scholarly journals 92 Patient with Lowe syndrome and fanconi tubulopathy undergoing surgery for scoliosis: anaesthetic implications and focus on a safe perioperative management of a platelet dysfunction

2021 ◽  
Author(s):  
M Chami ◽  
N Parisi
2017 ◽  
Vol 21 (4) ◽  
pp. 312-320 ◽  
Author(s):  
Patrick Odonkor ◽  
Archana Srinivas ◽  
Erik Strauss ◽  
Brittney Williams ◽  
Michael Mazzeffi ◽  
...  

Perioperative management of cardiovascular surgical procedures requiring cardiopulmonary bypass (CPB) in patients with hemophilia A poses a clinical challenge in coagulation management. Use of CPB requires the administration of an anticoagulant, usually unfractionated heparin, and also causes dilutional coagulopathy, platelet dysfunction or platelet consumption coagulopathy. Hypothermia and activation of the inflammatory cascade also affect coagulation. The effects of CPB on circulating levels of factor VIII have not been clearly defined. In this review, the effects of CPB and hemodilution on FVIII are shown in a case presentation, and perioperative laboratory testing in patients with hemophilia A having cardiac surgery is discussed along with perioperative and postoperative coagulation management.


2020 ◽  
Vol 5 (9) ◽  

Autosomal dominant macrothrombocytopenia with platelet dysfunction associated with reduction of surface αIIbβ3 (ADM) is an autosomal dominant form of Glanzmann’s thrombasthenia reported in few families in the literature. This case presents the first report of perioperative approach of a patient with ADM successfully submitted to a surgical procedure. The multidisciplinary approach including immunohaemotherapy consultation was considered the best clinical approach to avoid bleeding. The use of desmopressin and tranexamic acid in the preoperative period followed by desmopressin plus aminocaproic acid in the postoperative period showed to be safe and effective in the perioperative approach of the patient with ADM.


2009 ◽  
Vol 56 (1) ◽  
pp. 112
Author(s):  
Jin Yong Chung ◽  
Jae-hyoun Kwon ◽  
Kwi Chu Seo ◽  
Seok-Young Song ◽  
Woon Seok Roh ◽  
...  

Author(s):  
C. Eisenring ◽  
J. Sarnthein ◽  
D. Sabanés Bové ◽  
L. Held ◽  
N. Krayenbühl

1976 ◽  
Vol 36 (01) ◽  
pp. 200-207 ◽  
Author(s):  
Donald G. Corby ◽  
Thomas F. Zuck

SummaryPer cent aggregation, release and content of adenine nucleotides, and specific radioactivity were evaluated in citrated platelet-rich plasma (PRP) prepared from paired samples of maternal and cord blood. Platelets of newborn infants aggregated normally in response to high dose ADP (20 μM), strong collagen suspensions, and thrombin; however, when compared with PRP from the mothers or from normal adults, per cent aggregation in response to lower concentrations of ADP (2 μM), weak collagen, and part particularly epinephrine was markedly reduced. Nucleotide release after stimulation of the newborns’ PRP with the latter two inducers was also impaired. ATP and ADP content of the newborns’ platelets was also significantly less than that of their mothers or of normal adults, but specific activity was normal. The data suggest that the impairment of ADP release in the platelets of newborn infants is due to decreased sensitivity to external stimuli. Since metabolic ATP is necessary for the platelet release reaction, it is postulated that the platelet dysfunction results from a lack of metabolic ATP.


1982 ◽  
Vol 48 (01) ◽  
pp. 108-111 ◽  
Author(s):  
Elisabetta Dejana ◽  
Silvia Villa ◽  
Giovanni de Gaetano

SummaryThe tail bleeding time (BT) in rats definitely varies according to the method applied. Of the various variables that may influence BT, we have evaluated the position (horizontal or vertical) of the tail, the environment (air or saline), the temperature (4°, 23° or 37° C) and the type of anaesthesia. Transection of the tail tip cannot be used to screen drugs active on platelet function since it is sensitive to coagulation defects. Template BT in contrast is not modified by heparin and is sensitive to defects of platelet number and function (“storage pool disease”, dipyridamole-like drugs, exogenous prostacyclin). In contrast the test fails to detect aspirin-induced platelet dysfunction. The evidence reported indicates that thromboxane A2-prostacyclin balance is not a factor regulating BT. Aspirin treatment however may be a precipitating factor when associated with other abnormalities of platelet function.Template BT is a valid screening test for platelet disorders and for antiplatelet drugs.


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