Objective To investigate the characteristics of Wegener granulomatosis in children and to improve its recognition. Methods Ten pediatric patients with Wegener granulomatosis were studied retrospectively by clinical manifestations, serum examination, pathological data, treatment, etc. Results All children had clinical evidence of upper respiratory tract and lungs involvement; kidney lesion occurred in 6 cases(6/10); joints, skin, eyes and nervous system were also involved with different degree, cANCA (PR3) showed positive in 8 cases; Pathological examination (1 kidney sample, 2 nasal mucosa samples, 2 skin samples) showed granulomatous vasculitis and crescents were found in renal biopsy; 7 cases were treated with corticosteroid plus cyclophosphamide, 1 case was treated with corticosteroid plus methotrexate, and improvement were seen in all of them. Conclusions Wegener granulomatosis in children have diverse clinical manifestations and clinical diagnosis is difficult. The upper and lower respiratory tract and kidney are most commonly involved organs. ANCA inspection is characteristic. Treatment with corticosteroid and immunosuppressive can achieve good outcome.

Objective To explore the feature and the optimal treatment of kidney stones in children associ-ated with infant formula which had been aduherated with melamine.Method Total of 47 cases of urinary stone and 31 cases of acute renal failure caused by urinary multiple obstruction in children associated with infant formula which had been adulterated with mlelamine in Beijing Children's Hospital University of Medical Science,form Jan-uary to November,2008 were retrospectively analyzed.Results Infant formula which had been adultemted with melamine may lead to kindey stones in 15 days.The mean age was 11-month and the ratio of male to female was 2.6:1.The kindey stones were usually multiple and mostly located either in the ureteropelvic junction or all through the ureter.In the view of the renal function resuming duration,there was no statistic difference among the acute renal failure group,operation group and the conservative group(P=0.683～0.846).In children with uri-nary obstruction and acute renal failure,hemedialysis or peritoneal dialysis was performed.83.9%were relieved by posting catheter through panedoseope and blood purification.100% of the patients resumed normal renal func-tion.81.3% of the patients without acute renal failure had recovered after conservative therapies such as infusion.Conclusions The patients associated with infant formula which had been adulterated with melamine were Usually little infants.Male were more vulnerable than female.Most of them had recovered smoothly and the renal function resumed normal after effcient therapies.

Plasma exchange is a commonly used blood purification method, h includes plasma exchange,double filtration plasmapheresis and immunoadsorption.Many nervous system diseases have the antibody. Guillian-Barre syndrome is caused by antibodies of the nervous system demyelinafing disease; Myasthenia grads is caused by the acetylcholinc receptor antibodies. CIDP also has autoimmune-mediated mechanism. Plasma exchange can be apphed effectively to remove antibodies, and to quickly improve the patient' s clinical symptoms and shorten the course of disease.

Objective To investigate the treatment of atypical hemolytic uremic syndrome (aHUS) in their acute phase and relate the prognostic factors.Methods Twenty-eight patients with aHUS treated in Department of Nephrology,Beijing Children's Hospital Affiliated to Capital Medical University from January 2011 to March 2015 were collected,the outcomes of the treatment were summarized.Results All the patients were given plasma exchange (PE)treatment,21 patients with hemodialysis (HD) treatment;13 patients with H factor (FH) antibody were further treated with corticosteroid therapy,and 1 patient was treated with the combination of mycophenolate mofetil.Evaluation of patients' indices were conducted at month 3.Out of the 28 patients,there were 13 patients with complete remission;of the 14 patients showed improvement,6 patients were complicated with hypertension and 4 patients with renal dysfunction.One patient,with ongoing hemodialysis,did not show restoration of renal function.There was no death in children.Statistical analysis showed that the acute phase serum creatinine levels were (430.0 ± 124.7) μmol/L and (265.6 ± 90.8)μmol/L for the group with remaining kidney injury and the group with complete remission,respectively,the difference was statistically significant(P ＜0.01).The levels of FH for the 2 groups were (206.5 ± 134.9) mg/L and(415.4-±214.1) mg/L,and the difference was statistically significant (P ＜ 0.01) as well.Statistical analysis showed that differences were no statistically significant between the 2 groups of patients on their age,platelets,complement C3,complement C4,hemoglobin and lactate dehydrogenase (all P ＞ 0.05).For the group with positive antibody,its average serum creatinine was (346.4 ± 112.5) μmol/L.For the group with negative antibody,its average serum creatinine was (297.0 ± 89.3) μmol/L.The FH for group with positive antibody and group with negative antibody were (307.4 ±187.3) mg/L and (379.8 ± 203.5) mg/L,respectively.The differences were not statistically significant (all P ＞0.05).Conclusions PE is an effective treatment for aHUS at acute phase,and corticosteroid and immunosuppressive therapy are effective for patients with positive anti-FH antibody.For patients with severe kidney injury at acute phase,the possibility of long-term kidney damage is high.Low concentration of serum FH indicates a poor prognosis.

