scholarly journals How to manage bleeding disorders in aging patients needing surgery

Hematology ◽  
2021 ◽  
Vol 2021 (1) ◽  
pp. 529-535
Author(s):  
Mouhamed Yazan Abou-Ismail ◽  
Nathan T. Connell

Abstract With improvements in medical care, the life expectancy of patients with bleeding disorders is approaching that of the general population. A growing population of older adult patients with bleeding disorders is at risk of age-related comorbidities and in need of various elective and emergent age-related procedures. The increased risk of thrombosis and volume overload in older adults complicates perioperative hemostatic management. Furthermore, antithrombotic treatment such as antiplatelet or anticoagulant therapy, which is frequently required for various cardiovascular interventions, requires a meticulous individualized approach. Evidence-based guidelines for the management of aging patients with bleeding disorders are lacking, largely due to the underrepresentation of older adult patients in clinical trials as well as the rarity of many such bleeding disorders. We discuss the current guidelines and recommendations in the perioperative hemostatic management of older adult patients with hemophilia and von Willebrand disease as well as other rare bleeding disorders. The optimal management of these patients is often complex and requires a thorough multidisciplinary and individualized approach involving hematologists, surgeons, anesthesiologists, and the specialists treating the underlying disorder.

Bone Reports ◽  
2021 ◽  
Vol 14 ◽  
pp. 100833
Author(s):  
Graziana Colaianni ◽  
Lorenzo Sanesi ◽  
Giuseppina Storlino ◽  
Roberta Zerlotin ◽  
Patrizia Pignataro ◽  
...  

Open Heart ◽  
2019 ◽  
Vol 6 (1) ◽  
pp. e001056
Author(s):  
Sebastian Udholm ◽  
Camilla Nyboe ◽  
Andrew Redington ◽  
Jens Erik Nielsen-Kudsk ◽  
Jens Cosedis Nielsen ◽  
...  

BackgroundIn recent Danish nationwide register-based study, adults with small, unrepaired atrial septal defects (ASD) have increased risk of pneumonia, atrial fibrillation (AF) and stroke. Moreover, they revealed higher mortality than the background population.ObjectiveIn this nationwide study, we evaluate the hidden burden of atrial and ventricular arrhythmias in adult patients with a small, unrepaired ASD without a previous diagnosis of AF.MethodsAll Danish patients, aged 18–65, diagnosed between 1953 and 2011 with an unrepaired ASD and no documented AF were invited for 7 days Holter-recording, echocardiography and 6 min walk test. The first 48 hours Holter-recording was completely analysed, while only AF was screened for throughout all 7 days. Furthermore, the entire patient group were characterised using the unique Danish registries.ResultsA total of 151 patients (mean age 32 years) were included. Approximately 80% of the patients had spontaneous closure of their defect. Despite this, occult arrhythmias were frequent. The most common arrhythmia was supraventricular tachycardia (n=24, 16%) with non-sustained atrial arrhythmias in 21 patients and AF in two patients. A considerable number of patients had non-sustained ventricular tachycardia (n=12, 8%). Patients with ASD and tachyarrhythmias had increased right ventricular to left ventricular diastolic area in echocardiography and higher age when compared with ASD patients without arrhythmias.ConclusionAdult patients with small, unrepaired ASD have a hidden burden of both atrial and ventricular tachyarrhythmias. The mechanism likely relates to the residua of previous right-heart volume overload and incomplete reverse remodelling. Our results support guidelines recommending continued follow-up of patients with small, unrepaired ASD.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii80-ii81
Author(s):  
Mary Jane Lim-Fat ◽  
Jayne Vogelzang ◽  
Eleanor Woodward ◽  
Alana McGovern ◽  
Clement Ma ◽  
...  

