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Objective:To summarize the clinical data of anti-factor H antibody-associated atypical hemolytic uremic syndrome (aHUS) in children, and analyze the risk factors for disease recurrence and poor prognosis.Methods:A prospective cohort study was conducted on 52 children with anti-factor H antibody-associated aHUS in Beijing Children′s Hospital, Capital Medical University from November 2011 to November 2021.Patient information about the genetic background, clinical and renal pathological characteristics, treatment, and prognosis were collected.Then, the disease recurrence and prognosis were analyzed using the survival curve and Cox regression model. Results:In 52 children, there were 33 males and 19 females.The average age of onset for aHUS was 2.4-12.8 years, and 92.3%(48/52) of the children developed symptoms at the age of 4-12 years.The copy numbers of complement factor-H-related 1 (CFHR1) and complement factor-H-related 3 (CFHR3) genes were calculated in 42 children.Among the 42 cases, 18 cases (42.9%) had CFHR1 homozygous deletion, and 83.3% (15/18) of them also had CFHR3 homozygous deletion.All the patients were given plasma therapy.Besides, 76.9% (40/52) of the children were treated with immunosuppressive therapy (steroid and/or immunosuppressant) at the first onset of the disease.About 86.5%(45/52 cases) of the patients received immunosuppressive therapy in the course of disease, and the immunosuppressive treatment lasted for 6-20 months in total.The median follow-up time was 58 (28, 91) months.Among 52 patients, only 12 patients (23.1%) suffered disease recurrence.The relapse-free survival rate in children with CFHR1 homozygous deletion was significantly lower than that in children with non-homozygous deletion ( χ2=4.700, P=0.030). The relapse-free survival rate in children with CFHR1 and CFHR3 homozygous deletions was also significantly lower than that in other children ( χ2=4.181, P=0.041). At the end of the follow-up, 73.1%(38/52) of the children had normal renal function and no persistent proteinuria or hypertension.23.1%(12/52 cases) of the children had persistent proteinuria and/or hypertension.One child had Stage 3-4 chronic kidney disease, and 1 child was dialysis dependent. Conclusions:Anti-factor H antibody-associated aHUS is prone to occur in children aged between 4-12 years old, who respond well to plasma therapy and immunosuppressive therapy.Children with anti-factor H antibody-associated aHUS and CFHR1 and CFHR3 homozygous deletions have a high recurrence rate.Treatment with immunosuppressive therapy and assessment of the copy number of CFHR1 and CFHR3 genes in the early stage of the disease are important for preventing disease recurrence and improving prognosis.
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Objective:To understand clinical characteristics, treatment effects and prognosis of children with methylmalonic acidemia (MMA) presented with hemolytic uremic syndrome(HUS).Methods:The medical records of children with MMA were collected in Beijing Children′s Hospital, Capital Medical University from January 2012 to January 2019, the clinical manifestations, laboratory, imaging material, inspection results, renal pathological, gene analysis, treatment effect, and prognosis of MMA children with renal damage were analyzed, and were followed-up for 1-7 years.Results:Thirty cases were diagnosed as MMA with secondary renal damage.Eight cases(26.67%) showed as MMA-HUS.Age was from 1 month and 14 days to 12 years and 10 months old.There were 4 males and 4 females.The concentration of urine methylmalonic acid increased by 10-62 times.All were combined with hyperhomocysteine(HCY). The level of serum methylmalonic acid(1.5-11.8 mg/L), propylene carnitine(6.33-9.77 μmol/L)and the ratio of propylene /ethylene carnitine (0.24-0.29)were increased.Manifested as the mental and physical development retardation, anemia, jaundice, renal dysfunction, platelet reduction, hematuria, proteinuria in 8 cases, hypertension in 6 cases, frequent vomiting and convulsions in 2 cases.Two cases had a positive family history.Renal pathology showed that mesangial cells and mesangial matrix proliferation broadening, electron dense deposits no mesangial area, renal tubular epithelial cell swelling degeneration, immunofluorescence was negative.Two cases were genetically analyzed. One case was a CblC type MMACHC compound heterozygous mutation[c.80A>G(p.Q27R); c.217C>T(p.R73X)] and CblX type HCFC1 heterozygous mutation [c.3757G>A(p.R1253C)] double mutation; 1 case was a CblC type MMACHC compound heterozygous mutation[c.365A>T(p.H122L); c.609 G>A(p.W203X)]. Children diagnosed were treated with vitamin B 12, etc.Four cases of children gave up.The others, after treatment, were improved. Conclusions:MMA-HUS might be associated with multiple organ failure.Early diagnosis was the key, timely treatment could effectively control the disease, improve the prognosis.It should be followed up for ever.