Objective To analyze the clinical features of pediatric sepsis with initial symptoms of autoimmune hemolytic anemia(AIHA) and acute renal failure.Methods Ten cases of pediatric sepsis with initial symptoms of AIHA and acute renal failure were selected from January 2000 to January 2014 in Department of Nephrology,Beijing Children's Hospital Affiliated to Capital University of Medical Science.The clinical data were retrospectively analyzed and long-time follow-up was taken.Results There were 8 boys and 2 girls of the 10 cases.The ages ranged from 8 months to 12.8 years.There were 8 children less than 6 years old having a prevalence of 80％.There were sudden dark urine and progressive anemia in all the cases,jaundice in 9 cases,abdominal pain and vomiting in 6 cases.Urine volume decreased in 7 cases and anuria in 3 cases.Four cases showed disorder of consciousness and 2 developed shock.Respiratory failure and disseminated intravascular coagulation happened in 1 case respectively.In terms of pathogens,2 cases were infected by mycoplasma and 1 case was infected by hemolytic streptococcus.The rest of the pathogen was unknown.The Coomb's test was 3 +-4 + positive.Serum urea nitrogen and creatinine gradually increased.High level total serum bilirubin and unconjugated bilirubin were shown in 8 cases and low level complement in 7 cases.Bone marrow films showed hyperplasia in all the cases.Renal biopsy was taken in 3 cases and no microthrombus was found.Corticosteroid was used in all 10 cases for 6 months.Blood purification was taken in 8 cases.Respiratory support was used in 4 cases.Nine recovered and 1 case gave up treatment.Further follow-up was taken for 2.2-8.1 years.Eight cases had normal urine and blood routine test and renal function.Relapse happened in 1 girl 1 year later.One case lost follow-up.Conclusions Once sudden hemoglobinuria,anemia and progressively decreased urine output occur in sepsis cases,the clinicians should consider AIHA and acute renal failure.Based on active infection control,the early use of steroids and blood purification treatment can improve the prognosis.

Objective To explore the clinical feature of spontaneous bacterial peritonitis (SBP) in children with nephrotic syndrome (NS).Methods Eleven cases of SBP in children with NS from Department of Nephrology,Beijing Children's Hospital Affiliated to Capital University of Medical Science between January 2010 and June 2014 were analyzed retrospectively.The clinical features,laboratory data and efficacy of therapy were reviewed.Results In this study,12 episodes of primary peritonitis were detected in 11 patients.One patient had 2 attacks.There were 11 cases including 9 boys and 2 girls.The age of the patients ranged from 2 years and 11months to 14 years and 11 months.Nine cases of them received steroid therapy and/or cytotoxic drugs.Peritonitis was characterized by abdominal pain,mild abdominal pain occurred in 7 cases with slow onset.Ten cases had fever of varying degrees,8 cases had abdominal distention,5 cases had diarrhea and 4 cases had nausea and vomiting.Shock signs were present suddenly in 2 cases before therapy of antibiotics.Apparent edema and severe ascites were present in all cases.Five of them were presented with increasingly rapid severe ascites and showed no response to diuretic drugs.Blood investigation showed leukocytosis and high C-reactive protein.Serum albumin levels and IgG and CD4 ratio were very low in all cases.Ascites were like cloudy pus with raised neutrophil cells.Streptococcus pneumonia was positive in both blood culture and ascites culture in 2 cases.Cephalosporin was used empirically to all cases for 7 to 10 days in 9 cases and 4 weeks in 2 cases with positive blood culture.Paracentesis to release ascites fluid was performed in 5 cases with constant ascites.Eleven cases recovered from peritonitis completely.The proteinuria was negative in 2 cases after they recovered from peritonitis.Conclusions SBP was atypical in children with NS and sometimes patients might have to abdominal pain and rebound tenderness,which may be ignored and may induce deterioration suddenly and clinicians should be aware of it clearly.SBP could lead to relapse of NS and the cure of SBP by treatment could help the children recover from NS.

