Materiały źródłowe

• #1 Hemolytic Uremic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK556038/

  The prognosis of typical HUS is generally good with mortality estimated at 5% overall. However, up to 25% of patients with HUS develop long-term renal insufficiency with a glomerular filtration rate 80 mL/min/1.73 m2, hypertension, or proteinuria, which could predispose patients to increased renal insufficiency as they get older. The most significant prognostic indicator of ongoing renal dysfunction is the length of time on dialysis, with long-term complications evident after 2 to 3 weeks of dialysis dependency. One exception to the low mortality rate of typical HUS is in adults older than 60 who comprise most fatalities. […] The course of aHUS has traditionally been much less benign than typical HUS; however, the advent of eculizumab treatment has decreased progression to ESRD or death in children from 30% to 50% down to 9% and in adults from 60% to 6% down to 15%.

• #2 Shiga Toxin-Associated Hemolytic Uremic Syndrome: Specificities of Adult Patients and Implications for Critical Care Management

  https://www.mdpi.com/2072-6651/13/5/306

  Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) is a form of thrombotic microangiopathy secondary to an infection by an enterohemorrhagic E. coli. […] In this review, we review the epidemiology, most important outbreaks, physiopathology, clinical presentation and prognosis of STEC-HUS, focusing on the differential features between pediatric and adult disease. […] We show that the clinical presentation of STEC-HUS in adults is far from typical and marked by the prevalence of neurological symptoms and a poorer prognosis. […] The differences between pediatric and adult patients have implications for the treatment of this disease, which remains a public health threat and lack a specific treatment. […] The global burden of STEC infection worldwide has been estimated to be around 43.1 acute illnesses per 100,000 person-years, leading to 3890 annual cases of STEC-HUS, the vast majority of STEC infections remaining uncomplicated.

• #3 Hemolytic-Uremic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/201181-overview

  Stx-HUS prognosis is as follows: Acute kidney injury occurs in 55-70% of patients, but 85% recover kidney function with supportive therapy. […] NonStx-HUS prognosis is as follows: Patients collectively have a poor prognosis, and as many as 50-60% progress to ESRD (50% in those with the sporadic forms and 60% in those with the familial forms) or develop irreversible brain damage. About 25% die during the acute phase. […] A study by Balestracci et al comparing 145 cases of non-severe Stx-HUS with 71 severe cases found that a shorter prodromal phase is associated with worse prognosis. […] Factors predictive of poor prognosis are as follows: NonStx-HUS, Prolonged oliguria or anuria, Severe hypertension (especially delayed onset of hypertension), Involvement of medium-sized arteries, Severity of CNS symptoms, Persistent consumption of clotting factors, Extensive glomerular involvement (80%), Age older than 5 years.

• #4 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hemolytic-Uremic-Syndrome-Management-and-Complications.aspx

  In most cases, HUS is self-limited, and 95% of children recover with effective supportive treatment. The mortality has dropped from above 30% to as low as 1% in some studies. This is linked to close monitoring and effective support throughout the phases of the illness. […] Factors which predict the onset of complications and death include: Oliguria persisting beyond 10 days, Anuria for more than 5 days, Dehydration in the acute phase, High total count above 20 000 per mm3, Hematocrit over 23%. […] 5% of patients may die, most commonly during the acute renal injury phase. […] Atypical HUS may run in families, and has a worse prognosis than the typical form, resulting in end-stage renal disease in most patients. In this group, transplanted kidneys tend to develop the condition, ending in graft loss.

• #5 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hemolytic-Uremic-Syndrome-Management-and-Complications.aspx

  In up to a quarter of patients, complications persist for life, such as hypertension and chronic renal failure, occurring even 20 years after an apparently complete recovery. Kidney damage persists in a third of cases. Long-term monitoring of HUS patients is required to check for the later development of high blood pressure or protein loss in urine. The most accurate predictor of long-term prognosis is a renal biopsy which shows: more than half the glomeruli are destroyed by microangiopathic damage, arterial microangiopathy, cortical necrosis.

• #6

  https://link.springer.com/article/10.1007/s00431-021-04200-1

  Our objective was to establish the rate of neurological involvement in Shiga toxin-producing Escherichia coli hemolytic uremic syndrome (STEC-HUS) and describe the clinical presentation, management and outcome. […] Neurological involvement occurred in 22 (11%). […] Of the 21 surviving children with neurological involvement, 19 (91%) achieved a complete neurological recovery. […] Neurological involvement in STEC-HUS is associated with good long-term outcome (complete neurological recovery in 91%) and a low case-fatality rate (4.5%) in our cohort. […] We have identified that the rate of neurological involvement in STEC-HUS is 11%. Neurological involvement is associated with predominantly good long-term outcome (90%) and a reduced case-fatality rate (4.5%) compared to older reports. […] One in ten children with STEC-HUS will have neurological involvement and 90% will have a complete neurological recovery.

