Clinical and genetic characteristics of a young child with combined pituitary hormone deficiency type I caused by POU1F1 gene variation / 中国当代儿科杂志

This paper reports the clinical and genetic characteristics of a case of combined pituitary hormone deficiency type I (CPHD1) caused by POU domain, class 1, transcription factor 1 (POU1F1) gene variation. A 2 years and 3 months old girl mainly presented with short stature, special facial features of prominent forehead, enophthalmos, and short mandible, loose skin, central hypothyroidism, complete growth hormone deficiency, and anterior pituitary hypoplasia. Gene analysis identified a novel heterozygous mutation, c.889C>T (p.R297W), in POU1F1 gene, and this locus of her parents was wild-type. This mutation was analyzed as a possible pathogenic variant according to the guidelines of the American College of Medical Genetics and Genomics, which has not been previously reported in the literature and conforms to the autosomal dominant inheritance. This child was diagnosed with CPHD1. Her height increased by 19.8 cm and showed a catch-up growth trend after one year of combined treatment with growth hormone and euthyrox. This study enriches the mutation spectrum of POU1F1 gene and has important significance for the diagnosis and classification of combined pituitary hormone deficiency.

Recognizing pediatric acute kidney injury / 中国当代儿科杂志

Acute kidney injury (AKI) is characterized by a reversible increase in blood concentration of creatinine and nitrogenous waste products and by the inability of the kidney to regulate fluid and electrolyte homeostasis appropriately. AKI in hospitalized patients is independently associated with increased morbidity and mortality in pediatric and adult populations. Continued reliance on serum creatinine and urine output for the diagnosis of AKI has resulted in an inability to provide successful therapeutic and supportive interventions to prevent and mitigate AKI. Research efforts over the last decade have foused on the discovery and validation of novel biomarkers to detect AKI prior to a change in kidney function and to make a differential diagnosis of AKI.

Relationship between TAP gene promoter methylation and cervical lesions with HPV infection in Uyghur women / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the relationship between TAP (transporter associated with antigen processing) gene promoter regional methylation level and cervical lesions with HPV infection in Uyghur women.</p><p><b>METHODS</b>A specialized software was used to design specific primers of CpG island fragments of TAP1 and TAP2 gene promoter for PCR amplification, bisulfitemodified SiHa cancer cell DNA for PCR amplification, cloning and sequencing analysis to obtain the relevant information on the gene base sequence methylation of CpG sites. Seventy-eight fresh cervical tissue samples from Uyghur women with cervicitis (number = 15), cervical intraepithelial neoplasia (CIN, number = 30) and cervical squamous cell carcinoma (number = 33) were collected. The methylation level of TAP1 and TAP2 gene promoter regions was detected using MassArray DNA technology. HPV infection status was determined by HPV gene chips. The relationship between CpG-island methylation of gene promoter regions and HPV infection was then analyzed.</p><p><b>RESULTS</b>Each TAP1 and TAP2 gene corresponding target fragment contained 23 and 8 CpG sites. There were 5 and 8 CpG sites methylation occurred in SiHa cervical cancer cells genomic DNA respectively. The TAP1 methylation level increased steadily with the severity of cervical lesions. The methylation levels in cervical squamous cell carcinoma and CIN (0.048 ± 0.039 and 0.037 ± 0.026, respectively) were higher than that of normal cervical tissue (0.035 ± 0.029, P < 0.05). Although TAP2 gene methylation level also demonstrated similar changes, the difference however was not statistically significant (P > 0.05). HPV gene chip detected 13 HPV genotypes, with HPV16 infection rate being 66.7% (52/78). The methylated proportion of TAP1 positively correlated with HPV16 infection (χ(2) = 6.08, P = 0.039).</p><p><b>CONCLUSION</b>TAP1 methylation is a remarkable phenomenon occurring in a range of cervical lesions and significantly associated with cervical HPV infection.</p>

Meta-analysis of calcineurin inhibitor in the treatment of lupus nephritis / 中华儿科杂志

