Retrospective study of plasma exchange in patients with idiopathic rapidly progressive glomerulonephritis and vasculitis

A retrospective study of 48 patients was conducted to evaluate the efficacy of plasma exchange in children with idiopathic rapidly progressive glomerulonephritis (IRPGN), and renal or non-renal vasculitis. All patients were followed up at a single centre over a 15 year period. Treatment consisted of corticosteroids and/or cytotoxic agents. Plasma exchange was used in all patients because of severe renal involvement and/or clinical deterioration. One hundred per cent of patients with renal vasculitis who started plasma exchange within one month of disease onset and 58% of cases with IRPGN had significant improvement in renal function. No relapses of vasculitis were observed after treatment with plasma exchange in patients with renal and non-renal vasculitis. The results suggest that plasma exchange associated with immunosuppressive treatment could be of benefit in cases of IRPGN or vasculitis in terms of both renal and extrarenal recovery.     

血液净化治疗非肾脏疾病危重症患儿的临床分析

目的 总结血液净化(blood purification,BP)治疗非肾脏疾病危重症患儿的经验.方法 回顾性分析2009年1月至12月在我院PICU因非肾脏疾病而行BP治疗的10例危重症患儿的临床资料.10例患儿中,5例为急性肝能衰竭;2例自身免疫性疾病,其中1例吉兰-巴雷综合征,1例幼年特发性关节炎(全身型)并发MAS;2例重症脓毒症;1例由于代谢病出现持续性的严重代谢性酸中毒.结果 10例非肾脏疾病危重患儿中,实施CVVH联合PE治疗4例次,单纯CVVH治疗3例,单纯PE治疗3例.临床痊愈7例,好转1例,家长放弃2例.结论 BP作为一种脏器功能替代及支持的新疗法对PICU中肝功能衰竭及自身免疫性疾病等非肾脏病危重症患儿有较好的疗效.     

血液净化治疗非肾脏疾病危重症患儿的临床分析

目的 总结血液净化(blood purification,BP)治疗非肾脏疾病危重症患儿的经验.方法 回顾性分析2009年1月至12月在我院PICU因非肾脏疾病而行BP治疗的10例危重症患儿的临床资料.10例患儿中,5例为急性肝能衰竭;2例自身免疫性疾病,其中1例吉兰-巴雷综合征,1例幼年特发性关节炎(全身型)并发MAS;2例重症脓毒症;1例由于代谢病出现持续性的严重代谢性酸中毒.结果 10例非肾脏疾病危重患儿中,实施CVVH联合PE治疗4例次,单纯CVVH治疗3例,单纯PE治疗3例.临床痊愈7例,好转1例,家长放弃2例.结论 BP作为一种脏器功能替代及支持的新疗法对PICU中肝功能衰竭及自身免疫性疾病等非肾脏病危重症患儿有较好的疗效.     

Plasma exchange therapy in children and adolescents with systemic lupus erythematosus (SLE)

Five children (11.5-17.5 years of age) with severe systemic lupus erythematosus (SLE) were treated with plasma exchange. Three children suffered from renal failure and hypertension, one adolescent girl from gastrointestinal and arthritic pains with fever, and one patient from generalized paresis. All patients had excessive serological signs of disease activity. Forty-five sessions of plasma exchange were performed without serious complications. Four children showed improvement of SLE after initiation of plasma exchange in combination with immunosuppressive therapy in two of them renal replacement therapy could be stopped. In the 2 patients with non-renal SLE-complications a dramatic rapid improvement of the symptoms was observed. One girl succumbed to severe hypertension with cerebral bleeding and fungal sepsis after pulsE therapy a few days after start of plasma exchange. Plasma exchange should be started before observation of life threatening complications of SLE. Further information is needed about indication, frequency and duration of plasma exchange in children with SLE.     

