scholarly journals Incidence, Natural History and Outcomes of Transient and Persistent Antiphospholipid Antibodies in Children and Young Adults with Provoked Venous Thromboembolism: Analysis of the Kids-DOTT Trial

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 636-636
Author(s):  
Marisol Betensky ◽  
Anh Thy Nguyen ◽  
Cristina Tarango ◽  
Anupam R Verma ◽  
Rukhmi Bhat ◽  
...  
Keyword(s):  
Young Adults ◽  
Natural History ◽  
Antiphospholipid Antibodies ◽  
Anticoagulation Therapy ◽  
Total Duration ◽  
First Episode ◽  
Published Data ◽  
Increased Risk ◽  
Children And Young Adults ◽  
Recurrent Vte

Background: Antiphospholipid syndrome (APS) is characterized by the development of thromboembolic events in the setting of persistent antiphospholipid antibodies (APA). While several studies have investigated the clinical characteristics and venous thromboembolic (VTE) outcomes of pediatric patients with a diagnosis of APS, there is a scarcity of published data describing the incidence, natural history and VTE outcomes of children who present with positive APA at the time of an acute VTE episode. Objective: To describe the incidence, natural history and outcomes of transient and persistent APA in patients ≤21 years old with a first episode of provoked VTE. Methods: Patients with positive APA at the time of enrollment in an ongoing NHLBI-sponsored multicenter, parallel-cohort randomized controlled trial (RCT) on duration of anticoagulation therapy in provoked VTE (the Kids-DOTT Trial; NCT00687882) were included in this analysis. APA testing was obtained at the time of enrollment in all patients, and at 6 weeks post-VTE diagnosis in those with an initial positive APA result. Subsequent follow-up testing in patients whose APA persisted at 6 weeks post-VTE was performed at the discretion of the enrolling site's treating hematologist. Patients with persistent APA at 6 weeks were treated in a non-randomized parallel-cohort of the trial in which patients received at least a 3-month therapeutic course of anticoagulation. Those without persistent APA at 6 weeks were retained in the RCT and randomized to shortened-duration (no further therapy) versus conventional duration (total duration = 3 months) of therapy. Measured APA included lupus anticoagulant-sensitive activated partial thromboplastin time (LAS-aPTT), dilute Russell viper venom time (DRVVT), hexagonal phase phospholipid assay (STACLOT LA), anti-cardiolipin (ACL) IgM, and anti-beta-2-glycoprotein-1 (aß2GPI) IgG and IgM. Data were prospectively collected. Descriptive statistics were used to summarize data on demographic characteristics, VTE presentation, and outcomes of interest (persistent thrombus occlusion at 6 weeks post-VTE, post-thrombotic syndrome [PTS], recurrent VTE and clinically-relevant bleeding [CRB]). The blind was maintained in the Kids-DOTT trial throughout data transfer and analysis. Results: APA testing was performed in 476 patients at the time of enrollment. Twenty-one percent of patients (n=100) had at least one positive APA and were included in the present analysis. Demographics, VTE characteristics, and laboratory findings at enrollment are summarized in Table 1. Median age was 9.6 years (range 0.05-19). The most common thrombosis site was the lower extremity (43%). Presence of a central venous catheter was the provoking factor in 44% of cases. LA was the most common APA detected at the time of enrollment (36%, 35% and 13% of patients by STACLOT, DRVVT and LAS-aPTT, respectively). Twenty-four percent of patients had a persistent APA at 6 weeks post-VTE (Figure 1). Of these, 16 (66.7%) had repeat APA testing at least 12 weeks from first positive APA. Persistent APA at ≥12 weeks (i.e., APS) was determined in 12 of the 16 patients. Most of these children with APS had a single positive APA (aß2GPI IgG/IgM, n=4, LA, n=4, ACL IgM, n=2) while only 1 patient had triple-positive APA (Table 2). Rates of recurrent VTE, complete venous occlusion at 6 weeks post-VTE, development of PTS, and CRB were higher in APS patients as compared to those without APS (Table 3). Conclusions: This Kids-DOTT analysis reports for the first time that positive APA are found in nearly one quarter of children and young adults with provoked VTE at the time of the acute VTE episode, and that approximately 75% of these are transient (negative at 6 weeks post-VTE diagnosis). Patients with provoked VTE who have APS tended to have increased risk of adverse VTE outcomes when compared to those without APS; however, the size of these subgroups does not permit a definitively-powered comparison in outcomes. The putative role of persistently positive APAs as a prognostic factor for adverse VTE outcomes should be formally tested upon completion of the Kids-DOTT trial. Disclosures Tarango: Shire: Membership on an entity's Board of Directors or advisory committees; Bayer: Other: Study steering committee. Goldenberg:NIH: Other: research support and salary support.

