小儿特发性溶血尿毒症综合征13例临床分析

溶血尿毒症综合征（ＨＵＳ）的发病率近年来有逐渐升高趋势，对其发病机制的认识也有了进一步提高。ＨＵＳ可分为Ｓｈｉｇａ毒素相关性ＨＵＳ（典型ＨＵＳ，有腹泻病史）及特发性ＨＵＳ（无腹泻病史）两类。我们总结１９８２年至今本院收治的特发性ＨＵＳ患儿的临床资料。 临床资料 ｌ．一般资料：自１９８２年８月至今共收治特发性ＨＵＳ患儿１３例（１６例次，有３例因病情复发分别住院２次），其中男性９例，女性４例；平均年龄６．６岁（ｌ．２～１１岁）；发病季节ｌ～３月份９例次，４～６月份３例次，７～９月份３例次，１０～１２月…     

溶血性尿毒症综合征的血浆置换治疗

目的；观察血浆置地溶血性尿毒症综合征的治疗效果，方法：对５例明显诊断的溶血性尿毒症综合征病人进行单滤式膜式血浆置换治疗，每次置换冰冻新鲜血浆２５００ｍｌ，平均每倒置换５．２（３－９）次，结果５例病人在血浆置换术后迅速好转，血小板水平显著上升，肌酐水平显著下降。总胆     

儿童溶血尿毒综合征4例报道并文献回顾

溶血尿毒综合征(hemolytic uremic syndrome,HUS)是小儿急性肾衰竭常见原因之一,死亡率高。本文通过分析总结4例HUS患儿临床特点及诊治经过,为提高诊疗水平提供参考。方法将湖北省妇幼保健院PICU收治的4例HUS患儿临床表现、辅助检查、治疗及转归进行回顾性分析。结果4例患儿均进行血液净化治疗,2例患儿恢复良好;1例患儿出院后10个月复发,再次住院好转出院,随访5个月身体健康。1例患儿住院治疗68 d,间断透析治疗,因预后差放弃治疗后死亡。结论HUS为儿童致命性疾病,血浆置换能够缓解病情、改善预后,为HUS一线治疗。婴儿期发生非典型HUS可出现血浆置换治疗不敏感,建议尽早采用其他治疗如依库珠单抗。     

血浆置换在溶血性尿毒症综合征应用的研究进展

溶血性尿毒症综合征是机体在感染、药物等因素影响下,以微血管内血栓形成伴相应器官受累为表现的一组临床综合征,其发病率虽不高,但死亡率较高.本病无特效治疗.自从血浆置换应用于溶血性尿毒症综合征以来,其死亡率大大降低.本文就血浆置换在溶血性尿毒症综合征中的具体应用及进展做一综述.     

6例产后溶血性尿毒症综合征患者的护理

对6例产后溶血性尿毒症综合征患者行血液透析、血浆置换或输注新鲜冰冻血浆,纠正酸中毒与电解质紊乱、改善急性肾功能衰竭等综合治疗措施,结果6例均康复出院。提示对此类患者需严密观察有无烦躁不安、头痛、视物模糊等,警惕子痫的发生;重点观察尿量;遵医嘱及时准确进行各种治疗,同时准备好各种抢救器械及药品;严格执行无菌操作,做好血液透析和血浆置换时的配合和护理,防止出血和感染;认真做好基础护理和产褥期护理,以促进患者的康复。     

血浆置换加淋巴细胞去除抢救Goodpasture综合征并急性肾功能衰竭一例

Goodpasture综合征临床经过凶险，预后差，常常在疾病的早期因大咯血、呼吸功能衰竭而导致死亡。现报道1例用血浆置换加淋巴细胞去除的方法成功抢救Goodpasture综合征合并ARF、大咯血。     

