Hong Kong Journal of Nephrology

Volume 6 Number 1, 2004
Abstract

The Kidney in Sickle Cell Disease: Pathophysiology and Clinical Review

Ibrahiem Saeed Abdulrahman

Sickle cell disease is a major health problem in many countries. Sickled erythrocytes in the renal medullary vessels are the hallmark of the disease, and are associated with a variety of renal complications. Renal ischemia, microinfarcts and papillary necrosis occur, and renal tubular abnormalities follow. Glomerular disease with proteinuria may be observed. Renal failure, both acute and chronic, is the expected outcome of the disease. Hematuria, electrolyte and acid base disturbances are common throughout the course of the disease. End-stage renal disease as a complication of aggressive sickle cell disease should be treated with dialysis or kidney transplantation; both are effective. There is a tendency towards better survival in sickle cell nephropathy patients undergoing transplantation than in those treated with hemodialysis alone. Whether bone marrow transplantation in the early stage of the disease can halt the progression of sickle cell nephropathy is unknown and awaits clinical studies.

Key words: sickle cell, tubular, glomerular disease, papillary necrosis, renal failure, ESRD

鐮狀細胞疾病乃不少國家的共同且重要問題,其特徵為腎髓血管出現鐮刀狀的紅血球,並與多種腎臟的併發症有關,包括腎缺血、微梗塞、乳突壞死、及隨之而來的腎小管功能異常等。腎衰竭(急性或慢性)是鐮狀細胞疾病的可預期後果,同時亦可能會出現蛋白尿及腎小球病變。病人常有血尿、及電解質/酸鹼失衡等表現。透析療法或腎臟移植,均可有效治療鐮狀細胞疾病所致的末期腎病,唯腎臟移植的存活效益可能較佳。至於早期施行的骨髓移植,是否能阻止鐮狀細胞性腎臟病變的惡化,則仍有待臨床研究的證實。


Back to Previous Page