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Objective@#To analyze the Oxford classification (MESTC) and the International Study of Kidney Disease in Children (ISKDC) classification for evaluating the clinical manifestations, histological lesion and short-term prognosis of children with Henoch-Schönlein purpura nephritis (HSPN).@*Methods@#According to the Oxford classification and ISKDC classification, the histological lesions of children with HSPN diagnosed by renal biopsy from Beijing Children's Hospital affiliated to Capital Medical University from January 2018 to December 2018 were re-evaluated. The renal biopsy specimens of the selected subjects were scored according to the Oxford classification and the ISKDC classification. According to whether the first symptom was combined with renal performance, MESTC score and ISKDC classification, children were grouped. The differences in clinicopathological manifestations between the groups were compared. Correlation between MESTC and ISKDC grades was analyzed by nonparametric test rank correlation. Kaplan-Meier survival curve and Log-rank test were used to compare the difference of proteinuria remission rate between the two groups. Univariate and multivariate Cox regression equations were used to analyze the influencing factors of the proteinuria remission rate.@*Results@#A total of 78 children with HSPN were enrolled. There were 37 male patients (47.4%) with age of (10.4±2.9) years. When the patients were divided according to MESTC scores and ISKDC classification, the results showed that the proportion of children with nephrotic-range proteinuria in the group of endocapillary hypercellularity (E1, P=0.008), segmental glomerulosclerosis (S1, P=0.015) and ISKDCⅢ(P=0.041) was higher than that of E0, S0 and ISKDCⅡ groups. The proportion of children with E1 (P=0.015), crescents (C1&C2, P=0.025) or ISKDCⅢ(P=0.017) that had been treated with high-dose methylprednisolone was higher. The result of Kaplan-Meier survival curve showed more difficult for proteinuria remission in children with C2 are than C0&C1 group (P=0.026), while no difference were found when children were grouped by M, E, S, T and ISKDC. Multivariate Cox regression analysis showed that the C2 (HR=0.143, 95%CI 0.020-1.046, P=0.055) might be a risk factor for proteinuria remission, while the P value was close to 0.05.@*Conclusions@#Children with HSPN scored as ISKDCⅢ, E1 and S1 are more likely to show nephrotic-range proteinuria. C2 may indicate that patients are more difficult to achieve proteinuria remission.
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Objective To analyze the Oxford classification (MESTC) and the International Study of Kidney Disease in Children (ISKDC) classification for evaluating the clinical manifestations,histological lesion and short-term prognosis of children with Henoch-Sch(o)nlein purpura nephritis (HSPN).Methods According to the Oxford classification and ISKDC classification,the histological lesions of children with HSPN diagnosed by renal biopsy from Beijing Children's Hospital affiliated to Capital Medical University from January 2018 to December 2018 were re-evaluated.The renal biopsy specimens of the selected subjects were scored according to the Oxford classification and the ISKDC classification.According to whether the first symptom was combined with renal performance,MESTC score and ISKDC classification,children were grouped.The differences in clinicopathological manifestations between the groups were compared.Correlation between MESTC and ISKDC grades was analyzed by nonparametric test rank correlation.Kaplan-Meier survival curve and Log-rank test were used to compare the difference of proteinuria remission rate between the two groups.Univariate and multivariate Cox regression equations were used to analyze the influencing factors of the proteinuria remission rate.Results A total of 78 children with HSPN were enrolled.There were 37 male patients (47.4％) with age of (10.4+2.9) years.When the patients were divided according to MESTC scores and ISKDC classification,the results showed that the proportion of children with nephrotic-range proteinuria in the group of endocapillary hypercellularity (E1,P=0.008),segmental glomerulosclerosis (S1,P=0.015) and ISKDC Ⅲ (P=0.041) was higher than that of E0,S0 and ISKDC Ⅱ groups.The proportion of children with E1 (P=0.015),crescents (C1&C2,P=0.025) or ISKDC Ⅲ (P=0.017) that had been treated with high-dose methylprednisolone was higher.The result of Kaplan-Meier survival curve showed more difficult for proteinuria remission in children with C2 are than C0&C1 group (P=0.026),while no difference were found when children were grouped by M,E,S,T and ISKDC.Multivariate Cox regression analysis showed that the C2 (HR=0.143,95％C1 0.020-1.046,P=0.055) might be a risk factor for proteinuria remission,while the P value was close to 0.05.Conclusions Children with HSPN scored as ISKDC Ⅲ,E1 and S1 are more likely to show nephrotic-range proteinuria.C2 may indicate that patients are more difficult to achieve proteinuria remission.
