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Article ; Online: Fitusiran prophylaxis in severe haemophilia without inhibitors.

Jiménez-Yuste, Víctor / Álvarez-Román, María Teresa

The Lancet. Haematology

2023 Volume 10, Issue 5, Page(s) e308–e309

MeSH term(s)	Humans ; Hemophilia A/complications ; Hemophilia A/drug therapy ; Acetylgalactosamine/therapeutic use ; RNA, Small Interfering/therapeutic use ; Factor VIII/therapeutic use
Chemical Substances	fitusiran ; Acetylgalactosamine (KM15WK8O5T) ; RNA, Small Interfering ; Factor VIII (9001-27-8)
Language	English
Publishing date	2023-03-29
Publishing country	England
Document type	Journal Article ; Comment
ISSN	2352-3026
ISSN (online)	2352-3026
DOI	10.1016/S2352-3026(23)00057-1
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Article: Real-world experience of rIX-FP prophylaxis at dosing intervals of up to 14 days in a pediatric patient with hemophilia B during the COVID-19 pandemic.

Díaz-Jordán, Bolívar Luis / Cebanu, Tamara / García Barcenilla, Sara / Álvarez-Román, Maria Teresa

Clinical case reports

2023 Volume 11, Issue 12, Page(s) e8180

Abstract: Switching to rIX-FP prophylaxis at dosing intervals of up to 14 days in a hemophilia B pediatric patient decreased treatment burden by reducing the number of administrations and hospital visits, without affecting efficacy or treatment adherence. This is ... ...

Abstract	Switching to rIX-FP prophylaxis at dosing intervals of up to 14 days in a hemophilia B pediatric patient decreased treatment burden by reducing the number of administrations and hospital visits, without affecting efficacy or treatment adherence. This is particularly important in contexts of limited mobility and overloaded healthcare services.
Language	English
Publishing date	2023-11-28
Publishing country	England
Document type	Case Reports
ZDB-ID	2740234-4
ISSN	2050-0904
ISSN	2050-0904
DOI	10.1002/ccr3.8180
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Article ; Online: The 2021 guidelines on the diagnosis of von Willebrand disease: A comparison with current clinical practice in Spanish centers.

Álvarez-Román, María Teresa / Sierra-Aisa, Cristina / Jiménez-Yuste, Víctor

Haemophilia : the official journal of the World Federation of Hemophilia

2023 Volume 29, Issue 3, Page(s) 925–927

MeSH term(s)	Humans ; von Willebrand Diseases/diagnosis ; von Willebrand Factor
Chemical Substances	von Willebrand Factor
Language	English
Publishing date	2023-03-13
Publishing country	England
Document type	Letter
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.14778
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Zs.A 4249: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Care for children with haemophilia during COVID-19: Data of the PedNet study group.

Álvarez-Román, María Teresa / Kurnik, Karin

Haemophilia : the official journal of the World Federation of Hemophilia

2021 Volume 27, Issue 4, Page(s) e537–e539

MeSH term(s)	COVID-19/epidemiology ; Child ; Disease Management ; Hemophilia A/prevention & control ; Hemophilia A/therapy ; Hospitals, Special ; Humans ; Patient Care ; Telemedicine
Language	English
Publishing date	2021-03-08
Publishing country	England
Document type	Letter
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.14286
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Zs.A 4249: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Switching and increasing prophylaxis regimen with a genetically recombinant fusion of coagulation factor IX and albumin in haemophilia B: a case report.

Álvarez-Román, María Teresa / Merchán, Raquel Díaz / Mellado, Roberto Carlos Raynero / Jiménez-Yuste, Victor

Current opinion in hematology

2023 Volume 30, Issue 5, Page(s) 175–179

Abstract: Purpose of review: We present a case of a boy diagnosed in 2007 with severe haemophilia B [factor IX (FIX) concentration < 1%] at age of 9 months. He was initially treated with recombinant FIX concentrates, but changes in regimens were frequent due to ... ...

