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      Renal hemosiderosis secondary to intravascular hemolysis after mitral valve repair : A case report

      case-report

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          Abstract

          Rationale:

          Renal hemosiderosis is a disease in which hemosiderin deposits in the renal cortex as a form of iron overload. However, cases of renal hemosiderosis due to intravascular hemolysis following mitral valve repair have been rarely reported.

          Patient concerns:

          We present the case of a 62-year-old woman who developed asymptomatic urinary abnormalities including microscopic hematuria and proteinuria due to renal hemosiderosis following a mitral valve repair surgery performed two years earlier.

          Diagnoses:

          A percutaneous renal biopsy showed no specific glomerular abnormality, tubular atrophy, or interstitial fibrosis but extensive deposition of hemosiderin in the proximal tubule. The patient was diagnosed with renal hemosiderosis and chronic intravascular hemolysis following mitral valve repair.

          Interventions:

          Our patient refused a mitral valve repeat surgery and hence was treated with oral iron preparations, N-acetylcysteine, and a β-receptor blocker.

          Outcomes:

          Moderate mitral regurgitation with the regurgitant blood striking against the annuloplasty ring was confirmed on follow-up echocardiography. After the 24-month follow-up period, hemolytic anemia persisted, but there was no significant decline of renal function.

          Lessons:

          For cases of chronic intravascular hemolysis accompanied with asymptomatic urinary abnormalities, a renal biopsy is required to exclude underlying kidney pathology and predict potential renal insufficiency.

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          Most cited references18

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          The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease.

          The efficient sequestration of hemoglobin by the red blood cell membrane and the presence of multiple hemoglobin clearance mechanisms suggest a critical need to prevent the buildup of this molecule in the plasma. A growing list of clinical manifestations attributed to hemoglobin release in a variety of acquired and iatrogenic hemolytic disorders suggests that hemolysis and hemoglobinemia should be considered as a novel mechanism of human disease. Pertinent scientific literature databases and references were searched through October 2004 using terms that encompassed various aspects of hemolysis, hemoglobin preparations, clinical symptoms associated with plasma hemoglobin, nitric oxide in hemolysis, anemia, pulmonary hypertension, paroxysmal nocturnal hemoglobinuria, and sickle-cell disease. Hemoglobin is released into the plasma from the erythrocyte during intravascular hemolysis in hereditary, acquired, and iatrogenic hemolytic conditions. When the capacity of protective hemoglobin-scavenging mechanisms has been saturated, levels of cell-free hemoglobin increase in the plasma, resulting in the consumption of nitric oxide and clinical sequelae. Nitric oxide plays a major role in vascular homeostasis and has been shown to be a critical regulator of basal and stress-mediated smooth muscle relaxation and vasomotor tone, endothelial adhesion molecule expression, and platelet activation and aggregation. Thus, clinical consequences of excessive cell-free plasma hemoglobin levels during intravascular hemolysis or the administration of hemoglobin preparations include dystonias involving the gastrointestinal, cardiovascular, pulmonary, and urogenital systems, as well as clotting disorders. Many of the clinical sequelae of intravascular hemolysis in a prototypic hemolytic disease, paroxysmal nocturnal hemoglobinuria, are readily explained by hemoglobin-mediated nitric oxide scavenging. A growing body of evidence supports the existence of a novel mechanism of human disease, namely, hemolysis-associated smooth muscle dystonia, vasculopathy, and endothelial dysfunction.
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            A comparison of outcomes of mitral valve repair for degenerative disease with posterior, anterior, and bileaflet prolapse.

            We sought to compare the clinical and echocardiographic outcomes of mitral valve repair for mitral regurgitation in patients with degenerative disease of the mitral valve with posterior, anterior, or bileaflet prolapse. Patients underwent operations from 1981 through 2001: 359 had posterior (mean age, 60.4 years), 92 had anterior (mean age, 53.3 years), and 250 had bileaflet (means age, 56.4 years) prolapse. Patients with anterior prolapse were younger (P = .04) and had more associated aortic valve disease (P = .02), particularly bicuspid aortic valve disease (P < .001). Anterior prolapse was corrected by using chordal replacement with Gore-Tex sutures in most patients, but early on in this series, leaflet resection, chordal shortening, and chordal transfer were also used. Echocardiograms were done annually, and clinical follow-up was complete at a mean of 6.9 +/- 4.0 years (range, 0-23 years). The overall survival at 12 years was 75% +/- 5%, with no difference among the posterior, anterior, and bileaflet prolapse groups (P = .3). The freedom from reoperation at 12 years was 96% +/- 2% for posterior, 88% +/- 4% for anterior, and 94% +/- 2% for bileaflet prolapse (P = .019). Anterior prolapse was the only independent predictor of reoperation. The freedom from moderate or severe mitral regurgitation at 12 years was 80% +/- 4% for posterior, 65% +/- 8% for anterior, and 67% +/- 6% for bileaflet prolapse (P = .001). Anterior and bileaflet prolapse, age, ejection fraction of less than 40%, and aortic valve disease were independent predictors of recurrent moderate or severe mitral regurgitation. The pathophysiology of mitral regurgitation affects the durability of mitral valve repair for degenerative disease, and the results of posterior prolapse are better than those of anterior and bileaflet prolapse. This study indicates that rates of reoperation underscore the rates of failure of mitral valve repair.
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              Hemolysis and acute kidney failure.

              Deposits of iron and hemosiderosis in the kidney have been observed in diseases with intravascular hemolysis, including paroxysmal nocturnal hemoglobinuria, and valvular heart diseases and prosthetic heart valve implants. However, the decrease in kidney function associated with hemolysis caused by cardiac valvular disease or prostheses is less well recognized. We present a case of intravascular hemolysis after repair and banding of the mitral valve that resulted in massive renal tubular deposition of hemosiderin with decreased kidney function. We discuss the pathophysiologic process of both acute and chronic tubular injury from heme and heme proteins, including injury to organelles resulting in autophagic vacuoles containing damaged organelles, such as mitochondria. We conclude that tubular injury resulting from heme proteins should be considered as a cause of decreased kidney function in all patients with a cardiac valvular disease or prosthesis. Copyright © 2010 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                17 January 2020
                January 2020
                : 99
                : 3
                : e18798
                Affiliations
                [a ]Department of Internal Medicine
                [b ]Department of Pathology, Daegu Catholic University School of Medicine, Daegu
                [c ]Department of Pathology, Dongguk University College of Medicine, Gyeongju
                [d ]Department of Internal Medicine, Hansung Union Internal Medicine Clinic and Dialysis Center, Daegu, Republic of Korea.
                Author notes
                []Correspondence: In Hee Lee, Department of Internal Medicine, Daegu Catholic University School of Medicine, 17-Gil 33, Duryugongwon-ro, Nam-gu, Daegu 42472, Republic of Korea (e-mail: ihlee@ 123456cu.ac.kr ).
                Article
                MD-D-19-03747 18798
                10.1097/MD.0000000000018798
                7220361
                32011483
                779bc3d1-c449-4ddc-b5e0-ed0e3844f34f
                Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0

                History
                : 15 May 2019
                : 18 November 2019
                : 17 December 2019
                Categories
                5200
                Research Article
                Clinical Case Report
                Custom metadata
                TRUE

                asymptomatic urinary abnormality,intravascular hemolysis,mitral valve repair,renal hemosiderosis

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