scholarly journals Antiphospholipid antibodies and venous thromboembolism

Blood ◽  
1995 ◽  
Vol 86 (10) ◽  
pp. 3685-3691 ◽  
Author(s):  
JS Ginsberg ◽  
PS Wells ◽  
P Brill-Edwards ◽  
D Donovan ◽  
K Moffatt ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Anticoagulant Therapy ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Odds Ratio ◽  
Warfarin Therapy ◽  
Strong Association ◽  
First Episode ◽  
Increased Risk ◽  
Recurrent Vte

The clinical relevance of antiphospholipid antibodies (APLA) in patients without systemic lupus erythematosus who have venous thromboembolism (VTE) in unknown. Limited evidence suggests that there is an association between the presence of APLA and both initial and recurrent episodes of VTE and that patients with APLA and VTE are resistant to warfarin therapy. Unselected patients with a first episode of clinically suspected deep vein thrombosis or pulmonary embolism were evaluated with objective tests for VTE and with laboratory tests for APLA; the latter included tests for the lupus anticoagulant (LA) and anticardiolipin antibodies (ACLA). Patients with VTE were treated with anticoagulant therapy and observed during and after discontinuation of anticoagulants for symptomatic recurrence of VTE. There was a strong association between LA and VTE (odds ratio, 9.4; 95% confidence interval [CI], 2.1 to 46.2) and 9 to 65 (14%; 95% CI, 7% to 25%) patients with VTE had LA. There was no association between the presence of ACLA and VTE (odds ratio, 0.7; 95%CI, 0.3 to 1.7) because of the high frequency of positive ACLA assays in patients without VTE. None of the 16 patients with VTE and APLA developed recurrent VTE while receiving warfarin therapy. There was no difference in rates of recurrent VTE in patients with or without APLA after anticoagulant therapy was discontinued. The strong association between LA and VTE suggests that testing for LA in patients with VTE is useful. The measurement of ACLA in patients with VTE has no clinical usefulness because the results are abnormal in a high proportion of patients without VTE. Although the presence of APLA in patients with VTE was not associated with resistance to a conventional intensity of warfarin or an increased risk of recurrent VTE after discontinuation of warfarin, a larger study should address these issues in a subgroup of patients with VTE and LA.

scholarly journals Coeliac disease is associated with depression in children and young adults with type 1 diabetes: results from a multicentre diabetes registry

2021 ◽  
Author(s):  
Sascha René Tittel ◽  
◽  
Désirée Dunstheimer ◽  
Dörte Hilgard ◽  
Burkhild Knauth ◽  
...  
Keyword(s):  
Type 1 Diabetes ◽  
Young Adults ◽  
Coeliac Disease ◽  
Diabetes Patient ◽  
Routine Care ◽  
Diabetes Registry ◽  
Increased Risk ◽  
Treatment Data ◽  
Children And Young Adults

