Haemolytic Uraemic Syndrome in Childhood: Clinical, Cognitive and Psychological Aspects
Condition: Hemolytic-Uremic Syndrome; Children; Parents; Psychological Adjustment
MeSH terms: Azotemia; Hemolytic-Uremic Syndrome; Syndrome
Study type: Observational

Summary: 
      The purpose of this study is to investigate the clinical, cognitive outcome and psychosocial
      outcome of haemolytic uraemic syndrome in childhood.

      The haemolytic uraemic syndrome (HUS) is the leading cause of acute renal failure in
      childhood. The more common typical HUS is mostly caused by Shigatoxin-producing
      enterohaemorrhagic Escherichia coli (EHEC). The rarer atypical HUS is mainly caused by
      different genetic abnormalities in complement regulatory proteins.

      About 50 till 60 percent of all patients with HUS develop a severe acute renal failure and
      require dialysis. Resulting from new diagnostic and therapeutic approaches the survival rate
      increased during the last years. Despite this, there are only few data concerning long-term
      prognosis, cognitive and motoric development, as well as psychological coping and
      health-related quality of life of affected children and their parents.
    

Detailed description: 
      The main purposes of this study are: the evaluation of the long-term renal function of
      pediatric HUS-patients; the evaluation of the dialysis method in the acute phase of the
      disease and its impact on the duration of the renal-placement-therapy, as well as the
      long-term renal function; the evaluation of the intellectual and motoric performance of
      affected children and the evaluation of the health-related quality of life and psychological
      processing of the patients themselves and their parents.

      All cases of HUS treated at the University Children's Hospital Zurich between 1995 and 2012
      will be analyzed retrospectively. In the course of a routine-follow-up-examination the
      clinical data of patients up to the age of 17 years will be actualized and completed. The
      results of clinical and paraclinical investigations related to renal function will be
      evaluated to describe the clinical features of haemolytic uraemic syndrome in childhood.

      Intellectual performance will be analyzed with the Wechsler-Intelligence Scale for
      Children-IV (WISC-IV) and motoric performance with the Zurich Neuromotor Assessment in
      children at the age from 6 to 16 years.

      The health-related quality-of-life of all parents and affected children from the age of 7
      years will be assessed by different generic quality-of-life-instruments.
    

Observational model: Cohort
Time perspective: Retrospective
Sampling method: Probability Sample
Study population: 
        children and youth from 2 months to 17 years in who had suffered from haemolytic uraemic
        syndrome
      
Biospecimen description: 
      urine, native-blood, blood plasma, EDTA stabilized blood, heparin blood
    
Keywords: haemolytic uraemic syndrome; complications of HUS; renal replacement treatment; renal function outcome; neuropsychological sequelae; psychological adjustment; quality of life