Cases reported "Hematuria"

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1/18. microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?

    We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.
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2/18. Primary localized amyloidosis of the urinary bladder. A case report.

    We present case 96 (the first one in bulgaria) of primary localized amyloidosis of the urinary bladder in a 72-year-old woman with episodes of painless hematuria. The amyloid tumor is situated on the anterior wall of the bladder--a yellowish prominence 15 mm in diameter with superficial erosion that is surrounded by a reddish ring and bullous edema of the mucosa. Rough and regularly pink perivascular deposits, thickened vessel walls and focal lymphoplasmocyte infiltrates are present in the submucosa. After preliminary treatment with KHMnO4, AL-amyloid is detected in polarized light by Congorot staining and in ultraviolet light using thioflavin. immunoglobulins and C3 deposits are not found. On electron microscopy deposits of amyloid fibrils are seen in abundance close to collagen bundles. Diseases that may be associated with systemic amyloidosis have been clinically excluded. Nodular amyloidosis resulting from a local immune dyscrasia is accepted in the case. The patient is in good health 11 months after the first biopsy. A literature review on this rare form of localized amyloidosis has been made. Generally, the disease has a favorable prognosis and non-active behavior is recommended. Intravesical instillations of dimethyl sulfoxide, sectional resection or plastic surgery that preserve the urinary tract are used in the rare case of progressive course.
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3/18. Massive hematuria after cystoscopy in a patient with an internal iliac artery aneurysm.

    An unusual case is reported here of a patient with internal iliac artery aneurysm who developed massive hematuria after cystoscopic examination. A 75-year-old man presented with asymptomatic gross hematuria. Cystoscopic examination revealed that the bladder neck was congested and that the right-side wall was being pressed on by an extrinsic mass. Computed tomography showed a right internal iliac artery aneurysm and tortuous perivesical vessels. Three days after the cystoscopic examination the patient suffered massive hematuria. hemorrhage due to an arteriovesical or arterio-ureteral fistula secondary to rupture of the internal iliac artery aneurysm was suspected, and an emergency operation was performed. At operation the aneurysm had not ruptured but overswelling perivesical vessels were found to have developed, and these fed a high blood flow to the bladder neck. In the present case cystoscopic examination injured the mucosa and led to massive hemorrhage from the bladder neck.
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4/18. An uncommon association of abdominal vascular compression syndromes: Dumbar and Nutcracker.

    abdominal pain associated with nausea and vomiting in a young patient led to a diagnosis of median arcuate ligament syndrome. The presence of mild haematuria was associated with a concomitant Nutcracker syndrome. diagnosis was achieved by a computed tomography scan, which showed compression of the vessels of the coeliac axis and left renal vein. These syndromes are very rare, and their association in the same patient has not been described before. There is no relationship in the aetiology of these entities. In this report we discuss the diagnosis and therapeutic options, and review the literature.
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5/18. Subconjunctival hemorrhage: the first presenting clinical feature of idiopathic thrombocytopenic purpura.

    BACKGROUND: Subconjunctival hemorrhage as the first presenting clinical feature of idiopathic thrombocytopenic purpura, to the best of our knowledge, has not been reported earlier. CASE: A 60-year-old woman presented with an isolated finding of subconjunctival hemorrhage. She later developed hemorrhage from retinal vessels and had a single episode of hematuria. OBSERVATIONS: Her blood cell count showed an extremely low platelet count. Her medical history and clinical examination for any other systemic or ophthalmic pathology were negative. Even after treating the patient with blood and platelet transfusion and maintaining her on high doses of systemic steroids, she did not respond well. CONCLUSION: The appearance of spontaneous subconjunctival hemorrhage in a patient should be thoroughly investigated as it can be an initial sign of a grave systemic disorder.
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6/18. Spontaneous thrombosis of the renal vessels. Rare entities to be considered in differential diagnosis of patients presenting with lumbar flank pain and hematuria.

    We report 2 cases of spontaneous thrombotic occlusion of the main renal vessels presenting with acute lumbar flank pain and hematuria suspect of nephrolithiasis. Clinical and laboratory signs of blood hypercoagulability, generalized arterial embolism, nephrotic syndrome or glomerulonephritis were absent. Excretory urography, nephrosonography and retrograde ureteropyelography showed no evidence of upper urinary tract calculi or other causes of obstruction. Renal angiography and cavography demonstrated an acute renal vein thrombosis in 1 patient and a thrombotic occlusion of all but one of the segmental renal arteries in the other patient. These 2 cases demonstrate that thrombotic occlusion of the renal artery or renal vein has to be considered in patients who are presenting with lumbar flank pain and hematuria, in whom the excretory urogram shows severe malfunction of one of the kidneys, and stone disease can be excluded. Renal angiography and cavography as well as CT scan should be carried out in these patients. When the disease is diagnosed at an early stage, an intra-arterial thrombolysis can be attempted.
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7/18. Recurrent macroscopic haematuria due to bladder blood vessels after exercise induced haematuria.