Abstract Aims To analyse the association between coeliac disease (CD) and depression in children, adolescents, and young adults with type 1 diabetes (T1D). Methods We included 79,067 T1D patients aged 6–20 years, with at least six months of diabetes duration, and treatment data between 1995 and 2019 were documented in the diabetes patient follow-up registry. We categorized patients into four groups: T1D only (n = 73,699), T1 + CD (n = 3379), T1D + depression (n = 1877), or T1D + CD + depression (n = 112). Results CD and depression were significantly associated (adjusted OR: 1.25 [1.03–1.53]). Females were more frequent in both the depression and the CD group compared with the T1D only group. Insulin pumps were used more frequently in T1D + CD and T1D + depression compared with T1D only (both p < .001). HbA1c was higher in T1D + depression (9.0% [8.9–9.0]), T1D + CD + depression (8.9% [8.6–9.2]), both compared with T1D only (8.2% [8.2–8.2], all p < .001). We found comorbid autism, attention deficit hyperactivity disorder, anxiety, schizophrenia, and eating disorders more frequently in the T1D + CD + depression group compared with T1D only (all p < .001). Conclusions CD and depression are associated in young T1D patients. The double load of T1D and CD may lead to an increased risk for depression. Depression was associated with additional psychological and neurological comorbidities. Aside from imperative CD screening after T1D diagnosis and regular intervals, depression screening might be helpful in routine care, especially in patients with diagnosed CD.

The Ozaki Procedure With CardioCel Patch for Children and Young Adults With Aortic Valve Disease: Preliminary Experience – a Word of Caution

2019 ◽  
Vol 10 (6) ◽  
pp. 724-730 ◽  
Author(s):  
Sian C. Chivers ◽  
Carine Pavy ◽  
Ricky Vaja ◽  
Cesare Quarto ◽  
Olivier Ghez ◽  
...  
Keyword(s):  
Young Adults ◽  
Aortic Valve ◽  
Adult Population ◽  
Published Data ◽  
Bovine Pericardial Patch ◽  
Valve Position ◽  
Children And Young Adults ◽  
Ischemic Attack

Background: The Ozaki procedure is a surgical technique for patients with significant aortic stenosis or regurgitation or both where valve repair cannot be performed. Individual cusps are cut from glutaraldehyde-treated autologous pericardium or bovine pericardium and implanted into the aortic valve position. Encouraging results have been reported within the adult population. There are limited published data on success of this procedure in younger patients. Methods: We present a series of five children and young adults who underwent the Ozaki procedure with neoaortic valve cusps made from CardioCel, a decellularized bovine pericardial patch treated with a monomeric glutaraldehyde. Results: There were no complications in the initial postoperative period and short inpatient stay. At a mean follow-up of 29.6 months (range: 22-36 months), 4 patients had no evidence of stenosis and 3 patients had trivial or no regurgitation from the neoaortic valve. Overall, two patients had complications related to the valve and underwent reintervention during the follow-up period with a Ross procedure. One of these patients who was not taking long-term anticoagulation experienced a transient ischemic attack. Conclusions: Our experience demonstrates that the Ozaki procedure with CardioCel in pediatric and young adult patients should be approached with caution. Further research with larger groups of pediatric patients, comparison of different graft materials, and longer follow-up is required to ascertain long-term success in children.

"ProC Global": A functional screening test that predicts recurrent venous thromboembolism

2005 ◽  
Vol 93 (03) ◽  
pp. 600-604 ◽  
Author(s):  
Shannon Bates ◽  
Marilyn Johnston ◽  
Simon McRae ◽  
Jeffrey Ginsberg ◽  
Anne Grand’Maison
Keyword(s):  
Venous Thromboembolism ◽  
Protein C ◽  
Specific Inhibitor ◽  
Factor V Leiden ◽  
First Episode ◽  
Functional Screening ◽  
Factor V ◽  
Increased Risk ◽  
Global Result ◽  
Recurrent Vte

SummaryAbnormalities of the Protein C (PC) pathway are found in the majority of patients with thrombophilia. ProC Global is a coagulation assay that reflects the net effect of the PC pathway by measuring the activated partial thromboplastin time (APTT) of patient and control plasma, before and after activation of endogenous PC by Protac, a snake venom. Previous studies have suggested that abnormalities in this test are associated with an increased risk of venous thromboembolism (VTE). A retrospective analysis was performed using frozen plasma samples from 140 patients with confirmed VTE to determine whether an abnormal ProC Global result (in the presence and in the absence of known abnormalities in the PC pathway) is a predictor of initial and recurrent VTE. Patients were tested for the presence of activated protein C resistance, Factor V Leiden, PC and protein S (PS) deficiency, and non-specific inhibitor positivity. Mean ProC Global results were significantly lower in patients with recurrent VTE than in patients without recurrent VTE. The association between abnormal ProC Global result and recurrent VTE showed a strong trend, before (odds ratio, OR 3.6) and after (OR 3.1) exclusion of known thrombophilic abnormalities. Patients with a first episode of idiopathic VTE also expressed significant lower ProC Global results than those with secondary VTE. After exclusion of known PC pathway abnormalities, there was a statistically significant association between abnormal ProC Global and initial idiopathic VTE (p=0.04). These results suggest that ProC Global may serve as a predictor of recurrent VTE and potentially for first episode of idiopathic VTE. ProC Global may help identify patients at increased risk of initial and recurrent VTE.