甲巯咪唑与抗中性粒细胞胞浆抗体阳性血管炎的关系

目的 研究抗甲状腺药物甲巯咪唑(MMI)与抗中性粒细胞胞浆抗体(ANCA)阳性血管炎的关系.方法 对33例Graves病患儿在服药前及服药后检测血ANCA.同时对我院近年来诊治的2例毒性弥漫性甲状腺肿(Graves病)患儿在服用MMI期间出现ANCA阳性血管炎进行临床病理分析.结果 2例患儿在服用MMI第5～6年出现抗中性粒细胞胞浆抗体阳性血管炎,临床出现血尿、肾功能减退,肾活检示肾血管炎改变,急性肾血管坏死伴新月体形成.经免疫抑制剂等治疗临床缓解;33例Graves病患儿服药前血ANCA均为阴性.有3例患儿分别在服用MMI第2、3个月及2年时出现ANCA阳性,阳性率9%(3/33).临床出现镜下血尿,服药后ANCA阳性率高于服药前(x2=5.3,P<0.05).3例患儿经对症治疗后镜下血尿消失,血ANCA复查转阴性.结论 MMI与ANCA阳性血管炎可能有关,可产生从轻到重的肾脏受累临床表现,采用不同的治疗方法可使症状消失.     

儿童继发性血栓性血小板减少性紫癜1例并文献复习

目的 提高对儿童不典型继发性血栓性PLT减少性紫癜（TTP）的认识。 方法 总结1例无神经系统受累的继发性TTP患儿的临床资料、实验室检查结果、ADAMTS13酶活性和Anti-ADAMTS13抗体检测结果,行系统文献检索并文献复习。 结果 男性患儿,12岁,急性起病,病初有发热,双下肢可见瘀点,PLT及Hb进行下降,血涂片可见破碎RBC,高胆红素血症,LDH明显升高,镜下血尿,肾功能正常,补体正常,考虑血栓性微血管病(TTP或非典型溶血尿毒综合征)。为进一步明确诊断, 行ADAMTS13酶活性检测2.3%（正常值40％~130%）,ADAMTS13抗体检测90 U·mL-1（正常值＜12 U·mL-1）,确诊继发性TTP,予血浆置换和激素治疗。4个月后患儿停用所有药物,目前停药6月无复发。系统检索中国知网、万方和PubMed数据库,共有14篇英文文献中40例继发性TTP进入本文分析,发病年龄（10.2±5.2）岁,男19例,女21例,发热36例（90%）,神经系统受累28例（70%）,肾脏受累18例（45%）,均有贫血和PLT降低。3例死亡,37例血浆置换+激素治疗,31例（83.8%）对血浆置换治疗即时反应好,1例因血浆过敏和1例血浆置换导管相关感染改为激素+利妥昔单抗治疗反应好,1例难治性继发性TTP加长春新碱（利妥昔单抗上市前）随访时复发,2例发生血浆置换依赖,加环孢素后治疗反应好,1例治疗反应不好,加长春新碱后治疗反应好,,4例失访（10.8%）,平均随访时间29月（3~72个月）,13例（39.4%）出现复发,9/13例加利妥昔单抗中仍有2例复发。 结论 贫血和PLT降低应怀疑TTP,需行ADAMTS13酶活性及其抗体的检测,有助于区别遗传性和获得性TTP;血浆置换+激素,或+利妥昔单抗是TTP的治疗组合选项。     

Outcomes of renal transplant in patients with anti‐complement factor H antibody‐associated hemolytic uremic syndrome

Atypical HUS associated with anti‐CFH autoantibodies is an uncommon illness associated with high risk of progression to end‐stage renal disease. Disease relapses after transplantation, observed in one‐third cases, often lead to graft loss. We report four patients with anti‐CFH antibody‐associated HUS who underwent renal transplantation 16–62 months from initial presentation. Two patients each received organs from deceased and living‐related donors. Anti‐CFH antibody titers were monitored during the illness and following transplantation. All patients received two doses of IV rituximab before or after transplantation; three patient each received 1–2 g/kg of IV immunoglobulin or underwent 2–5 sessions of plasma exchanges. The use of therapeutic plasma exchange, IV immunoglobulin, and rituximab in two cases enabled two‐third reduction in anti‐CFH antibody titers before transplantation. At 5‐ to 26‐month follow‐up, all patients showed satisfactory graft function without recurrence of HUS. This is the first report of patients with anti‐CFH antibody‐associated HUS who underwent living‐related renal transplantation. Clearance of anti‐CFH antibody by therapeutic plasma exchange and adjuvant immunosuppression aimed at decreasing antibody levels may enable successful transplantation and recurrence‐free survival.     

