6例小儿溶血尿毒综合征的护理体会

溶血尿毒综合征（HUS）是小儿少见且严重病症之一。其主要特征为急性’育功能衰竭，溶血性贫血及血小板减少［‘］，发病急、病死率高，加强对本病的护理，尤其少尿期的护理是治疗成功的关键。1989—1996年，我科共收治6例HUS患儿，现将护理体会报告如下。Ill＄床资料6例中男4例，女2例，年龄3—11岁。患儿出现不同程度的贫血、乏力、水肿、腹水、少尿。血尿、恶心、呕吐、腹痛、便血。其中4例烦躁不安、嗜睡、惊厥，2例出现心力衰竭或顽固性高血压。Hb30～90g／L，RBCI．0X10‘2～30X10‘2／L，网织红细胞计数超过1．5％，可见异形…     

溶血尿毒综合征的诊治进展

溶血尿毒综合征(HUS)属于血栓性微血管性疾病。以微血管性溶血性贫血(可找到红细胞碎片)、血小板减少、肾功能损伤为特点。微血栓主要分布于肾脏。感染、多种毒素、抗内皮细胞抗体、药物等因素使内皮损伤是发病的关键。近年来随着对HUS的病因和发病机制有了新的认识,输注血浆和血浆置换是目前治疗HUS最有效的方法,已大大提高了HUS患者的生存率。     

溶血尿毒综合征的最新进展

溶血尿毒综合征的最新进展龚新顺综述朱起之审校一、引言典型的溶血尿毒综合征（Ｈｅｍｏｌｙｔｉｃ－ｕｒｅｍｉｃｓｙｎｄｒｏｎｍｅＨＵＳ）系一种伴有红细胞形态异常临床以溶血性贫血，血小板减少，与急性肾衰竭为特征的综合征。在婴幼儿多见，其前驱症状有腹泻与便血...     

连续性血液净化治疗儿童溶血尿毒综合征的临床观察

目的探讨溶血尿毒综合征患者应用连续性血液净化治疗的临床效果。方法选择2013年1月至2017年12月南京医科大学附属儿童医院收治的57例溶血尿毒综合征患者为研究对象,根据治疗方法分为对照组(n=21)、观察组(n=36)。对照组患者采用血浆置换法进行治疗,观察组患者采用连续性血液净化进行治疗,比较两组患者的实验室指标、不良反应、患者满意度。结果两组患者治疗后血尿素氮(BUN)、肌酐(Scr)水平明显降低,血小板(PLT)、血红蛋白(Hb)水平明显升高,差异有统计学意义(P0.05)。两组间比较,差异无统计学意义(P0.05)。观察组患者不良反应的发生率、治疗的满意度分别为11.4%、91.7%,与对照组患者的11.4%、90.4%相比,差异无统计学意义(P0.05)。结论连续性血液净化与血浆置换法治疗溶血尿毒综合征效果值得肯定,有效的护理干预可减少不良反应的发生,提高患者满意度。     

成人溶血尿毒综合征的诊断及综合治疗探讨

成人溶血尿毒综合征的诊断及综合治疗探讨曹书华，李瑾，刘天飚ＲＥＳＥＡＲＣＨＯＦＤＩＡＧＮＯＳＩＳＡＮＤＣＯＭＰＲＥ－ＨＥＮＳＩＶＥＴＲＥＡＴＭＥＮＴＯＦＡＤＵＬＴＨＥＭＯＬＹＴＩＣＵＲＥＭＩＡＳＹＮＤＲＯＭＥＣｈａｏＳｈｕｈｕａ；ＬｉＪｉｎ；ＬｉｕＴ...     

儿童溶血尿毒综合征合并人类细小病毒B19感染

溶血尿毒综合征（HUS）好发于儿童，起病急、病情重、病死率高，且发病机制不明。临床分为腹泻后HUS（D＋HUS）和无腹泻HUS，且以D＋HUS多见。我院于2005年收治两例HUS合并人类细小病毒B19（HPVB19）感染，报告如下。     

恶性疟合并溶血尿毒综合征的诊疗探讨

目的探讨恶性疟引起的溶血尿毒综合征（HUS）的诊断及治疗要点。 方法对本院2008至2014年13例恶性疟引起的溶血尿毒综合征患者的临床特点、辅助检查及治疗情况进行回顾性分析。 结果入组患者中男性12例,女性1例,年龄22～60岁,均符合恶性疟疾合并溶血尿毒综合征的诊断。其中10例合并脑型疟,1例合并消化道出血,2例出现呼吸循环衰竭。经抗疟原虫治疗、激素治疗、补液对症治疗以及呼吸机、血液透析滤过等,入组的13例患者中1例死亡,1例自动出院,11例治愈出院,其中5例因急性肾功能衰竭行血液透析滤过治疗肾功能恢复,随访11例治愈患者均未出现慢性肾功能损害,2例病例出现再燃。 结论疟疾的早期诊断及治疗,对控制溶血及阻止脏器损害具有重要作用;大剂量长疗程使用蒿甲醚可增强抗疟原虫效果,早期应用激素可有效阻止溶血,减轻肾脏损害,对严重溶血、急性肾功能衰竭病例应及时采取血液透析滤过治疗,以降低病死率。     

