International AIDS Society


Abstract



ATYPICAL HIV-1 ASSOCIATED HEMOLYTIC UREMIC SYNDROME (HIV-HUS) AND HIV-HELPP SYNDROME IN CHILDREN

RAY P, CHANDRA R, SELBY D, RAKUSAN T

HIV-HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Very little is known about the pathogenesis of HIV-HUS in children. To define the clinical and pathological features of HIV-HUS in children from the Washington DC area, we followed 280 HIV-infected children from the period between January 1985 and June 2000. All children showing signs of systemic infections and DIC were excluded from this diagnosis. A total of 5 children who developed an atypical form of HIV-HUS were followed prospectively. These children lacked a diarrhea prodrome and showed a clinical course characterized by an insidious clinical onset, preserved urine output, elevated liver and pancreatic enzymes, severe renal tubular injury, and rapid progression to chronic renal failure and death (4/5). The pathologic findings support the diagnosis of atypical HUS in that all patients had microangiopathic changes in renal arterioles, accumulation of fibrin in glomerular capillaries, endothelial cell detachment and proliferation. In addition, sixteen children developed an incomplete form of HUS, which we named HIV-associated HELPP syndrome, due to the presence of hemolytic anemia (H), hypertriglyceridemia (H), elevated liver and pancreatic enzymes (EL), plaquetopenia (P) and proteinuria (P), without renal failure. This incomplete form of HIV-HUS had a better clinical outcome. Both HIV-HUS, and HIV-HELPP were usually associated with the presence of opportunistic infections (i.e, CMV, candida, parvovirus B19). Urine samples from patients with HIV-HUS induced the proliferation of cultured renal endothelial cells, and plasma samples revealed high HIV-1 viral loads and elevated levels of bFGF (an angiogenic growth factor released by injured endothelial cells). We conclude that the HIV-HELPP syndrome may identify HIV-infected children at risk of developing an atypical form of HUS characterized by a rapid progression to chronic renal failure and death. 




The 1st. IAS Conference on HIV Pathogenesis and Treatment
Abstract no. 136


Suggested Citation
"RAYP, et al. ATYPICAL HIV-1 ASSOCIATED HEMOLYTIC UREMIC SYNDROME (HIV-HUS) AND HIV-HELPP SYNDROME IN CHILDREN. Oral Presentation: The 1st. IAS Conference on HIV Pathogenesis and Treatment : Abstract no. 136"