Clinical features of children with acute focal bacterial nephritis / 中华实用儿科临床杂志

Objective To investigate the clinical features of acute focal bacterial nephritis (AFBN) in children,and to evaluate the strategy of diagnosis and therapy on children with AFBN,avoiding any blind spots of clinical treatment.Methods The clinical features of 12 cases of AFBN children admitted in Shenzhen Children's Hospital from Jan.2006 to Dec.2012 were analyzed.The clinical features and treatment strategy of AFBN were summarized.ResultsSeven in 12 cases(58.3％) were younger than 1 year old,fever was the initial and cardinal symptom,most cases were lack of typical or local urinary symptoms and signs.Peripheral blood leukoeytes,C-reactive protein and procalcitonin were highly increased in all cases,comparing with simple urinary tract infection.Five cases combined with pyeloureterectasis.One case combined with vesicoureteral reflux (VUR).Blood culture:staphylococcus aureus (1 case),staphylococcus epidermidis (1 case),E.Coli (2 cases).Urine culture:E.Coli (4 cases),E.Coli and enterococcus faecalis (1case),staphylococcus aureus (1 case),white candida yeast (1 case),mixed flora (2 cases).Urinary ultrasound:renal swelling was showed in involved kidney.Echo of renal parenchyma were enhanced or nonuniform,multiple hypoechoic area were easily visible in involved renal parenchyma,with boundary ambiguity.CT or MRI scan:typical findings were reducing perfusion with multiple wedge-shaped or quasi-circular lesions.Broad-spectrum antibacterial treatment was effective and necessary.The medication of antimicrobial was under the guidance of drug susceptibility test.The average course of treatment was 14-55 days.Eleven cases were cured by conservative treatment.Conclusions The morbility of AFBN in young infants are high.The combination of urinary tract deformity,pyeloureterectasis or VUR should be highly vigilant in AFBN children.Imageological inspection of urinary tract is the necessary method on AFBN diagnosis.The major pathogen is E.Coli,which shows a high resistance to antibiotics.Conservative antibacterial treatment is effective in AFBN.

Clinical and pathological analysis of 41 children with lupus nephritis / 中华实用儿科临床杂志

Objective To analyze the correlation between clinical characteristics and pathological findings lupus nephritis (LN) in children,together with the correlation of the renal vascular lesion respectively with the glomerular lesion and tubulointerstitial lesion.Methods Forty-one cases of LN in children diagnosed by percutaneous renal biopsy from Jan.2008 to Sep.2012 in Pediatrics of the Second Xiangya Hospital of Central South University were collected.Clinical manifestations and the lab findings of the blood and urine of all the patients were analyzed,and all the frozen sections were evaluated according to the standard of ISN/RPS2003 for LN.The glomerular lesion and tubalointerstitial lesion separately were also evaluated,respectively.The wall thickening/outer diameter ratio,intima thickening/outer diameter ratio and medial thickening/outer diameter ratio of the arterioles were measured.Results The percentage of clinical manifestation with LN increased coupled with the degree of pathological damage.In the different stages of pathological damage,both the glomerular lesion and tubulointerstitial lesion were getting much more serious along with the progressiveness of pathological damage,and what's more,they had a positive correlation(r =0.959,P ＜ 0.05).The wall thickness/outer diameter ratio in all cases was greater than 0.5,in dicating the thickness of vessel wall.Conclusion Renal vascular lesion really existed and is characterized by the progressive loss of integrity of the intima and the medial thickening.

Status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia.</p><p><b>METHODS</b>Serum concentrations of ferritin (SF), transferrin receptor (sTfR) and erythropoietin (EPO) were measured in 158 children with thalassemia. The differences in the concentrations of the three indices among children with different genotypes of thalassemia were compared. The correlations of the hemoglobin level with sereum SF, sTfR and EPO levels were assessed.</p><p><b>RESULTS</b>Among the 158 children with thalassemia, 52(32.9%) were diagnosed with alpha-thalassemia minor, 27(17.1%) with HbH disease, 59(37.4%) with beta-thalassemia minor, 13(8.2%) with beta-thalassemia major, and 7(4.4%) with combining alpha beta thalassemia. The SF levels in children with HbH disease or beta-thalassemia major were significantly higher than those in the other thalassemia groups (P<0.01). The sTfR levels in children with beta-thalassemia major were the highest when compared with those in the other thalassemia groups (P<0.05). The EPO levels in children with beta-thalassemia major were also the highest when compared with those in the other thalassemia groups (P<0.01). There was a negative correlation between hemoglobin and EPO levels in children with HbH disease (r=-0.656, P<0.01) and beta-thalassemia major (r=-0.641; P<0.05).</p><p><b>CONCLUSIONS</b>The status of iron metabolism and erythropoietic proliferation is different in children with different genotypes of thalassemia. A combined measurement of SF, sTfR and EPO may reflect the status of erythropoietic proliferation.</p>