Abstract BACKGROUND Prognostic significance of IDH-mutation in glioma is incompletely understood in children and adolescents/young adults (YAs). We compared the clinico-genomic features, outcomes and prognostic factors observed in IDH-mutant gliomas across age groups. METHODS Clinical, histologic and molecular data of patients with IDH-mutant gliomas from 8 pediatric institutions (spanning twenty years) and adult patients from two institutions (from 2013–2019) were identified. Patients were grouped as pediatric (< 19y), YA (19y to < 40y) or older adult (≥ 40y). Genomic alterations, including somatic mutations and copy number variants, were captured with institutional next generation sequencing. Factors were compared across age categories using Fisher’s exact test or analysis-of-variance. Cox proportional-hazards regression tested factors for association with overall (OS) and progression-free survival (PFS). RESULTS Of 379 patients, 48(13%) were pediatric, 204(54%) YA and 127(33%) older adult. Histological subtype differed significantly by age group (p< 0.0001). YAs had higher rates of malignant transformation (p=0.01) and shorter time-from-diagnosis-to-malignant transformation (p=0.01) compared to other age groups. Analysis of genomic alterations revealed an age-related difference in distribution in ATRX mutations only (p=0.0018). Median PFS and OS for the entire cohort were 4.62 and 17.19 years. In univariate models, PFS differed by age group (p=0.0012), with YAs having the worst outcomes. Lack of MGMT methylation (p=0.024) predicted poorer OS. Upfront observant management was predictive of poorer PFS. Gene mutations were not associated with PFS. In multi-variable models, YAs had shorter PFS compared to pediatric (hazard ratio [HR]=2.03, p=0.01) and older adults (HR=1.59, p=0.003) after adjusting for histology, extent of resection, and initial therapy. Age at diagnosis was not associated with OS in multi-variable analysis. CONCLUSIONS Within our cohort, YA with IDH-mutant tumors progressed more quickly compared to their pediatric counterparts. Further study of YA patients with IDH-mutant glioma is critical to better define best practices for this group.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jakob Lederman ◽  
Veronica Lindström ◽  
Carina Elmqvist ◽  
Caroline Löfvenmark ◽  
Gunnar Ljunggren ◽  
...  

Abstract Background Older adults (age ≥ 65 years) represent a significant proportion of all patients who are not transported to hospital after assessment by ambulance clinicians (non-conveyed patients). This study aimed to fill the knowledge gap in the understanding of the prevalence of older adult non-conveyed patients and investigate their characteristics and risk factors for subsequent and adverse events with those of younger non-conveyed patients comparatively. Methods This population-based retrospective cohort study included all adult non-conveyed patients who availed the ambulance service of Region Stockholm, Sweden in 2015; they were age-stratified into two groups: 18–64 and ≥ 65 years. Inter-group differences in short-term outcomes (i.e. emergency department visits, hospitalisations, and mortality within 7 days following non-conveyance) were assessed using multivariate regression analyses. Results Older adult patients comprised 48% of the 17,809 non-conveyed patients. Dispatch priority levels were generally lower among older non-conveyed patients than among younger patients. Non-conveyance among older patients occurred more often during daytime, and they were more frequently assessed by ambulance clinicians with nonspecific presenting symptoms. Approximately one in five older adults was hospitalised within 7 days following non-conveyance. Patients presenting with infectious symptoms had the highest mortality risk following non-conveyance. Oxygen saturation level < 95% or systolic blood pressure > 160 mmHg had significantly higher associations with hospitalisation within 7 days following non-conveyance in older adult patients. Conclusions Older adult patients have an increased risk for adverse events following non-conveyance. In combination with a complex and variating presentation of symptoms and vital signs proved difficult for dispatch operators and ambulance clinicians to identify and assess, the identified risks raise questions on the patient safety of older adult non-conveyed patients. The results indicate a system failure that need to be managed within the ambulance service organisation to achieve higher levels of patient safety for older non-conveyed patients.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4657-4657
Author(s):  
Manuela Krause ◽  
Daniele Pillitteri ◽  
Ann-Kathrin Pilgrimm ◽  
Thomas Scholz ◽  
Rainer Schwerdtfeger ◽  
...  

