scholarly journals The ISTH bleeding assessment tool as predictor of bleeding events in inherited platelet disorders: Communication from the ISTH SSC Subcommittee on Platelet Physiology

2021 ◽  
Vol 19 (5) ◽  
pp. 1364-1371
Author(s):  
Paolo Gresele ◽  
Emanuela Falcinelli ◽  
Loredana Bury ◽  
Alessandro Pecci ◽  
Marie‐Christine Alessi ◽  
...  
2019 ◽  
Vol 18 (3) ◽  
pp. 732-739 ◽  
Author(s):  
Paolo Gresele ◽  
Sara Orsini ◽  
Patrizia Noris ◽  
Emanuela Falcinelli ◽  
Marie Christine Alessi ◽  
...  

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1061-1061 ◽  
Author(s):  
Juliana Perez Botero ◽  
Deepti M. Warad ◽  
Rong He ◽  
Rajiv K. Pruthi ◽  
Dong Chen

Introduction Patients with inherited and acquired platelet disorders (PD) frequently present with thrombocytopenia and/or mucocutaneous bleeding. Diagnosis of PDs relies on both clinical and laboratory investigation, which encompasses both routine and esoteric platelet testing. The former includes platelet indices, light transmission aggregation (LTA) and platelet function analyzer (PFA-100). The latter includes platelet glycoprotein (GP) assessment by flow cytometry and platelet transmission electron microscopy (PTEM). Several recent studies have demonstrated limited sensitivities of routine platelet testing. It is uncertain if a combination of both routine and esoteric testing can vastly improve the sensitivity. Our goal is to assess the value of platelet esoteric testing and the ultimate sensitivity of laboratory platelet testing in diagnosing PDs. Methods Patients (between year 2012 and 2015) with a known or suspected inherited or acquired platelet disorders were recruited for platelet routine and esoteric testing. Patients' clinical features including ISTH bleeding assessment tool (BAT) scores, platelet count, LTA, PFA-100 ADP (CADP) and epinephrine (CEPI) cartridge closure times, GP expression by flow cytometry and PTEM were performed. All clinical and laboratory variables were collected and statistically analyzed. Results A total of 69 patients (51 females; median age 40 years; range: 8 months to 76 years) were enrolled to this cohort study. By retrospective chart review, 34 patients (49%) had a positive ISTH-BAT score (range 0 to 16). The most common bleeding manifestations (on any degree of severity) were cutaneous bleeding present in 53% of patients (n=37), menorrhagia in 47% of females (n=24), epistaxis in 36% (n=25) and postoperative bleeding in 35% (n=24). Joint, muscle and CNS hemorrhage were the least frequent with 7%, 6% and 1% of patients reporting bleeding in these sites. A total of 22 patients (32%) had thrombocytopenia at the time of their evaluation. Fifty-eight patients (84%) had platelet aggregation studies and of those 15 (26%) had an abnormal study. PFA-100 was performed in 57 patients (83%) of whom 42% (n=24) had prolonged closure times with either ADP or epinephrine. PTEM was performed on all samples. Twenty-seven patients (39%) had abnormal platelet structure including 17 patients who had dense granule deficiency. GP by flow cytometry was performed in 41 patients (59%) and abnormal glycoprotein expression was detected in 6 patients (15%). Of the 55 patients who had all tests performed, 22 patients (40%) had completely normal results. Of the routine tests, only PFA-CEPI or -CADP prolongation is associated with the likelihood of a PTEM abnormality (P<0.005). With the assistance of molecular testing, there were 2 cases of MYH-9 mutation-related platelet disorders, one of RUNX1 mutation-associated thrombocytopenia, one of York platelet syndrome, two of Bernard Soulier variants, one of Quebec syndrome and three of gray platelet syndrome or variants. Bleeding scores showed no statistically significant association with severity of any laboratory abnormalities. Conclusion In this cohort, the sensitivity of routine platelet testing in clinically suspected PDs is about 30-40%. Addition of platelet esoteric testing improves the sensitivity to 60%. However, the degree of platelet laboratory abnormalities do not predict severity of bleeding. The findings of this cohort underscore the importance of clinical assessment and both platelet routine and esoteric testing in investigation of patients with suspected PDs. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3728-3728
Author(s):  
Janaki Iyer ◽  
Matthew Badin ◽  
Lucas Graf ◽  
Georges E. Rivard ◽  
A. D. Paterson ◽  
...  