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Galactosemia is a metabolism abnormality caused by enzyme deficiency in the process of lactose metabolism.Patients with galactosemia usually have growth retardation, liver and kidney dysfunction and sepsis.Sometimes, they may also suffer from cataract, neurodevelopmental abnormalities and premature ovarian failure.In this article, the clinical data of a patient with galactosemia and heavy proteinuria who was hospitalized in Beijing Children′s Hospital Affiliated to Capital Medical University were collected.The etiology, clinical manifestations, treatment and follow-up data were analyzed retrospectively.The patient presented with massive proteinuria, and he was diagnosed with galactosemia by blood and urine metabolism screening and genetic testing.After dietary adjustment, the prognosis was good.Children with galactosemia and heavy proteinuria are rare, who should be considered with metabolic diseases in clinical practice, and the timely diagnosis and intervention are required.
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Objective:To summarize the clinical characteristics of children with Streptococcus pneumoniae associated hemolytic uremic syndrome(SP-HUS). Methods:Clinical data, laboratory results, treatment and prognosis of patients with SP-HUS in Beijing Children′s Hospital, Capital Medical University from January 2010 to December 2017 were retrospectively analyzed.Results:Seven 7 children (5 boys and 2 girls) aged 18-43 months were enrolled.All of them had toxic symptoms and dyspnea.The clinical symptoms were fever and cough in 5 cases, and abdominal pain and vomiting in 2 cases.Five of them had fever and cough for onset, and two had abdominal pain and vomit for onset, hemolytic anemia, thrombocytopenia and decreasing renal function in 3-9 days.Clinical examination showed cardiac dysfunction in 4 cases, septic shock in 2 cases and neurological damage in 2 cases.Five cases had only Streptococcus pneumoniae infection, 2 cases had both Streptococcus pneumoniae and Acinetobacter infections.Both C-reactive protein and procalcitonin were significantly high(80-200 mg/L and 6.43-100.00 μg/L, respectively) in 7 cases.Peripheral blood smear demonstrated fragmented red blood cells.Marrow smear showed inhibition of erythroid proliferation in 4 cases and toxic granules in the granulocytes.The direct Coombs tests were positive in all but 1 case.Serum complement C 3 decreased in acute phase(0.42-0.66 g/L). In the acute stage chest X-ray showed massive consolidation, followed by liquefaction necrosis, cystic segmented encapsulation and void changes.Ultrasound with Doppler revealed enlarged liver and kidney which normalized in 2-3 months and 6-9 months, respectively.All the patients were treated with antimicrobial therapy for 4-8 weeks and received respiratory support, 5 cases received blood purification treatment, and 4 cases received plasmapheresis.Deteriorating hemolysis was not observed following the infusion of frozen plasma.washed type red blood cells or suspension red blood cells in all 7 cases.Platelet numbers gradually recovered in 2-3 weeks.Kidney function improved and normalized in 2-4 weeks.Radiographic demonstrations of the chest normalized gradually in almost 3-6 months. Conclusions:It is necessary to consider the possibility of SP-HUS in very young children with severe pneumonia who showed microangiopathic hemolytic anemia, thrombocytopenia and decreasing renal function.Clinicians should identify high-risk children.Timely respiratory support and blood purification are important to improve prognosis.