Objective To analyze the clinical characteristics,prognosis and the risk factors in 48 children with lupus nephritis combined with renal insufficiency.Methods The clinical data from 48 pediatric lupus nephritis with renal insufficiency from January 2000 to January 2013 were retrospectively analyzed.Results Forty-eight cases showed renal insufficiency among 168 children with lupus nephritis,and the incidence rate was 28.6％.The age ranged from 5.8 to 16.2 years.The male to female ratio was 1.0 ∶ 2.2.Among 48 children,43 cases had hematuria,41 cases had heavy proteinuria,25 cases had anaemia and 23 cases had persistent hypertension.Totally 20 cases underwent renal biopsy,and among them,15 cases(75.0％) were diagnosed as diffuse proliferative lupus nephritis(class Ⅳ).The histological activity index was ≥7 in 13 cases and the histological chronicity index was ≥4 in 3 cases.Corticosteroid and/or cytotoxic drugs were used in all of 48 cases.Thirty-five cases had normal urine and renal function,5 cases had stable renal function with persistent proteinuria,4 cases developed into chronic renal failure and 4 cases died.Persistent hypertension (x2 =4.274,P =0.039),the time of starting therapy (x2 =28.830,P =0.000),and histologic chronicity index(P =0.008 8 by Fisher's exact probability test)were the prognostic factors.Conclusions Among pediatric lupus nephritis with renal insufficiency,class Ⅳ (diffuse proliferative lupus nephritis) is the most frequent finding.Persistent hypertension,the time of starting therapy,and histologic chronicity index are the prognostic factors.The outcomes of lupus nephritis with insufficiency can be improved by adequate and appropriate treatment.

Objective To translate the Eating Behavior Scale (EBS) into Chinese and test its reliability and validity. Methods The Chinese Version of the scale (C-EBS) was developed through translation, back-translation, cross-cultural adaption and pre-test. A convenient sample of 100 old people with dementia was investigated with C-EBS to test its reliability and validity. Results The Cronbach′s α, Guttman Spilt and test-retest reliability of C-EBS were 0.842, 0.865 and 0.840, respectively. The I-CVIand S-CVI of C-EBS were 1.00, and the score of C-EBS was negatively correlated to the total score of The Chinese Edinburgh Feeding Evaluation in Dementia scale(C-EdFED) (r = 0.727,P < 0.01). Conclusion The C-EBS is reliable and valid and can be used as a valid tool to measure the eating ability among the old people with dementia in China.

Objective To study the patients' clinical characteristics and prognosis when only C3 deposition exists in endocapillary proliferative glomerulonephritis and try to understand deeply the role of C3 in kidney damage deeply. Methods The patients who were diagnosed with endocapillary proliferative glomerulonephritis but only had C3 deposited in immunofluorescence(to avoid false positive,C3≥2 ﹢ was included)were selected from Beijing Children's Hospital Affiliated to Capital Medical University during November 2010 to October 2014. Their clinical manifestations,la-boratory examinations,treatments,prognosis,and pathological changes were analyzed,and literature review was performed. Their clinical characteristics and prognosis were summarized. Results There were 11 patients diagnosed with endocapil-lary proliferative glomerulonephritis which had only C3 deposition(≥2 ﹢ ). Nine of them had onset with acute nephritis syndrome(81. 8% ),and 2 cases presented recurrent paroxysmal gross hematuria(18. 2% ). Seven cases were diagnosed with acute post streptococcal glomerulonephritis(63. 6% ). Eleven cases' clinical manifestations were relatively severe, and the complement C3 was significantly lower than the normal(100. 0% ). Their light microscope showed capillary proli-ferative glomerulonephritis,and the electron microscope showed the immune complexes were deposited in the endothelium,the epithelium or the mesangial area. The patients received corresponding treatment respectively,and all the patients had good prognosis during following up of 7 months up to 39 months. Conclusions Streptococcus infection is a common cause in endocapillary proliferative glomerulonephritis with only C3 deposition. The clinical manifestations of some children are similar to post streptococcal glomerulonephritis but relatively severe. Only deposition of C3 without IgG may be involved in another complement activation mechanism.

This study investigated the effect of epigenetic modification of maspin on extravillous trophoblastic function. The mRNA expression of maspin in placentae from normotensive and preeclamptic pregnant women was detected by RT-PCR. TEV-1 cells, a human first-trimester extravillous trophoblast cell line, were cultured and treated with CoCl(2) (300 μmol/L) to induce chemical hypoxia and with 5-aza (500 nmol/L) to induce demethylation. The mRNA expression of maspin in TEV-1 cells subjected to different treatments was determined by RT-PCR, and the proliferative and migratory abilities of TEV-1 cells were assessed by cell counting kit-8 (CCK-8) and Transwell assays. Our results showed that the maspin mRNA expression level in placentae from preeclamptic women was much higher than that from normotensive women. CoCl(2) or 5-aza could up-regulate the mRNA expression of maspin and significantly suppress the proliferation and migration of TEV-1 cells. It was concluded that the epigenetic modification in promoter region of maspin contributes to incomplete trophoblast invasion, which offers a novel approach for predicting and treating placental dysfunction.