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The kidney in sickle cell anemia

1975; Elsevier BV; Volume: 7; Issue: 6 Linguagem: Inglês

10.1038/ki.1975.54

1523-1755

George A.O. Alleyne, L.W. Statius van Eps, S Addae, George D. Nicholson, H. Schouten,

Autopsy Techniques and Outcomes

In 1910, Herrick described the first authentic case of sickle cell in a young student from Grenada in the West Indies [1]. He records that he was uncertain whether the blood picture represented merely a freakish poikilocytosis or is dependent on some peculiar physical or chemical condition of the blood or is characteristic of some particular disease. It is obvious that this was not a new disease and Konotey-Ahulu [2] notes that the bears many different tribal names in Africa, and was probably well recognized in that area for many centuries. In 1846, Lebby [3] had described the clinical case of a Negro who had bilious intermittent and remittent fevers and at autopsy no spleen could be found. This patient probably had sickle cell anemia and the spleen had become atrophic as a result of numerous infarcts. The prevalence of sickle cell anemia varies in different parts of the world. In Jamaica it is estimated that approximately 11% of the population carry the gene for S hemoglobin and the homozygous occurs in 0.2% of the people. In Curacao the comparable figures are 6.5% and 0.3%. The genetic basis of the was recognized by Emmel [4] and was firmly established by the study of Pauling et al [5]. It is now known that the abnormality of the sickle cell hemoglobin consists of the substitution of valine for glutamic acid in position 6 in the beta-chain. The fact that the kidney might be involved in sickle cell anemia was recognized very early, and in Herrick's original reports he mentioned the presence of urinary casts, leukocyturia, a slightly increased diuresis and urine of low specific gravity. In 1923 Sydenstricker, Mulherin and Houseal first described macroscopic and microscopic studies of the kidney on necropsy [6], and after a hiatus of 25 yr, reports of functional studies began to appear. The present review examines some of the older as well as the more recent information on the morphology of the kidney in sickle cell disease, the functional changes which occur, as well as the clinically significant syndromes with which the patient may present. In addition, some attempt is made to correlate the anatomic and functional changes.