Abstract Abstract 4657 Introduction: Pregnancy is a hypercoagulable state, and thromboembolism is the leading cause of antepartum and postpartum maternal mortality. Women with thrombophilic mutations (factor V leiden, prothrombin, and MTHFR) and inherited bleeding disorders, such as deficiency of factor XIII and fibrinogen, have been shown to be at increased risk of pregnancy loss. However, the risk of miscarriage in women with other inherited bleeding disorders has been discussed controversially. Due to the lack of data, it cannot be determined if the risk of miscarriage is increased in women with von Willebrand disease (vWD). The aim of our study was to clarify the association between inherited bleeding disorders and pregnancy loss. Patients and Methods: Subjects Concerning this investigation we included 91 female patients with two [n=46] or more [n=45] miscarriages occurring prior to 28 weeks of gestation and/or stillbirth without apparent reason. The median age of the examined group at the time of first fetal loss was 29 years, ranging from 17 to 41 years. Methods At first we compiled a detailed clinical history of bleedings of all patients. Subsequently, we performed various tests to gather information regarding coagulation abnormalities and thrombophilic defects. Therefore a molecular and functional assessment of the following data was performed: Coagulation factors, vWF:Ag, vWF:RCo, phospholipid antibodies, hyperhomocysteinaemia (HHCY), protein S (PS), protein C (PC), antithrombin (AT) and FV-Leiden mutation (G1691A), FII mutation (G20210A) and MTHFR C677T. Results: In our investigated population consisting of 91 women we registered 299 pregnancies of which 240 resulted in fetal loss, 232 prior to week 28 of pregnancy and 8 stillbirths. Seven out of 91 patients (8%) were carriers of inherited coagulation disorders; vWD: n=2 (2%), FVII deficiency: n=3 (3%), thrombocytopathy: n=2 (2%). In our study collective there was no increased rate of patients with vWD. None of the patients showed a FXIII- or fibrinogen deficiency. However, 17 patients (19%) have a bleeding diathesis. In 55 patients (60%) we could detect the following thrombophilic defects: FV-Leiden (G1691A): n=10, MTHFR C677T: n=42, PS: n=1, PC: n=1, APS: n=1. Conclusion: The incidence of vWD patients in our miscarriage collective is the same as the overall incidence of vWD patients in the general population. Therefore vWD is not associated with an increased risk of fetal loss. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2020 ◽  
Vol 136 (19) ◽  
pp. 2143-2150
Author(s):  
Frank W. G. Leebeek ◽  
Johannes Duvekot ◽  
Marieke J. H. A. Kruip

Abstract Women with inherited bleeding disorders, including carriers of hemophilia A and B, or with von Willebrand disease, have an increased risk of bleeding during pregnancy and delivery. The unborn child may also be affected by the bleeding disorder for which specific measures have to be considered. This requires a multidisciplinary approach, with a team that includes a hematologist, a pediatric hematologist, a clinical geneticist, an obstetrician-perinatologist, and an anesthesiologist. An optimal approach includes prepregnancy genetic counseling, prenatal diagnostic procedures, and a treatment plan for delivery for both the mother and child. Recent retrospective studies show that even if strict guidelines are followed, these women are still at risk of postpartum bleeding. This occurs even if coagulation factor levels are normalized, either due to the pregnancy-induced rise of factor levels or by infusion of coagulation factor concentrates at the time of delivery. In this article, we describe our current diagnostic and clinical management of pregnancy and delivery in women with inherited bleeding disorders. We also briefly discuss possible interventions to improve the outcome of current strategies by increasing target factor levels during and after delivery.


Author(s):  
Nidal Karim Al-Rahal

Background: Consanguineous marriage is defined as inbreeding between second cousins or closer. In such families there will be a potential increase in the autosomal recessive traits with its lethal effect, with an increased risk of morbidity and mortality in the new generation.  Inherited bleeding disorders (InBDs) are rare complicated diseases, difficult and expensive to treat, the defect usually due to quantitative or qualitative deficiency of clotting factors, platelets or fibrinolysis. This study attempts to assess the diversity, the frequency and the clinical features of inherited bleeding disorders (InBDs) in central part of Iraq and to determine the state of consanguineous marriage. Materials and Methods: This is a prospective cross-sectional study conducted in the National Center of Hematology NCH, Baghdad, Iraq between June2014 and June 2017. In total, 256 pediatrics and adult patients were included. Full bleeding history, family history, drug history and consanguineous marriage were recorded and followed by medical examination.  First-line laboratory tests were performed and then were followed by further tests included mixing study, lupus anticoagulant testing, clotting factor activity assay, von Willebrand Antigen (VW: Ag), Ristocetin co factor vWF: RiCoF activity and platelet function test. Results: The range of age was from 1 month to 57 years, with mean age 8.424±8.623 years and median age of 6.5years. The male to female ratio was 1.1:1. The most common age group was in the range of 1-10 years (46.45%). Family history was positive in 55.07% of patients (P >0.05). The consanguinity was found in 76.95% of the families studied (P <0.0001).  The most prevalent InBD was von Willebrand disease (42.98%) with majority type 3VWD (86.4%). The second most prevalent was thrombasthenia (36.71%) and the majority had Glanzmann’s thrombosthenia (86.2%). Rare bleeding disorders (RBDs) were observed in 6.25% of patients and the most common factor deficiency was FVII.  Conclusion: Consanguinity is high in patients with inherited bleeding disorders in Iraq, leading to emergence of life-threatening autosomal recessive inherited diseases. Genetic counselling is recommended besides education and awareness to minimize such rare illnesses in the community.


