Abstract
HUS is not a discrete clinicopathologic entity but a syndrome conceptually akin to the nephrotic syndrome. Based on our studies we present this classification:
1) The typical, idiopathic HUS of infancy and childhood: Commonest form. Mainly in endemic areas, but also sporadic. Usually <4 yrs. Prognosis:mild cases, excellent;severe,very good. If sib affected, occurs within days. Pathology, electron microscopy(EM):endothelial cell swelling and formation of subendothelial space. Thromboses rare. 2)HUS with a possible genetic predisposition:Sibs with onsets > 1 yr apart. Mainly nonendemic areas. Poor prognosis with inexorable course.EM:no apparent changes to severe damage.Possibly some overlap with Group 3. May be inherited in some cases as an autosomal dominant gene. 3)HUS with recurrent episodes:Data on 29 cases,24 from nonendemic areas.Rarely have typical prodrome. Only 50% < 4 yr at onset. Sib or cousin involvement in 27%; prognosis poor, > 30% die. EM pathology ranges from no apparent lesion to severe glomerular injury. 4)HUS associated with pregnancy:Pre-eclamptic toxemia,postpartum renal failure. 5)HUS with a putative etiology:Oral contraceptives,viruses,Shigella,Salmonella. 6)Atypical HUS:Classical clinical findings but subepithelial deposits on EM and no aubendothelial lesions. 7)HUS as a feature of other syndromes:Malignant hypertension,TTP,malignancy,immunodeficiency syndromes,postrenal transplantation.
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Kaplan, B., De Chadarevian, JP. CLASSIFICATION OF HEMOLYTIC UREMIC SYNDROME(HUS). Pediatr Res 11, 553 (1977). https://doi.org/10.1203/00006450-197704000-01099
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DOI: https://doi.org/10.1203/00006450-197704000-01099