Abstract Platelet function disorders represent a heterogeneous group of bleeding disorders with diverse molecular causes that are frequently associated with platelet dense granule deficiency and/or impaired aggregation responses. With the exception of Quebec platelet disorder (QPD), bleeding risks for common platelet disorders have not been estimated. This led us to study a Canadian cohort with uncharacterized platelet function disorders and confirmed abnormalities in validated assays for platelet dense granule deficiency and/or light transmission aggregometry (reduced aggregation with ≥2 agonists). Subjects were assessed using: (i) the International Society for Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT) to determine scores and categorize symptom severity, and (ii) CHAT-P, a clinical history assessment tool for assessment of platelet disorders that included questions about general health and bleeding symptoms/problems and questions previously used to assess bleeding risks for QPD. CHAT-P was completed by subjects (or parent in the case of young children) before review by a hematologist. Participants included: 29 individuals with confirmed platelet function disorders of unknown cause (from 7 families, 10 "sporadic" cases), 12 unaffected relatives and 60 general population controls. A one-way ANOVA was used to compare the overall ISTH-BAT scores between the affected individuals, unaffected relatives and healthy controls. Bleeding risks were estimated as odds ratio (OR) with 95% confidence intervals (CI) using CHAT-P data for general population controls as the comparison group. The total number of affected subjects reporting a bleeding problem/symptom from the group of affected individuals was added up and compared with the corresponding numbers of responses for general population controls and unaffected relatives using ANOVA. Summative bleeding scores for CHAT-P items with OR>1 were used to compare the number and range of abnormal bleeding symptoms experienced by subjects. Individuals with confirmed platelet abnormalities had higher ISTH-BAT scores (median: 9, range: 0-18) than unaffected family members (median: 0, range: 0-1) and general population controls (median: 0, range: 0-6) (p < 0.01), and their most severe symptom scores were for: epistaxis, cutaneous bleeding, menorrhagia, bleeding from dental extractions, surgery and a subdural hematoma at birth. Affected individuals had higher risks for bleeding (OR, 95% CI, p value) including: bleeding from minor cuts/wounds lasting >1 hour (56, 3.1-1000, p<0.01); abnormal bruising (15-65, 1.8-140 to 3.7-1200, p<0.01); prolonged nosebleeds (23, 5.9-92, p<0.01) and nosebleeds requiring medical attention (40, 1.5-520, p<0.01), packing (33, 1.8-620, p<0.01) or cautery (27, 1.5-510, p<0.01); wound healing problems (13, 3.4-53, p<0.01); excessive bleeding from injuries/trauma (9.5, 1-87, p=0.03), oral/dental challenges (44, 5.3-370, p<0.01) and surgery (17, 4.1-68, p<0.01). Affected females reported: bleeding interfering with their sex life (6.5, 1.1-38, p=0.04); menses >7 days (11, 2.5-49, p<0.01); flooding/gushing accidents (3.8, 1.2-12, p=0.04 ) and/or clots with menses (13, 2.6-63, p<0.01); menses requiring treatment (7.8, 2.1-29, p<0.01); and excessive bleeding during childbirth (17, 2.7-105, p<0.01), sometimes requiring surgical intervention (41, 2-810, p<0.01). Affected individuals reported more of these bleeding symptoms (median: 15, range: 0-24) than unaffected family members (median: 2, range: 0-6; p<0.01) and general population controls (median: 1, range: 0-14, p<0.01), although there was overlap. Our study illustrates that uncharacterized platelet function disorders are associated with significantly increased bleeding risks and mild rather than severe bleeding problems. It will be important to translate our study findings for patients and healthcare providers to promote evidence-based care of individuals with confirmed dense granule deficiency and/or impaired aggregation responses, which are common amongst individuals tested for bleeding problems. Disclosures No relevant conflicts of interest to declare.


Healthcare ◽  
2021 ◽  
Vol 9 (2) ◽  
pp. 148
Author(s):  
Crischentian Brinza ◽  
Alexandru Burlacu ◽  
Grigore Tinica ◽  
Adrian Covic ◽  
Liviu Macovei

Dual antiplatelet therapy (DAT) is recommended for all patients undergoing percutaneous coronary intervention (PCI), as it significantly reduces the ischemic risk at the cost of increasing the incidence of bleeding events. Several clinical predictive models were developed to better stratify the bleeding risk associated with DAT. This systematic review aims to perform a literature survey of both standard and emerging bleeding risk scores and report their performance on predicting hemorrhagic events, especially in the era of second-generation drug-eluting stents and more potent P2Y12 inhibitors. We searched PubMed, ScienceDirect, and Cochrane databases for full-text studies that developed or validated bleeding risk scores in adult patients undergoing PCI with subsequent DAT. The risk of bias for each study was assessed using the prediction model risk of bias assessment tool (PROBAST). Eighteen studies were included in the present systematic review. Bleeding risk scores showed a modest to good discriminatory power with c-statistic ranging from 0.49 (95% CI, 0.45–0.53) to 0.82 (95% CI, 0.80–0.85). Clinical models that predict in-hospital bleeding events had a relatively good predictive performance, with c-statistic ranging from 0.70 (95% CI, 0.67–0.72) to 0.80 (95% CI, 0.73–0.87), depending on the risk scores and major hemorrhagic event definition used. The knowledge and utilization of the current bleeding risk scores in appropriate clinical contexts could improve the prediction of bleeding events.