    The case is reported of exercise induced asymptomatic macroscopic haematuria, which became recurrent haematuria no longer induced by exercise. The cause, diagnosis, and management are discussed. An overview of the potential causes of sport related haematuria is presented.
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8/18. Nutcracker syndrome associated with severe anemia and mild proteinuria.

    A 70-year-old man was referred to our hospital with the chief complaint of gross hematuria. urinalysis revealed gross hematuria (3 , RBC 100/HPF or more) and mild proteinuria (3 , 1.8 g/day) with no urinary casts. Computed tomography of the abdomen showed compression of the left renal vein between the superior mesenteric artery and the aorta. ultrasonography showed an increased flow velocity at the stenotic portion of the left renal vein. An aortography and selective left renal arteriography showed that there was no evidence of tumor vessels or arterial abnormalities in the arterial phase. However, the venous phase revealed a stenosis of the left renal vein just lateral to the aorta as well as a reflux of contrast material toward the left gonadal vein which was dilated. In addition, cystoscopy revealed left ureteral bleeding. Based on these findings, we made the diagnosis of gross hematuria caused by nutcracker syndrome (NCS). We concluded that the main cause of the anemia and proteinuria in our patient was leakage of blood and this is confirmed by the relationship of red blood cells to protein in the urine because we proved whole blood and plasma protein loss in the urine by calculation. Fourteen months after discharge, both the gross hematuria and proteinuria spontaneously disappeared. This case strongly suggested that the first therapy for hematuria and proteinuria with NCS should be observation.
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9/18. Characterization of a myeloma patient with a life-threatening hemorrhagic diathesis: presence of a lambda dimer protein inhibiting shear-induced platelet aggregation by binding to the A1 domain of von willebrand factor.

    We have identified a patient with IgD lambda-type multiple myeloma who was characterized by a severe bleeding tendency, especially after puncture of arterial vessels. Both the bleeding time (>25 min) and activated partial thromboplastin time (APTT) were prolonged. To clarify the underlying pathogenesis, we purified the APTT-prolonging activity from the patient's serum. The purified protein was a highly negatively-charged homodimer of the lambda light chain. The lambda dimer protein (M-protein) inhibited ristocetinand high shear-induced platelet aggregation, dependent on platelet glycoprotein Ibalpha (GPIbalpha), but not epinephrine-, collagen-, ADP-, thrombin-, or botrocetin-induced platelet aggregation. The lambda dimer protein inhibited the binding of platelets to immobilized or ristocetin-treated von willebrand factor (VWF). Furthermore, a 39/34 kD fragment of VWF encompassing the A1 domain specifically bound to the immobilized lambda dimer protein in the presence of ristocetin, suggesting that the lambda dimer protein directly binds to the A1 domain of VWF. To help elucidate the binding site within the A1 domain, binding of ristocetin-treated VWF to the immobilized lambda dimer protein was assayed in the presence of various anti-A1 domain monoclonal antibodies. Based on these data, we conclude that the lambda dimer protein binds to the region of the A1 domain composed of helices alpha3 and alpha4 and thus interferes with VWF-GPIbalpha interaction. The existence of a protein that inhibits high shear-induced platelet aggregation in acquired von Willebrand disease (VWD) has only rarely been reported. The results suggest that the hemostatic function in arteries with high shear force is profoundly disrupted if the binding of GPIbalpha to VWF is abrogated, supporting the relevance of shear-induced VWF interaction with GPIbalpha in the initiation of the hemostatic process.
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10/18. Superselective embolization of bladder arteries in the treatment of intractable bladder haemorrhage.

    Bladder hemorrhage following radiation therapy is a serious complication in patients undergoing this treatment. Several methods have been proposed to control this particular situation; however, results have been far from satisfactory, with the exception of drastic measures such as hypogastric artery ligation and radical cystectomy. We recently used a method of superselective embolization of the bladder arteries which enabled us to control severe intractable bleeding in a patient submitted to bladder irradiation for a transitional cell infiltrating carcinoma. Compared to selective embolization and other methods, the advantages of superselective embolization are a lower recurrence rate concerning bleeding, fewer side-effects and the possibility of using adaptable embospheres (150-1000 micron) which, on account of their marked plasticity, offer better occlusion of the vessels. For these reasons, superselective embolization of the bladder arteries should be considered as the treatment of choice in intractable bladder hemorrhage.
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