<p><b>OBJECTIVE</b>To systematically evaluate the clinical effects of cyclosporine A (CsA) and tacrolimus, which are calcineurin inhibitors, on lupus nephritis.</p><p><b>METHOD</b>In this study, the clinical trials on treatment of lupus nephritis with cyclosporine A and tacrolimus published until May 2010 were searched at www.guideline.gov, www.nice.org.uk, mdm.ca/cpgsnew/cpgs/index.asp, www.show.scot.nhs.uk, www.nzgg.org.nz, www.eguid elines.co.uk, www.gin.net, Cochrane library, EMBASE, MEDLINE, Wanfang database, Chinese Journal full-text Database, Chongqing Weipu Database by using the methods of Cochrane systematic review. At the same time the information from related journals, professional data and network were hand-searched. The homogeneous evaluation was performed by meta-analysis.Statistical analysis of clinical data was performed by using RevMan 4.2 software provided by the Cochrane Collaboration.</p><p><b>RESULT</b>A total of 214 reports were found, while only 7 randomized controlled trials met the inclusion criteria, 4 of them were on the treatment with CsA (treatment group) and cyclosporine (CTX) group (control group), and 3 of them were the on treatment with FK506 (treatment group) and CTX group (control group). There were 148 reports in the treatment of CsA and CTX group, while 185 reports in the treatment of FK506 and CTX group. Both CsA and tacrolimus group could decrease daily urinary protein. Tacrolimus group was good at reducing daily urinary protein as compared with CTX group, and the difference was statistically significant (Z = 2.8, P = 0.005), but there was no significant difference between CsA and CTX groups (Z = 1.08, P = 0.28). Tacrolimus group was good at complete remission as compared with CTX group (Z = 3.64, P = 0.0003), partial remission was similar in both groups (Z = 0.53, P = 0.6), and tacrolimus group was good at total remission (Z = 2.2, P = 0.03). There was no significant difference between CsA and CTX group in side effect within a short period, while FK506 had less side-effect than CTX group.</p><p><b>CONCLUSION</b>Compared with the treatment with CTX, tacrolimus was good at reducing daily urinary protein. CsA and CTX were similar in reducing daily urinary protein in the treatment of lupus nephritis. Tacrolimus resulted in better total remission than CTX and had less side effect. CsA and CTX groups were similar in side effect. On the whole, calcineurin inhibitor could significantly decrease daily urinary protein, and tacrolimus was better in treatment and had less side-effect than CTX. However, large scale, multicenter, well-designed clinical trials should be adopted to further confirm the conclusions.</p>

Relationship of 24-hour ambulatory blood pressure and rennin-angiotensin-aldosterone system in children with primary nephrotic syndrome / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the changes of blood pressure by 24-hour ambulatory blood pressure (ABP) monitoring in children with primary nephrotic syndrome (PNS) and explore the relationship of the changes in blood pressure with rennin-angiotensin-aldosterone system (RAAS) in these children.</p><p><b>METHODS</b>ABP and casual blood pressure (CBP) monitoring were performed in 114 children with PNS. Plasma levels of rennin activity (PRA), angiotensin II (AngII) and aldosterone (ALD) were measured. The correlation of plasma levels of PRA, AngII and ALD with ABP was evaluated.</p><p><b>RESULTS</b>Of the 114 children with PNS, 101 (88.6%) presented elevated blood pressure. Mild or severe masked hypertension was found in 45 children (39.5%). Eighty (70.2%) children showed non-dipper blood pressure. The index and load of systolic blood pressure were higher than those of diastolic blood pressure. The blood pressure index and blood pressure load during sleep were higher than those during wakefulness. The boy presented higher diastolic blood pressure index and load than girls. Decubitus blood PRA, AngII and ALD levels in children with PNS were significantly higher than normal controls. The group with elevated blood pressure presented significantly higher decubitus blood PRA, AngII and ALD levels than the group with normal blood pressure. AngII level was significantly positively correlated with the index and load of both systolic blood pressure and diastolic blood pressure.</p><p><b>CONCLUSIONS</b>The children with PNS present a high incidence of hypertension, with a large percentage of masked hypertension and non-dipper blood pressure. Systolic blood pressure increases more significantly than diastolic blood pressure. Blood pressure during sleep increases more significantly than that during wakefulness. Diastolic blood pressure increases more significantly in boys than in girls. RAAS activity is elevated and the elevated RAAS activity might increase the blood pressure mainly by AngII in children with PNS.</p>

