Mechanisms of Acquired Defects of the Stabilization of Fibrin

Abstract

Summary. The causes of undemonstrable plasma fibrin‐stabilizing‐factor (FSF, factor XIII) activity in various diseases have been investigated. Results suggest impaired synthesis of the enzyme in three patients with portal cirrhosis, and one with acute hepatic necrosis. Impaired activation of factor XIII seems a possible explanation in one patient with portal cirrhosis. Evidence of a plasma inhibitor of factor XIII was obtained in six patients with chronic renal failure and two with acute hepatic necrosis. The inhibitor present in renal failure was non‐dialysable, relatively heat labile and not adsorbed by either aluminium hydroxide or bentonite.

Plasma Factor XIII Levels in Children with Renal Disease

“…This is in accord with previous observations [7][8][9], The lowered factor XIII levels were also found in our patients with rapidly progressive glome rulonephritis. In chronic renal failure, the presence of a uremic inhibitor of factor XIII which was reported by Losowsky and Walls [13] could explain the reduction of plasma factor XIII activity, the mechanism of these findings in the others is still obscure, however. Several explanations including consumption, destruction and diminished synthesis are probable [8,13,14].…”

“…In chronic renal failure, the presence of a uremic inhibitor of factor XIII which was reported by Losowsky and Walls [13] could explain the reduction of plasma factor XIII activity, the mechanism of these findings in the others is still obscure, however. Several explanations including consumption, destruction and diminished synthesis are probable [8,13,14]. The most attractive one to us is a hypothesis that it results from enhanced fibrin formation in the kidney.…”

Factor XIII Deficiency in Adult Polycystic Kidney Disease

“…In adult polycystic kidney disease, we found significantly (p< 0.001) decreased fac- tor XIII levels (method of Sigg), but factor XIII concen trations were normal (data not shown). In 5/11 patients, we could confirm factor XIII inhibitors [6,7], This special inhibitor persisted in 1 patient after bilateral nephrec tomy (due to severe bleeding). The inhibitor present in renal failure was non-dialysable.…”