Pathology of the Glomerulus in Sickle Cell Anemia with and Without Nephrotic Syndrome

Overview

Journal Am J Pathol

Publisher Elsevier

Specialty Pathology

Date 1974 Dec 1

PMID 4611224

Citations 15

Authors

I B Elfenbein

A PATCHEFSKY

W Schwartz

A G Weinstein

Affiliations

Soon will be listed here.

Abstract

Glomeruli from 6 cases of sickle cell disease (SS) with the nephrotic syndrome (NS) were compared histologically and quantitatively with glomeruli from 9 cases of SS, 10 cases of sickle cell trait (SCT), 4 cases of other hemoglobinopathies, all without NS, and normal controls. Five of 6 patients with SS and NS had extensive reduplication of their glomerular basement membranes and mild mesangial proliferation. Similar but milder lesions occurred in SS without NS but not in SCT or controls. Incidental renal disease occurred in 1 patient with SS and NS. Nephrotic syndrome was probably secondary to effects of sickle cell disease. Glomeruli in SS were significantly larger (>70%) than in SCT and controls. Mean total glomerular area per unit area of cortex in SS with normal BUN significantly exceeded that of SCT, which, in turn, was significantly greater than that of controls. Mechanisms for the histologic lesions and hypertrophy of the glomeruli were suggested.

Citing Articles

Sickle Cell Disease and CKD: An Update.

Zahr R, Saraf S Am J Nephrol. 2023; 55(1):56-71.

PMID: 37899028 PMC: 10872505. DOI: 10.1159/000534865.

Improvement of hemolytic anemia with GBT1118 is renoprotective in transgenic sickle mice.

Ren G, Setty S, Zhang X, Susma A, Ruiz M, Minshall R Blood Adv. 2022; 6(15):4403-4407.

PMID: 35759756 PMC: 9636314. DOI: 10.1182/bloodadvances.2022007809.

Hemodynamic and biological correlates of glomerular hyperfiltration in sickle cell patients before and under renin-angiotensin system blocker.

Haymann J, Hammoudi N, Livrozet M, Santin A, Mattioni S, Letavernier E Sci Rep. 2021; 11(1):11682.

PMID: 34083624 PMC: 8175337. DOI: 10.1038/s41598-021-91161-y.

Mechanisms of haemolysis-induced kidney injury.

Van Avondt K, Nur E, Zeerleder S Nat Rev Nephrol. 2019; 15(11):671-692.

PMID: 31455889 DOI: 10.1038/s41581-019-0181-0.

Receptor for Advanced Glycation End Products Antagonism Blunts Kidney Damage in Transgenic Townes Sickle Mice.

Charrin E, Faes C, Sotiaux A, Skinner S, Pialoux V, Joly P Front Physiol. 2019; 10:880.

PMID: 31396093 PMC: 6663971. DOI: 10.3389/fphys.2019.00880.

References

Mccoy R . Ultrastructural alterations in the kidney of patients with sickle cell disease and the nephrotic syndrome. Lab Invest. 1969; 21(2):85-95. View

PITCOCK J, MUIRHEAD E, HATCH F, Johnson J, Kelly B . Early renal changes in sickle cell anemia. Arch Pathol. 1970; 90(5):403-10. View

Vitsky B, Suzuki Y, Strauss L, Churg J . The hemolytic-uremic syndrome: a study of renal pathologic alterations. Am J Pathol. 1969; 57(3):627-47. PMC: 2013642. View

Morita T, WENZL J, McCoy J, Porch J, KIMMELSTIEL P . Bilateral renal hypoplasia with oligomeganephronia: quantitative and electron microsopic study. Am J Clin Pathol. 1973; 59(1):104-12. DOI: 10.1093/ajcp/59.1.104. View

Bernstein J, WHITTEN C . A histologic appraisal of the kidney in sickle cell anemia. Arch Pathol. 1960; 70:407-18. View

ETTELDORF J, Smith J, Tuttle A, DIGGS L . Renal hemodynamic studies in adults with sickle cell anemia. Am J Med. 1955; 18(2):243-8. DOI: 10.1016/0002-9343(55)90239-4. View

Walker B, Alexander F, Birdsall T, Warren R . Glomerular lesions in sickle cell nephropathy. JAMA. 1971; 215(3):437-40. View

Miller R, HARTLEY M, Clark E, LUPTON Jr C . SICKLE CELL NEPHROPATHY. Ala J Med Sci. 1964; 1:233-8. View

Vassalli P, Simon G, ROUILLER C . ELECTRON MICROSCOPIC STUDY OF GLOMERULAR LESIONS RESULTING FROM INTRAVASCULAR FIBRIN FORMATION. Am J Pathol. 1963; 43:579-617. PMC: 1949744. View

10.

NAEYE R . CHILDREN AT HIGH ALTITUDE: PULMONARY AND RENAL ABNORMALITIES. Circ Res. 1965; 16:33-8. DOI: 10.1161/01.res.16.1.33. View

11.

TELLEM M, RUBENSTONE A, FRUMIN A . Renal failure and other unusual manifestations in sickle-cell trait. AMA Arch Pathol. 1957; 63(5):508-12. View

12.

HATCH Jr F, Azar S, Ainsworth T, Nardo J, CULBERTSON J . Renal circulatory studies in young adults with sickle cell anemia. J Lab Clin Med. 1970; 76(4):632-40. View

13.

BARNETT H . Clinicopathological conference. Albert Einstein College of Medicine, Bronx, N. Y. J Pediatr. 1968; 73(6):936-41. DOI: 10.1016/s0022-3476(68)80254-9. View

14.

BERMAN L, TUBLIN I . The nephropathies of sickle-cell disease. AMA Arch Intern Med. 1959; 103(4):602-6. DOI: 10.1001/archinte.1959.00270040088011. View

15.

BERMAN L, SCHREINER G . Clinical and histologic spectrum of the nephrotic syndrome. Am J Med. 1958; 24(2):249-67. DOI: 10.1016/0002-9343(58)90314-0. View

16.

Arakawa M, KIMMELSTIEL P . Circumferential mesangial interposition. Lab Invest. 1969; 21(3):276-84. View

17.

ETTELDORF J, TUTTLE A, Clayton G . Renal function studies in pediatrics. 1. Renal hemodynamics in children with sickle cell anemia. AMA Am J Dis Child. 1952; 83(2):185-91. DOI: 10.1001/archpedi.1952.02040060051005. View

18.

Susmano S, Lewy J . Sickle cell disease and acute glomerulonephritis. Am J Dis Child. 1969; 118(4):615-8. DOI: 10.1001/archpedi.1969.02100040617015. View

19.

Hutchins G, Kutchemeshgi A . Renal glomerular enlargement in chronic passive congestion. Johns Hopkins Med J. 1973; 132(5):292-300. View

20.

BAUER W, ROSENBERG B . A quantitative study of glomerular enlargement in children with tetralogy of Fallot. A condition of glomerular enlargement without an increase in renal mass. Am J Pathol. 1960; 37:695-712. PMC: 1942281. View