https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(05)71144-2.pdf

Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome

KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases

The hemolytic–uremic syndrome, which is characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal impairment, occurs most frequently in young children. Most cases are secondary to infection with Escherichia coli O157:H7 and other Shiga-toxin–producing strains. However, approximately 10% of cases are atypical and not associated with infection. This article reviews current concepts about the pathobiology of atypical hemolytic–uremic syndrome and its diagnosis and management.

https://www.nejm.org/doi/suppl/10.1056/NEJMra0902814/suppl_file/nejm_noris_1676sa1.pdf

Atypical Hemolytic–Uremic Syndrome

KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases.

ContextThe long-term renal prognosis of patients with diarrhea-associated hemolytic
uremic syndrome (HUS) remains controversial.ObjectivesTo quantify the long-term renal prognosis of patients with diarrhea-associated
HUS and to identify reasons for different estimates provided in the literature.Data SourcesWe searched MEDLINE and Experta Medica (EMBASE) bibliographic databases
and conference proceedings, and we contacted experts until February 2003.
We also searched the Institute for Scientific Information index and reference
lists of all studies that fulfilled our eligibility criteria. The search strategy
included the terms hemolytic-uremic syndrome, purpura, thrombotic thrombocytopenic, Escherichia coli O157, longitudinal studies, kidney diseases, hypertension, and proteinuriaStudy SelectionAny study that followed up 10 or more patients with primary diarrhea-associated
HUS for at least 1 year for renal sequelae.Data ExtractionTwo authors independently abstracted data on study and patient characteristics,
renal measures, outcomes, and prognostic features. Disagreements were resolved
by a third author or by consensus.Data SynthesisForty-nine studies of 3476 patients with a mean follow-up of 4.4 years
(range, 1-22 years at last follow-up) from 18 countries, 1950 to 2001, were
summarized. At the time of recruitment, patients were aged 1 month to 18 years.
In the different studies, death or permanent end-stage renal disease (ESRD)
ranged from 0% to 30%, with a pooled incidence of 12% (95% confidence interval
[CI], 10%-15%). A glomerular filtration rate lower than 80 mL/min per 1.73
m2, hypertension, or proteinuria was extremely variable and ranged
from 0% to 64%, with a pooled incidence of 25% (95% CI, 20%-30%). A higher
severity of acute illness was strongly associated with worse long-term prognosis.
Studies with a higher proportion of patients with central nervous system symptoms
(coma, seizures, or stroke) had a higher proportion of patients who died or
developed permanent ESRD at follow-up (explaining 44% of the between-study
variability, P = .01). Studies with a greater proportion
of patients lost to follow-up also described a worse prognosis (P = .001) because these patients were typically healthier than those
followed up. One or more years after diarrhea-associated HUS, patients with
a predicted creatinine clearance higher than 80 mL/min per 1.73 m2,
no overt proteinuria, and no hypertension appeared to have an excellent prognosis.ConclusionsDeath or ESRD occurs in about 12% of patients with diarrhea-associated
HUS, and 25% of survivors demonstrate long-term renal sequelae. Patients lost
to follow-up contribute to worse estimates in some studies. The severity of
acute illness, particularly central nervous system symptoms and the need for
initial dialysis, is strongly associated with a worse long-term prognosis.

Long-term renal prognosis of diarrhea-associated hemolytic uremic syndrome: a systematic review, meta-analysis, and meta-regression.

Seventy-nine children with haemolytic uraemic syndrome (mean age 28 months) were randomly assigned either to a group receiving plasma infusions (plasma group, n = 39) or to a group treated conservatively (control group, n = 40). The duration of haemolysis, thrombocytopenia and anuria was similar in the two groups. Serum creatinine levels were similar in the two groups at the 1-month follow-up but were higher in the control group at 3 months (plasma group 49 +/- 14, control group 66 +/- 28 mumol/l; P less than 0.02) and at 6 months (plasma group 48 +/- 13, control group 63 +/- 21 mumol/l; P less than 0.005). The prevalence of proteinuria was also higher in the control group at the 6-month follow-up (plasma group 17%, control group 46%; P less than 0.02). However, differences were no longer significant after 1 year. Renal tissue was examined in 54 cases (plasma group, n = 27; control group, n = 27). Diffuse cortical necrosis was present in 7 cases in the control group but was absent in the plasma group (P less than 0.02). Taking into consideration the higher serum creatinine levels, the higher prevalence of proteinuria during the first 6 months of follow-up in the control group and the presence of diffuse cortical necrosis in this group compared with the plasma group, we conclude that plasma infusions should be regarded as beneficial. Further study is needed to determine which plasma fraction is involved.

Treatment of the childhood haemolytic uraemic syndrome with plasma. A multicentre randomized controlled trial. The French Society of Paediatric Nephrology.

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Treatment of the childhood haemolytic uraemic syndrome with plasma. A multicentre randomized controlled trial. The French Society of Paediatric Nephrology.