Cases reported "Kidney Failure, Chronic"

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1/7. polycythemia vera in an anephric man.

    The effect of renal failure and bilateral nephrectomy on erythropoiesis and plasma erythropoietic activity was observed in a patient with polycythemia vera. For eight years the patient's hematocrit was maintained between 45 and 50 per cent by phlebotomy and in spite of the development of renal failure the hematocrit did not decline. Following rejection of a renal transplant, the hematocrit fell to 18 per cent but rose to 40 per cent with oral iron therapy. Following bilateral nephrectomy, the hematocrit fell to 29 per cent but subsequently increased to 37 per cent. After an episode of gastrointestinal bleeding the hematocrit was 21 per cent but subsequently rose to 32 per cent. erythropoietin could not be detected in the plasma either before or after nephrectomy. In addition, erythropoietin failed to stimulate 59Fe incorporation into heme in vitro in the patient's marrow cells. The data incidate that, in polycythemia vera, erythropoiesis does not require erythropoietin.
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2/7. Management of porphyria cutanea tarda in the setting of chronic renal failure: a case report and review.

    The treatment of porphyria cutanea tarda (PCT) in patients with chronic renal failure poses a therapeutic challenge. In the absence of renal failure, phlebotomy and oral antimalarials have been the standard of care for PCT. However, in the presence of renal failure, associated chronic anemia often precludes the use of phlebotomy, and oral antimalarials are usually ineffective. We describe a patient with severe symptomatic PCT and chronic renal failure whose disease was successfully managed with a combination of high-dose erythropoietin and small volume phlebotomy. We also review several previously reported approaches to management of PCT in the setting of renal failure, which include small repeated phlebotomy, erythropoietin, deferoxamine, chloroquine, plasma exchange, high-efficiency/high-flux hemodialysis, cholestyramine, charcoal hemoperfusion, and kidney transplantation. An algorithm for the management of these patients is proposed.
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keywords = phlebotomy
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3/7. Treatment of porphyria cutanea tarda with phlebotomy in a patient on peritoneal dialysis.

    porphyria cutanea tarda (PCT) is frequently described among patients with chronic renal failure undergoing hemodialysis. One patient who was receiving peritoneal dialysis and had PCT develop has been described in the literature. However, it was later determined that the patient's PCT was related to the hepatotoxic drug she was receiving rather than her peritoneal dialysis. Our patient is the first reported case, to our knowledge, of a patient with end-stage renal disease with negative hepatitis serology who was not receiving a hepatotoxic drug, or on hemodialysis, who had PCT develop while receiving peritoneal dialysis.
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ranking = 4
keywords = phlebotomy
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4/7. Treatment of a patient with end-stage renal disease, severe iron overload and ascites by weekly phlebotomy combined with recombinant human erythropoietin.

    A 41-year-old hemodialyzed woman developed ascites and was found to have secondary iron overload. The dose of administered iron was approximately 11-12 g, and her serum ferritin level was 15,000 ng/ml (15,000 micrograms/l). There were no signs of congestive heart failure, fluid overload, or liver cirrhosis. A program of weekly phlebotomy combined with recombinant human erythropoietin (rhEPO) therapy was tried to eliminate the iron congestion. After 9 months of this therapy, about 5 g of iron had been removed. The ascites completely disappeared, and her serum ferritin level fell to 5,800 ng/ml (5,800 micrograms/l). This suggests that such combined therapy would be useful when iron overload must be corrected rapidly. Before therapy, the sterile ascitic fluid showed exudative characteristics with 3.7 g/dl (37 g/l) of total protein. The serum-ascites albumin difference was 0.6 g/dl (6 g/l), and the fluid contained 1,400 inflammatory cells/mm3 (1.4 X 10(9)/l). Notably, the serum-ascites albumin difference increased in parallel with iron elimination. These findings suggested that iron deposition may have played a role in changing the permeability of the peritoneum, or in impairing lymphatic drainage, both of which are presumed to be pathogenetic factors of nephrogenic ascites.
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keywords = phlebotomy
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5/7. Iron management during recombinant human erythropoietin therapy.

    Treatment with recombinant human erythropoietin (r-HuEPO; EPOGEN [epoetin alfa], AMGEN Inc, Thousand Oaks, CA) rapidly corrects the anemia associated with end-stage renal disease during the acute phase of therapy and supports hematocrit levels throughout the maintenance phase. However, during the acute phase of therapy, iron deficiency will develop in most patients; it is therefore initially essential to monitor body iron stores monthly. A plasma ferritin level of less than 30 ng/mL or a transferrin saturation level of less than 20% confirms the diagnosis of iron deficiency. Microcytic, hypochromic red cell morphology appears only after prolonged iron deficiency due to inadequate monitoring and insufficient iron supplementation; alternatively, microcytosis in the presence of adequate iron stores suggests aluminum toxicity. In all patients except those with transfusional iron overload, prophylactic supplementation with ferrous sulfate (325 mg up to three times daily) is recommended. When oral supplements, which are poorly tolerated at high doses, are insufficient to meet the extraordinary needs resulting from r-HuEPO-induced erythropoiesis, intravenous iron dextran (500 to 1,000 mg administered in five to ten doses) may be required. During the maintenance phase of therapy, it may be necessary to continue iron supplementation to counteract ongoing loss of iron associated with blood loss through dialyzers and gastrointestinal bleeding. At the other extreme of iron balance, iron overload in transfusion-dependent patients, recent studies suggest that the ability of r-HuEPO to mobilize iron stores can be harnessed with therapeutic phlebotomy to reverse transfusional iron overload.
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6/7. Polycythemia in pediatric renal transplantation.

    Post-transplant polycythemia is not uncommon in adult patients and is usually transient, responding to phlebotomy. Five pediatric patients developed erythrocytosis post-transplantation. Three patients had end-stage renal disease due to cystinosis, one had reflux glomerulopathy and one had focal glomerular sclerosis. The probable causes of the polycythemia were graft arterial stenosis in three patients. In one, polycythemia occurred with nephrosis. Polycythemia with hypertension may indicate the presence of arterial stenosis in children post-transplantation.
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keywords = phlebotomy
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7/7. Reversible acute pulmonary edema due to uncontrolled hyperglycemia in diabetic individuals with renal failure.

    On eight separate occasions, four functionally anephric diabetic patients (on maintenance hemodialysis) experienced episodes of severe hyperglycemia with acute interstitial and alveolar pulmonary edema demonstrated clinically and by chest x-ray without electrocardiographic or enzymatic evidence of an acute myocardial lesion. Three patients had normal stress 201T1 scanning. The fourth patient, who experienced three such episodes, had normal coronary angiograms and only a mild elevation of the left-ventricular end-diastolic pressure. Clinical and chest x-ray improvement were immediate following insulin therapy and control of hyperglycemia, without phlebotomy or dialysis. Since these episodes were observed during a 1-yr period, this syndrome may be more common than suspected. It is concluded that in functionally anephric diabetic individuals: (1) pulmonary edema can be precipitated by uncontrolled diabetes; (2) endogenous fluid shifts may contribute to the cause of acute pulmonary edema; (3) clinical and radiologic improvement can be achieved with adequate insulin therapy; and (4) blood glucose levels should be monitored and controlled in diabetic patients with renal failure.
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