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??Abstract: Objectives??To investigate the clinical characteristics of the children with hemolytic-uremic syndrome??HUS?? and to verify the effect of plasma exchange in HUS. Methods??We analyzed 9 patients with HUS in renal service in Shengjing Hospital of China Medical University from July 2001 to September 2007. All patients were analyzed with main symptoms??lab examinations?? treatment efficiency of steroid and IVIG and plasma exchange??PE??. All patients had been followed up from 0.5??6 years with treatment of oral administration of steroid. Results??It seemed that there was no difference in the clinical characteristics and the lab examinations between the patients with D+ HUS and D- HUS. The patients should be treated with plasma exchange as soon as possible if there was no effect with steroid or IVIG. Blood dialysis or plasma exchange would be added if there was no obvious effect with PE for the first time. Steroid would be orally administered by the HUS patients with proteinuria after PE?? which was effective for proteinuria. Among the following 9 patients?? 1 patient was followed with proteinuria and was done with re-renal biopsy?? which showed increased antigen and renal interstitial injury?? 1 patient with hypertention. There was no abnormal signs for the other 7 patients. Conclusion??It is effective for the patients with HUS treated with plasma exchange. It seems that oral administration of steroid is effective for the HUS patients with proteinuria. All patients with HUS should be paid more attention to in the long-term follow up.     

Outcome of reaching end stage renal failure in children under 2 years of age.

AIMS: To determine the outcome of children who reach end stage renal failure before the age of 2 years. METHODS: Using a retrospective questionnaire, 10 years' data were collected from the paediatric nephrology units in Britain and Ireland (1988 to 1997, follow up 1.3-11.5 years). RESULTS: A total of 192 children were identified; 0.31/million/year. Most had congenital or inherited conditions, and there were more boys. Latterly, half were diagnosed antenatally. Ninety per cent were dialysed initially, most using home peritoneal cyclers, some by haemodialysis through central lines. Five per cent recovered sufficient function to come off dialysis. Most required tube feeding (often gastrostomy) and erythropoietin; some needed growth hormone. A total of 56% received a transplant (2% without prior dialysis) at (medians) 2.6 years and 12.3 kg. The 2 and 10 year survival of first kidneys was 78%. Growth improved following transplantation. Fourteen per cent died because treatment was not started or was withdrawn. Most had particularly complex renal conditions, or additional major non-renal diagnoses. Typically, decisions not to treat were made mutually between clinicians and families. When treatment was continued, 71% survived, and few had serious non-renal conditions. Most attended normal schools, and by 6 years of age, less than 10% still required dialysis. Infants starting treatment under and over 1 month of age fared equally well. CONCLUSIONS: By school age, most infants treated for end stage renal failure will have a functioning transplant, reasonable growth, and will attend a normal class, regardless of the age at which they commence treatment. Treatment is seldom sustained in children who have serious additional medical conditions. It is reasonable to treat infants with uncomplicated renal failure.     

Outcome of reaching end stage renal failure in children under 2 years of age

Aims: To determine the outcome of children who reach end stage renal failure before the age of 2 years. Methods: Using a retrospective questionnaire, 10 years'' data were collected from the paediatric nephrology units in Britain and Ireland (1988 to 1997, follow up 1.3–11.5 years). Results: A total of 192 children were identified; 0.31/million/year. Most had congenital or inherited conditions, and there were more boys. Latterly, half were diagnosed antenatally. Ninety per cent were dialysed initially, most using home peritoneal cyclers, some by haemodialysis through central lines. Five per cent recovered sufficient function to come off dialysis. Most required tube feeding (often gastrostomy) and erythropoietin; some needed growth hormone. A total of 56% received a transplant (2% without prior dialysis) at (medians) 2.6 years and 12.3 kg. The 2 and 10 year survival of first kidneys was 78%. Growth improved following transplantation. Fourteen per cent died because treatment was not started or was withdrawn. Most had particularly complex renal conditions, or additional major non-renal diagnoses. Typically, decisions not to treat were made mutually between clinicians and families. When treatment was continued, 71% survived, and few had serious non-renal conditions. Most attended normal schools, and by 6 years of age, less than 10% still required dialysis. Infants starting treatment under and over 1 month of age fared equally well. Conclusions: By school age, most infants treated for end stage renal failure will have a functioning transplant, reasonable growth, and will attend a normal class, regardless of the age at which they commence treatment. Treatment is seldom sustained in children who have serious additional medical conditions. It is reasonable to treat infants with uncomplicated renal failure.     