A prospective multicenter clinical control trial on treatment of refractory nephrotic syndrome with mycophenolate mofetil in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of mycophenolate mofetil (MMF) plus prednisone on refractory nephrotic syndrome (RNS) in children.</p><p><b>METHODS</b>One hundred and forty-two children with RNS from ten clinical trial centers were divided into two groups: MMF (n=87) and control (n=55). The MMF group patients were administered with oral MMF (30-40 mg/kg daily) for at least 6 months. Afterwards the patients who responded to MMF received another 6 months MMF treatment at a dosage of 10-20 mg/kg daily. The controls were treated with pulse intravenous infusion of cyclophosphamide (CTX) (10 mg/kg daily) for 2 days every 2 weeks for 3 months. Then CTX was administered at a dosage of 500 mg/m2 once a month 4, 7 and 10 months after treatment. While the patients received MMF or CTX treatment, they were treated with oral prednisone (0.5-1 mg/kg daily) for 2 to 3 months, and then the dosage of prednisone was gradually reduced. Urinary protein, liver and renal functions, and side effects of drugs were examined at regular intervals for one year.</p><p><b>RESULTS</b>Of the 87 patients, 58 achieved complete remission, 16 achieved partial remission, 9 achieved early remission and 4 had no response to treatment. In the control group, 35 achieved complete remission, 9 achieved partial remission, 1 achieved early remission and 10 had no response to treatment. The total remission rate in the MMF group (95.4%) was significantly higher than that in the control group (81.8%) (P<0.01). After treatment 67 patients (65.4%) in the MMF group had negative proteinuria compared with 36 patients (65.4%) in the control group (P>0.05). MMF was found to be more effective in reducing proteinuria, and improving hypoproteinemia, oliguria, hyperlipemia, and edema than CTX. MMF was better tolerated with lower incidences of adverse reactions than CTX.</p><p><b>CONCLUSIONS</b>The combined therapy of MMF and prednisone is more effective and tolerable than pulse intravenous infusion of CTX for treatment of RNS in children.</p>

Effects of astragalus on renal tubulointerestitial lesions and expression of NF-kappaB and MCP-1 in renal tissues in rat experimental IgA nephropathy / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the effects of astragalus on tubulointerstitial lesions in rats with IgA nephropathy (IgAN) and to explore the possible mechanism.</p><p><b>METHODS</b>Twenty-eight Sprague-Dawley rats were randomly assigned to three groups. The rat model of IgA nephropathy was induced by intragastric administration of bovine serum albumin and injections of LPS and CC14. Six weeks later, the rats with IgAN were randomly treated with oral astragalus (3 g/kg/d, for 6 weeks) or normal saline. Normal control rats which were not subjected to IgAN were treated with normal saline. The number of urinary erythrocytes and urinary protein and B-D-N-Acetyl glucosaminidase (NAG) contents were determined by Pan-automatic biochemistry analyzing meter. Expression of monocyte chemotactic protein-1 (MCP-1) and nuclear factor-kappa B (NF-kappaB) in tubulointerstitial tissues were analyzed by immunohistochemistry. A semiquantitative score was used to evaluate the degree of renal pathologic lesions.</p><p><b>RESULTS</b>The number of urinary erythrocytes (74.02+/-16.58 / microL vs 383.23+/-4.94 /microL) and urinary protein (13.88+/-4.94 vs 59.82+/-14.73 mg/L) and NAG contents (2.84+/-0.31 vs 5.24+/-0.80 U/L) in the astragalus-treated IgAN rats decreased remarkably compared with those in the IgAN rats without astragalus treatment (P<0.01). Expression of the NF-kappaB and MCP-1 in the renal tissues in the IgAN rats without astragalus treatment was significantly higher than that in the astragalus-treated IgAN rats and normal control rats (P<0.01). There were significant differences in the scores of renal pathologic lesions between the IgAN rats with or without astragalus treatment (6.03+/-0.46 vs 10.57+/-1.23; P<0.01).</p><p><b>CONCLUSIONS</b>Astragalus can decrease the number of urinary erythrocytes and urinary protein and NAG contents, and relieves tubulointerstitial lesions, possibly through the down-regulation of NF-kappaB and MCP-1 expression in rats with IgAN.</p>