Abstract	Purpose of review: We present a case of a boy diagnosed in 2007 with severe haemophilia B [factor IX (FIX) concentration < 1%] at age of 9 months. He was initially treated with recombinant FIX concentrates, but changes in regimens were frequent due to spontaneous hemarthros. In 2013, he entered a phase III trial (NCT01662531) and received rIX-FP, IDELVION at 50 IU/kg once a week. Although the boy was safely maintained with this regimen (2015-2017), the number of hemarthros increased after he started to play football. Thus, rIX-FP regimen was modified (40 IU/kg twice/week) to optimize therapy. This modification was efficient on maintaining patient's thought levels (33%), helped during his fully incorporation at school and social life, and significantly improved synovial hypertrophy. In the last year, the boy has not suffered any bleeding episode and his joint situation improved significantly, which allowed reducing doses to weekly recommended doses. Recent findings: FIX replacement therapies with intravenous plasma-derived FIX (pdFIX) or standard half-life recombinant FIX (rFIX) concentrates are hampered by the relatively short terminal elimination half-life (t1/2) of these substances (around 17-34 h), resulting in the need for frequent infusions (e.g. once every 3 or 4 days) to maintain protective FIX levels. In the past years, the first genetically recombinant fusion of rFIX with another protein - a recombinant human albumin - was developed (albutrepenonacog-alfa or rIX-FP; IDELVION) as a strategy to extend the t1/2 of rFIX-FP (around 95 h). Summary: We provide information about the difficult management of a patient with a major bleeding haemorrhagic phenotype, which caused serious limitations in the patient's daily life, impacting his quality of life at his young age, and how the switch to IDELVION allowed the situation to improve considerably.
MeSH term(s)	Humans ; Infant ; Male ; Factor IX/genetics ; Factor IX/therapeutic use ; Hemophilia A/drug therapy ; Hemophilia B/drug therapy ; Hemorrhage/etiology ; Hemorrhage/prevention & control ; Hemorrhage/drug therapy ; Quality of Life
Chemical Substances	Factor IX (9001-28-9)
Language	English
Publishing date	2023-08-09
Publishing country	United States
Document type	Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1153887-9
ISSN	1531-7048 ; 1065-6251
ISSN (online)	1531-7048
ISSN	1065-6251
DOI	10.1097/MOH.0000000000000775
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Zs.A 3703: Show issues

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Article ; Online: Predicting joint involvement through tailored prophylaxis in severe haemophilia A, is it possible?

Meijon-Ortigueira, Maria Del Mar / Alvarez-Roman, Maria Teresa / De La Corte, Hortensia / Butta, Nora / Jimenez-Yuste, Victor

Haemophilia : the official journal of the World Federation of Hemophilia

2024

Abstract: Introduction: Tailored prophylaxis is the current treatment regimen for patients with severe haemophilia A. Recently, published guidelines describe two possible approaches, based on clinical characteristics or estimation of pharmacokinetic parameters. ... ...

Abstract	Introduction: Tailored prophylaxis is the current treatment regimen for patients with severe haemophilia A. Recently, published guidelines describe two possible approaches, based on clinical characteristics or estimation of pharmacokinetic parameters. However, both have strengths and weaknesses, and their characteristics need to be integrated to optimize treatment appropriately. In this paper, we present a model that considers together the characteristics of prophylaxis and the relevance of each. Methods: The age at initiation of prophylaxis, number of bleeding events, treatment regimen, therapeutic adherence, FVIII trough levels, and joint status were analyzed in 59 patients followed at La Paz University Hospital between January 2000 and December 2019. Results: The mean duration of primary prophylaxis of 113.37 ± 57.79 months. Eighty-three percent (n = 49) had no joint status involvement at the end of follow-up (HJHS and HEAD-US = 0). The median ABR was 0.7 (IQR 0.2 -1.0) and 54.2% presented trough levels of FVIII during follow-up >1 IU/dL. 72,9% engaged in some type of physical activity and overall adherence was over 85% in all patients evaluated. The regression analysis performed, considering all these factors, showed that the initiation of prophylaxis before 21 months of age was the most relevant protective factor against the appearance of joint involvement (OR 88.33 p.031 CI 95% 1.49-5224.40) CONCLUSION: Early initiation of prophylaxis was the most relevant factor in the protection of joint status. More comprehensive analysis models adapted to the characteristics of each population, are needed to adequately individualize treatment.
Language	English
Publishing date	2024-04-04
Publishing country	England
Document type	Journal Article
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.15014
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Zs.A 4249: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Arthroscopic ankle surgery in people with haemophilia.

Rodriguez-Merchan, E Carlos / De la Corte-Rodriguez, Hortensia / