Outcome Of Hemolytic Uremic Syndrome Research Articles

Changing Epidemiology and Outcomes of Hemolytic Uremic Syndrome in Children: A Prospective National Cohort Study from the Polish Pediatric HUS Registry and the Polish Registry of Renal Replacement Therapy in Children.

Background/Objectives: Hemolytic uremic syndrome (HUS) is a known cause of acute kidney injury in children, but there are few recent reports on its epidemiology and outcome. We aimed to investigate trends in the incidence and the long-term outcomes of both Shiga toxin-producing Escherichia coli -HUS (STEC-HUS) and atypical HUS (aHUS) in Poland over the last 12 years (2012-2023), based on the Polish Pediatric HUS and Pediatric Renal Replacement Therapy (RRT) Registries. Methods: A total of 436 patients (301 with STEC-HUS and 135 with aHUS) were included. Results: The incidence of STEC-HUS increased during the observation period, with a mean of 3.9 cases per million age-related population (marp). The incidence of aHUS was relatively constant with a mean of 1.8/marp. The majority of patients fully recovered, although kidney sequelae were observed at 5-year follow-ups in 31% of children with STEC-HUS, 57% of aHUS subjects in the pre-eculizumab era, and 37% of aHUS subjects who had received eculizumab. The overall mortality rate was 2% for STEC-HUS and 3.7% for aHUS, with no deaths reported in children on eculizumab and mortality mainly attributed to neurological damage. A decreasing incidence of chronic kidney disease stage 5 (CKD5) due to HUS was observed. Conclusions: Despite an unchanging incidence of aHUS and an increasing incidence of STEC-HUS, the kidney outcomes of both diseases have improved significantly over the last 12 years. Mortality from HUS has dropped due to improved symptomatic treatment and the introduction of anti-C5 therapy. The development of CKD5 in childhood as a consequence of HUS has become exceptional.

Hemolytic Uremic Syndrome in Egyptian Children: A Single Center Experience across 10 Years.

Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and is the most common cause of AKI in children. We aimed to demonstrate the clinical patterns, laboratory findings, management, and outcomes of HUS in Egyptian children. This was a retrospective cohort study carried out in the Nephrology Unit of the Pediatric Department at Tanta University Hospitals. Hospital-based records of HUS cases between January 2009 and January 2019 were used to obtain the disease history, clinical manifestations, investigations, treatment, and outcomes. Sixty-eight children were included in the study: 63 (96.56%) with Shiga-toxin-producing Escherichia coli (STEC) HUS and five (7.53%) with atypical HUS. The boy-to-girl ratio was 1.19:1. The age at the onset of the disease ranged from 0.5 to 13 years, with a median of 2.25 years. The main presenting manifestations were pallor (80.88%), diarrhea (67.65%), oliguria (54.41%), and convulsions (19.21%). The survival rate was 85.29%, whereas the mortality rate was 14.71%. Thirty-seven patients (54.41%) recovered completely, 17 (25%) patients survived but with chronic kidney disease, and four patients (5.88%) progressed to end-stage renal disease and are currently maintained on dialysis. The risk factors for mortality were female gender, age <5 years, anuria, and an affected central nervous system (CNS). STEC-HUS had a higher incidence than atypical HUS with better outcomes. Early dialysis improved the outcome in terms of mortality in young patients, females, and those with an affected CNS.

Hemolytic uremic syndrome associated with Streptococcus pneumoniae

Hemolytic uremic syndrome associated with Streptococcus pneumoniae is a rare disease that occurs in 5-10% of all cases of hemolytic uremic syndromes in childhood. Pneumonia and meningitis are among the main diseases that can be complicated by St. pneumoniae-associated hemolytic uremic syndrome. The widespread introduction of polymerase chain reaction into clinical practice has increased the frequency of detection of this pathology. The article presents the epidemiology, clinical picture, features of pathogenesis, diagnosis and treatment of St. pneumoniae-associated hemolytic uremic syndrome. The authors described 3 clinical cases of this syndrome in children with pneumonia. In 2 patients, pneumonia proceeded with destruction of lung tissue, pleural empyema, bacteremia, and severe renal failure, which required hemofiltration therapy to replace renal function. Outcomes of hemolytic uremic syndrome associated with St. pneumoniae is worse than in typical hemolytic uremic syndrome, and is associated with the severity of the underlying disease (pneumonia, meningitis).