Regulatory effect of Astragalus membranaceus on the immune disorder in rats with IgA nephropathy / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the regulattory effect of Astragalus membranaceus on immune disturbance of the rats with IgA nephropathy.</p><p><b>METHODS</b>Rats IgA nephropathy (IgAN) model was duplicated by oral feeding of bovine serum albumin (BSA), subcutaneous injection of carbon tetrachloride (CCl4) and injection of lipopolysaccharide (LSP) into vena caudalis. The rats were divided into three groups randomly for the normal, IgAN model group and the group treated with Astragalus membranaceus (treatment group). The treatment group was given the Astragalus membranaceus granules via intragastric administratsion, the normal group and the IgAN model group were given the equal amount of aqua destillata by gastric perfusion. The rats were examined for albuminuria, hematuria and pathological changes of renal tissue and the distribution of TGF-beta and interleukin-5 in renal tissue was determined by immunohistochemistry and the IFN-gamma and IL-4 of cytokine of Th1 and Th2 types were detected in rats IgA nephropathy model by sandwich enzyme linked immunosorbent assay (ELISA).</p><p><b>RESULTS</b>(1) The hematuria in rats with IgA nephropathy significantly increased compared with normal control group and Astragalus treatment group (P < 0.05). There was significant increase in albuminuria in rats with IgA nephropathy, compared with normal control group and astragalus treatment group (P < 0.01). (2) The pathological change of glomerular mesangium, renal tubules and renal interstitia became serious in rats IgA nephropathy model when compared with normal control group and astragalus treatment group. Immumofluorescence showed renal IgA density in rats IgA nephropathy model was significantly higher than that in the normal control group (P < 0.001) and astragalus treatment group (P < 0.001). (3) The result of immuno histochemistry showed that there was only weak expression of TGF-beta and interleukin 5 in normal renal tissue. The expression of TGF-beta and interleukin 5 in IgA nephropathy model was significantly stronger than those in normal control group (P < 0.05) and astragalus treatment group (P < 0.05). (4) The serum IL-4 levels were (33.74 +/- 7.52) pg/ml in rats IgA nephropathy model, significantly higher than that in normal control group (2.36 +/- 0.85) pg/ml and astragalus treatment group (3.24 +/- 1.13) pg/ml. The IFN-gamma level in serum of rats IgA nephropathy model was (18.79 +/- 3.80) pg/ml, which was significantly higher than that in normal control group (46.53 +/- 5.56) pg/ml and astragalus treatment group (41.28 +/- 2.95) pg/ml.</p><p><b>CONCLUSIONS</b>The astragalus could lower the level of hematuria and 24 hours-albuminuria of the IgAN model, and amelioratse the change of the renal pathology and reduce the deposit of IgA in glomerular mesangium. The possible mechanism of the effect is that astragalus could regulate the derangement of Th1, Th2, accordingly could improve the level of IL-4 and IFN-gamma in the serum and diminish the expression of cytokine Th2 TGF-beta1 and IL-5 of the renal tissue, and thereby could postpone the development of IgAN.</p>

Assessment of mycophenolate mofetil for treatment of frequently relapsing nephrotic syndrome in children / 中南大学学报(医学版)