血液透析治疗尿毒症脑病20例

尿毒症脑病是指尿毒症患者并发神经、精神异常，是急、慢性肾功能衰竭的严重并发症。我院应用血液透析技术治疗20例尿毒症脑病患者，疗效满意。现报告如下。     

重症急性胰腺炎并溶血尿毒症综合征一例报告

患者,女,38岁。因“饮白酒后意识不清伴恶心、呕吐1d”入院。入院前1d患者2h内饮烈性白酒约500ml后意识不清,呕吐、尿少且呈酱油色,血淀粉酶1600U／L。外院诊断为急性胰腺炎。给予对症治疗并行床边血液透析滤过（CRRT）治疗6h后意识转清转入我院。查体：上腹部压痛明显。实验室检查：血淀粉酶1728u／L、PT23．2S、     

H因子蛋白家族与非典型溶血尿毒综合征

H因子蛋白家族包括H因子、H因子样蛋白1和H因子相关蛋白1-5,均为由肝脏合成的糖蛋白,是补体旁路途径的重要调节蛋白,本文主要就其结构、生理作用、与非典型溶血尿毒综合征(atypical hemolytic uremic syndrome,aHUS)的关系以及临床应用前景作一综述.     

Analysis of 66 children with atypical hemolytic uremic syndrome

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 cases (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.     

Staphylococcus aureus-associated nephritis with histologic features resembling hemolytic uremic syndrome

A case that revealed the clinical and histologic features of superantigen-related nephritis and hemolytic uremic syndrome is reported. The patient was admitted with purulent arthritis due toStaphylococcus aureus infection, and showed rapidly progressive glomerulonephritic syndrome Clinical and histologic findings were similar to those of cases of superantigen-related nephritis previously reported, but our case had histologic evidence of various endothelial damage resembling that of hemolytic uremic syndrome. We suggest that such hemolytic uremic syndrome-like lesions may be one of the features of superantigen-related nephritis.     

A case of atypical hemolytic uremic syndrome with a transient decrease in complement factor H

We report a case of sporadic atypical hemolytic uremic syndrome (HUS) with a transient decrease in complement factor H. Referred for hemolysis and azotemia without diarrhea prodrome, this 31-month-old boy showed a decreased complement 3 (C3) and complement factor H (FH) level. However, the factor H gene (HF1) mutation was missing. After the hemolysis was controlled with plasma infusion, the C3 and FH levels recovered. The patients renal function fully recovered and remained normal, and there was no recurrence of the HUS.     

Association of systemic lupus erythematosus and hemolytic uremic syndrome in a child

We describe a 10-year-old girl with systemic lupus erythematosus (SLE) who first presented with hemolytic uremic syndrome (HUS). Diagnoses were based on the classic HUS triad, including the observation of fragmented red blood cells, and on the American College of Rheumatology criteria for SLE. Plasma exchange may have been effective against both HUS and SLE in this patient, as it was associated with improvement of platelet counts, renal function, and serological findings. Received: 24 September 1998 / Revised: 4 January 1999 / Accepted: 4 January 1999     

Childhood hemolytic uremic syndrome in Argentina: long-term follow-up and prognostic features

From January 1968 to December 1984, 312 infants and children with hemolytic uremic syndrome were admitted to our unit; 8 patients died (2.5%) during the acute phase; 118 children were followed as outpatients at yearly intervals for at least 10 years (mean follow-up 13 years, range 10 – 19.8 years). Four evolution patterns at the end of the follow-up were defined: group 1, complete recovery, 74 (62.7%); group 2, proteinuria with/without hypertension, 21 (17.7%); group 3, reduced creatinine clearance, often in conjunction with proteinuria and hypertension, 19 (16.1%); group 4, end-stage renal failure, 4 (3.4%). We investigated the association between several variables of the acute stage and the long-term evolution. Most non-anuric patients recovered completely (92.5%), while 38.4% of those with 1 – 10 days and 69.2% of those with 11 or more days of anuria had chronic renal sequelae. Similar results were found when analyzing the requirement for peritoneal dialysis. Of the patients with proteinuria at the 1-year control, 86% had renal abnormalities at the end of the follow-up. In our experience, although the final outcome was not predictable in every instance, the severity of acute renal failure – as determined by the days of anuria – and the presence of proteinuria 1 year after the acute phase were the most useful prognostic indicators. Received January 9, 1996; received in revised form April 16, 1996; accepted May 10, 1996     