2020 ◽  
Vol 63 (4) ◽  
pp. 1071-1082
Author(s):  
Theresa Schölderle ◽  
Elisabet Haas ◽  
Wolfram Ziegler

Purpose The aim of this study was to collect auditory-perceptual data on established symptom categories of dysarthria from typically developing children between 3 and 9 years of age, for the purpose of creating age norms for dysarthria assessment. Method One hundred forty-four typically developing children (3;0–9;11 [years;months], 72 girls and 72 boys) participated. We used a computer-based game specifically designed for this study to elicit sentence repetitions and spontaneous speech samples. Speech recordings were analyzed using the auditory-perceptual criteria of the Bogenhausen Dysarthria Scales, a standardized German assessment tool for dysarthria in adults. The Bogenhausen Dysarthria Scales (scales and features) cover clinically relevant dimensions of speech and allow for an evaluation of well-established symptom categories of dysarthria. Results The typically developing children exhibited a number of speech characteristics overlapping with established symptom categories of dysarthria (e.g., breathy voice, frequent inspirations, reduced articulatory precision, decreased articulation rate). Substantial progress was observed between 3 and 9 years of age, but with different developmental trajectories across different dimensions. In several areas (e.g., respiration, voice quality), 9-year-olds still presented with salient developmental speech characteristics, while in other dimensions (e.g., prosodic modulation), features typically associated with dysarthria occurred only exceptionally, even in the 3-year-olds. Conclusions The acquisition of speech motor functions is a prolonged process not yet completed with 9 years. Various developmental influences (e.g., anatomic–physiological changes) shape children's speech specifically. Our findings are a first step toward establishing auditory-perceptual norms for dysarthria in children of kindergarten and elementary school age. Supplemental Material https://doi.org/10.23641/asha.12133380


2020 ◽  
Vol 29 (4) ◽  
pp. 1944-1955 ◽  
Author(s):  
Maria Schwarz ◽  
Elizabeth C. Ward ◽  
Petrea Cornwell ◽  
Anne Coccetti ◽  
Pamela D'Netto ◽  
...  

Purpose The purpose of this study was to examine (a) the agreement between allied health assistants (AHAs) and speech-language pathologists (SLPs) when completing dysphagia screening for low-risk referrals and at-risk patients under a delegation model and (b) the operational impact of this delegation model. Method All AHAs worked in the adult acute inpatient settings across three hospitals and completed training and competency evaluation prior to conducting independent screening. Screening (pass/fail) was based on results from pre-screening exclusionary questions in combination with a water swallow test and the Eating Assessment Tool. To examine the agreement of AHAs' decision making with SLPs, AHAs ( n = 7) and SLPs ( n = 8) conducted an independent, simultaneous dysphagia screening on 51 adult inpatients classified as low-risk/at-risk referrals. To examine operational impact, AHAs independently completed screening on 48 low-risk/at-risk patients, with subsequent clinical swallow evaluation conducted by an SLP with patients who failed screening. Results Exact agreement between AHAs and SLPs on overall pass/fail screening criteria for the first 51 patients was 100%. Exact agreement for the two tools was 100% for the Eating Assessment Tool and 96% for the water swallow test. In the operational impact phase ( n = 48), 58% of patients failed AHA screening, with only 10% false positives on subjective SLP assessment and nil identified false negatives. Conclusion AHAs demonstrated the ability to reliably conduct dysphagia screening on a cohort of low-risk patients, with a low rate of false negatives. Data support high level of agreement and positive operational impact of using trained AHAs to perform dysphagia screening in low-risk patients.


Author(s):  
Matthew L. Hall ◽  
Stephanie De Anda

Purpose The purposes of this study were (a) to introduce “language access profiles” as a viable alternative construct to “communication mode” for describing experience with language input during early childhood for deaf and hard-of-hearing (DHH) children; (b) to describe the development of a new tool for measuring DHH children's language access profiles during infancy and toddlerhood; and (c) to evaluate the novelty, reliability, and validity of this tool. Method We adapted an existing retrospective parent report measure of early language experience (the Language Exposure Assessment Tool) to make it suitable for use with DHH populations. We administered the adapted instrument (DHH Language Exposure Assessment Tool [D-LEAT]) to the caregivers of 105 DHH children aged 12 years and younger. To measure convergent validity, we also administered another novel instrument: the Language Access Profile Tool. To measure test–retest reliability, half of the participants were interviewed again after 1 month. We identified groups of children with similar language access profiles by using hierarchical cluster analysis. Results The D-LEAT revealed DHH children's diverse experiences with access to language during infancy and toddlerhood. Cluster analysis groupings were markedly different from those derived from more traditional grouping rules (e.g., communication modes). Test–retest reliability was good, especially for the same-interviewer condition. Content, convergent, and face validity were strong. Conclusions To optimize DHH children's developmental potential, stakeholders who work at the individual and population levels would benefit from replacing communication mode with language access profiles. The D-LEAT is the first tool that aims to measure this novel construct. Despite limitations that future work aims to address, the present results demonstrate that the D-LEAT represents progress over the status quo.


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