scholarly journals Cardiovascular and metabolic risk of antipsychotics in children and young adults: a multinational self-controlled case series study

2021 ◽  
Vol 30 ◽  
Author(s):  
Kenneth K. C. Man ◽  
Shih-Chieh Shao ◽  
Yu-Chuan Chang ◽  
Mei-Hung Chi ◽  
Han Eol Jeong ◽  
...  
Keyword(s):  
Young Adults ◽  
Cardiovascular Events ◽  
Case Series ◽  
Real World Evidence ◽  
Case Series Study ◽  
Increased Risk ◽  
Children And Young Adults ◽  
Exposure Windows ◽  
The Uk

Abstract Aims The risk of antipsychotic-associated cardiovascular and metabolic events may differ among countries, and limited real-world evidence has been available comparing the corresponding risks among children and young adults. We, therefore, evaluated the risks of cardiovascular and metabolic events in children and young adults receiving antipsychotics. Methods We conducted a multinational self-controlled case series (SCCS) study and included patients aged 6–30 years old who had both exposure to antipsychotics and study outcomes from four nationwide databases of Taiwan (2004–2012), Korea (2010–2016), Hong Kong (2001–2014) and the UK (1997–2016) that covers a total of approximately 100 million individuals. We investigated three antipsychotics exposure windows (i.e., 90 days pre-exposure, 1–30 days, 30–90 days and 90 + days of exposure). The outcomes were cardiovascular events (stroke, ischaemic heart disease and acute myocardial infarction), or metabolic events (hypertension, type 2 diabetes mellitus and dyslipidaemia). Results We included a total of 48 515 individuals in the SCCS analysis. We found an increased risk of metabolic events only in the risk window with more than 90-day exposure, with a pooled IRR of 1.29 (95% CI 1.20–1.38). The pooled IRR was 0.98 (0.90–1.06) for 1–30 days and 0.88 (0.76–1.02) for 31–90 days. We found no association in any exposure window for cardiovascular events. The pooled IRR was 1.86 (0.74–4.64) for 1–30 days, 1.35 (0.74–2.47) for 31–90 days and 1.29 (0.98–1.70) for 90 + days. Conclusions Long-term exposure to antipsychotics was associated with an increased risk of metabolic events but did not trigger cardiovascular events in children and young adults.

scholarly journals Risk of recurrent venous thromboembolism in COPD patients: results from a prospective cohort study

2017 ◽  
Vol 50 (1) ◽  
pp. 1700094 ◽  
Author(s):  
Raphael Le Mao ◽  
Cécile Tromeur ◽  
Amélie Bazire ◽  
Maelenn Gouillou ◽  
Marie Guegan ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Prospective Cohort ◽  
Multivariate Analyses ◽  
Anticoagulation Therapy ◽  
Secondary Outcome ◽  
Risk Of Death ◽  
Copd Patients ◽  
Increased Risk ◽  
Recurrent Venous Thromboembolism ◽  
Recurrent Vte

