溶血性尿毒综合征

溶血性尿毒综合征（hemolytic uremic syndrome,HUS）是一种非免疫性溶血性贫血。具有突然出现的溶血性贫血、血小板减少、急性肾衰竭三大特征,并排除其他原因引起的急性肾衰竭、肾小球肾炎、血小板减少和溶血性贫血。近年来在儿童呈增加趋势,成为急性肾功能衰竭的常见原发病之一。目前对该病的发病机制并不十分清楚,且尚乏有效的治疗方法,预后严重。     

Hemolytic uremic syndrome

Hemolytic uremic syndrome is primarily a disease of childhood. Seventy three children presented with clinical signs and symptoms were studied in detail. Seventy four percent of them were under two years of age. Many of these children had clinical evidence of involvement of organs other than the kidney. Mortality among the children studied was 60%. The high mortality is possibly due to involvement of non renal organs in addition to factors like malnutrition, delay in seeking hospitalization etc. Some of the recent trends in the pathogenesis, pathology and management have been reviewed.     

Hemolytic uremic syndrome: Events of the past decade

This article describes the birth of the Canadian Pediatric Kidney Disease Research Centre (CPKDRC) in 1985 and the activities that have transpired as a result of collaborative research at paediatric centres across Canada. These include the National Retrospective Study of Childhood Hemolytic Uremic Syndrome (HUS), National Prospective Study of Risk Factors for Developing Escherichia coli O157:H7 Infection, and Intervention Studies for the Prevention of HUS. A look to the future describes possible studies to determine potential factors (surrogate markers) to identify children who are at risk for developing HUS following verotoxin-producing E coli gastroenteritis, other intervention studies and a more accurate understanding of permanent renal insufficiency in children who have had HUS.     

Hemolytic uremic syndrome associated with cisplatin therapy

An adolescent with a small round cell tumor of the chest wall, who was treated with cisplatin, developed hemolytic uremic syndrome with severe hypertension, which ultimately contributed to her death. Cisplatin's role as a possible causative agent of this syndrome is discussed. Recommendations are made for monitoring abnormalities that may signal the onset of this potential complication.     

Hemolytic uremic syndrome recurrence after renal transplantation

Abstract:  About 60% of non-Stx-associated aHUS are due to the defect of protection of endothelial cells from complement activation, secondary to mutations in the genes of CFH, MCP, IF, BF, or C3. In addition, 10% of patients have anti-CFH antibodies. While the risk of post-transplant recurrence is less than 1% in Stx-HUS patients, it is approximately 80% in CFH or IF -mutated patients, 20% in MCP -mutated patients, and 30% in patients with no mutation . Patients with anti-CFH antibodies probably also are at risk of recurrence. While MCP -mutated patients can reasonably go to transplantation, recent reports suggest that plasmatherapy started before surgery and maintained life-long may prevent recurrence in CFH -mutated patients. Four successful liver–kidney transplantation utilizing plasmatherapy in CFH -mutated children have been reported recently. In summary, the risk of post-transplant recurrence can now be approached according to genotype. Therefore, aHUS patients should undergo complement determination, screening for anti-CFH antibodies, and genotyping before transplantation. Kidney or kidney + liver transplantation with concomitant plasmatherapy need to be evaluated by prospective trials in patients with hereditary complement abnormalities.     

Hemolytic uremic syndrome: A case presenting with acute colitis

A case of hemolytic uremic syndrome (HUS) in a 3 year old boy, who presented with diffuse rectal bleeding is discussed. On barium enema, there was evidence of acute colitis with submucosal hemorrhage. The probable relationship of these findings to the pathogenetic intravascular coagulopathy and the prodromal stage of HUS is discussed.     

Hemolytic uremic syndrome associated with Corynebacterium diphtheria infection

Although hemolytic uremic syndrome (HUS) is usually idiopathic, it follows a number of infections. The pathogenesis of post-infectious HUS is endothelial cell damage by either circulating endotoxin or exotoxin. Diphtheria exotoxin has never been implicated in HUS. We report HUS following diphtheria infection in a 9 yr old un-immunized white female admitted with a short history of sorethroat and thrombocytopenia. There were hemorrhages in sclera, gums and left tonsillar area and a grayish exudate on right tonsil. Laboratory values revealed Hgb 14.4 g/dl, decreasing to 7.6 g/dl, WBC/26,900 mm3, platelet count 7,000/mm3. Bone marrow examination revealed normal megakaryocytes. She was oliguric with BUN 214 mg/dl, serum creatinine 12.4 mg/dl and serum uric acid 19.2 mg/dl. Despite peritoneal dialysis, red cell and platelet transfusions and exchange transfusion she expired. A postmortem examination was refused. A throat culture done on admission grew corynebacterium species which was later confirmed to be toxigenic C. diphtheriae. Diphtheria exotoxin inactivates an enzyme in cytoplasm which is necessary for peptide chain elongation. This may have interfered with prostacyclin synthesis thereby allowing the development of HUS.     