Perinatal asphyxia may present with features of neonatal atypical hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is the consequence of platelet consumption at sites of endothelial injury. Perinatal asphyxia (PA) may cause renal failure after birth and can be associated with disseminated intravascular coagulopathy (DIC) with platelet consumption. No biological investigation permits us to distinguish clearly between neonatal HUS and DIC. We report on three neonates with renal failure due to different degrees of PA. They presented biological features compatible with HUS, such as fragmentocytes (∼2%), thrombopenia (<50,000/mm³), and anemia (<8 g/dl). One patient required peritoneal dialysis. Haptoglobin was undetectable for all three patients. Factor H and factor I, as well as components of the complement system (C3 and C4) and ADAMTS13 activity, were decreased. Two patients received daily fresh frozen plasma infusions over the first 4 weeks. Renal function improved in two patients; one patient had chronic renal failure. No neurological sequelae were noted. All blood parameters suggestive of thrombotic microangiopathy (TMA) were normal on days 12, 30, and 60. We hypothesize that endothelial cell damage concomitant with PA may lead to a vicious circle that results in consumption of platelets and plasma factors involved in hemostasis and/or fibrinolysis. In conclusion, PA, DIC and HUS are difficult to distinguish, and endothelial cell damage may be their common pathophysiological pathway.     

Complement and the atypical hemolytic uremic syndrome in children

Over the past decade, atypical hemolytic uremic syndrome (aHUS) has been demonstrated to be a disorder of the regulation of the complement alternative pathway. Among approximately 200 children with the disease, reported in the literature, 50% had mutations of the complement regulatory proteins factor H, membrane cofactor protein (MCP) or factor I. Mutations in factor B and C3 have also been reported recently. In addition, 10% of children have factor H dysfunction due to anti-factor H antibodies. Early age at onset appears as characteristic of factor H and factor I mutated patients, while MCP-associated HUS is not observed before age 1 year. Low C3 level may occur in patients with factor H and factor I mutation, while C3 level is generally normal in MCP-mutated patients. Normal plasma factor H and factor I levels do not preclude the presence of a mutation in these genes. The worst prognosis is for factor H-mutated patients, as 60% die or reach end-stage renal disease (ESRD) within the first year after onset of the disease. Patients with mutations in MCP have a relapsing course, but no patient has ever reached ESRD in the first year of the disease. Half of the patients with factor I mutations have a rapid evolution to ESRD, but half recover. Early intensive plasmatherapy appears to have a beneficial effect, except in MCP-mutated patients. There is a high risk of graft loss for HUS recurrence or thrombosis in all groups except the MCP-mutated group. Recent success of liver–kidney transplantation combined with plasmatherapy opens this option for patients with mutations of factors synthesized in the liver. New therapies such as factor H concentrate or complement inhibitors offer hope for the future.     