In-Hospital Outcomes of Hemolytic Uremic Syndrome: A Perspective from the Last Decade

Introduction: Hemolytic Uremic Syndrome (HUS) is a condition characterized by progressive renal failure that is associated with microangiopathic hemolytic anemia and thrombocytopenia. It is a clinical syndrome which occurs as an immune reaction, most commonly after a gastrointestinal infection. Several studies have reported the prevalence of HUS among children but it is not well studied among adults. Studies have shown mortality rates ranging up to 20% and may go up to to 50% if HUS is complicated by end stage renal failure. There is limited data regarding HUS, especially among the adult population in regards to hospitalization trends and outcomes. In this study we aimed to describe the hospitalization trend, patient characteristics and in-hospital outcomes of HUS using a nationally representative database.Methods:Study cohort was from the Nationwide Inpatient Sample (NIS) for the years 2008-2017 for hospitalizations due to HUS by using International Classification of Diseases (9th/10th Editions) Clinical Modification diagnosis codes ICD-9-CM/ICD-10-CM). Other diagnosis and comorbidities were also identified by ICD-9/10-CM codes and Elixhauser comorbidity software. Our primary outcome was a discharge disposition following HUS hospitalization. We utilized multivariable survey logistic regression models to analyze and identify predictors of poor outcomes.Results:Between 2007-2018, a total of 8,043 hospitalizations occurred due to HUS. Hospitalization trend due to HUS increased from 528 (6.57%) in 2007 to 800 (9.95%) in 2013 and then reduced to 620 (7.7%) in 2018. Study cohort consisted of children <10 years of age (56.2%) followed by 18-65 years of age (24.7%). Out of total HUS patients, 74.4% were Caucasians and 59.3% were females. Overall in-hospital mortality of hospitalization due to HUS was 3.0%, and discharge to the facility was 15.7%. In trend analysis, the proportion of discharge to facility decreased from 23.1% in 2007 to 14.5% in 2018 but in-house mortality increased from 1% in 2007 to 3.2% in 2018. Median Length of hospital stay was 8-days (interquartile range 3-day to 15-days). Furthermore, in multivariable logistic regression analysis, age above 65 years (OR 2.5; 95%CI 1.4-5.8; p=0.0023), rural hospital (OR 17.9; 95%CI 8.9-36.1; p<0.001) and urban-non teaching hospital (OR 5.2; 95%CI 3.4-8.1; p<0.0001) and small-medium size hospitals were also associated with higher odds of discharge to facility among HUS patients.Conclusion:Our study estimates the epidemiology of hospitalizations due to HUS in the United States from a nationally representative database. In this study we observe that the burden of hospitalizations due to HUS has been increased over the study period. We also identified factors associated with poor discharge outcomes and some of which are modifiable. Further studies are warranted for developing strategies for better risk stratification of HUS patients to improve the overall outcomes. DisclosuresNo relevant conflicts of interest to declare.

