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Therapy of Renal Disorders in Sickle Hemoglobinemia

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Therapy of Renal Diseases and Related Disorders

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Abstract

Sickle hemoglobinemia, a term which refers to the presence of sickle hemoglobin (Hb-S) in either the heterozygous or homozygous form, has been associated with numerous and widely varying forms of disordered renal function, the majority of which are ultimately a consequence of the sickling process. In the presence of hypoxia, acidosis, or hyperosmolality (which causes red cells to shrink, thereby increasing intracellular hemoglobin concentration), the rate of gelation and tactoid formation increases and red cells become sickled (1–5). When this morphologic change occurs in the capillary bed there is an increase in blood viscosity. Resistance to blood flow is thereby increased, passage of red cells through capillaries is further delayed, and more deoxygenation and sickling ensue. This process, which has been described as ‘a vicious cycle of erythrostasis’(6), can eventually lead to ischemia and infarction of tissue. In the relatively hypoxic, acidic, and hyperosmolar environment of the renal medulla, such a process will eventually produce obliteration of the vasa recta, focal scarring, medullary interstitial fibrosis, papillary necrosis, and tubular atrophy which are the pathologic hallmarks of sickle-cell nephropathy (7–11). It is this widespread pathologic disruption of renal medullary anatomic integrity that accounts for most of the renal disorders that afflict patients with sickle hemoglobinemia.

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  1. Robert M. Rosa
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Editors and Affiliations

  1. The Bernard J. Hanley, Los Angeles, CA, 90033, USA

    Wadi N. Suki M.D. (Professor of Medicine Professor of Physiology and Biophysics Chief) (Professor of Medicine Professor of Physiology and Biophysics Chief)

  2. Division of Nephrology, University of Southern California School of Medicine, Los Angeles, CA, 90033, USA

    Wadi N. Suki M.D. (Professor of Medicine Professor of Physiology and Biophysics Chief) (Professor of Medicine Professor of Physiology and Biophysics Chief)

  3. Renal Section Baylor College of Medicine, The Methodist Hospital, Houston, TX, 77030, USA

    Shaul G. Massry M.D. (Professor of Medicine and Physiology Chief) (Professor of Medicine and Physiology Chief)

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© 1984 Martinus Nijhoff Publishers, Boston / The Hague / Dordrecht / Lancaster

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Rosa, R.M. (1984). Therapy of Renal Disorders in Sickle Hemoglobinemia. In: Suki, W.N., Massry, S.G. (eds) Therapy of Renal Diseases and Related Disorders. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-3807-9_29

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  • DOI: https://doi.org/10.1007/978-1-4613-3807-9_29

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