We aimed to assess the risk of recurrent venous thromboembolism (VTE) in patients with chronic obstructive pulmonary disease (COPD) following cessation of anticoagulation therapy.In a prospective cohort of 1468 patients with a documented episode of VTE, followed for up to 5 years after cessation of anticoagulation therapy, the diagnosis of COPD was confirmed in 136. The main outcome was recurrent VTE. The secondary outcome was overall mortality. Univariate and multivariate analyses were performed to identify the risk factors of recurrence.Of the 1468 patients included, recurrent VTE was observed in 306 (34 with COPD and 272 without) during a median follow-up period of 36.5 months. The incidence rate of recurrent VTE was 9.1% (95% CI 6.5–12.8) for COPD patients and 7.0% (95% CI 6.2–7.9) for non-COPD patients. COPD was not associated with an increased risk of VTE recurrence on univariate or multivariate analyses (hazard ratio: 1.0 (95% CI 0.7–1.4)). The risk of death, adjusted for demographic and clinical characteristics, showed no increase in COPD patients, as compared to non-COPD patients.In patients with COPD who had an acute episode of VTE, the risk of recurrent VTE was not any higher than that in non-COPD patients.

Thromboembolic Events in Children and Young Adults With Pediatric Sarcoma

2007 ◽  
Vol 25 (12) ◽  
pp. 1519-1524 ◽  
Author(s):  
Ido Paz-Priel ◽  
Lauren Long ◽  
Lee J. Helman ◽  
Crystal L. Mackall ◽  
Alan S. Wayne
Keyword(s):  
Pulmonary Embolism ◽  
Young Adults ◽  
Vena Cava ◽  
Thromboembolic Events ◽  
Pediatric Sarcoma ◽  
Increased Risk ◽  
Venous Thromboembolic Events ◽  
Venous Thromboembolic ◽  
Localized Disease ◽  
Children And Young Adults

Purpose Adults with malignancy are at increased risk for venous thromboembolic events (TEs). However, data in children and young adults with cancer are limited. Patients and Methods To determine the risk and clinical features of TEs in children and young adults with sarcoma, we reviewed records on 122 consecutive patients with sarcoma treated from October 1980 to July 2002. Results Twenty-three TEs were diagnosed in 19 of 122 (16%; 95% CI, 10% to 23%) patients. Prevalence by diagnosis was Ewing sarcoma, eight of 61 (13%); osteosarcoma, two of 20 (10%); rhabdomyosarcoma, four of 26 (15%); and other sarcomas, five of 15 (33%). TEs developed in 23% of patients with metastases at presentation versus 10% with localized disease (odds ratio, 2.59; 95% CI, 0.9 to 7.1; P < .06). Fifty-three percent of patients with thrombosis had a clot at presentation. A lupus anticoagulant was detected in four of five evaluated patients. There was a single fatality due to pulmonary embolism. Patients who were diagnosed with cancer after 1993 had a higher rate of TE (7% v 23%; P < .015). Of the 23 events, 43% were asymptomatic. Main sites of thromboses were deep veins of the extremities (10 of 23; 43%), pulmonary embolism (five of 23; 22%), and the inferior vena cava (four of 23; 17%). TEs were associated with tumor compression in eight of 23 (35%) and with venous catheters in three of 23 (13%). Conclusion Thromboembolism is common in pediatric patients with sarcomas. Thromboses are detected frequently around the time of oncologic presentation, may be asymptomatic, and seem to be associated with a higher disease burden. Children and young adults with sarcoma should be monitored closely for thrombosis.

A diagnostic approach to recurrent myalgia and rhabdomyolysis in children

2015 ◽  
Vol 100 (8) ◽  
pp. 793-797 ◽  
Author(s):  
Eunice K Chan ◽  
Andrew J Kornberg ◽  
Monique M Ryan
Keyword(s):  
Young Adults ◽  
Malignant Hyperthermia ◽  
Diagnostic Algorithm ◽  
Diagnostic Approach ◽  
Extensive Investigation ◽  
Muscle Disorders ◽  
Metabolic Myopathies ◽  
Common Complaint ◽  
Increased Risk ◽  
Children And Young Adults

Episodic myalgia is a common complaint in children and young adults. While many cases remain idiopathic even after extensive investigation, myalgia can be the first symptom of an underlying serious neuromuscular condition, and can be associated with an increased risk of such serious complications as rhabdomyolysis and malignant hyperthermia. We review and highlight the metabolic myopathies and other increasingly recognised muscle disorders that may present to paediatricians with episodic myalgia or isolated episodes of rhabdomyolysis, and propose a diagnostic algorithm for investigation of these complaints.

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