OBJECTIVE@#To investigate the efficacy and adverse effect of mycophenolate mofetil (MMF) in the treatment of frequently relapsing nephrotic syndrome in children.@*METHODS@#The study population consisted of 37 children (24 simple nephrotic syndrome and 13 nephritis-type syndrome) suffering from frequently relapsing nephrotic syndrome. Patients received 20-30 mg/(kg d) of MMF in conjunction with 1 mg/(kg d) prednisone for 3-6 months.@*RESULTS@#Out of 24 patients suffered from simple nephrotic syndrome, 17 patients (70.8%) with complete relief, 4 patients (16.7%) with partial relief and 3 patients (12.5%) with non-relief, whereas out of 13 patients suffered from nephritis-type syndrome 6 patients (46.2%) with complete relief, 3 patients (23.1%) with partial relief and 4 patients (30.7%) with non-relief. Eight patients with Minimal Change Disease (MCD) achieved complete relief. Of 23 patients with Mesangial Proliferative Glomerulonephritis (MsPGN) or Membranoproliferative Glomerulonephritis (MPGN), complete relief was observed in 17 patients (73.9%), partial relief in 4 patients (17.4%) and non-relief in 2 patients.@*CONCLUSION@#These Results suggest that MMF has better efficacy against simple renal disease than against nephritis-type syndrome, and MMF may be more suitable for the treatment of frequently relapsing nephrotic syndrome characterized by proliferative lesions.

Effect of glucocorticoid receptor beta on glucocorticoid action in glomerular mesangial cells / 中南大学学报(医学版)

OBJECTIVE@#To construct mesangial cell lines over- or under- expressing glucocorticoid receptor beta (GRbeta), to investigate the effect of GRbeta on glucocorticoid biological function, and to determine whether the overexpression of GRbeta explains the glucocorticoid-resistant in glomerular mesangial cells (GMCs).@*METHODS@#The recombinant human sense or anti-sense gene of GRbeta was transferred into the rat GMCs by retrovirus-mediated stable transfection technique. Expression of hGRbeta mRNA in GMCs was determined by reverse transcription of total RNA followed by quantitative reverse transcription-polymerase chain reaction (RT-PCR), and the product of RT-PCR was then analyzed by gene sequencing. The expression of hGRbeta protein in GMCs was tested by Western blot. The inhibitory rate of dexamethasone-mediated cells on lipopolysaccharide (LPS)-stimulated GMC proliferation was detected to assess the effect of GRbeta at different expression levels on the glucocorticoid action. The cell proliferative activity in different cells with different levels of GRbeta was tested by MTT chromatometry. The change of cell cycle was analyzed by flow cytometry.@*RESULTS@#RT-PCR and gene sequencing showed that the recombinant sense and anti-sense genes were correctly integrated into genomic DNA of mesangial cells. The protein expression tested by Western blot showed that GRbeta in cells inserted with the sense hGRbeta gene was higher than those cells inserted with the anti-sense hGRbeta gene (109.74+/-10.63 vs. 19.08+/-1.01, P<0.05). The inhibitory rate of cell proliferation induced by dexamethasone was lower in GMCs transfected with sense hGRbeta gene than those transfected with anti-sense hGRbeta gene (18.47%+/-2.12% vs. 60.33%+/- 5.29%,P<0.05). Under the inhibition of dexamethasone, the decreased cell number of S-stage cells was significantly lower, and the increased cell number of G1- stage cells was significantly higher in GMCs transfected with sense hGRbeta gene than those of non-transfected cells.@*CONCLUSION@#The overexpression of GRbeta may inhibit the glucocorticoid action in GMCs. The GRbeta level in mesangial cells may be an important factor in determining whether they are sensitive or resistant to glucocorticoid.

Glucocorticoid administration in steroid sensitive nephritic syndrome: a meta-analysis / 中南大学学报(医学版)