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目的探讨原发性肾病综合征患儿(PNS)血脂代谢紊乱与肾功能改变的关系。方法2004-01—2006-01,根据肾功能检查结果,将广西医科大学第一附属医院儿科收治的76例PNS患儿分成无肾功能损害组(46例)与肾功能损害组(30例),检测血浆总胆固醇(TC)、甘油三酯(TG)、高密度脂蛋白(HDL)、非-高密度脂蛋白(non-HDL)、低密度脂蛋白(LDL)、载脂蛋白A1(ApoA1)、载脂蛋白B(ApoB)、ApoA1/B、血浆尿素氮(BUN)、肌酐清除率(Ccr)和尿酸(UA)等指标。结果(1)肾功能损害组TG、BUN、Ccr和UA均明显高于无肾功能损害组;(2)76例PNS患儿TG与Ccr及UA呈高度正相关(P<0.01),ApoA1与Ccr呈中度正相关(P<0.05),ApoB与UA呈中度正相关(P<0.05);(3)76例PNS患儿non-HDL与LDL呈高度正相关(P<0.01)。结论PNS合并肾功能损害的血脂代谢紊乱以TG显著增高为特征;TG和non-HDL变化可以作为临床判断PNS患儿肾功能损害程度及调脂措施有效性的指标。     

Acute Hemorrhagic Edema of Infancy; a Report of Five Iranian Infants and Review of the Literature

Background

Acute hemorrhagic edema of infancy (AHEI) is a benign self limiting leukocytoclastic vasculitis in young children. Serious complications, e.g. renal and gastrointestinal involvement, are not usually detected in AHEI patients.

Case Presentation

We report five patients with AHEI. Our patients were 17 to 21 months old. One patient presented with gastrointestinal bleeding due to this syndrome, the other one experienced second attack and scrotal edema due to epididymo-orchitis, while the third patient had renal involvement as hematuria and the other one had bilateral auricular chondritis. One of our cases was a typical case of AHEI without any complications, so a skin biopsy was not necessary. In this study, we describe the symptoms, probable triggering factors and treatment of choice for each patient.

Conclusion

Although AHEI is a childhood vasculitis with no impairment of the general condition, some organ involvements such as gastrointestinal, renal or scrotal lesions and rarely chondritis are probable in these patients.     

Management of major blunt pediatric renal trauma: Single-center experience

ObjectiveTo review the impact of major pediatric renal trauma and its management on long-term function and morphology of the injured kidney.MethodsThirty-six blunt trauma patients (20 males, 16 females) presented in 2004–2007 (age range 2 days to 14 years; mean 6.2 years). Thirty-seven renal units were included: 13 grade III, 14 grade IV, and 10 grade V injuries. Follow up was for 3–38 (mean 14) months. Patients were managed non-operatively unless vitally unstable. The most common causes of trauma were motor vehicle accidents and falls. Fourteen patients had associated non-renal injuries. Four patients had pre-existing renal problems.ResultsThe surgical intervention group (13 patients, 36%) included 9/10 grade V and 4/14 grade IV renal injuries. Surgical repair of lacerations was performed in seven cases, partial nephrectomy in four cases and nephrectomy in two cases. Follow up showed no significant change in renal function, and none developed hypertension. The non-operative group (24 patients, 63.2%) included all grade III injuries, 10 grade IV injuries, and one grade V injury. There was an excellent outcome for 18/24 patients (75%) with kidney preservation, no complications from urinary extravasation and hematoma resolution. The remaining patients had lower polar infarction (1), renal atrophy (1), persistent subcapsular collection (2), recurrent hematuria requiring angioembolization (1), and there was one death related to central nervous system injury.ConclusionThe outcome of our management of pediatric major renal trauma was favorable overall. Longer follow up is needed with regard to renal function and development of hypertension.     