P0334HEMOLYTIC UREMIC SYNDROME: CLINICAL PRESENTATION, LABORATORY FINDINGS, MANAGEMENT AND OUTCOMES

Abstract Background and Aims Hemolytic-uremic syndrome (HUS) is a disease that has been described as a triad of hemolytic anemia, thrombocytopenia and renal impairment. There are two types of HUS, typical and atypical. HUS remains a leading cause of acute renal injury in children and is increasingly recognized as a cause of renal failure in adults. In our study we evaluated the demographic features, clinical characteristics, management and outcome of HUS. Method This is a descriptive study evaluating all cases of Hemolytic Uremic Syndrome (HUS) that have been admitted at Royal Hospital (RH) Oman in the period between 2008 and 2017. Results Thirty-six patients identified. The mean age (SD) of 10.68 (14.07) years. Eighteen (50%) presented with abdominal pain, nausea, vomiting and diarrhea, 9 (25 %) with hypertension, 23 (64 %) with acute kidney injury (AKI), 6 (16.67%) with seizure, 2 (5.56%) with confusion and 1 (2.78%) with cerebrovascular accident (CVA). Twenty-one (58.33 %) diagnosed as typical HUS of which 19 with Shiga toxin producing Escherichia Coli (STEC) HUS and 2 had post Streptococal HUS. Six (16.67%) diagnosed with aHUS, 2 (5.56%) with HELLP, 2 (5.56%) with G6PD, 3 (8.33%) with Autoimmune hemolytic anemia (AIHA ), one (2.78%) with congenital TTP and 1 (2.78%) with post partum HUS. Twenty three (63.89%) needed renal replacement therapy (RRT), while remaining 13 (36.11%) did not require RRT. Eleven (30.56%) received plasma exchange, 5 (13.89%) received Eculizumab while 2 (5.56%) received plasma infusion and 1 (2.78%) patient received Rituximab. The majority 22 (61.11%) had partial recovery after treatment, 5 (13.89 %) had compelte recovery and 3 (8.33% ) ended with end stage kidney disease (ESKD) and 1 (2.78%) died from hypertensive crises. Conclusion The study results showed that HUS population were mostly due to Shiga toxin producing Escherichia Coli (STEC). It showed that HUS population were young, mostly male and only 25% have known medical comorbidities at time of presentation. Also, the majority presented with AKI requiring dialysis, of which PD was main stay of therapy. The duration of RRT and recovery time was almost a month period.

Characteristics and outcome of hemolytic uremic syndrome in Sudanese children in a single Centre in Khartoum State.

Hemolytic uremic syndrome (HUS) is one of the important causes of acute kidney injury (AKI) and chronic kidney disease (CKD) in children. Proposed prognostic features are controversial. We reviewed, retrospectively, the records of children with HUS seen at Soba hospital, Khartoum (2004-2012). We aimed to study demographics, clinical/ laboratory features, outcome and prognostic risk factors. Thirty-nine children with HUS were recorded; 59% had diarrhoea positive (D+) and 41% diarrhoea negative (D-) HUS. The mean age was 65.4 months and males were 61.5%. At the acute phase seizures, coma, anuria/oliguria and hypertension were present in 25%, 17.9%, 51.3% and 53.8% respectively. Severe anaemia, thrombocytopenia, and leukocytosis were present in 71.8%, 97.4%, and 28.2% respectively. On discharge, hypertension was detected in 23.1%. Clinical and laboratory features were not significantly different in D+ and D- cases (P > 0.05 for all parameters). Dialysis was undertaken in 84.6% and acute mortality was 12.8% being significantly higher in D+ (P = 0.002). Demographic, clinical and laboratory features, late referral or need for dialysis were not significantly associated with higher risk of acute mortality (p > 0.05 for all). At short-term follow up (mean period ± SD of 18.54 ± 13.21 months), 51.3% had complete renal recovery, 15.4% CKD 3-4, 12.8% CKD 5 requiring renal replacement therapy (RRT), and 20.5% died. Higher mean serum creatinine and hypertension on discharge were risk factors for adverse outcome (CKD5 requiring RRT or death), P = 0.011 and 0.00 respectively. In spite of institution of RRT and supportive therapy, our data showed less favourable outcome of HUS.

ANNUAL INCIDENCE AND CLINICO-LABORATORY STUDY OF HEMOLYTIC UREMIC SYNDROME AMONG ATTENDANTS OF PEDIATRIC ZAGAZIG UNIVERSITY HOSPITAL.