Application of PCR-DGGE technique in G-6-PD deficiency / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To detect gene mutations of children with glucose-6-phosphorate dehydrogenase (G-6-PD) deficiency and of carriers of G-6-PD deficiency gene with the technique of polymerase chain reaction and denatured gradient gel electrophoresis (PCR-DGGE), and to explore the value of the technique in the diagnosis of G-6-PD deficiency and G-6-PD deficiency gene carrying.</p><p><b>METHODS</b>cDNAs were harvested by reverse transcription method after RNAs had been extracted from peripheral blood of 43 children with G-6-PD deficiency and of their family members (36 lineages). Electrophoresis behaviors of the fragment from exons 11-12 of G-6-PD cDNA were detected with the technique of PCR-DGGE. Gene sequencing was then performed for the abnormal electrophoresis bands.</p><p><b>RESULTS</b>Abnormal electrophoresis bands were found in the 1304-1520 fragment of G-6-PD cDNA in 33 out of 36 family lineages. The G-6-PD/6-PGD ratio was below 1.00 in 9 mothers of patients. Three of them had the G-6-PD/6-PGD ratio lower than 0.50. The PCR-DGGE bands were the same in the 3 mothers. Gene sequencing showed double heterozygote in the 3 mothers, but the maternal carriers of G-6-PD deficiency gene who had normal G-6-PD/6-PGD ratio showed mono-heterozygote in gene sequencing. Three mutational sites were found in the 1304-1520 fragment, i.e., C1311TG1376T and G1388A. The electrophoresis behaviors were different among the 3 gene mutational sites.</p><p><b>CONCLUSIONS</b>PCR-DGGE is a sensitive and reliable technique in the screening of gene mutations. It is useful in the diagnosis of G-6-PD deficiency, especially in the diagnosis of female G-6-PD deficiency gene carrying.</p>

Expression of G-6-PD mRNA in children with G-6-PD deficiency / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To detect the level of glucose-6-phosphate dehydrogenase (G-6-PD) mRNA expression in children with G-6-PD deficiency and their lineal family members in order to explore possible mechanisms of the disease at the transcriptional level.</p><p><b>METHODS</b>RNA was extracted from peripheral blood of 41 children with G-6-PD deficiency and of their lineal family members (29 father lineages and 40 mother lineages). cDNA was then harvested using the reverse transcription method. G-6-PD mRNA expression was detected by quantitative real-time PCR (QRT-PCR). The detection results of G-6-PD mRNA expression in three groups (Patient, Father lineage and Mother lineage) were compared using Statistical Software SPSS 10.0 system.</p><p><b>RESULTS</b>The mean G-6-PD mRNA value in the Patient, Father lineage and Mother lineage groups were 0.57 +/- 0.19, 0.74 +/- 0.21 and 0.67 +/- 0.21 respectively. The G-6-PD mRNA value in the Patient group was significantly lower than both Father lineage (t = -3.18, P < 0.01) and Mother lineage groups (t = -2.54, P < 0.05).</p><p><b>CONCLUSIONS</b>The expression of G-6-PD mRNA decreased in children with G-6-PD deficiency, suggesting that the pathogenesis of this disorder relates to the varying levels of gene transcription.</p>

Expression of proliferating cell nuclear antigen in renal tissues of children with primary nephrotic syndrome / 实用儿科临床杂志

Objective To investigate the expression of proliferating cell nuclear antigen(PCNA) in renal tissues of children with primary nephrotic syndrome(NS),and elucidate the relationship between PCNA expression and cell proliferation in renal tissues from the children with primary NS.Methods Paraffin-embedded renal biopsy tissue sections from 39 patients with primary NS were examined by immunohistochemical staining with anti-PCNA monoclonal antibody,normal renal tissue sections from 6 nephrectomized patients with nephroma were selected as control. Possible correlation between the percentage of PCNA positive cells and the pathologic type , histopathological score, clinical indices (serum albumin ,serum cholesterol ,serum creatinine and 24 hours urine protein ) before renal biopsy of NS were evaluated separately .Results The percentage of PCNA positive cells in glomeruli and tubulom terstitium of NS patients was significantly higher than that of the control (P