Systemic lupus erythematosus of childhood onset: correlation between T cells expressing early and late activation antigens and disease activity

Correlation between T cell phenotypes, especially activated T cells expressing early (EA1) and late (HLA-DR) activation antigens and clinical features were investigated in 22 patients with systemic lupus erythematosus (SLE) of childhood onset. Percentages of T cells expressing EA1 and HLA-DR in 22 patients with SLE were significantly higher than those in controls. Comparison of T cell phenotypes between patients with active and inactive SLE showed that eight patients with active disease had significantly increased percentages of HLA-DR positive T cells than 14 with inactive disease (P<0.01). Serial examinations showed that the percentages of HLA-DR positive T cells were decreased after therapy in seven with active non-renal or active non-renal and renal diseases but not in one with only active renal disease. A possible significance of T cells expressing EA1 and HLA-DR in the management of patients with SLE is discussed.Abbreviations HLA-DR human leukocyte antigens-DR - SLE systemic lupus erythematosus     

Red blood cell exchange transfusion as an adjunct treatment for severe pediatric falciparum malaria, using automated or manual procedures

Pediatric falciparum malaria is associated with high morbidity and mortality rates. Cerebral malaria and renal failure are common among children with a high percentage of malaria-infected red blood cells. We report 3 cases of imported pediatric falciparum malaria with central nervous system involvement and/or renal failure that were treated initially with intravenous antimalarial therapy, with no clinical improvement. Red blood cell exchange transfusion (RBCET) was started; this resulted in decreases in the percentages of parasitized red blood cells of 80% to 90%. The RBCET was performed with either an automated 1-blood volume or manual 1.5-blood volume exchange. Most cases of falciparum malaria can be treated with intravenously administered antimalarial agents alone. However, for children who have high percentages of parasitized red blood cells with central nervous system involvement and/or renal failure, the use of RBCET as an adjunct treatment should be considered.     

The lungs in congenital bilateral renal agenesis and dysplasia.

A detailed quantitative analysis was made of the lungs from 8 infants dying with bilateral renal agenesis or dysplasia. Total lung volume was reduced in all cases, particularly in those with renal agenesis. In both groups there was a reduction in number of airway generations, indicating interference with development at between 12 and 16 weeks'' gestation. The alveoli in each acinus were reduced in size and, in some cases, number--although their stage of differentiation was normal for age--pointing to a disturbance of growth during later fetal life also. As liquor is largely non-renal in origin at least up to 16 weeks'' gestation, it seems that there are factors other than the oligohydramnios interfering in early lung growth in these cases, such as reduced proline production by the kidney.     

Juvenile polyarteritis: results of a multicenter survey of 110 children

OBJECTIVE: To characterize pediatric patients who had been diagnosed with polyarteritis nodosa (PAN) through necrotizing vasculitis of the small and mid-size arteries or those with characteristic findings on angiograms data were collected. STUDY DESIGN: Pediatricians were asked to classify their patients into one of the four suggested groups for juvenile PAN. Twenty-one pediatric centers worldwide participated with 110 patients. RESULTS: The girl:boy ratio was 56:54, with a mean age of 9.05 +/- 3.57 years. The cases were classified as: 33 (30%) cutaneous PAN; 5 (4.6%) classic PAN associated with hepatitis B surface antigen (HBs Ag); 9 (8.1%) microscopic polyarteritis of adults associated with antineutrophil cytoplasmic antibodies (ANCA); and 63 (57.2%) systemic PAN. Cutaneous PAN was disease confined to the skin and musculoskeletal system. All patients with HBs Ag-associated classic PAN were diagnosed with renal angiograms. Antiviral treatment was administered in most cases. Microscopic PAN patients had pulmonary-renal disease, in combination or separately. ANCA was present in 87%, and 2 patients progressed to end-stage renal failure. Patients classified with systemic PAN had multiple system involvement, almost all had constitutional symptoms, and all had elevated acute phase reactants. Corticosteroids and cyclophosphamide were the first choices of immunosuppressive treatment. The overall mortality was 1.1%. CONCLUSIONS: There were remarkable differences among pediatric patients with PAN, with different clinical manifestations and overall better survival and lower relapse rates when compared with adults.     