A clinicopathological study of 24 children with hemolytic uremic syndrome

This study reports the pattern of renal injury in 24 North American children with hemolytic uremic syndrome (HUS), and the extent of extrarenal involvement in 9 of these children examined at autopsy. Fifteen of the 24 children were studied during the first 16 days of hospital-ization; their renal specimens demonstrated glomerular thrombotic microangiopathy (TMA) in 8 children, cortical necrosis in 1, and varying degrees of glomerular TMA and cortical necrosis in 6 children. Nine of the children were studied after 16 or more days of hospitalization; these patients had prominent renal arterial lesions. Of 9 children examined at autopsy, extrarenal microthrombi were identified in 8. In 4 children who died during the acute phase of the disease, hemorrhagic colonic necrosis (3 children) and pancreatic islet cell necrosis (2 children) were the principal extrarenal lesions encountered. Rare microthrombi were present in the brains of the 3 children who manifested seizures. Southwest Pediatric Nephrology Study Group (SPNSG Central Office, Baylor University Medical Center, Dallas, Tex. USA). Director: Ronald J. Hogg; Associate Directors: Fred G. Silva, F. Bruder Stapleton; Statistician: Joan S. Reisch; Administrative Assistant: Kaye Green. Participating Centers: Baylor College of Medicine, Houston, Tex., Phillip L. Berry, L. Leighton Hill, David Powell, Edith Hawkins; Baylor University Medical Center, Dallas, Tex., Ronald J. Hogg, Kaye Green; Tulane University Medical Center, New Orleans, La., Frank Boineau, John E. Lewy, Patrick Walker; University of Arkansas, Little Rock, Ark., Watson Arnold, Eileen Ellis, M. Melinda Sanders; University of Colorado Health Science Center, Denver, Colo., Gary M. Lum, William Hammond; University of Oklahoma Medical Center, Oklahoma City, Okla., James Wenzl, Geoffrey Altshuler, Sarah Johnson; University of Tennessee, Memphis, Tenn., F. Bruder Stapleton, Shane Roy, III, Robert J. Wyatt, Charles McKay, William Murphy; University of Texas Southwestern Medical Center, Tex., Billy S. Arant, Jr, Michel Baum, Fred G. Silva, Arthur Weinberg, Craig Argyle, Joseph Rutledge, Ed Eigenbrodt; University of Texas Medical School, Houston, Tex., Susan B. Conley, Jacques Lemire, Ron Portman, Ann Ince, Regina Verani; University of Texas Health Science Center at San Antonio, Tex., Michael foulds, Sudesh Makker, Kanwal Kher, Melanie Sweet, Victor Saldivar, Fermin Tio; University of Texas Medical Branch, Galveston, Tex., Ben H. Brouhard, Alok Kalia, Luther B. Travis, Lisa Hollander, Tito Cavallo, Srinivasan Rajaraman; University of Utah Medical Center, Salt Lake City, Utah, Eileen Brewer, Richard Siegler, Elizabeth Hammond, Theodore Pysher     

溶血性尿毒症性综合征14例临床分析

目的探讨溶血性尿毒症性综合征（HUS）的病因及临床诊疗。方法分析我院近10年来14例HUS患者的临床资料。结果13例患者具备HUS的典型临床表现,包括溶血性贫血、血小板减少和急性肾衰竭。1例患者虽不具有HUS的典型临床表现,但肾活检病理显示为典型的HUS肾脏微血管病变。经过包括血液净化在内的综合治疗,9例患者获得临床治愈,5例患者死亡。结论该病诊断主要依据病史、临床表现及病理检查进行综合分析,早期改善肾衰竭、纠正重度贫血并积极进行抗凝是治疗关键。     

Challenges in the management of infantile factor H associated hemolytic uremic syndrome

We describe a 1-year old with four episodes of recurrent hemolytic uremic syndrome (HUS). Family history suggested an autosomal dominant mode of inheritance. Factor H concentrations in the blood were normal in the affected family members. Mutation screening in the human complement factor H gene (HF-1) revealed a novel mutation in exon 23 (c.3546_3581dup36). The HF-1 gene encodes complement factor H and the mutation leads to the insertion of 12 additional amino acids after codon 1176 in factor H. The recurrent HUS responded to plasma infusions and renal function improved from a glomerular filtration rate of 21 to 50 ml/min per 1.73 m². The infusions of fresh-frozen plasma were necessary at once-weekly intervals at a dose of 40–45 ml/kg in order to maintain remission and resulted in significant hyperproteinemia. This was addressed by intermittent plasma exchange through an arterio-venous fistula. The prognosis and therapeutic dilemmas are discussed.