REVIEW OF COMPLEMENT FUNCTION TESTING AND CORRELATION OF ABNORMAL RESULTS WITH PRIMARY AND SECONDARY COMPLEMENT DEFICIENCY

Author(s):  
Matthew Buckland
Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2068-2068
Author(s):  
Adam Bryant ◽  
Dawn Sheppard ◽  
Christopher Bredeson ◽  
Mitchell Sabloff

Abstract Abstract 2068 Introduction Current first-line therapy for acute myeloid leukemia (AML) involves a regimen of anthracycline-based induction chemotherapy. Cardiotoxicity is a rare but serious complication of such regimens and although it is a well-established phenomenon there is minimal evidence to inform the optimal frequency or nature of cardiac monitoring in these patients. The Ottawa Hospital measures baseline ejection fraction in all AML patients potentially eligible for induction chemotherapy. In this chart review we evaluated the effectiveness of this practice by weighing the clinically relevant information obtained from this testing against its costs and its potential to delay therapy. Methods Electronic charts of all patients admitted to the Ottawa Hospital with a primary diagnosis of AML between January 2009 and March 2012 were included for review. Charts were excluded only if initial AML management pre-dated 2009 or occurred at other institutions. We sought to: 1. Determine to what extent cardiac function testing delays chemotherapy administration and 2. Assess the utility of cardiac function testing by a) determining if pre-test risk factor profiles were predictive of abnormal cardiac function, and b) quantifying the instances in which cardiac function testing results altered management. To address these objectives data regarding timing from AML diagnosis to cardiac function testing and induction, individual cardiac risk factor profiles and cardiac function test results, and reasons for deciding against induction chemotherapy were extracted from all included electronic charts and analyzed. Results A total of 124 patient charts were included for review. Median patient age was 61 (range 18–97) and patents were more often male (62%). Approximately two thirds (65%) were induced. Patients had a median of two cardiac risk factors (mean 2.3 +/− 1.7), the most common of which were age over 60 (59%), smoking (52%), and obesity (36%). A majority of patients (92, 74%) had cardiac function testing performed, 43 (47%) of which were echocardiograms and 47 (53%) of which were multigated acquisition scans (MUGAs). For patients in whom AML was diagnosed on admission, a bone marrow aspirate was performed within a median of one day. It took a median two days to complete cardiac function testing. Induction proceeded within one day of completion of cardiac function testing in 40 patients (75%) and on the same day in 22 patients (42%). Time courses for patients who were diagnosed with AML prior to admission were shorter than those diagnosed after admission but reflected a similar pattern. Cardiac function tests were available for 92 patients and 4 abnormal results were documented. There were no instances in which results of cardiac function testing lead to changes in dosing or cancellation of planned induction therapy. Patients with abnormal baseline cardiac function tended to have more risk factors than those with normal cardiac function (3.3 +/− 1.3 vs 2.1 +/− 1.7) but this difference was not statistically significant (p = 0.217). Of note 41% of those with normal cardiac function had fewer than two risk factors, while none of those with abnormal cardiac faction met this cutoff. Discussion The clinical utility of baseline cardiac function testing in our population of AML patients is questionable. Of 124 patients, abnormal results were rare (3%) and in no instances did these results change management decisions. Cardiac risk factor profiles were not significantly predictive of abnormal cardiac function, but given the rarity of abnormal results in our population it is difficult to draw conclusions from this data. Cardiac function testing ultimately lead to a delay in induction by an estimated median of 2 days. Given the cost and resources used for cardiac function testing (estimated $200–300/test), the additional hospital days spent waiting for its completion (estimated $1,500/day), its potential to delay the administration of emergent induction chemotherapy, and its non-contribution to management decisions, this chart review puts into question the necessity of baseline cardiac function testing in newly diagnosed AML patients. These conclusions merit broader exploration in other centers and patient populations. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4726-4726
Author(s):  
Dharmesh Gopalakrishnan ◽  
Heesun J Rogers ◽  
Paul Elson ◽  
Keith R. McCrae

