SILVANA VIGANO-D'ANGELO, M.D.; ARMANDO D'ANGELO, M.D.; CHRISTIAN E. KAUFMAN Jr., M.D.; CHRIS SHOLER, M.D.; CHARLES T. ESMON, Ph.D.; PHILIP C. COMP, M.D., Ph.D.
Protein S activity may be compromised in patients with the nephrotic syndrome and contribute to a thrombotic diathesis. Protein S is found in two forms in plasma as free and functionally active protein S, and complexed to C4b-binding protein. When compared with controls, patients with nephrotic syndrome had reduced functional levels of protein S (69% ± 27% [SD], p < 0.001) despite having elevated levels of total protein S antigen (139% ± 42%, p < 0.001). Decreased protein S activity was caused by significant reductions in free (active) protein S levels (90% ± 38%, p < 0.05) due to the selective urinary loss of free protein S and elevation of C4b-binding protein levels (170% ± 52%, p < 0.001) that favors complex formation; and in the specific activity of the circulating free protein S (0.76; p < 0.001). Along with this reduction in specific activity, we noted the abnormal electrophoretic mobility of the protein S in the presence of calcium ions. We conclude that acquired protein S deficiency occurs in the nephrotic syndrome and may be a risk factor for the development of the thromboembolic complications.
VIGANO-D'ANGELO S, D'ANGELO A, KAUFMAN CE, SHOLER C, ESMON CT, COMP PC. Protein S Deficiency Occurs in the Nephrotic Syndrome. Ann Intern Med. ;107:42–47. doi: 10.7326/0003-4819-107-1-42
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Published: Ann Intern Med. 1987;107(1):42-47.
Coagulopathies, Hematology/Oncology, Nephrology, Nephrotic Syndrome.
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