Inhibit conversion of angiotensin I to angiotensin II but also inhibition of the breakdown of bradykinin Bradykinin is produced as the blood is exposed to the extracorporeal surface With build-up of bradykinin, may have anaphylaxis: vasodilatation, hypotension, flushing, bradycardia Generally occurs within the first few minutes of the procedure; resolves when procedure stopped Don’t return blood in line Withold ACE-I 24-48 hours prior to pheresis
Absorption of mestinon occurs in 30-60min, peaks in 2 hrs and declines after 4 hours; usually dosed every 4-6 hrs
If pheresis precipitates cholinergic crisis, be prepared to suction patient, evaluate for need to intubate, once stabilized, tensilon test can differentiate between MC and CC. Tensilon will relieve MC but will have little effect or worsen a CC
von Willebrand factor (vWf) is synthesized in endothelial cells and assembled in larger multimers that are present in normal plasma. The larger multimers, called unusually large von Willebrand factor (ULvWf), are rapidly degraded in the circulation into the normal size range of vWf multimers by a specific von Willebrand factor-cleaving protease (or cleaving metalloproteinase) Nature: October 2001 Thrombotic thrombocytopenic purpura (TTP) is a life-threatening systemic illness of abrupt onset and unknown cause. Proteolysis of the blood-clotting protein von Willebrand factor (VWF) observed in normal plasma is decreased in TTP patients. However, the identity of the responsible protease and its role in the pathophysiology of TTP remain unknown. We performed genome-wide linkage analysis in four pedigrees of humans with congenital TTP and mapped the responsible genetic locus to chromosome 9q34. A predicted gene in the identifed interval corresponds to a segment of a much larger transcript, identifying a new member of the ADAMTS family of zinc metalloproteinase genes (ADAMTS13). Analysis of patients' genomic DNA identified 12 mutations in the ADAMTS13 gene, accounting for 14 of the 15 disease alleles studied. We show that deficiency of ADAMTS13 is the molecular mechanism responsible for TTP, and suggest that physiologic proteolysis of VWF and/or other ADAMTS13 substrates is required for normal vascular homeostasis.
TBV: infant-child 100-75ml/kg teen 70-75ml/kg adult 65-80ml/kg Example: RBC volume in a 36kg child with Hct 22% TBV=36kg x 80ml/kg=2880ml RBCV=TBV x Hct = 2880ml x .22 = 633.6ml Unit of leukopoor RBC with 300cc and Hct 55% has RBCV of 165cc so for this child, 3.8 units of PRBCs would be needed for exchange
TBV: infant-child 100-75ml/kg teen 70-75ml/kg adult 65-80ml/kg Example: RBC volume in a 36kg child with Hct 22% TBV=36kg x 80ml/kg=2880ml RBCV=TBV x Hct = 2880ml x .22 = 633.6ml Unit of leukopoor RBC with 300cc and Hct 55% has RBCV of 165cc so for this child, 3.8 units of PRBCs would be needed for exchange