血浆置换与血液透析串联治疗血栓性血小板减少性紫癜及溶血尿毒综合征

血栓性血小板减少性紫癜（TTP）及溶血尿毒综合征（HUS）均属于以血栓性微血管病为主的综合征，传统血浆置换（PE）治疗副反应发生率较高。2003年以来我们采用血浆置换与血液透析串联（PE＋HD）的方法并结合药物，治疗了4例TTP及HUS患者，取得了较好效果。     

溶血尿毒综合征有关问题的研究进展

溶血尿毒综合征（HUS）以内皮细胞受损继而血小板性血栓形成、微血管性溶血、肾功能损伤为特点，可以由许多种因素诱发。近年来HUS的病因、诊断和治疗取得显著进展，同时也存在一些争议。本文结合国内外研究进展，就HUS分类、感染导致HUS、补体调节异常HUS以及治疗方面的有关问题展开讨论。     

Hemolytic uremic syndrome in solid-organ transplant recipients

Post-transplant hemolytic uremic syndrome characterized by microangiopathic hemolysis, thrombocytopenia, and renal failure is an infrequent but potentially serious complication in organ transplant recipients. Hemolytic uremic syndrome developed in 2% (2/100) of our consecutive liver transplants. We report our patients and review a total of 91 cases of hemolytic uremic syndrome in adult solid organ transplant recipients reported in the literature. Ninety percent were observed in renal transplant recipients, 8% in liver, and 1% each in lung and heart transplant recipients. Eighty percent and 96% of cases occurred within 90 days and 1 year, respectively, post-transplantation. In renal transplant recipients, 23% of cases were due to post-transplant recurrence of hemolytic uremic syndrome. In 50% of renal transplant recipients and in all norenal solid-organ transplant recipients, hemolytic uremic syndrome was attributed to cyclosporin or tacrolimus therapy. Notably, infections were not a significant precipitating factor for post-transplant hemolytic uremic syndrome. Graft loss attributable to hemolytic uremic syndrome occurred in 43% of renal transplant recipients while renal transplantation and hemodialysis were required in the lung and heart transplant recipients due to hemolytic uremic syndrome induced renal failure. The overall mortality was 13% (12/91). Physicians caring for transplant recipients need to be aware of this potentially severe graft and life-threatening disorder since prompt recognition and removal of identifiable risk factors is critical in the management of post-transplant hemolytic uremic syndrome.     

Hemolytic uremic syndrome linked to infectious mononucleosis

A 12-month-old boy developed a mild hemolytic uremic syndrome with no acute diarrheal prodrome. The typical clinical, hematological, and serological features of infectious mononucleosis were also noted. The clinical course of both hemolytic uremic syndrome and infectious mononucleosis was uneventful. A review of the literature disclosed that hemolytic uremic syndrome has been noted in two adolescents with infectious mononucleosis.     

Hemolytic uremic syndrome in children in northern India

We observed 73 patients with the hemolytic uremic syndrome (HUS) in 9 years (1980–1988), comprising 34% of patients with acute renal failure treated over the same period. There were 53 boys and 20 girls; 59% were below the age of 2 years and 33% between 2 and 5 years. Acute, usually severe dysentery, responding poorly to various antibiotics, was the prodromal illness in 80%, whereas 12% had watery diarrhea. Most patients had severe renal involvement with anuria in 56% and oliguria in 30%. A polymorphonuclear leukocytosis was present in 85% of cases, but had no correlation with the highest levels of blood urea. Coagulation abnormalities suggesting consumption coagulopathy were found in 24 of 30 cases. The results of stool culture showedShigella species in 7 cases and nontyphoidalSalmonella in 9.Escherichia coli were isolated in 11 cases, but were not further characterized. Renal biopsy showed total or patchy cortical necrosis in 20 of 50 cases. The patients were managed with supportive care, including transfusion of fresh blood or plasma and dialysis as required. The mortality was 60%, being chiefly related to the duration of renal failure and presence of renal cortical necrosis, whereas persistent dysentery and infections were complicating factors. The presence of convulsions and coagulation defects had no relation to the outcome. Our observations indicate that HUS in children in northern India is mostly related to dysentery, likely to be shigellosis, and is usually associated with severe renal damage and a high death rate.     

