Childhood haemolytic uraemic syndrome: long-term outcome and prognostic features

A Kelles1, M Van Dyck, W Proesmans

  • 1Department of Paediatrics, Renal Unit, University Hospital Gasthuisberg, Leuven, Belgium.

Insights

Most children with haemolytic uraemic syndrome (HUS) survive, but long-term outcomes are unpredictable. Initial symptoms like hypertension or CNS issues don't predict later complications, highlighting the need for continued monitoring.

Area of Science:

  • Pediatric Nephrology
  • Critical Care Medicine
  • Epidemiology

Background:

  • Haemolytic uraemic syndrome (HUS) is a serious condition affecting infants and children.
  • Understanding the long-term prognosis and predictors of sequelae in HUS is crucial for patient management.

Purpose of the Study:

  • To evaluate the long-term outcomes and sequelae in a cohort of children diagnosed with HUS.
  • To identify clinical and laboratory factors during the acute phase that predict the development of long-term complications.

Main Methods:

  • A retrospective cohort study of 95 infants and children with HUS admitted between January 1970 and December 1982.
  • Patients were followed for up to 10 years, with assessments of renal function, arterial hypertension, and neurological status.
  • Clinical and laboratory data from the acute phase were analyzed for prognostic significance.

Main Results:

  • The overall mortality rate in the acute phase was 6.3%, with 4.2% progressing to end-stage renal failure.
  • After 10 years, 65% of survivors had no sequelae, 26% had mild defects, and 9% had severe sequelae.
  • Neither initial arterial hypertension nor central nervous system involvement predicted immediate outcome, nor did any single variable or combination of complications predict long-term sequelae.

Conclusions:

  • While the immediate outcome of HUS has improved, long-term sequelae remain a concern.
  • Predicting long-term sequelae based on acute-phase clinical or laboratory data is not currently possible.
  • Long-term, regular follow-up is essential for all HUS survivors to manage potential late-onset complications.

Related Concept Videos

Urinary Tract Infection II: Pathophysiology01:25

Urinary Tract Infection II: Pathophysiology

The pathophysiology of urinary tract infections (UTIs) encompasses several progressive stages, beginning with bacterial colonization and culminating in potential systemic complications if untreated. UTIs are primarily initiated by bacteria, such as Escherichia coli, which often originate from the gastrointestinal tract and migrate to the urinary system through the periurethral area. This migration can occur via several routes, including improper hygiene practices, sexual activity, or...
Kidney Transplant I: Introduction01:28

Kidney Transplant I: Introduction

A kidney transplant is a surgical approach that involves replacing a non-functioning kidney with a healthy one from a donor. This procedure is often a treatment option for end-stage renal disease (ESRD) patients. The method requires careful recipient selection, including evaluating various medical and psychosocial factors. These criteria vary between transplant centers but generally include assessments of the patient's overall health, adherence to medical recommendations, and lifestyle...
Acute Kidney Injury I: Introduction01:22

Acute Kidney Injury I: Introduction

Introduction:Acute Kidney Injury (AKI) describes a swift decrease in kidney function occurring over hours to days, characterized by the kidneys' failure to remove waste products from the bloodstream. This leads to dangerous complications like metabolic acidosis, fluid overload, and electrolyte imbalances, such as hyperkalemia, which can cause life-threatening arrhythmias. AKI is common in both hospital and outpatient settings, often triggered by dehydration, sepsis, or exposure to nephrotoxic...
Acute Kidney Injury III: Clinical Manifestations01:29

Acute Kidney Injury III: Clinical Manifestations

Acute Kidney Injury (AKI) progresses through distinct clinical phases: the oliguric, diuretic, and recovery phases, each marked by unique manifestations and challenges.Oliguric Phase:The oliguric phase is the initial stage of AKI, typically lasting 10 to 14 days. This phase is marked by a significant reduction in urine output, usually less than 400 mL per day, indicating decreased kidney function. Fluid retention is a prominent feature, leading to symptoms such as edema, hypertension, and...
Rocky Mountain Spotted Fever01:26

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever (RMSF) is a severe tick-borne illness caused by Rickettsia rickettsii, a Gram-negative, coccobacillary bacterium. This pathogen is an obligate intracellular parasite, requiring a host cell for replication. Transmission occurs through the bite of an infected tick. In the United States, the most important vectors are Dermacentor variabilis (American dog tick) and Dermacentor andersoni (Rocky Mountain wood tick), though other tick species may also serve as vectors.
Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...