Non-enteropathic hemolytic uremic syndrome: causes and short-term course.

Abstract

BACKGROUND:

Nondiarrheal or Streptococcus pneumoniae-related hemolytic uremic syndrome (HUS) represents a heterogeneous group of disorders. This study was performed to: (1) describe the current incidence, causes, demographic features, hospital courses, and short-term outcomes of non-enteropathic HUS; (2) compare findings in patients with non-enteropathic HUS with those obtained from a contemporaneous cohort of children with enteropathic or diarrhea-associated HUS (D+ HUS) diagnosed and treated at the same clinical sites; and (3) identify clinical or laboratory features that differentiate these 2 groups and predict disease severity and the short-term outcome in patients with non-enteropathic HUS.

METHODS:

Data were collected from patients screened between 1997 and 2001 for enrollment in a multicenter trial of SYNSORB Pk (SYNSORB Biotech Inc, Calgary, Alberta, Canada) in D+ HUS, but who were ineligible because of lack of a diarrhea prodrome. The following features were recorded: age; sex; ethnicity; prodromal symptoms; cause; nadir values for hemoglobin, hematocrit, and platelet count; use of dialysis; and length of hospitalization.

RESULTS:

Twenty-seven of 247 children with HUS had non-enteropathic HUS (11%). Twenty-four patients (15 boys, 9 girls), whose medical records were complete and available for review, comprise the study cohort. Mean age at onset was 4.2 +/- 0.9 (SE) years. Infection caused by S pneumoniae was diagnosed in 9 patients (38%). Dialysis was performed in 17 patients (71%) for 40 +/- 27 days. Median length of hospitalization was 22 days (range, 2 to 71 days). Children with S pneumoniae-related HUS had a longer hospital stay than those with other causes of non-enteropathic HUS, but all patients with S pneumoniae-related HUS recovered kidney function. Dialysis therapy was required more often (17 of 24 versus 59 of 145 children; P = 0.025) and hospital stays were longer (median, 22 versus 9 days; P = 0.002) in children with non-enteropathic HUS compared with patients with D+ HUS who were enrolled in the SYNSORB Pk clinical trial.

CONCLUSION:

(1) The incidence of non-enteropathic HUS is approximately one tenth that of D+ HUS; (2) patients with non-enteropathic HUS require dialysis therapy more often and are hospitalized more than twice as long during the acute episode compared with those with D+ HUS; (3) infection caused by S pneumoniae accounts for nearly 40% of cases of non-enteropathic HUS; and (4) although S pneumoniae-related HUS is associated with a less favorable short-term course than other types of non-enteropathic HUS or D+ HUS, the long-term prognosis for recovery of renal function appears to be good in these patients.

• Authors: Alex R Constantinescu, Martin Bitzan, Lynne S Weiss, Erica Christen, Bernard S Kaplan, Avital Cnaan, Howard Trachtman
• Journal: American journal of kidney diseases : the official journal of the National Kidney Foundation (Am J Kidney Dis) Vol. 43 Issue 6 Pg. 976-82 (Jun 2004) ISSN: 1523-6838 [Electronic] United States
• PMID: 15168377 (Publication Type: Comparative Study, Journal Article, Multicenter Study, Research Support, U.S. Gov't, P.H.S.)

Topics

• Adolescent
• Age of Onset
• Canada
• Child
• Child, Preschool
• Cohort Studies
• Diarrhea (complications)
• Female
• Hemolytic-Uremic Syndrome (drug therapy, epidemiology, ethnology, therapy)
• Humans
• Infant
• Kidney (pathology, surgery)
• Kidney Transplantation (methods)
• Male
• Renal Dialysis (methods)
• Renal Insufficiency (etiology, therapy)
• Streptococcus pneumoniae (isolation & purification)