OBJECTIVE@#To evaluate the benefits and toxicities of different corticosteroid regimes in preventing relapse in children with steroid sensitive nephrotic syndrome (SSNS).@*METHODS@#MEDLINE (Jan. 1963-Mar. 2007), elsevier (Jan. 1997-Aug. 2006), OVID databank (Jan. 1993-Aug. 2006), Springer databank (Jan. 1994-March 2007), the Cochrane Controlled Trials Register (Cochrane Library, Issue Feb. 2006), Cochrane Renal Group Specialised Register (Jul. 2006), EMBASE (Jan. 1980-Mar. 2007) and CNKI (Jan. 1994-Mar. 2007) etc, were searched by the terms primary nephrotic syndrome, glucocorticoid, corticosteroid, steroid, prednisone, methylprednisolone, dexamethasone and children etc for the human clinical trials about glucocorticoid (GC) administration in primary nephrotic syndrome (PNS) (aged 3 months to 18 years), controlled or semi-controlled ones, including unpublished documents from scientific meetings and theses, and similar documents listed in the references of the above documents were also included. All the studies were evaluated strictly according to Jadad Standard, and the Meta-analysis were adopted. Review manager 4.2 software was used to analyze the data. The odds ratio was calculated for the relapse rate and side effect from the initial episode to the end of follow-up between the patients treated with corticosteroids and the controls.@*RESULTS@#Totally 12 trials with 868 subjects meeting the criteria were included in this review. A Meta-analysis of 7 trials, which compared between 2 months of prednisone and 3 months or more in the first episode, showed that longer treatment duration significantly reduced the risk of relapse at 12-24 months (RR=0.70,95% CI:0.60-0.89),without an increase of side effect. There was a negative linear relationship between the duration of treatment and risk of relapse (r2 =0.66, P=0.05).@*CONCLUSION@#(1) Children in their first episode of SSNS should be treated for at least 3 months of GC. The therapeutic effect of patients in the primary nephrotic syndrome treated with GC for 12 weeks was prior to that for 8 weeks, compared with that in the controls. It could reduce the relapse rate of half year, the longer treatment duration in the NS patients at the first relapse was, the lower relapse risk was.(2) Compared with alternative GC administration, standard GC administration can reduce the side effects; Course more than 1 year of GC administration can reduce the 2-year relapse rate. Hence in children who relapse frequently, multicentre, well-designed experiments should be adopted.

Clinicopathologic characteristics of 1,316 children with renal disease / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic characteristics of childhood renal diseases.</p><p><b>METHODS</b>A retrospective analysis of 1316 renal biopsies performed over the past 20 years was performed.</p><p><b>RESULTS</b>Of the 1316 patients, 383 (29.09% ) were diagnosed as nephrotic syndrome, 291 (22.00%) as acute nephritis syndrome, 224 (17.21%) as isolated hematuria, 209(15.87%) as purpura nephritis, and 96 (7.30% ) as hepatitis B virus-associated nephritis . Mesangial proliferation was the most common pathological change (756 cases; 57.45%), followed by IgA nephropathy (113 cases; 8.59%), endothelial capillary proliferation(112 cases; 8.51%), membranous nephropathy (66 cases; 5.02%), and various minor and minimal changes (59 cases; 4.48%). Alport syndrome, congenital nephrotic syndrome, thin basement membrane nephropathy, fibrillary glomerulopathy disease, and Fabry disease were confirmed by electronic microscopy. IgA, IgM and C1q nephropathy were definitely diagnosed using immune histochemistry or immunofluorescent. A diagnosis of primary glomerular disease was made in 69.53% of the cases (915 cases); secondary glomerular disease was noted in 26.14% (344 cases). Of the 915 cases of primary glomerular disease, 375 (41.0%) had nephrotic syndrome. Secondary glomerular disease due to purura nephritis was common (209/344; 60.8%).</p><p><b>CONCLUSIONS</b>Primiary glomerular disease predominates in children. Nephrotic syndrome is the most common clinical diagnosis. Mesangial proliferation is the most common pathological patterns in children with renal disease.</p>

