Outcome and Prognostic Determinants in the Hemolytic Uremic Syndrome of Children

Abstract

The late outcome in 89 children with the hemolytic-uremic syndrome (HUS) oberved from 1971 to 1988 was analyzed up to 17 years after onset in relationship to various clinical and pathologic features at the onset of the disease. In the first 3 months after onset (acute phase) 69% of all children needed dialysis therapy. Fifteen children died, 9 during the acute phase and 6 subsequently. All surviving patients except 7 were reexamined and divided into five prognostic categories: recovery, residual renal symptoms with normal kidney function, moderate renal insufficiency, preterminal chronic renal failure (CRF) and end-stage renal disease (ESRD). The rate of recovery calculated by the life table method increased from 35% after 10 years in 1971-1979 to 68% in 1980-1988 (p < 0.001); it was lower in infants than in older children (44 vs. 63%; nonsignificant). Children with an atypical HUS experienced more often preterminal CRF, ESRD or death than those with a typical (postenteropathic) form (33 vs. 17%; p < 0.05). If oliguria lasted < 7 days, 74% of patients recovered after 10 years versus 13% in the case of oliguria > 14 days or anuria > 7 days (p < 0.0005). The rate of recovery was also significantly smaller with the duration of dialysis treatment > 7 days, central nervous system involvement and requirement for antihypertensive therapy. In the entire series 7 patients developed preterminal CRF and 5 ESRD. Of 27 cases serially followed for 5-10 years after onset, a stable course was noted in 16, a subsequent improvement in 8 and deterioration in 3 leading to ESRD in 2. In conclusion, the study demonstrates a high incidence of chronic kidney disease in patients who have suffered from HUS. Late deterioration of renal function after apparent recovery underlines the need for continuing follow-up examinations for at least 10 years.

Relative Nephroprotection DuringEscherichia coliO157:H7 Infections: Association With Intravenous Volume Expansion

“…Oligoanuria or its duration during HUS is associated with worse outcomes [6][7][8][9][10][11][12][13][14] and should be avoided. 15 At our institutions, we encourage starting intravenous isotonic volume expansion as early as possible in E coli O157:H7 infections, even before culture results are known.…”

“…[1][2][3][4][5] Oligoanuric renal failure during HUS usually necessitates dialysis and lengthens hospitalization; its occurrence and duration are risk factors for chronic sequelae. [6][7][8][9][10][11][12][13][14][15] Ideally, the oligoanuric form of HUS should be prevented, because oligoanuria seems to represent a more severe form of renal injury.Vascular injury and prothrombotic coagulation abnormalities, such as thrombin generation, fibrinolysis inhibition, intravascular fibrin accretion, 3 elevated circulating platelet activating factor, 16 and degraded von Willebrand factor multimers, 17 precede renal injury in infected children. These findings suggest that vascular occlusion, a process that conceivably could be attenuated by volume expansion, at least partly underlies renal insufficiency during HUS.…”

“…[1][2][3][4][5] Oligoanuric renal failure during HUS usually necessitates dialysis and lengthens hospitalization; its occurrence and duration are risk factors for chronic sequelae. [6][7][8][9][10][11][12][13][14][15] Ideally, the oligoanuric form of HUS should be prevented, because oligoanuria seems to represent a more severe form of renal injury.…”

Hemolytic Uremic Syndrome

“…A recent meta-analysis of 49 published studies (3476 patients, mean follow-up of 4.4 yr) describing long-term prognosis of patients who survived an episode of Stx-HUS reported death or permanent ESRD in 12% of patients and GFR Ͻ80 ml/min per 1.73 m 2 in 25% (38). The severity of acute illness, particularly central nervous system symptoms, and the need for initial dialysis were strongly associated with a worse long-term prognosis (4,38). Stx-HUS that is precipitated by S. dysenteriae infection is almost invariably complicated by bacteremia and septic shock, systemic intravascular coagulation, and acute cortical necrosis and renal death and has a high mortality rate (approximately 30%) (39).…”

Shiga Toxin/Verocytotoxin-Producing Escherichia coli Infections: Practical Clinical Perspectives

“…Unfortunately, these risk factors and biomarkers were measured at different points of illness, often after poor outcomes are becoming self-evident. Nonetheless, the greatest determinant of short-(75) and long-term (62,(76)(77)(78)(79)(80)(81)(82)(83)(84)(85)(86)(87) outcome of E. coli-related acute kidney injury remains oligoanuria.…”