Child ; Hemolytic-Uremic Syndrome ; therapy ; Humans ; Plasma Exchange

Complement System Proteins ; metabolism ; Hemolytic-Uremic Syndrome ; diagnosis ; therapy ; Humans

The precise etiology of hemolytic uremic syndrome (HUS) is unknown. However, it has been associated with bacterial (Shigella, Salmonella, E. coli, S. pneumoniae), Bartonella, and viral (coxsackie, ECHO, influenza, varicella. Epstein-Barr) infections and with endotoxemia. Recently, we experienced a case of HUS in a 16-year-old boy who was in the acute phase of an Epstein-Barr virus (EBV) infection. He had typical manifestations of HUS and EBV infection. He also transiently presented disseminated intravascular coagulation. His renal dysfunction recovered by supportive care, including hemodialysis, plasmapheresis, antihypertensive medication and aspirin. We present this case with a review of the literature as the second report of HUS associated with EBV infection.
Adolescence ; Follow-Up Studies ; Hemolytic-Uremic Syndrome/virology* ; Hemolytic-Uremic Syndrome/therapy ; Herpesviridae Infections/diagnosis* ; Herpesvirus 4, Human/isolation & purification* ; Human ; Male ; Renal Dialysis ; Tumor Virus Infections/diagnosis*

OBJECTIVETo observe the therapeutic effect of continuous blood purification (CBP) combined with hemoperfusion (HP) in children with hemolytic-uremic syndrome (HUS) and to investigate its possible mechanism.

METHODSEight children with HUS received CBP combined with HP on the basis of internal medicine treatment in the acute stage. Before and after treatment, serum levels of interleukin-6 (IL-6), IL-8 and tumor necrosis factor-α (TNF-α) were measured by chemiluminescence method, and levels of blood urea nitrogen (BUN), serum creatinine (SCr), alanine aminotransferase (ALT), aspartate aminotransferase (AST), creatine kinase (CK), creatine kinase MB (CKMB), hemoglobin (Hb), platelet (PLT) and C-reactive protein (CRP) were measured. Eight healthy children undergoing physical examination were used as controls.

RESULTSThe 8 children with HUS all survived after CBP combined with HP and showed improved conditions. They had increased Hb and PLT levels and decreased serum levels of IL-6, IL-8, TNF-α, BUN, SCr, ALT, CK and CRP after treatment (P<0.05).

CONCLUSIONSCBP combined with HP can quickly remove pathogenic factors, continually eliminate inflammatory mediators and toxins, and reverse multiple organ dysfunction, and is one of effective methods for treating HUS in children.

Child ; Child, Preschool ; Female ; Hemofiltration ; Hemolytic-Uremic Syndrome ; therapy ; Hemoperfusion ; Humans ; Infant ; Male

Acute Kidney Injury ; etiology ; Hemolytic-Uremic Syndrome ; complications ; etiology ; therapy ; Humans ; Shiga Toxin ; toxicity

Antibodies, Monoclonal, Humanized/therapeutic use* ; Atypical Hemolytic Uremic Syndrome/drug therapy* ; Humans

Hemolytic uremic syndrome (HUS) is clinically rare, with high mortality and case fatality rates. In recent years, the research on HUS has been intensified and the pathophysiological mechanism has been continuously improved. At present, the main mechanism of pathogenesis is the excessive activation of complement alternative pathways mediated by complement-related gene mutations or the existence of antibodies. The treatment methods and strategies are also constantly updated, mainly including complement-blocking drugs such as Eculizumab, Lavalizumab, and Ravulizumab. In this review, the new developments in the pathogenesis and treatment of HUS is summarized, and provide references for the clinical treatment of HUS.
Complement System Proteins/therapeutic use* ; Hemolytic-Uremic Syndrome/therapy* ; Humans ; Mutation

PURPOSE: Cyclosporine associated hemolytic uremic syndrome (HUS) is a serious complications following kidney transplantation. In this study, 2 renal transplant patients, treated by the dual drugs -Mycophenolate Mofetil (MMF) and steroid (Deflazacort), without cyclosporine- due to development of HUS, were followed-up. Additionally, IFN-gamma and IL-10 as Th1 and Th2 cytokines, respectively, and their serum levels investigated. METHODS: Following their recovery from HUS, the 2 patients have been followed for 37 and 45 months, respectively, with MMF and steroid as maintenance immunosuppressants. The serum IFN-gamma and IL-10 levels were measured simultaneously in the 2 patients on dual drug, 10 on triple drug (cyclosporine, MMF, steroid) therapies and 18 normal volunteers. The 10 patients on the triple drug therapy were selected from 14 patients, that had undergone renal transplantations in the same year as the 2 dual drug therapy patients. RESULTS: At 37 and 45 months post-transplantation, the 2 pdual drug therapy patients showed serum creatinine levels less than 1.8 and 1.7 mg/dl, respectively. The serum IFN-gamma and IL-10 levels of the 12 (2 dual and 10 triple drug therapy) renal transplant patients (11.83+/-5.01 and 5.96+/-6.02 pg/ml, respectively) were significantly higher than those of the 18 normal volunteers (7.25+/-0.84 and 1.40+/-0.81 pg/ml, respectively), (IFN-gamma: P=0.000, IL-10: P=0.000). However a comparison of IFN-gamma and IL-10 levels between the 2 dual(11.56+/-3.35 and 4.91+/-1.66 pg/ml, respectively) and 10 triple drug therapy patients (11.89+/-5.43 and 6.17+/-6.61 pg/ml, respectively) showed no significant difference (IFN-gamma: P=0.606, IL-10: P=0.485). CONCLUSION: Long-term maintenance treatment with MMF and steroid is an effective alternative therapy in case of cyclosporine induced HUS.
Creatinine ; Cyclosporine ; Cytokines ; Drug Therapy ; Healthy Volunteers ; Hemolytic-Uremic Syndrome ; Humans ; Immunosuppressive Agents ; Interleukin-10 ; Kidney Transplantation*

Post-partum hemolytic uremic syndrome (PHUS) is a severe thrombotic microangiopathy clinically characterized by hemolytic anemia, renal dysfunction, and low platelets after birth with rapid progression and poor prognosis. Here, we reported a rare case of severe preeclampsia diagnosed as hemolytic uremic syndrome after birth. The patient was diagnosed with PHUS and underwent intermittent plasma exchange with supportive treatment including glucocorticoid injections and transfusion of suspended red blood cells. After these treatments, the patient experienced no apparent remission and chronic renal dysfunction occurred on her. PHUS is a severe emergency with acute onset, rapid progress, and poor prognosis. Early detection, diagnosis, and treatment can significantly improve the prognosis.
Adult ; Female ; Hemolytic-Uremic Syndrome ; diagnosis ; therapy ; Humans ; Infant, Newborn ; Pre-Eclampsia ; diagnosis ; Pregnancy ; Puerperal Disorders ; diagnosis

Antibodies, Monoclonal, Humanized ; therapeutic use ; Atypical Hemolytic Uremic Syndrome ; drug therapy ; surgery ; China ; Humans ; Kidney Transplantation ; Male