Role of mast cells in the development of renal interstitial fibrosis in children with Henoch-Schonlein purpura nephritis / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the role of mast cells in the development of renal interstitial fibrosis in children with Henoch-Schonlein purpura nephritis (HSPN) and possible mechanisms.</p><p><b>METHODS</b>Paraffin-embedded renal biopsy tissue sections from 20 children with HSPN were examined for the levels of tryptase-beta and transforming growth factor-beta1 (TGF-beta1) by immunohistochemical staining. Mast cells were counted by toluidine blue staining. Masson staining was used to assess the level of renal interstitial fibrosis and renal histopathological scores. Normal renal tissue sections from 5 nephrectomized children for nephroma were used as control group.</p><p><b>RESULTS</b>The percentages of positive tryptase-beta cellsand mast cells and the TGF-beta1 expression in the HSPN group were significantly higher than those in the control group (P < 0.05). The percentages of positive tryptase-beta cells and mast cells and the TGF-beta1 expression in renal tissue were positively correlated with the glomeruli histopathological score (r =0.940, 0.920, 0.937, respectively; P < 0.05) and were also positively correlated with the histopathological score of renal interstitium (r=0.903, 0.859, 0.948, respectively; P < 0.05). The level of renal interstitial fibrosis was positively correlated with the percentages of positive tryptase-beta cells and mast cells and the expression of TGF-beta1 (r =0.790, 0.766, 0.858, respectively; P < 0.05). There was a positive correlation between the percentages of positive tryptase-beta cells and mast cells (r =0.941, P < 0.05), between the percentage of positive tryptase-beta cells and the TGF-beta1 expression (r =0.897, P < 0.05) and between the percentage of positive mast cells and the TGF-beta1 expression (r=0.942, P < 0.05).</p><p><b>CONCLUSIONS</b>Tubulointerstitial mast cell infiltration is associated with the development of renal interstitial fibrosis in children with HSPN. Mast cells together with TGF-beta1 and mast cell-derived tryptase-beta may be involved in the development of the renal interstitial fibrosis in HSPN.</p>

Relationship between clinical manifestations and renal pathology in children with Henoch-Schonlein purpura nephritis / 中国当代儿科杂志

<p><b>OBJECTIVE</b>This study investigated the clinical manifestations and renal pathological findings of 95 children with Henoch-Schonlein purpura nephritis (HSPN) in order to explore the relationship between clinical manifestations and renal pathology in HSPN.</p><p><b>METHODS</b>According to clinical manifestations, 95 HSP patients were classified into six clinical groups: 1) normal urine analysis; 2) isolated hematuria or proteinuria; 3) proteinuria with hematuria; 4) acute nephritis; 5) nephrotic syndrome; 6) acute nephritis with over 50 mg/(kg.d) of proteinuria. The severity of the renal pathological findings was determined based on the classification of the International Study of Kidney Disease (ISKDC), including grades I-VI. The relationship between clinical manifestations and the severity of renal pathological findings was studied.</p><p><b>RESULTS</b>Nephrotic syndrome was the most common clinical diagnosis (26 cases), followed by proteinuria with hematuria (23 cases), normal urine analysis (20 cases), isolated hematuria or proteinuria (15 cases), acute nephritis with over 50 mg/(kg.d) of proteinuria (7 cases) and acute nephritis (4 cases). Twenty-five out of 26 patients with nephrotic syndrome had an ISKDC classification of grade III-IV. All of the four patients with acute nephrits had a classification of grade IIIb. The 7 cases of acute nephritis with over 50 mg/(kg.d) of proteinuria had a classification of grade IIIa-V. The 20 patients with normal urine analysis had a classification of grade Iia- IIIb. There were no significant differences in ISKDC classification among the patients with normal urine analysis, isolated hematuria or proteinuria, and hematuria plus proteinuria. As the course progressed, the degree of renal pathological changes in patients with isolated hematuria or proteinuria and hematuria plus proteinuria became more serious. Of all the 95 patients, 58% had co-deposition of immunoglobulins A, G and M. The percentage of co-deposition of immunoglobulins A, G and M was related to the disease course and the severity of renal pathological findings.</p><p><b>CONCLUSIONS</b>HSPN children with nephrotic syndrome or acute nephritis with or without proteinuria had relatively severe renal pathological changes. The clinical manifestations were not always in parallel with the severity of renal pathological findings in HSPN children. With the course progressing, the renal pathological changes tended to be serious. The severe renal pathological manifestations came with co-deposition of immunogolobulins A, G and M in the glomerulin.</p>

