Literature DB >> 33451335

Real-world evidence on Kovaltry (81-8973) in children with moderate or severe hemophilia A in Europe: a nested cohort analysis.

Jamie O'Hara1,2, Ceri Hirst3, Jose Francisco Cabre Marquez4, Tom Burke2.   

Abstract

BACKGROUND: Untreated hemophilia A patients may experience recurrent bleeding events leading to debilitating joint damages. While RCT and pharmacokinetic data support the value of Kovaltry [an unmodified full-length recombinant factor VIII (FVIII) product], real world evidence in children is lacking. This report describes a descriptive and multivariate analysis of the effectiveness of Kovaltry in children with hemophilia A in the real-world setting, using data from medical chart abstraction and cross-sectional surveys of physicians, patients, and caregivers.
RESULTS: Male patients aged < 18 years with moderate or severe hemophilia A, residing in five European countries and treated with FVIII were studied. The co-primary endpoints were the annualized bleeding rate (ABR) and the annual FVIII utilization rate. Twenty nine patients treated with Kovaltry were included, of whom 93% had severe disease and 75% were on continuous prophylactic treatment. The mean ABR was 2.66 ± 2.06, with rates decreasing with age. The children received on average 2.45 infusions per week, consistent across age groups (median 3; range 1-3). There were no reports of inhibitor development or adverse events in the study (AEs), and all patients were satisfied or very satisfied with the treatment. An exploratory multivariate analysis suggests no significant difference in ABR or units utilized between Kovaltry and some extended half life products in children with severe hemophilia A, though characteristics of these patient cohorts were markedly different.
CONCLUSION: This analysis demonstrates the effectiveness and safety of Kovaltry in a pan-European pediatric population with severe hemophilia A.

Entities:  

Keywords:  Clinical burden; Factor utilization; Hemophilia A; Kovaltry; Real world evidence

Mesh:

Substances:

Year:  2021        PMID: 33451335      PMCID: PMC7809751          DOI: 10.1186/s13023-021-01676-w

Source DB:  PubMed          Journal:  Orphanet J Rare Dis        ISSN: 1750-1172            Impact factor:   4.123


  14 in total

1.  Life expectancy in hemophilia outcome.

Authors:  C Mejia-Carvajal; E E Czapek; L A Valentino
Journal:  J Thromb Haemost       Date:  2006-03       Impact factor: 5.824

Review 2.  Blood-induced joint disease: the pathophysiology of hemophilic arthropathy.

Authors:  L A Valentino
Journal:  J Thromb Haemost       Date:  2010-09       Impact factor: 5.824

Review 3.  The past and future of haemophilia: diagnosis, treatments, and its complications.

Authors:  Flora Peyvandi; Isabella Garagiola; Guy Young
Journal:  Lancet       Date:  2016-02-18       Impact factor: 79.321

4.  Understanding the experience of caring for children with haemophilia: cross-sectional study of caregivers in the United States.

Authors:  M DeKoven; S Karkare; L A Kelley; D L Cooper; H Pham; J Powers; W C Lee; T Wisniewski
Journal:  Haemophilia       Date:  2014-01-29       Impact factor: 4.287

5.  Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV.

Authors:  Sarah C Darby; Sau Wan Kan; Rosemary J Spooner; Paul L F Giangrande; Frank G H Hill; Charles R M Hay; Christine A Lee; Christopher A Ludlam; Michael Williams
Journal:  Blood       Date:  2007-04-19       Impact factor: 22.113

6.  BAY 81-8973 safety and efficacy for prophylaxis and treatment of bleeds in previously treated children with severe haemophilia A: results of the LEOPOLD Kids Trial.

Authors:  R Ljung; G Kenet; M E Mancuso; V Kaleva; L Rusen; D Tseneklidou-Stoeter; L A Michaels; A Shah; W Hong; M Maas Enriquez
Journal:  Haemophilia       Date:  2015-12-09       Impact factor: 4.287

7.  Increased burden on caregivers of having a child with haemophilia complicated by inhibitors.

Authors:  Karin Lindvall; Sylvia von Mackensen; Sölve Elmståhl; Kate Khair; Ann Marie Stain; Rolf Ljung; Erik Berntorp
Journal:  Pediatr Blood Cancer       Date:  2013-11-26       Impact factor: 3.167

8.  Prophylaxis vs. on-demand treatment with BAY 81-8973, a full-length plasma protein-free recombinant factor VIII product: results from a randomized trial (LEOPOLD II).

Authors:  K Kavakli; R Yang; L Rusen; H Beckmann; D Tseneklidou-Stoeter; M Maas Enriquez
Journal:  J Thromb Haemost       Date:  2015-03       Impact factor: 5.824

9.  Randomised controlled trials and population-based observational research: partners in the evolution of medical evidence.

Authors:  C M Booth; I F Tannock
Journal:  Br J Cancer       Date:  2014-01-14       Impact factor: 7.640

10.  The cost of severe haemophilia in Europe: the CHESS study.

Authors:  Jamie O'Hara; David Hughes; Charlotte Camp; Tom Burke; Liz Carroll; Daniel-Anibal Garcia Diego
Journal:  Orphanet J Rare Dis       Date:  2017-05-31       Impact factor: 4.303

View more
  1 in total

1.  Enhanced pharmacokinetics and reduced bleeds in boys with hemophilia A after switching to Kovaltry from other standard half-life factor VIII concentrates.

Authors:  Kun Huang; Yingzi Zhen; Gang Li; Xinyi Wu; Zhenping Chen; Runhui Wu
Journal:  Res Pract Thromb Haemost       Date:  2022-03-25
  1 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.