Cases reported "Hemoglobinuria"

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1/20. Conservative treatment of hemolytic complication following coil embolization in two adult cases of patent ductus arteriosus.

    Two adult cases of relatively large patent ductus arteriosus (PDA) were treated by coil embolization, but were complicated by hemolysis that was successfully managed by medical treatment. Case 1 was a 67-year-old woman and Case 2 was a 71-year-old woman with a PDA of minimal diameter of 5.3 mm and 5.5 mm, respectively. The approach was via the pulmonary artery and 2 coils were delivered simultaneously into the ductus, known as the 'kissing coil technique'. Although immediately after the procedure only a small residual shunt was revealed by aortogram, hemolysis occurred for several hours after the procedure in both cases. A hemolytic complication usually needs additional coil embolization or surgical treatment, but in these 2 cases it was successfully treated by haptoglobin infusion to prevent nephropathy and by antiplasmin infusion to promote thrombus formation. Hemolytic complications of coil embolization of PDA can managed by medication when the residual shunt is minimal and the degree of hemolysis is mild.
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2/20. Case report. An immediate hemolytic transfusion reaction apparently caused by anti-Dia.

    The Diego blood group system has had its primary applications in population genetics and anthropology, although it can also give rise to clinical problems. Anti-Dia has ofter been reported to cause hemolytic disease of the newborn. The patient presented in the report experienced an immediate hemolytic transfusion reaction apparently due to anti-Dia. We believe it to be the only such case reported since the Diego system was first discovered in 1956.
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3/20. Severe central nervous system thrombotic events in hemoglobin Sabine patient.

    Hemoglobin (Hb) Sabine is a rare, unstable Hb variant resulting from the point mutation in codon 91 (CTG --> CCG) of beta-globin gene. We report a case of Hb Sabine patient with mild hemolytic anemia, unusually high Hb F level and severe central nervous system thrombotic disturbances. We have tried to elucidate possible genetic background of this unusual Hb Sabine phenotype. Extremely high level of Hb F and rather mild anemia in our patient could be partially explained by the presence of G gamma Xmn I polymorphism. This case of Hb Sabine, unlike all other reported to date, shows extremely severe thromboembolic complications. It is our opinion that the hypercoagulable state described in thalassemia is not the only factor responsible for this specific clinical state. The presence of MTHFR C677T mutation in heterozygous state found in our patient and unstable Hb Sabine molecules could contribute to development of thromboembolic phenomena. However, it remains unclear whether other factors participate in pathogenesis of the disease. In this paper we emphasize different genetic background of father and son both affected with Hb Sabine, but with markedly different severity of the disease.
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4/20. Acute haemolytic crisis in a Thai patient with homozygous haemoglobin Constant Spring (Hb CS/CS): a case report.

    Acute haemolysis associated with mild upper respiratory tract infection was observed in a Thai boy who presented with a rapid decline in haemoglobin (Hb) levels, haemoglobinuria and evidence of intravascular haemolysis. Several possible causes giving rise to such a condition were excluded including G6PD deficiency, which is extremely common in thailand. Subsequent haematological and molecular analyses demonstrated that the patient was homozygous for Hb Constant Spring (Hb CS/CS), an a globin haemoglobinopathy. It has been shown previously that patients with homozygous Hb CS had mild haemolytic anaemia secondary to an accumulation of alpha(CS) chains, which are toxic to red blood cell membrane cytoskeletons. Increased body temperature might induce more precipitation of this a globin variant. This report highlights the importance of Hb CS/CS as a potential predisposing cause of acute haemolysis in children that might be aggravated by acute bacterial or viral infections. This is particularly relevant for patients of Southeast Asian descent where this abnormal haemoglobin is highly prevalent.
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keywords = anaemia
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5/20. Aural involvement in loxoscelism: case report and literature review.