Mobilization effects of SCF along with G-CSF on bone marrow stem cells and endothelial progenitor cells in rats with unilateral ureteral obstruction / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the mobilization effects of stem cell factor (SCF) along with granulocyte colony-stimulating factor (G-CSF) on bone marrow stem cells and endothelial progenitor cells in rats with unilateral ureteral obstruction (UUO).</p><p><b>METHODS</b>Fifty-six healthy male Wistar rats were randomly divided into seven groups: control, SCF, G-CSF, SCF+G-SCF, Sham-operated, UUO and UUO+SCF+G-CSF groups (n=8 each). The rats from the control, SCF, G-CSF and SCF+G-CSF groups were hypodermically injected with normal saline (2 mL/kg), SCF (200 microg/kg), G-CSF (200 microg/kg) and SCF along with G-CSF respectively for 5 days. The rats from the UUO and UUO+SCF+G-CSF groups were subjected to the ligation of right ureter and then were hypodermically injected with normal saline (2 mL/kg) and SCF (200 microg/kg)+G-CSF (200 microg/kg) respectively for 5 days. The sham-operated group had the same operative approach as the UUO and the UUO+SCF+G-CSF groups but the right ureter was not ligated. After operation they received a hypodermical injection of 2 mL/kg normal saline for 5 days. Five days later blood samples were collected. The percentages of CD34+ and CD34+/CD133+ cells in intravenous blood mononuclear cells were detected by flow cytometry. Serum contents of glutamate-pyruvate transaminase (GPT), glutamic oxalacetic transaminase (GOT), urea nitrogen and creatinin were measured.</p><p><b>RESULTS</b>Except for the sham-operated group, the other five groups (SCF, G-CSF, SCF+G-SCF, UUO and UUO+ SCF+G-CSF groups) had significantly higher percentage of CD34+ cells and CD34+/CD133+ cells in intravenous blood mononuclear cells than the control group (P < 0.05). There were significant differences in the percentage of CD34+ cells and CD34+/CD133+ cells among the five groups (P < 0.05). The UUO+SCF+G-CSF group showed the highest percentage of CD34+ cells and CD34+/CD133+ cells, followed by the SCF+G-CSF group. There were no significant differences in serum contents of GPT, urea nitrogen and creatinin among the seven groups. Except the UUO group showed higher GOT contents, there were no significant differences in the GOT contents among the other six groups.</p><p><b>CONCLUSIONS</b>The mobilization effects of SCF and G-CSF on bone marrow stem cells and endothelial progenitor cells were not always in paraller. A combination of SCF and G-CSF can effectively mobilize stem cells and endothelial progenitor cells, and side effects were not found in the liver and the kidney.</p>

Effects of siRNAs silencing transforming growth factor-beta1 on fibronectin in SD rat masangial cells / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate fibronectin synthesis in SD rat mesangial cells after transforming growth factor-beta1 (TGF-beta1) is silenced by the short interfering RNA (siRNA) expressed by reconstructed pGEFP-C1 vectors.</p><p><b>METHODS</b>Depending upon the 538th - 556th (A) and 895th - 913th (B) nucleotides of rat TGF-beta1 gene, a nucleotide (A or B) was constructed into a small hairpin nucleotide which was separately (A or B) or together (A plus B) inserted into a pGEFP-C1 vector with three reconstructed pGEFP-C1 vectors separately expressing the siRNAs for A or/and B. TGF-beta1 and fibronectin were dynamically investigated for their interrelationship by ELISA in the supernatant and RT-PCR in their extracted total RNA.</p><p><b>RESULTS</b>The siRNA hairpin-like molecules were constructed according to the 538th - 556th nucleotides of rat TGF-beta1 gene were able to markedly silence the expression of TGF-beta1 mRNA (P < 0.01) and protein (P < 0.01) at 48 h. Lipfectamin 2000 transfection stimulated the peak secretion of fibronectin at 24 h in the control and the experimental group whose TGF-beta1 was not silenced, but the silence of TGF-beta1 in both experimental groups delayed the top values of fibronectin to 48 h (P < 0.01).</p><p><b>CONCLUSION</b>The silence of TGF-beta1 by siRNA decreased the fibronectin expression, but the latter was possibly not completely TGF-dependent.</p>

Prolonged Hyperoxia Exposure Induced Pathological Changes and Apoptosis of Neonatal Rats Kidney / 实用儿科临床杂志

75%) in P 21 d in group HO.2.The apoptotic index in group HO was higher than that of group Air on P 3 d(Pa