hemoglobinuria/atrofiere

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Total hemiatrophy. Association with localized scleroderma, Schönlein-Henoch nephritis, and paroxysmal nocturnal hemoglobinuria.

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In the clinical course of a patient with progressive facial hemiatrophy associated with ipsilateral body atrophy (total hemiatrophy), signs and symptoms of localized scleroderma were noted. The patient subsequently was found to have Schönlein-Henoch purpura with renal involvement and, later,

Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH).

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Focal choroidal excavation (FCE) is an unusual configuration characterized by focal thinning and pitting of the choroid in the absence of staphyloma or scleral ectasia. The etiology and clinical implications of FCE are currently unknown. We report a case of bilateral multiple FCEs in a patient with

[Acute renal failure in a patient with myelodysplastic syndrome and paroxysmal nocturnal hemoglobinuria phenotype].

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Hemoglobin and myoglobin heme pigments and iron have acute and chronic nephrotoxic effects, which are often associated with massive hemolysis and rhabdomyolysis. We report a patient with a myelodysplastic syndrome and paroxysmal nocturnal hemoglobinuria phenotype who developed an acute renal failure

Dural sinus thrombosis in paroxysmal nocturnal hemoglobinuria.

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Dural sinus thrombosis developed in a young man with paroxysmal nocturnal hemoglobinuria (PNH). His neurological disorder developed in the face of anemia and a urinary tract infection. Progressive neurological deterioration occurred in spite of anticoagulation, antibiotic and steroid therapy. The

Urologic manifestations of paroxysmal nocturnal hemoglobinuria.

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A long-term study of 17 patients with paroxysmal nocturnal hemoglobinuria revealed an unexpectedly high incidence of functional and anatomic renal abormalities. All patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. All patients also had granular casts

Eculizumab in paroxysmal nocturnal hemoglobinuria with Budd-Chiari syndrome progressing despite anticoagulation.

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Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, life-threatening disorder characterized by chronic intravascular hemolysis caused by uncontrolled complement activation. Hepatic vein thrombosis (Budd-Chiari syndrome) is common in PNH patients. This case report describes the response to

Recurrent acute renal failure in a patient with aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome: a case report.

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Acute renal failure (ARF) is one of the renal expressions in patients with aplastic anemia (AA)-paroxysmal nocturnal hemoglobinuria (PNH) syndrome following hemolytic crisis. We report the case of an AA patient who experienced recurrent episodes of ARF, in association with evidence of PNH. A

Complement Inhibition Therapy and Dialytic Strategies in Paroxysmal Nocturnal Hemoglobinuria: The Nephrologist's Opinion.

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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease that presents an estimated incidence of 1.3 cases per million per year, with a prevalence of 15.9 cases per million. It is characterized by hemolysis, bone marrow dysfunction with peripheral blood cytopenia, hypercoagulability,

Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients.

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This study investigated for the first time brain ischemic involvement in 19 consecutive neurologically asymptomatic PNH patients by non-enhanced cerebral MRI, and by intracranial arterial and venous angio-MRI. Eleven cases (58%, 7 aged <65) showed pathological findings: 9 white matter (WM)

Complement research in the 18th-21st centuries: Progress comes with new technology.

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The complement system has been studied for about 120 years. Progress in defining this large and complex system has been dependent on the research technologies available, but since the introduction of protein chromatography, electrophoresis, and antibody-based assay methods in the 1950s and 60s, and

Low fractional excretion of sodium. Occurrence with hemoglobinuric- and myoglobinuric-induced acute renal failure.

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Ten patients with myoglobinuric and hemoglobinuric acute renal failure demonstrated low fractional excretion of sodium (FENa) values (less than 1%) during the oliguric phase of their course. Acute renal failure secondary to hemoglobinuria developed in five patients, and five demonstrated acute

[Reoperation for malfunction of Ionescu-Shiley bovine pericardial bioprosthetic valves in the mitral position--special consideration with emergency operation].

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Sixty-eight patients have had mitral valve replacement with Ionescu-Shiley bovine pericardial bioprosthesis from 1981 to 1984. Thirty five patients were required reoperation due to primary tissue failure of the bioprosthesis, 10 (28.6%) were on emergency basis and 25 (71.4%) were on elective.

[Systemic pathologic physiology parameters changes in sheep drowning: a control study in freshwater and seawater].

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OBJECTIVE To compare the systemic pathologic physiology parameter changes in sheep drowning in freshwater and seawater. METHODS The experimental animals were healthy crossbred sheep. According to the envelope method, 24 sheep were randomly divided into two groups, with 12 animals in each group. The

Buried Hydrogen Bond Interactions Contribute to the High Potency of Complement Factor D Inhibitors.

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Aberrant activation of the complement system is associated with diseases, including paroxysmal nocturnal hemoglobinuria and age-related macular degeneration. Complement factor D is the rate-limiting enzyme for activating the alternative pathway in the complement system. Recent development led to a

Histopathology of acute toxic responses in selected tissues from rats exposed by inhalation to methyl bromide.

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To determine and characterize the histological changes induced in selected tissues from the Fischer 344 rat by acute inhalation exposure to methyl bromide (MeBr), groups of 10 male rats (11-13 weeks old) were exposed to 0, 90, 175, 250, or 325 ppm MeBr 6 hr/day for 5 days. Animals were anesthetized

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