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Objective@#To observe the value of serum IgG combined with IgE in the diagnosis of steroid-sensitive nephrotic syndrome (SSNS) children.@*Methods@#Children with primary nephrotic syndrome diagnosed in Beijing Children's Hospital affiliated to Capital Medical University from January 2018 to March 2019 were enrolled. According to the 4 weeks steroid therapy, the patients were divided into steroid sensitive group and steroid resistance group. The receiver operating characteristic curve (ROC) was used to analyze the diagnostic value of serum IgG combined with IgE for SSNS.@*Results@#There were no significant differences in age, gender, course of the disease, hemoglobin, platelet, urea nitrogen, creatinine, albumin, globulin, 24 h urine protein, IgA level, IgM level and hematuria between steroid sensitive group and steroid resistance group (all P>0.05). There were significant differences in C3, IgG and IgE levels (all P<0.05). ROC curve analysis showed that the cut-off values of IgG and IgE in the diagnosis of SSNS were 2.57 g/L (AUC=0.717, 95%CI 0.623-0.812, P<0.001) and 174.3 IU/ml (AUC=0.828, 95%CI 0.751-0.905, P<0.001). The area under the ROC curve for IgG<2.57 g/L in combination with IgE>174.3 IU/ml in the diagnosis of SSNS was 0.904 (95%CI 0.834-0.953, P<0.001). Logistic regression showed that children who met both IgG<2.57 g/L and IgE>174.3 IU/ml were 51.00 times more likely to have SSNS than those who did not (P<0.001).@*Conclusion@#IgG combined with IgE can be used as a simple and practical clinical marker for predicting SSNS.
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Objective To understand the distribution of cancer deaths in China in 2015 and provide reference for the prevention and control of cancer.Methods Based on the results of Global Burden of Disease 2015,the cancer death distributions in different age groups,sex groups,provinces or by different malignant tumor in Chinese were described.Results The age-standardized mortality rate of cancer was 159.01/100 000 in China in 2015.The mortality rate was highest in age group ≥70 years (1 102.73/100 000),and lowest in age group 5-14 years (5.40/100 000).The mortality rate in males was 2.15 times higher than that in females.The first 5 provinces with high cancer mortality rate were Anhui,Qinghai,Sichuan,Guangxi and Henan.Lung cancer,liver cancer,stomach cancer,esophageal cancer and colorectal cancer ranked 1-5 in term of mortality rate.Conclusion The cancer mortality differed with age,gender,area and different malignant tumors,suggesting the necessity to develop targeted prevention and control strategies.
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Objective To summarize the clinical data of atypical hemolytic uremic syndrome (aHUS) and analyze the treatment and prognosis.Methods A prospective cohort study was conducted on 66 cases in Beijing Children's Hospital affiliated to Capital Medical University from January 2011 to December 2017.The children were divided into positive and negative auto-antibody groups according to the results of anti-factor H autoantibody test.The clinical characteristics,treatment plan and prognosis of the two groups were compared.Results Among the 66 children who met the inclusion criteria,there were 43 cases (65.2％) in the positive group,with an average onset age of (8.0±2.9) years.There were 23 eases (34.8％) in the negative group,with an average onset age of (3.0± 2.6) years.On the basis of plasma treatment,in the positive group,the usage rate of hormone was 83.3％ (35/42) and the usage rate of immunosuppressive agents was 42.9％(18/42),while in the negative group,the rates were 63.6％(14/22) and 13.6％(3/22) respectively.The average follow-up time was 19.3 months.One child in each group was lost to follow-up.In the positive group,8 cases recurred (19.0％)and the average recurrence interval time was 16.1 months.In the negative group,7 cases recurred (31.8％) and the average recurrence interval time was 9.3 months.And the recurrent interval time in the positive group was more longer than the negative group (P ＜ 0.05).A total of 85.9％(55/64) children had complete hemolysis control and complete recovery of renal function,in which the positive group was 85.7％(36/42) and negative group was 86.4％(19/22).However,7.8％(5/64) children had abnormal renal function,in which the positive group was 9.5％(4/42) and the negative group was 4.5％(1/22).And 4.7％(3/64) children died,in which the positive group was 2.4％(1/42) and the negative group was 9.1％ (2/22).The one left (1.6％) showed dialysis dependence,which was positive for the auto-antibody.Multifactor Cox regression analysis showed that the age of less than 3 years old was the risk factor of poor prognosis (HR=4.651,95％CI 0.988-21.898,P=0.047).Conclusions The positive proportion of anti-factor H autoantibody in children with aHUS is high.The age of these children is older.Individualized therapy based on anti-factor H autoantibody and immunosuppressive therapy is of great significance for disease remission,preventing recurrence and improving the prognosis.Age less than 3 years old is the risk factor for poor prognosis.