Background: Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in young children. It is characterized by microangiopathic hemolytic anemia (MAHA), acute renal failure, and thrombocytopenia. Objective: This work aims to study causes, clinical presentation, annual incidence, and outcome of hemolytic uremic syndrome in children admitted to Pediatric Zagazig University Hospital. Subjects and methods: This is a descriptive study, carried out at Children hospital, faculty of medicine, Zagazig University during the period from January 2012 to January 2014.This study included 42 patients younger than 15 years with diagnosis of hemolytic uremic syndrome. Results: Most of cases were younger than 2 years of age (57%). Males (74%) were more affected than females (26%) and most of patients were detected during summer. The overall incidence in pediatric age group ( <15 years) was 1.2 per 100,000 children. Incidence was highest below 5 years old (2.69 per 100,000 children). Most of patients were presented clinically by bloody diarrhea, pallor, oliguria & anuria. Laboratory abnormalities included hemolytic anemia, thrombocytopenia, leukocytosis, hyponatremia, hypokalemia or hyperkalemia, metabolic acidosis, elevated blood urea and serum creatinine. Stool culture was positive in only 7 cases (17%).The mortality rate was 21.4%. Conclusion: Unfavorable outcome was associated with young age, anuria, intractable seizures, high WBC count more than 30,000 per mm3 ,and hyperkalemia above 5 m mol/ L. Key words: Hemolytic Uremic Syndrome, Shiga-toxin, Acute Kidney Injury, Hemodialysis, Complement, TMA, MAHA.

Plasma exchange combined continuous veno-venous hemodiafiltration in the treatment of critical hemolytic uremic syndrome

Objective To investigate the efficacy of bedside plasma exchange(PE) combined continuous veno-venous hemodiafiltration(CVVHDF) in children with critical hemolytic uremic syndrome(HUS). Methods Eight patients with HUS from Pediatric Intensive Care Unit of Shanghai Children′s Hospital were included in the present study.The severity of children was graded according to pediatric critical illness score and pediatric risk of score mortality Ⅲ.Four of them received continuous blood purification treatment.Meanwhile, the clinical manifestation and outcome of HUS were analyzed. Results Eight children with HUS were initially treated with diuretic and blood transfusion for 12-24 hours.Four cases who deteriorated aggressively were performed PE and CVVHDF.Plasauto iQ21 and Prisma flex were used with Prisma TPE 2000 membrane plasma separator and AN69 M60 membrane filter respectively.All the 4 patients with critical HUS survived after bedside continuous blood purification treatment.Clinical symptoms and serum biochemistry were improved significantly as follows.The average levels of serum creatinine and lactate dehydrogenase decreased obviously(318 μmol/L vs. 162 μmol/L; 1 963 U/L vs. 407 U/L, respectively). In addition, platelet count increased significantly(40×109/L vs. 97×109/L). Eventually, symptoms disappeared in these 4 patients. Conclusion The combined therapy of PE and CVVHDF in HUS could stabilize fluid acid-base equilibrium, prevent hemolysis and improve the renal function. Key words: Plasma exchange; Continuous veno-venous hemodiafiltration; Hemolytic uremic syndrome; Critically ill; Children

Early Volume Expansion and Outcomes of Hemolytic Uremic Syndrome.

Hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS) is a severe acute illness without specific treatment except supportive care; fluid management is concentrated on preventing fluid overload for patients, who are often oligoanuric. Hemoconcentration at onset is associated with more severe disease, but the benefits of volume expansion after hemolytic uremic syndrome (HUS) onset have not been explored. All the children with STEC-HUS referred to our center between 2012 and 2014 received intravenous infusion targeted at inducing an early volume expansion (+10% of working weight) to restore circulating volume and reduce ischemic or hypoxic tissue damage. The short- and long-term outcomes of these patients were compared with those of 38 historical patients referred to our center during the years immediately before, when fluid intake was routinely restricted. Patients undergoing fluid infusion soon after diagnosis showed a mean increase in body weight of 12.5% (vs 0%), had significantly better short-term outcomes with a lower rate of central nervous system involvement (7.9% vs 23.7%, P = .06), had less need for renal replacement therapy (26.3% vs 57.9%, P = .01) or intensive care support (2.0 vs. 8.5 days, P = .02), and needed fewer days of hospitalization (9.0 vs 12.0 days, P = .03). Long-term outcomes were also significantly better in terms of renal and extrarenal sequelae (13.2% vs 39.5%, P = .01). Patients with STEC-HUS had great benefit from early volume expansion. It is speculated that early and generous fluid infusions can reduce thrombus formation and ischemic organ damage, thus having positive effects on both short- and long-term disease outcomes.