Hemolytic uremic syndrome following juvenile lupus erythematodes disseminatus

A 12.5 year old girl was admitted to hospital with the typical signs of hemolytic uremic syndrome, and systemic lupus erythematodes as well. On the basis of clinical, blood chemistry, and histological findings we assumed an hemolysis-induced form of hemolytic-uremic syndrome as the most likely pathogenic mechanism. The child also suffered from congenital IgA-deficiency and produced an inhibitor against coagulation factor VIII. Congenital IgA-deficiency, systemic lupus erythematodes, inhibitor-induced hemophilia and hemolytic uremic syndrome are suggested to form a pathogenic sequence.     

Hemolytic uremic syndrome complicated by vitamin K deficiency

A 5-year-old child with hemolytic uremic syndrome developed bleeding due to vitamin K deficiency 9 days after the onset of a diarrheal prodrome. Vitamin K deficiency was documented by rapid correction of the PT and PTT and cessation of bleeding following administration of vitamin K, as well as by the detection of noncarboxylated prothrombin in plasma. The case is instructive because it suggests that previously healthy older children who become acutely ill may develop vitamin K deficiency more rapidly than heretofore has been appreciated.     

Hemolytic uremic syndrome followed by acute lymphocytic leukemia

A 4 6/12-year-old boy presented with severe hemolytic uremic syndrome (HUS) and then developed acute lymphocytic leukemia (ALL) 8 months later. Although other syndromes of hematopoietic dysfunction have been reported to precede the overt and classic findings of leukemia, this report describes a patient who developed ALL preceded by HUS, association not previously described.     

Hemolytic uremic syndrome in children: platelet aggregation and membrane glycoproteins

PURPOSE: Several mechanisms have been proposed to explain the fibrin-platelet thrombosis at the microcirculation level in the different clinical conditions of hemolytic uremic syndrome (HUS). The relationships between platelet structure and function during the first 4 weeks of evolution of the disease were studied to understand the mechanism of platelet alteration. PATIENTS AND METHODS: Coagulation parameters, platelet counts, and aggregation were studied in 49 children, and membrane glycoproteins (GPs) in 20 of the 49 children (mean age, 17 months) with HUS (Group 2) were studied during the first 4 weeks of evolution of the disease. RESULTS: No disseminated intravascular coagulation was found in patients with recurrent or persistent thrombocytopenia. Platelet aggregation was sequentially performed during the first weeks of evolution. All patients had a functional decrease in the acute period of HUS. Platelet GPs GPIb, GPIIbIIIa, GPIIb, and GPIIIa were evaluated. GPIIbIIIa complex presented low level and never reached normal values during the first 4 weeks of disease. CONCLUSIONS: Platelet alterations are probably caused by multiple mechanisms: "exhausted" platelets, structural membrane alterations caused by arginine-glycine-aspartic peptide blockade, or diminished or nonfunctional membrane GPIb and GPIIbIIIa complexes.     

Hemolytic uremic syndrome associated with pneumococcal pneumonia in Taiwan

Abstract Streptococcus pneumoniae ( S. pneumoniae ) has been associated with hemolytic uremic syndrome (HUS), which is an unusual but serious disease in childhood. We conducted a retrospective review of children aged less than 18 years with S. pneumoniae -associated HUS in northern Taiwan from January 2000 to June 2005. The demographic characters, clinical courses, and outcomes were analyzed. Seven children (three girls, four boys) with S. pneumoniae -associated HUS were studied. The median age at onset of HUS was 40 months (range: 25–60 months). The median duration of hospital stay was 36 days (range: 15–50 days). The interval between the onset of illness attributable to S. pneumoniae and the development of HUS was around 1–2 weeks. The onset of oliguria developed within 2 weeks after illness. Six patients required dialysis with median duration of 16 days. Three patients had leukopenia as the initial presentation. All seven patients had pneumococcal pneumonia complicating with empyema, and two of them received decortication via video-assisted thoracoscopic surgery. Between patients who needed dialysis or not, there was no significant difference in age, sex, duration of thrombocytopenia, incidence of extra-renal complications, such as hepatitis, pancreatitis, and hypertension, and length of hospital stay. The seven patients survived with normal renal function. HUS is a potentially fatal complication of S. pneumoniae infection. Clinicians managing patients with pneumococcal pneumonia with empyema accompanied by leukopenia should beware of the development of HUS. The long-term prognosis for recovery of renal function appears to be good in these patients in northern Taiwan.