Clinical features of children with anti-CFH autoantibody-associated hemolytic uremic syndrome: a report of 8 cases

ObjectiveTo explore the clinical characteristics, treatment protocol and prognosis of children with anti-complement factor H (CFH) autoantibody (Ab)-associated hemolytic uremic syndrome (HUS).MethodsClinical data of 8 patients with anti-CFH Ab-associated HUS who were admitted to Shandong Provincial Hospital from January 2011 to December 2020 were collected retrospectively.ResultsThe age at disease onset ranged between 5.83 and 13.5 years, with a male: female ratio of 1.67:1. The time of onset was distributed from May to June and November to December. Digestive and upper respiratory tract infections were common prodromal infections. Positivity for anti-CFH Ab and reduced C3 levels were observed among all patients. Heterozygous mutation of the CHFR5 gene (c.669del A) and homozygous loss of the CFHR1 gene [loss2(EXON:2-6)] were found in two patients. All patients received early treatment with plasma exchange and corticosteroid therapy. Six patients were given immunosuppressive agents (cyclophosphamide and/or mycophenolate mofetil) for persistent proteinuria. The follow-up period was 12–114 months. Four of 8 patients achieved complete remission, 3 achieved partial remission, and 1 died. Relapse occurred in two patients.ConclusionChildren with anti-CFH Ab-associated HUS were mainly school-aged and predominantly male, with onset times of summer and winter. Digestive and upper respiratory tract infections were common prodromal infections. Plasma exchange combined with methylprednisolone pulse therapy in the acute phase and cyclophosphamide or mycophenolate mofetil treatment for maintenance can be utilized in children with anti-CFH Ab-associated HUS if eculizumab is not available.     

Hemolytic uremic syndrome due to Capnocytophaga canimorsus bacteremia after a dog bite

The hemolytic uremic syndrome (HUS) is known to have several causes, including infectious diseases, drugs, pregnancy, and malignant disease. We report a patient who developed acute renal failure attributable to HUS in the course of Capnocytophaga canimorsus bacteremia. Acute tubular necrosis as well as HUS should be considered as a cause of acute renal failure in the setting of Capnocytophaga canimorsus bacteremia.     

8例溶血性尿毒症综合征临床分析

目的探讨溶血性尿毒症综合征（HUS）的临床特点和治疗方法。方法回顾性分析自1997年12月至2007年12月收治的8例HUS患者的临床特点及治疗方法。结果成人7例,儿童1例。诱因为产后3例,呼吸道感染2例,肠道感染1例,呼吸道合并肠道感染1例,无明显诱因1例。8例均表现为溶血性贫血、血小板减少和急性肾衰竭。病情属重型5例,轻型3例。肾损害表现为少尿1例,无尿2例,肉眼血尿3例,高血压8例,水肿6例。8例均采用综合治疗：抗感染、降压等一般治疗;血液透析、改善肾循环、血液灌流、血浆置换、新鲜冰冻血浆输注以及肾上腺皮质激素等。2例治愈,4例好转,2例病情恶化放弃治疗。治疗后血液系统及生化指标均有改善,血红蛋白（Hb）、血小板（PLT）、血肌酐（SCr）、总胆红素（TBIL）、血乳酸脱氢酶（LDH）与治疗前比较,差异有统计学意义（P〈0．05）。结论HUS较罕见,属临床急症,早期诊断、及时正确治疗有助于改善短期预后。     

Hemolytic uremic syndrome in small-bowel transplant recipients: the first two case reports

Post-transplant hemolytic uremic syndrome (HUS) is an uncommon but well-described complication in solid organ transplant recipients. Believed to be secondary to immunosuppressive therapy, it has been reported after kidney, liver, pancreas, heart, and lung transplants. In all reported cases, the primary organ affected was the kidney (transplant or native). But until now, no cases after small-bowel transplants and no cases in which the kidney was not the primary organ affected have been reported. We report two cases of HUS in small-bowel transplant recipients. In our first case, clinical presentation was with renal failure; biopsy of the native kidney demonstrated the typical histological changes seen with HUS, namely occlusion of the microcirculation by thrombi and platelet aggregation. Immunosuppression was changed from tacrolimus to cyclosporin, but with no improvement in renal function. In our second case, the transplanted bowel was the primary organ affected. This recipient presented with ulcers in the bowel mucosa, which were believed to be ischemic in origin, secondary to occlusive vascular lesions affecting the small vessels in the transplanted bowel. Her tacrolimus dose was decreased with resolution of ulcers and no evidence of rejection. These two cases represent the first reports of HUS after small-bowel transplants; in addition, our second case represents the first report of an extrarenal graft as the primary organ affected. When caring for small-bowel transplant recipients, physicians must be alert to the possibility of HUS and its various presentations. Received: 29 October 1998 Received after revision: 12 March 1999 Accepted: 3 May 1999     

Hemolytic uremic syndrome and thymic dysplasia in an infant

A 33-day-old male infant was admitted to the neonatal intensive care nursery because of respiratory distress, grunting, cyanosis, and radiological findings of bilateral bronchopneumonia. He responded well to intensive therapy, but 11 days later developed hemolytic uremic syndrome, which was treated conservatively with prednisone and plasma transfusions with good response. The hemolytic uremic syndrome resolved, but he subsequently developed severe recurrent infections of unknown etiology and died at the age of 78 days. Necropsy findings revealed necrotizing enterocolitis as well as dysplasia of the thymus and other lymphoid tissues, compatible with the diagnosis of immunodeficiency disorder. Received February 7, 1997; received in revised form November 12, 1997; accepted November 13, 1997     

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