    An 11-year-old male presented with fever, rash, and a necrotic lesion on the lobule of the left ear. The lesion became tender and formed an eschar over 4 days. The patient developed leukocytosis, hemolytic anemia, and proteinuria, and was diagnosed with systemic loxoscelism from a brown recluse spider bite. He was managed with supportive therapy and improved in 4 days. Loxoscelism is a clinical diagnosis which should be suspected in an otherwise healthy patient with a necrotic wound, particulary in the endemic Southern and midwestern united states. physicians should be aware of this disease entity and its complications.
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6/20. Fragmentation hemolytic anemia 8 years after replacement of ascending aorta with a sutureless intraluminal graft.

    A 56-year-old man underwent replacement of the ascending aorta with a sutureless intraluminal graft, for a Stanford type A aortic dissection. Eight years after the operation, he developed gross hemoglobinuria, associated with an intravascular hemolytic anemia. Due to numerous schistocytes in the peripheral blood, the hemolysis was attributed to mechanical injury of the red blood cells at the site of the vascular graft. The patient's course was complicated by an infection of the aortic graft, which led to an urgent graft replacement. The hemolytic anemia resolved completely shortly after the reoperation. physicians should consider this etiology in the differential diagnosis of fragmentation hemolytic anemia.
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7/20. Hemolytic transfusion reaction following transfusion of frozen and washed autologous red cells.

    A case of hemolytic transfusion reaction, accompanied by hypotension and followed by transient renal failure, occurred after the transfusion of 1 unit of previously frozen autologous red cells. Subsequent investigation revealed the probable cause of the hemolysis to be inadequate deglycerolization of the unit. The cause of the associated symptoms is unknown. Possibilities include nephrotoxic effects of hemoglobin or stroma, toxic effects of glycerol, or release of vasoactive or thrombogenic substances from lysed red cells. This case of a hemolytic reaction adds to the known risks of autologous transfusion.
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8/20. methyldopa, intravascular haemolysis and renal disease. A case report.

    A 74-year-old man, who was receiving methyldopa to control systemic hypertension, presented to hospital in biventricular cardiac failure. He was found to be severely anaemic and jaundiced as a result of acute intravascular haemolysis with prominent haemoglobinuria. The cardiac failure and anaemia were initially refractory to fluid restriction and diuretics, but responded to red cell transfusion. Renal failure supervened, probably on the basis of hypoperfusion as a consequence of diuresis and hypotension. The patient died suddenly, possibly from myocardial infarction. Acute intravascular destruction of red cells in association with methyldopa appears not to have been previously reported.
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keywords = anaemia
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9/20. Neonatal ABO incompatibility. Complicated by hemoglobinuria and acute renal failure.

    The authors present two infants with isoimmune hemolytic disease due to ABO incompatibility complicated by massive hemoglobinuria and secondary acute renal failure. This represents an incidence of 0.36% of all neonates with ABO hemolytic disease in the author's newborn population. Only two patients have been reported previously to have similar complications. Analysis of data of these four infants revealed the clinical characteristics of this complication of ABO incompatibility: 1) very low frequency; 2) early onset of hemoglobinuria (first voided urine) and of acute renal failure (first 2 days of life); 3) lack of correlation between the clinical presentation of hemolytic disease and appearance and severity of renal failure; 4) complete recovery of renal functions following intravenous fluid administration; and 5) normal renal radiologic investigations.
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10/20. Extracorporeal hemolysis in orthopedic patients. Report of two cases.

    Two patients who had extracorporeal hemolysis of their blood transfusions are reported. In both cases, accidental overheating and hemolysis of the transfused blood caused a gross hemoglobinuria. Because the etiology of the hemolysis was not readily apparent at the time, both patients were managed as though they had had an acute hemolytic transfusion reaction. Because there was no activation of the complement cascade by antigen-antibody complexes, both patients suffered no ill effects. Differentiation of extracorporeal hemolysis from hemolytic transfusion reactions requires a careful inspection of all blood administration equipment, as well as an analysis of the untransfused blood in both bag and tubing. patients undergoing orthopedic procedures account for 15% of all blood transfused in the United States. awareness of adverse effects of transfusion is therefore important to orthopedic surgeons.
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