2020 ◽  
Author(s):  
Jakob Lederman ◽  
Veronica Lindström ◽  
Carina Elmqvist ◽  
Caroline Löfvenmark ◽  
Gunnar Ljunggren ◽  
...  

Abstract Background: Older adults (age ≥65 years) represent a significant proportion of all patients who are not transported to hospital after assessment by ambulance clinicians (non-conveyed patients). This study aimed to fill the knowledge gap in the understanding of the prevalence of older adult non-conveyed patients and investigate their characteristics and risk factors for subsequent and adverse events with those of younger non-conveyed patients comparatively.Methods: This population-based retrospective cohort study included all adult non-conveyed patients who availed the ambulance service of Region Stockholm, Sweden in 2015; they were age-stratified into two groups: 18–64 and ≥65 years. Inter-group differences in short-term outcomes (i.e. emergency department visits, hospitalisations, and mortality within 7 days following non-conveyance) were assessed using multivariate regression analyses.Results: Older adult patients comprised 48% of the 17,809 non-conveyed patients. Dispatch priority levels were generally lower among older non-conveyed patients than among younger patients. Non-conveyance among older patients occurred more often during daytime, and they were more frequently assessed by ambulance clinicians with nonspecific presenting symptoms. Approximately one in five older adults was hospitalised within 7 days following non-conveyance. Patients presenting with infectious symptoms had the highest mortality risk following non-conveyance. Oxygen saturation level <95% or systolic blood pressure >160 mmHg had significantly higher associations with hospitalisation within 7 days following non-conveyance in older adult patients.Conclusions: Older adult patients have an increased risk for adverse events following non-conveyance. In combination with a complex symptom presentation which proves difficult for dispatch operators and ambulance clinicians to identify, this risk raises questions on the patient safety of older adult non-conveyed patients.


2013 ◽  
Vol 14 (2) ◽  
pp. 78-83 ◽  
Author(s):  
Adam G. Golden ◽  
Judith Ortiz ◽  
Thomas T.H. Wan

Potentially avoidable hospitalizations are associated with high costs and an increased risk for iatrogenic conditions in older adult patients. Although care managers may be aware of the common potential pitfalls that may arise in the transfer of patients to and from the hospital, defining best practice models has been difficult. Many current models of geriatric care have had little or no impact on lowering the rates of hospitalizations and rehospitalizations when formally studied. Health care reform legislation mandates initiatives involving new models of coordinated or guided care such as the medical home model and the accountable care organization. These new models too will face significant challenges in their attempt to provide the financial incentives and systematic changes needed to successfully address transitional care in older adults.


1996 ◽  
Vol 1 (3) ◽  
pp. 166-179 ◽  
Author(s):  
Bo Molander ◽  
Lars Bäckman

Highly skilled miniature golf players were examined in a series of field and laboratory studies. The principal finding from these studies is that young and young adult players (range = 15-38 years) score equally well or better in competition than in training whereas older adult players (range = 46-73 years) perform worse in competitive events than under training conditions. It was also found that the impairment in motor performance on the part of the older players is associated with age-related deficits in basic cognitive abilities, such as memory and attention. These results support the hypothesis that older players may be able to compensate for age-related deficits under relaxed conditions, but not under conditions of high arousal. The possibility of improving the performance of the older players in stressful situations by means of various intervention programs is discussed.


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