scholarly journals Practolol therapy associated with a systemic lupus erythematosus-like syndrome and an inhibitor to factor XIII.

1977 ◽  
Vol 30 (8) ◽  
pp. 770-773 ◽  
Author(s):  
G R Milner ◽  
P J Holt ◽  
J Bottomley ◽  
J E Maciver
2002 ◽  
Vol 88 (12) ◽  
pp. 919-923 ◽  
Author(s):  
Pauline Velasco ◽  
John Hill ◽  
Karen Hoffmeister ◽  
Fredric Kaye ◽  
Laszlo Lorand

SummaryIntracranial hemorrhage in a young woman with systemic lupus erythematosus necessitated two surgical evacuations. In the absence of a family history of bleeding, clot solubility in urea suggested a factor XIII (FXIII) inhibitor. The patient’s IgG bound well to the virgin and the thrombin-modified zymogen ensemble (A2B2 and A2’B2) and to the free rA2 but reacted poorly with the thrombin-modified rA2’. Since the IgG did not block the thrombin-catalyzed proteolysis of A subunits nor the dissociation of the A2’B2, its action might be to interfere with the release of activation peptides from the thrombincleaved zymogen, hindering the conformational change necessary for generating FXIIIa.Treatment with cryoprecipitate and cyclophosphamide arrested the hemorrhage and almost neutralized the antibody so that the patient’s clot became insoluble in urea and showed a close to normally cross-linked γ-γ and αn fibrin chain profile. Nevertheless, she still has detectable anti-FXIII antibody and may be at risk for hemorrhage.


Transfusion ◽  
2017 ◽  
Vol 57 (9) ◽  
pp. 2159-2163 ◽  
Author(s):  
Cara A. Rabik ◽  
Meredith A. Atkinson ◽  
Sangeeta Sule ◽  
John J. Strouse

Author(s):  
Francis R. Comerford ◽  
Alan S. Cohen

Mice of the inbred NZB strain develop a spontaneous disease characterized by autoimmune hemolytic anemia, positive lupus erythematosus cell tests and antinuclear antibodies and nephritis. This disease is analogous to human systemic lupus erythematosus. In ultrastructural studies of the glomerular lesion in NZB mice, intraglomerular dense deposits in mesangial, subepithelial and subendothelial locations were described. In common with the findings in many examples of human and experimental nephritis, including many cases of human lupus nephritis, these deposits were amorphous or slightly granular in appearance with no definable substructure.We have recently observed structured deposits in the glomeruli of NZB mice. They were uncommon and were found in older animals with severe glomerular lesions by morphologic criteria. They were seen most commonly as extracellular elements in subendothelial and mesangial regions. The deposits ranged up to 3 microns in greatest dimension and were often adjacent to deposits of lipid-like round particles of 30 to 250 millimicrons in diameter and with amorphous dense deposits.


2000 ◽  
Vol 6 (7) ◽  
pp. 821-825 ◽  
Author(s):  
ELIZABETH LERITZ ◽  
JASON BRANDT ◽  
MELISSA MINOR ◽  
FRANCES REIS-JENSEN ◽  
MICHELLE PETRI

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