BK nephropathy in pediatric hematopoietic stem cell transplant recipients

Abstract:  BK nephropathy is a known cause of renal insufficiency in kidney transplant recipients. Activation of the polyoma virus may also occur in the native kidneys of non-renal allograft recipients. BK nephropathy has only been reported in a few patients after HCT, most being adult patients, and the single reported pediatric case had evidence of hemorrhagic cystitis. The response to antiviral therapy also seems to differ widely. Here, we describe two cases of BK nephropathy in the native kidneys of HCT recipients exposed to high levels of immunosuppression because of GVHD. Neither of our patients had any evidence of hemorrhagic cystitis. We present definitive renal pathology and detailed chronological evidence of the rising serum creatinine with simultaneous serum and urine BK PCR titers. In one of our cases, antiviral therapy did not seem beneficial as documented by continued renal dysfunction and elevated serum/urine BK PCR titers. Based on our report, intense immunosuppression in pediatric HCT recipients seems to be involved in the activation of BK virus and BK nephropathy should be suspected even in the absence of hematuria in HCT recipients with unexplained renal dysfunction.     

Duplex sonography of renal arteries as a diagnostic tool in hypertensive children

Computed duplex sonography was used to examine the renal arteries in 36 hypertensive children and adolescents (ages 4–17 years) with arterial hypertension of either renal or non-renal origin. Time-averaged flow velocities, maximum blood flow velocities as well as absolute renal blood flow and renal blood flow per gram kidney weight were measured. Normal flow velocities and normal to elevated renal blood flow volume was found in patients with acute glomerulonephritis and those with signs of chronic glomerulonephritis onset. Patients having advanced stages of chronic glomerulonephritis, on the other hand, were characterized by lower levels of all parameters. Unilateral renal artery stenosis was diagnosed correctly in four patients, although one intra-renal artery stenosis escaped imaging. Scarred kidneys exhibited low-normal or reduced flow velocities and renal blood flow volumes corresponded roughly to kidney size and preservation of normal kidney structure. Hypertension in some patients with normal kidneys showed a tendency to cause higher renal blood flow without consistent acceleration of blood flow velocities. We conclude that duplex sonography is a suitable primary diagnostic tool in measuring blood flow velocities and absolute renal blood flow volume in hypertensive children, thus facilitating the choice of the next diagnostic step.     

Neonatal renal vein thrombosis: review of the English-language literature between 1992 and 2006

Renal vein thrombosis is a complication that occurs in neonates with various underlying risk factors. It carries a grave prognosis for affected kidneys. Anticoagulant and fibrinolytic therapies have been promoted in the past with anecdotal success in some circumstances. However, prospective controlled trials are still lacking, and to date there have been no evidence-based guidelines available for the treatment of neonates with renal vein thrombosis. We retrospectively reviewed all the available medical literature pertaining to renal vein thrombosis published in English during the past 15 years. A total of 271 patients from 13 case series were identified by using the terms "renal vein thrombosis" and "neonates" via PubMed and Cochrane Library searches. Data then were extracted from each of the studies for analysis. During the past 15 years, a male predominance (67.2%) in neonatal renal vein thrombosis has been reported. More than 70% of patients had unilateral renal vein thrombosis, which was more prevalent on the left side (63.6%). The thrombus involved the inferior vena cava and was associated with adrenal hemorrhage in 43.7% and 14.8% of neonates, respectively. Forty percent of the patients were treated conservatively with supportive care alone. Among those patients who received anticoagulation therapy, unfractionated heparin and low molecular weight heparin were used alone in 21.6% and 20.7% of the patients, respectively. Fibrinolytic treatment alone was used in 11.2% of the patients. Only a minority of patients were treated with antithrombin (1.7%), warfarin alone, (0.9%) or underwent surgical intervention (0.3%). The majority (70.6%) of the involved kidneys became atrophic. A total of 9 neonates died with non-renal vein thrombosis-related conditions during the study period. Evidence-based recommendations on treatment cannot be made at the present time. Cooperative prospective studies that involve multiple centers are needed to elucidate the optimal treatment for neonatal renal vein thrombosis.