December 1980

Hematuria in Hemoglobin S Disorders

Author Affiliations

Departments of Medicine and Preventive Medicine Washington University School of Medicine 4566 Scott Ave St Louis, MO 63110

Arch Intern Med. 1980;140(12):1573-1574. doi:10.1001/archinte.1980.00330230019004

Medical treatment of patients with hemoglobin S disorders severely tests the skill and judgment of their physicians. Dramatic and potentially serious symptoms and findings are common; the question is whether they are primary manifestations of the hemoglobinopathy (in which case conservative management is indicated) or whether they reflect serious independent pathologic entities (eg, intra-abdominal catastrophes, osteomyelitis, pulmonary emboli, pneumonia, and so forth), where prompt aggressive, and often invasive, diagnosis and treatment are required. This generalization is equally apt with respect to renal manifestations of hemoglobin S disorders (SS, SA, SC, and S-thalassemia). Recent reviews by Buckalew and Someren1 and by Sears2 describe a wide spectrum of abnormalities under the rubric of hemoglobin S "nephropathy," including hyposthenuria, hematuria, nephrotic syndrome, papillary necrosis, and end-stage renal failure (rare). Pathologic examination of kidneys removed surgically or at autopsy demonstrates vascular dilation and engorgement in both cortical and medullary areas, sometimes with

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