Abstract Introduction: Commonly used platelet function tests include Platelet Function Analyzer -100 Closure Time (PFA-100), used as a screening test, and platelet aggregation testing by Light Transmission Aggregometry (LTA), which can help differentiate between various types of platelet function defects and guide further testing. In this study, we assessed the indications for platelet aggregation testing at our center, and the effect of factors that may interfere with the outcomes of testing. Methods: This was a retrospective electronic medical records based study in which we included all patients in our healthcare network who had platelet aggregation testing done using LTA between August 2008 and August 2013. Routine descriptive measures were used for frequencies and proportions. McNemar test was used for comparison of paired nominal variables and Wilcoxon signed-rank test was used for comparison of paired continuous variables. P value < 0.05 was considered significant. Results: Four hundred ninety seven patients had platelet aggregation testing performed during the 5-yr study period. Among these, 315 (63%) patients had antecedent screening with PFA-100 with 157 (31%) showing at least one abnormality. Two hundred forty nine (50%) patients underwent some form of further testing after LTA, including VWF analysis (42%), platelet flow cytometry (19%) and/or electron microscopy (1%). Two hundred fifty six (51%) patients had at least one factor previously known or suspected to interfere with platelet aggregation testing (platelet count <100,000, hematocrit <25% or medications), and 205 (80%) of these patients had abnormal results in response to one or more agonists. Eighty three of these patients had repeat aggregation testing performed - 26 were re-tested after correction of 1 or more suspected factor (s), while 57 were re-tested without correction. The former group showed a significant improvement with subsequent testing, both in the average number of abnormalities in the LTA panel (P = 0.01) and in the number of patients with abnormal aggregation to ≥2 agonists (P = 0.02), while the outcomes of platelet function testing in the latter group did not significantly change on subsequent testing (P = 0.15 and 0.21 respectively for the above two parameters). Three hundred forty five (69%) patients had a current or past history of abnormal bleeding. Of these, 81% had history of spontaneous bleeding while 29% had surgical or procedure-related bleeding. The most common sites of spontaneous bleeding were skin (47%) and mucosa (45%). Eight percent of patients had a history of spontaneous bleeding in deeper sites. Fourteen percent had a family history of abnormal bleeding. The most common indication for testing was documented abnormal bleeding (62%) - recent or past. Twenty five percent underwent testing for peri-operative prediction of bleeding risk and 13% to monitor efficacy of anti-platelet therapy. Conclusions: A majority of the patients in our study did not have abnormalities in PFA-100 testing despite abnormal results on subsequent platelet aggregometry. Fifty one percent of patients who underwent aggregometry were on medications that may affect platelet function or had other factors known or suspected to interfere with aggregation testing. Eighty percent of these patients had abnormal results. Repeat testing after correction of the known factor(s) led to a significant improvements in the results of LTA. The most common indication for platelet aggregation testing was a current or past history of abnormal spontaneous bleeding and the most common sites of bleeding in these patients were the skin and mucosal surfaces. Disclosures McCrae: Momenta: Consultancy; Janssen: Membership on an entity's Board of Directors or advisory committees; Syntimmune: Consultancy; Halozyme: Membership on an entity's Board of Directors or advisory committees.


2015 ◽  
Vol 53 (05) ◽  
Author(s):  
K Gyorgyev ◽  
A Rudas ◽  
I Wagner ◽  
Á Altorjay ◽  
F Izbéki

Author(s):  
Mathias Poussel ◽  
Isabelle Thaon ◽  
Emmanuelle Penven ◽  
Angelica I. Tiotiu

Work-related asthma (WRA) is a very frequent condition in the occupational setting, and refers either to asthma induced (occupational asthma, OA) or worsened (work-exacerbated asthma, WEA) by exposure to allergens (or other sensitizing agents) or to irritant agents at work. Diagnosis of WRA is frequently missed and should take into account clinical features and objective evaluation of lung function. The aim of this overview on pulmonary function testing in the field of WRA is to summarize the different available tests that should be considered in order to accurately diagnose WRA. When WRA is suspected, initial assessment should be carried out with spirometry and bronchodilator responsiveness testing coupled with first-step bronchial provocation testing to assess non-specific bronchial hyper-responsiveness (NSBHR). Further investigations should then refer to specialists with specific functional respiratory tests aiming to consolidate WRA diagnosis and helping to differentiate OA from WEA. Serial peak expiratory flow (PEF) with calculation of the occupation asthma system (OASYS) score as well as serial NSBHR challenge during the working period compared to the off work period are highly informative in the management of WRA. Finally, specific inhalation challenge (SIC) is considered as the reference standard and represents the best way to confirm the specific cause of WRA. Overall, clinicians should be aware that all pulmonary function tests should be standardized in accordance with current guidelines.


2021 ◽  
Vol 11 (12) ◽  
pp. 5523
Author(s):  
Qian Ye ◽  
Minyan Lu

The main purpose of our provenance research for DSP (distributed stream processing) systems is to analyze abnormal results. Provenance for these systems is not nontrivial because of the ephemerality of stream data and instant data processing mode in modern DSP systems. Challenges include but are not limited to an optimization solution for avoiding excessive runtime overhead, reducing provenance-related data storage, and providing it in an easy-to-use fashion. Without any prior knowledge about which kinds of data may finally lead to the abnormal, we have to track all transformations in detail, which potentially causes hard system burden. This paper proposes s2p (Stream Process Provenance), which mainly consists of online provenance and offline provenance, to provide fine- and coarse-grained provenance in different precision. We base our design of s2p on the fact that, for a mature online DSP system, the abnormal results are rare, and the results that require a detailed analysis are even rarer. We also consider state transition in our provenance explanation. We implement s2p on Apache Flink named as s2p-flink and conduct three experiments to evaluate its scalability, efficiency, and overhead from end-to-end cost, throughput, and space overhead. Our evaluation shows that s2p-flink incurs a 13% to 32% cost overhead, 11% to 24% decline in throughput, and few additional space costs in the online provenance phase. Experiments also demonstrates the s2p-flink can scale well. A case study is presented to demonstrate the feasibility of the whole s2p solution.


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