scholarly journals Vaccination against COVID‐19: Rationale, modalities and precautions for patients with haemophilia and other inherited bleeding disorders

Haemophilia ◽  
2021 ◽  
Author(s):  
Radoslaw Kaczmarek ◽  
Magdy El Ekiaby ◽  
Daniel P. Hart ◽  
Cedric Hermans ◽  
Mike Makris ◽  
...  
Keyword(s):  
1964 ◽  
Vol 11 (02) ◽  
pp. 506-512 ◽  
Author(s):  
V. A Lovric ◽  
J Margolis

SummaryAn adaptation of “kaolin clotting time” and prothrombin time for use on haemolysed capillary blood provided simple and sensitive screening tests suitable for use in infants and children. A survey of three year’s experience shows that these are reliable routine laboratory tests for detection of latent coagulation disorders.


Author(s):  
Jared A. Warren ◽  
John P. McLaughlin ◽  
Robert M. Molloy ◽  
Carlos A. Higuera ◽  
Jonathan L. Schaffer ◽  
...  

AbstractBoth advances in perioperative blood management, anesthesia, and surgical technique have improved transfusion rates following primary total knee arthroplasty (TKA), and have driven substantial change in preoperative blood ordering protocols. Therefore, blood management in TKA has seen substantial changes with the implementation of preoperative screening, patient optimization, and intra- and postoperative advances. Thus, the purpose of this study was to examine changes in blood management in primary TKA, a nationwide sample, to assess gaps and opportunities. The American College of Surgeons National Surgical Quality Improvement Program database was used to identify TKA (n = 337,160) cases from 2011 to 2018. The following variables examined, such as preoperative hematocrit (HCT), anemia (HCT <35.5% for females and <38.5% for males), platelet count, thrombocytopenia (platelet count < 150,000/µL), international normalized ration (INR), INR > 2.0, bleeding disorders, preoperative, and postoperative transfusions. Analysis of variances were used to examine changes in continuous variables, and Chi-squared tests were used for categorical variables. There was a substantial decrease in postoperative transfusions from high of 18.3% in 2011 to a low of 1.0% in 2018, (p < 0.001), as well as in preoperative anemia from a high of 13.3% in 2011 to a low of 9.5% in 2016 to 2017 (p < 0.001). There were statistically significant, but clinically irrelevant changes in the other variables examined. There was a HCT high of 41.2 in 2016 and a low of 40.4 in 2011 to 2012 (p < 0.001). There was platelet count high of 247,400 in 2018 and a low of 242,700 in 201 (p < 0.001). There was a high incidence of thrombocytopenia of 5.2% in 2017 and a low of low of 4.4% in 2018 (p < 0.001). There was a high INR of 1.037 in 2011 and a low of 1.021 in 2013 (p < 0.001). There was a high incidence of INR >2.0 of 1.0% in 2012 to 2015 and a low of 0.8% in 2016 to 2018 (p = 0.027). There was a high incidence of bleeding disorders of 2.9% in 2013 and a low of 1.8% in 2017 to 2018 (p < 0.001). There was a high incidence of preoperative transfusions of 0.1% in 2011 to 2014 and a low of <0.1% in 2015 to 2018 (p = 0.021). From 2011 to 2018, there has been substantial decreases in patients receiving postoperative transfusions after primary TKA. Similarly, although a decrease in patients with anemia was seen, there remains 1 out 10 patients with preoperative anemia, highlighting the opportunity to further improve and address this potentially modifiable risk factor before surgery. These findings may reflect changes during TKA patient selection, optimization, or management, and emphasizes the need to further advance multimodal approaches for perioperative blood management of TKA patients. This is a Level III study.


2020 ◽  
Author(s):  
Elisabeth Kahre ◽  
Tina Adelhardt ◽  
Claudia Nitze ◽  
Ursula Range ◽  
Sylvia von Mackensen ◽  
...  

Author(s):  
Antonio Girolami ◽  
Diana Noemi Garcia de Paoletti ◽  
Marcelo Leonardo Nenkies ◽  
Silvia Ferrari ◽  
Hugo Guglielmone

Background: Investigation of rare bleeding disorders in Latin-America. Objective: The report of a new case of FX deficiency due to a compound heterozygosis. Methods: Accepted clotting procedures were used. Sequencing of DNA was carried out by means of Applied Biosystems Instruments. Results: A compound heterozygote due to the association of a new mutation (Gla72Asp) with an already known mutation (Gly154Arg) of the FX gene is reported. The proposita is a 38 year old female who had a moderate bleeding tendency (menorrhagia, epistaxis, easy bruising). The proposita has never received substitution therapy but in the occasion of a uterine biopsy. The mother was asymptomatic but was a heterozygote for the new mutation. The father was asymptomatic but had deserted the family and could not be investigated. After this abandonment the mother of the proposita re-married with an asymptomatic man and she gave birth to a son who was asymptomatic but was also heterozygous for the new mutation (Gla72Asp). As a consequence it has to be assumed that the first husband of the mother of the proposita was heterozygous for the known mutation (Gly154Arg). Conclusion: This is the third case of a new mutation in the FX gene reported, during the past few years, in Argentina.


Author(s):  
Mrinalini Kotru ◽  
Deepti Mutereja ◽  
Abhishek Purohit ◽  
Seema Tyagi ◽  
Manoranjan Mahapatra ◽  
...  

Abstract. Background: Bleeding is a common clinical presentation. Even patients with mild bleeding disorders are extensively investigated for ascertaining the cause. The present study was conducted in order to evaluate the extent of the possibility of diagnosis in mild bleeding disorders.Material and Methods: This was a prospective study of patients referred for work up of mild bleeding for a period of 13 months. A complete blood count, peripheral smear examination, Prothrombin time, Partial Thromboplastin time and Thrombin Time, Platelet Aggregometry test, tests for von Willebrand’s disease and Platelet function 3 availability were measured. Results: 164 patients presented with mild bleeding, in 114 of the  patients a single site of bleeding was present. Epistaxis was the most common presentation (39%). Cutaneous bleeding (petechiae and purpura) was the next common site. History of a major bleeding tendency in the family was present only in 11 patients. The investigations showed that VWD (17/164), followed by clotting disorders (CD) mainly mild hemophilia (15/164) were the most common diagnosable cause. There were also 4 cases of hypofibrinogenemia. The disorders of platelets (Platelet function defects/PFD) were the least common (9/164). Rest 123 (75%) patients could not be diagnosed on the basis of these investigations and were labeled as  Bleeding disorders – Unclassified (BDC). Conclusion: n our study, 75% of the patients with mild bleeding remained undiagnosed even after extensive laboratory workup, thus raising a very pertinent question that is it necessary that all mild bleeders submit to a broad battery of investigations, as the diagnosis continues to be elusive despite extensive workup.


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