• #7 Shiga Toxin-Associated Hemolytic Uremic Syndrome: Specificities of Adult Patients and Implications for Critical Care Management

  https://www.mdpi.com/2072-6651/13/5/306

  Despite the much lower incidence of STEC-HUS in adults compared to children, the majority of STEC-HUS related deaths occurs in people ≥60 years old. […] Hence, the clinical characteristics of STEC-HUS in adults, as well as the impact of therapeutic strategies on outcome, remain uncertain. […] While two thirds of enterohemorrhagic E. coli (EHEC)-infected patients fully recover, identification of the risk factors predicting a fatal outcome or long-term sequelae is of critical importance. […] STEC-HUS is associated with a relatively low fatality rate in children, under 3%. […] Mortality in the adult population offers a stark contrast, rising up to 15 to 33% in elderly and frail populations. […] Long-term sequelae have been increasingly recognized in both populations, affecting about one-third of pediatric patients and possibly more than 50% of adults. […] Overall, the impact of STEC-HUS on the natural history of a preexisting condition is a major concern in elderly and frail patients.

• #8

  https://link.springer.com/article/10.1007/s40124-020-00208-7

  We review the pathophysiology of Shiga-Toxin Enteropathogenic Hemolytic Uremic Syndrome (STEC-HUS), strategies to ameliorate or prevent evolution of STEC-HUS, management and the improved recognition of long-term adverse outcomes. […] The long-term outcomes of STEC-HUS, similarly to other causes of AKI, are not as benign as previously thought. […] The long-term sequelae from STEC-HUS are significant and patients with apparent full renal recovery remain at risk. […] The risk to children of developing renal failure following STEC-HUS in the short and medium-term is well understood. However, the long-term risks into adulthood are less well known, particularly for patients whose renal function appears to completely recover during the acute episode; it is unclear whether such patients require any form of long-term surveillance.

• #9

  https://link.springer.com/article/10.1007/s40124-020-00208-7

  One meta-analysis reviewing the development of long-term renal sequelae after STEC-HUS (with a minimum of 12 months from acute disease onset) reported mortality or end-stage renal disease (ESRD) in 0-30% of patients. Up to 64% of patients developed abnormal GFR, proteinuria or hypertension. […] A small cohort of 29 patients examined long-term follow-up into adulthood (15-25 years). Ten patients had no renal sequelae; the remaining 19 developed renal dysfunction (isolated hypertension n=7, proteinuria n=4) with four reaching ESRD. […] That cohort also reported that an acute requirement for RRT did not predict either subsequent CKD or those at long-term risk. […] These studies demonstrate that patients with less severe initial disease may still be at risk of long-term renal sequelae, consistent with other AKI literature.

• #10

  https://link.springer.com/article/10.1007/s40124-020-00208-7

  There are data supporting the supposition that children with a full renal recovery have ongoing detectable manifestations of renal disease. […] Clarifying the underlying pathogenesis for these persisting abnormalities may assist in informing future therapies. […] The long-term outcome of STEC-HUS is less optimistic than earlier reports suggested, as longitudinal follow-up reveals patients remain at risk into adulthood. Recognition may allow earlier management of proteinuria and hypertension.

• #11 Predictive features of chronic kidney disease in atypical haemolytic uremic syndrome | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0177894

  Chronic kidney disease (CKD) is a frequent and serious complication of atypical haemolytic uremic syndrome (aHUS). […] Prognosis of aHUS is poor with a global mortality rate of 25% and a frequent progression to chronic kidney disease (CKD). Over half of patients develop end-stage renal disease (ESRD) after the first aHUS presentation and quality of life is consequently significantly impacted. […] Patients with mutations of CFH have the poorest prognosis with an up to 79% mortality or ESRD rate at 3 years and extra-renal lesions in 20% of cases, whereas in patients with MCP mutations mortality/ESRD rate is 20%. […] We report that severe renal impairment, high blood pressure and a mildly decreased platelet count in aHUS at diagnosis are associated with significant 1-year renal impairment.

• #12 Predictive features of chronic kidney disease in atypical haemolytic uremic syndrome | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0177894

  A model to predict CKD was constructed using these 3 parameters and showed a good discriminative ability (AUC = .84). The CKD score was based on the regression coefficient of the 3 predictors in the multivariate model. The score ranged from 0 to 5, with 4 categories for serum creatinine at admission, 2 categories for platelet count and 2 categories for MAP. The corresponding risk for 1-year CKD ranges from 18 to 100%. […] In conclusion, we identified the magnitude of renal dysfunction, high blood pressure and a higher platelet count on diagnosis as predictors of unfavourable renal outcome in aHUS. We also established a clinical score able to predict persistent renal failure that should help identifying patients suitable for a rapid treatment with complement blockers.

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