The case of a 6-year-old Inuit female with the epidemic form of hemolytic uremic syndrome (HUS) with myocardial involvement and probable cardiac tamponade is presented. This case illustrates the multisystemic nature of the syndrome, and to our knowledge, cardiac tamponade as a probable terminal event in HUS has not been reported previously.
We undertook a 1-year prospective point prevalence study to test the hypothesis that there is an excess of non-diabetic renal disease in native American children; 29.6% (73/247) of the population attending the only regional pediatric nephrology clinic in 1993 were native compared with 8.2% of the Manitoba population in this age group (odds ratio = 4.4, P
P1 blood group positivity has been postulated as a host factor which may provide protection against the development of post-enteropathic hemolytic uremic syndrome (HUS). In this study, blood group status in 20 Inuit survivors of Escherichia coli O157:H7-associated HUS was compared with age- and sex-matched controls from the same community who had experienced uncomplicated diarrheal illness due to the same pathogen. Of 20 HUS survivors, 6 were P1 antigen positive compared with 8 of the 20 controls (P = 0.7). We conclude that P1 antigen positivity was not protective against HUS in this population. Further studies of this condition to clarify the role of host factors in verotoxin-induced endothelial damage are indicated.
The use of pediatric ambulatory care facilities to perform invasive procedures that have low morbidity is increasingly popular. Over a 2-year period, 46 pediatric renal biopsies were performed in an ambulatory care setting at the Winnipeg Children's Hospital, with the patient discharged the same day. There was no serious complications and adequate tissue was obtained in 45 cases. Renal biopsy may be safely performed on an outpatient basis on carefully selected patients by experienced operators in properly equipped facilities.
Clinical reports have suggested that therapy with fresh frozen plasma is a useful adjunct in the management of the hemolytic-uremic syndrome (HUS). We reviewed the charts of 36 children with severe HUS who were treated at the Izaac Walton Killam Hospital for Children, Halifax, over 10 years to assess the effectiveness of plasma therapy. All children who required specific supportive therapy for renal dysfunction, hemolysis or serious extrarenal complications were included. We compared the outcome of 18 children who received plasma therapy from 1982 to 1987 with that of 18 children who did not. The two groups were similar with regard to the severity of HUS, the length of hospital stay, the duration of renal dysfunction and the incidence of disease-related complications, such as seizures, enterocolitis and cardiomyopathy. At discharge the prevalence of hypertension was higher in the plasma therapy group than in the control group. Plasma therapy did not demonstrate any benefit that would outweigh the risk of fluid overload, hyperproteinemia and transmission of viral infection.
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We undertook a case-control study to evaluate the renal health of survivors of hemolytic-uremic syndrome (HUS) from the 1991 Arctic epidemic of Escherichia coli O157:H7 gastroenteritis 4 years after the epidemic. Eighteen children who developed HUS during the 1991 epidemic and 18 age- and sex-matched controls from the same community who had uncomplicated gastroenteritis were compared in 1995 for height, weight, blood pressure, urinalysis, and glomerular filtration rate (GFR), measured using continuous subcutaneous infusion of non-radioactive iothalamate. HUS survivors did not differ from controls in height, weight, systolic (HUS 118 mmHg, control 117 mmHg) or diastolic (HUS 64 mmHg, control 62 mmHg) blood pressures. Hematuria was detected more frequently in HUS survivors (11/18 vs. 4/18, P