Abstract
A unique etilogy for erythrocytosis was found in a 6-years-old boy. He was normotensive with increased erythropoietin and renin levels. His hemoglobin was 24 g% and hematocrit 73%. The red cell mass by 51Cr tagging was greater than 2 standard deviations above expected values. WBC, platelet count, hemoglobin electrophoresis and serum electrolyte values were normal. No tumor was demonstrated and he increased normally his urinary erythropoietin excretion after phlebotomy. Arterial oxygen saturation was 98% and his hemoglobin did not have increased oxygen affinity. Despite normal creatinine clearance, urine concentration to 1,260 milliosmoes/I, and normal renal biopsy, intrarenal narrowing of distal arteries was found on selective renal arteriography. Increased levels of renin (0.105 and 0.133 au/ml) and erythropoietin (2.6 and 2.5% 59Fe incorporation) were found in renal vein blood. During simultaneous steady state infusion with inulin and PAH, renal plasma flow (282 ml/min) and glomerular filtration rate (85 ml/min) were normal. However, inulin extraction was high normal (30%) while PAH extraction was abnormally low (80%). These values are best explained by intrarenal shunting of blood, establishing areas of ischemia resulting in increased erythropoietin and renin excretion and thus causing the clinical and laboratory characteristics of primary erythrocytosis.
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Kisker, C., Kleinman, L. & Mauer, A. Primary Erythrocytosis, Increased Erythropoietin Excretion and an Intrarenal Perfusion Abnormality. Pediatr Res 4, 468 (1970). https://doi.org/10.1203/00006450-197009000-00136
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DOI: https://doi.org/10.1203/00006450-197009000-00136