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Objective To understand the distribution of cancer deaths in China in 2015 and provide reference for the prevention and control of cancer.Methods Based on the results of Global Burden of Disease 2015,the cancer death distributions in different age groups,sex groups,provinces or by different malignant tumor in Chinese were described.Results The age-standardized mortality rate of cancer was 159.01/100 000 in China in 2015.The mortality rate was highest in age group ≥70 years (1 102.73/100 000),and lowest in age group 5-14 years (5.40/100 000).The mortality rate in males was 2.15 times higher than that in females.The first 5 provinces with high cancer mortality rate were Anhui,Qinghai,Sichuan,Guangxi and Henan.Lung cancer,liver cancer,stomach cancer,esophageal cancer and colorectal cancer ranked 1-5 in term of mortality rate.Conclusion The cancer mortality differed with age,gender,area and different malignant tumors,suggesting the necessity to develop targeted prevention and control strategies.
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Objective@#In order to learn the serum level of galactose-deficient IgA1 (GdIgA1), IgG antibody to galactose-deficient IgA1(GdIgA1-IgG) and the clinical role of them in IgA nephropathy(IgAN) children.@*Method@#We compared blood levels of GdIgA1, GdIgA1-IgG in 33 children with IgAN, 38 children with other renal disease (including focal segmental glomerular nephritis, minimal change disease, Alport syndrome and thin basement membrane nephropathy) as disease controls, 35 healthy children as normal controls with enzyme-linked immunosorbent assay(ELISA). Twenty-four-hour urine protein content and Lee′s pathological classification were collected in IgAN cases. The difference among the three groups were examined by analysis of variance. The specificity and sensitivity of GdIgA1, GdIgA1-IgG to IgA nephropathy were evaluated by ROC curve.The correlations were analyzed by correlation analysis among GdIgA1, GdIgA1-IgG and urine protein content and pathological classification . All statistical analyses were performed by SPSS 18.0. P value of <0.05 was considered statistically significant.@*Result@#The levels of GdIgA1 and GdIgA1-IgG significantly elevated in children with IgAN compared to disease controls and normal controls. GdIgA1(mg/L): 1.728±0.456, 1.123±0.461, 1.410±0.905 (F=15.04, P<0.001); GdIgA1-IgG(g/L): 1.299±0.499, 1.111±0.549, 0.628±0.142 (F=76.96, P<0.001). Area under the curve of ROC of GdIgA1 between IgAN and normal controls was 0.765, with specificity 0.943 and sensitivity 0.697, while the area under the curve of GdIgA1-IgG was 0.969, with specificity 0.971 and sensitivity 0.939.The blood level of GdIgA1-IgG was mildly correlated with both 24-hour urine protein(r=0.364, ) and renal pathological grading(r=0.392) in children with IgAN.@*Conclusion@#The levels of GdIgA1 and GdIgA1-IgG significantly elevated in children with IgAN compared to disease controls and normal controls. GdIgA1-IgG had higher sensitivity in distinguishing IgAN from normal controls, GdIgA1-IgG was mildly correlated with 24-hour urine protein volume and pathological grading in children with IgAN.
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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.
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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.
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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.
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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.
