Paroxysmal nocturnal hemoglobinuria (PNH) may present with acute anemia, thrombocytopenia and, if hemoglobin nephropathy or dehydration is present, azotemia. Thus PNH may be confused with the hemolytic uremic syndrome (HUS). Recurrent episodes, though common in PNH, are unusual in HUS. A positive acid hemolysis test can be used to differentiate between the two diseases.
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References
1.
Rubin H.Paroxysmal nocturnal hemoglobinuria with renal failure. JAMA1971;215:433-6.
2.
Gelfand JA, Frank MMRenal failure in paroxysmal hemoglobinuria. JAMA1974;227:440.
3.
Saito T., Furuyama T., Onodera S., et al. Renal impairment in patients with paroxysmal nocturnal hemoglobinuria. TohokJ Exp Med1975;116:267-75.
4.
Shumway CN, Miller G.An unusual syndrome of hemolytic anemia, thrombocytopenia purpura and renal disease. Blood1957;12:1045-60.
5.
Fong Jsc, de Chadarevian JP, Kaplan BSHemolytic-Uremic Syndrome. Current Concepts and Management. Pediatr Clin North Am1983;29:835-6.
6.
Drummond KNHemolytic Uremic Syndrome: then and now. N Engl J Med1985;312:116-8.
7.
Olgaard K., Madsen S., Jacobsen SVRecurrent attacks of hemolytic anemia, thrombocytopenia and uremia, hemolytic uremic syndrome?Acta Med Scand1974;196:541-3.
8.
Drukker A., Winterborn M., Bennett B., et al. Recurrent hemolytic-uremic syndrome: a case report. Clin Nephrol1975;4:68-72.
9.
Folman R., Arbus GS, Churchill B., et al. Recurrence of the hemolytic uremic syndrome in a 3.5-year-old child, 4 months after second renal transplantation. Clin Nephrol1978;10:121-7.
10.
Tune BM, Groshong T., Plumer LB, et al. The hemolytic-uremic syndrome in siblings: a prospective survey . J Pediatr1974;85:682-3.
11.
Hagge WW, Holley KE, Burke EC, et al. Hemolytic-uremic syndrome in two siblings. N Engl J Med1970;227:138-9.
12.
Kaplan BS, Chesney RW, Drummond KNHemolytic uremic syndrome in families. N Engl J Med1975;292:1090-3.
13.
Braun SR, Weiss FR, Keller AI, et al. Evaluation of the renal toxicity of heme proteins and their derivatives: a role in the genesis of acute tubule necrosis. J Exp Med1970;131:443-60.