Characteristics and Outcomes of Renal Transplant Recipients with Hemolytic Uremic Syndrome in the United States.

Hemolytic uremic syndrome (HUS) accounts for <1% of renal transplants in the US. There are limited data on the characteristics and outcomes of HUS in pediatric and adult kidney transplant recipients in the US. This study included all renal transplant recipients identified with HUS (N=1,233) as a cause of end-stage renal disease between 1987 and 2013 using the UNOS/OPTN database. The cohort was divided into two age groups: pediatric (N=447) and adult (N=786). Main outcomes were acute rejection rate at one-year, allograft and patient survival, and recurrence of HUS post-transplant. Both age groups were then compared with a propensity score (1:2 ratio) matched control group with an alternative primary kidney disease (non-HUS cohort: pediatric [N= 829] and adult [N=1,547]). In pediatric cohort, when compared to the PS matched controls, acute rejection, death censored allograft and patient survival was similar in the HUS group. However, in the adult cohort, the graft and patient survivals were significantly worse in the HUS group. HUS was associated with allograft loss (HR=1.40, 95%CI 1.14-1.71) in adult recipients. Patients with HUS recurrence had significantly lower allograft and patient survival rates compared to the non-recurrent group in both age groups. Acute rejection was one of the major predictor of HUS recurrence in adults (OR=2.64, 95%CI 1.25-5.60). Calcineurin inhibitors (CNI) were not associated HUS recurrence in both age groups. Pediatric HUS-patients, unlike adult recipients, have similar outcomes compared to the PS matched controls. Recurrence of HUS is associated with poor allograft and patient survival in pediatric and adult patients. Use of CNIs seem to be safe as a part of maintenance immunosuppression post-transplantation. A comprehensive national registry is urgently needed.

Outcome of hemolytic uremic syndrome

Outcome of hemolytic uremic syndrome

Intracerebroventricular Shiga toxin 2 increases the expression of its receptor globotriaosylceramide and causes dendritic abnormalities

Neurological damage caused by intoxication with Shiga toxin (Stx) from enterohemorrhagic Escherichia coli is the most unrepairable and untreatable outcome of Hemolytic Uremic Syndrome, and occurs in 30% of affected infants. In this work intracerebroventricular administration of Stx2 in rat brains significantly increased the expression of its receptor globotriaosylceramide (Gb 3) in neuronal populations from striatum, hippocampus and cortex. Stx2 was immunodetected in neurons that expressed Gb 3 after intracerebroventricular administration of the toxin. Confocal immunofluorescence of microtubule-associated protein 2 showed aberrant dendrites in neurons expressing increased Gb 3. The pro-apoptotic Bax protein was concomitantly immunodetected in neurons and other cell populations from the same described areas including the hypothalamus. Confocal immunofluorescence showed that Gb 3 colocalized also with glial fibrillary acidic protein only in reactive astrocytic processes, and not in vehicle-treated normal ones. Rats showed weight variation and motor deficits as compared to controls. We thus suggest that Stx2 induces the expression of Gb 3 in neurons and triggers neuronal dysfunctions.

Cortical echogenicity in the hemolytic uremic syndrome: clinical correlation.

The initial renal sonograms of 15 patients, aged 8 months to 5 years, with hemolytic uremic syndrome (HUS) were reviewed. Ultrasound studies were graded according to cortical echogenicity relative to the liver, they were compared to the severity of the clinical syndrome at admission and to the ultimate outcome of the disease. The degree of cortical echogenicity correlated with the clinical outcome of HUS in 12 of the patients, whereas clinical assessment alone predicted outcome in 13 patients. Sonography overestimated severity in three patients with mild disease correctly assessed clinically, whereas clinical assessment overestimated severity in two patients with moderate disease in whom the sonographic assessment proved correct. The sonographic changes are most likely multifactorial. They appear to reflect a combination of platelet-thrombus deposition in the renal cortex, as well as the general fluid status of the patient. Ultrasound is useful in ruling out other causes of acute renal failure such as obstruction or congenital diseases. It cannot replace laboratory tests and clinical judgement, but nevertheless provides another index of severity in patients with HUS.