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Objective To explore the clinical features of invasive fungal infection in 27 children treated at nephrology department between 1999 and 2014.Methods Twenty-seven cases of invasive fungal infection at nephrology department were analyzed retrospectively.Results Candida urinary infection was found in 9 cases,pneumocystis carinii pneumonia were found in 8 cases,invasive pulmonary aspergillosis and urinary aspergillosis found in 4 cases and 1 case respectively,cryptococcus neoformans meningitis and pulmonary candidiasis found respectively in 2 cases,and pulmonary filamentous fungal disease was found in 1 case.These 27 cases showed different features of illness:10 primary nephrotic syndrome,7 secondary nephrotic syndrome,6 malformation of urinary development and 4 preterm birth ＜ 32 weeks gestational age with low birth weight.All of the cases had the history of taking antibiotics.Seventeen cases of them had used corticosteroid and cytotoxic drugs in a long-term.Sixteen cases had experienced invasive procedures.All children had fever in varying degrees,14 cases showed gasp,7 cases had progressive hypoxia and respiratory failure,and 5 cases developed into multiple organs failure.Chest computed tomographic (CT) imaging data showed diffuse ground-glass opacity with mosaic sign of pneumocystis carinii pneumonia(8 cases).The features of pulmonary aspergillosis included multiple nodules and cavity in bilateral lungs (4 cases).The radiologic findings showed patching lesions with indistinct edge and uncertained density of bilateral middle-lower lung fields with pulmonary candidiasis (2 cases).Full dose of antifungal drug was given to 23 cases of them,16 cases recovered completely,3 got better,4 cases died.Four cases gave up full dose antifungal therapy.Conclusions Long-term use of corticosteroid and cytotoxic drugs,use of broad-spectrum antibiotics,invasive therapies,such as indwelling centralvenous catheters,endotracheal intubation with mechanical ventilation,and preterm birth with low birth weight were risk factors in predicting invasive fungal infection.Chest CT findings were different among these cases.Even though not specific,the relatively differences were helpful to the differential diagnosis of these diseases.
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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.
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Objective To understand the clinical characteristics, treatment effect and prognosis of children with methylmalonic acidemia (MMA) combined with renal damage, and to provide experiences about how to improve the level of diagnosis and treatment.Methods The medical records of children with MMA were collected from January of 2007 to December of 2013 in Beijing Children's Hospital Affiliated to Capital University of Medical Sciences,while the clinical manifestations,laboratory findings, imaging material, inspection results were analyzed, and the renal pathological gene analysis, treatment effect, and prognosis of MMA children with renal damage were studied, and follow-up was carried out for 3 months up to 7 years.Results Among the two hundred and ninety-six cases of MMA,28 cases (9.5％) with renal damage,including 19 boys and 9 girls, 18 patients with hyperhomocysteinemia.Their ages varied from 2 days to 13 years old when confirmed.The first symptoms of 16 cases were proteinuria, hematuria, and edema, 9 cases were complicated with acute renal failure,5 cases were diagnosed as hemolytic uremic syndrome,6 patients were diagnosed as nephrotic syndrome, receiving enough glucocorticoids treatment without improvement.The routine examination of the urine showed that proteinuria (241-3 060 mg/d), methylmalonic acid levels and urinary protein were positively correlated (r =0.982, P =0.003), as urinary β2-microglobulin [(360.386 ± 231.496) μg/L], retinol binding protein [(338.537 ± 243.293) μg/L] increased.Extrarenal clinical manifestations were the spiritual, mental and physical development retardations, seizures, anemia etc.Two cases had a positive family history.Renal pathology showed mesangial cells and mesangial matrix proliferation broadening, no electron dense deposits in mesangial area, renal tubular epithelial cell swelling degeneration, and immunofluorescence was negative.Gene analysis of 3 cases showed exon1:c.80A ＞ G,p.Q27R and exon1 :c.365A ＞ T,p.H122L/c.609 G ＞ A,p.W203X.Children with MMA were treated with vitamin B12 treatment.Two cases of multiple organ failure died during hospitalization, and 2 cases died after leaving hospital.After treatment, the level of urinary methylmalonic acid was significantly decreased, while nervous system symptoms improved significantly, and the mental state and the renal function were improved.Conclusions MMA may be associated with kidney damage,usually with severe renal tubular and glomerular impairment.Early diagnosis is the key,and timely treatment can effectively control the disease, improve the prognosis.