissue and PBMCs were higher in the GC-resistant group (13.8 +/- 3.0 and 12.1 +/- 4.1) and in the GC-sensitive group (6.5 +/- 1.9 and 5.9 +/- 1.0) than that in control group (2.3 +/- 0.4 and 3.2 +/- 1.1, P < 0.01, respectively). GR beta expressions in renal tissue and PBMCs were higher in the GC-resistant group than that in the GC-sensitive group (P < 0.01). Compared with control group, GR beta expressions in PBMCs and in renal tissue were lower than those in mild renal lesion group (5.4 +/- 2.8, 6.46 +/- 2.50), midmedium renal lesion group (8.7 +/- 2.4 and 11.4 +/- 3.7) and (17.1 +/- 0.4 and 18.7 +/- 0.7) in severe renal lesion group (F = 5.8, 15.6, P < 0.01, respectively). GR beta expression of PBMCs had a positive correlation with GR beta expression of renal intrinsic cells (r = 0.651, P < 0.01). GR beta expressions by PBMCs and renal intrinsic cells were positively correlated with renal pathologic scores (r = 0.579 and 0.623, P < 0.01, respectively). CONCLUSION: GC-resistant children with PNS were related to the increased GR beta expression in PBMCs and renal intrinsic cells. There was no correlation between the GR alpha expressions in PBMCs and in renal intrinsic cells. Increased GR beta expression might decrease the effect of GC via inhibiting the activity of GR alpha.