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The Hemolytic-Uremic Syndrome
264
Citations
0
References
1973
Year
Glomerular DiseaseKidney BiopsiesAcute StageDialysisRenal PathologyPathologyGlomerulonephritisRenal FunctionIga GlomerulonephritisHematologyHemolytic-uremic SyndromeAcute Kidney InjuryChronic Kidney DiseaseHemodialysisRenal CareKidney FailurePediatric NephrologyEnd-stage Renal DiseaseUrologyRenal DiseaseHemostasisMedicineNephrologyKidney Research
The acute stage of HUS is a significant and emerging topic in pediatric nephrology. The study characterizes acute HUS through clinical and pathological data from 678 patients, 52 autopsies, 3 biopsies, and long‑term follow‑up of 124 patients, with detailed pathological analysis of 27 cases. Laboratory and pathological evidence implicates DIC, likely viral‑driven, as the main pathogenic mechanism; mortality remains 6.25% despite dialysis, heparin is ineffective, and long‑term outcomes show 60 recoveries, 64 with persistent renal disease, and nine late deaths from uremia.
The clinical and pathological characteristics of the acute stage of the HUS are described on the basis of 678 patients and the study of 52 autopsies and 3 kidney biopsies. The laboratory and pathological studies point to DIC as a basic pathogenic mechanism, probably related to a viral infection. The recent mortality rate has been 6.25% due to the exacting dialysis treatment employed. Heparin was ineffective in the 26 patients in which it was used. 124 patients have a follow-up of more than 5 years and up to 13 years. 60 patients are completely recovered, while 64 have persistent signs of renal disease. Nine other children died in uremia 1–12 years after onset. The pathological findings are discussed on the basis of 6 autopsies and 24 biopsies in 27 patients. This stage of the HUS appears as an important and new aspect of pediatric nephrology.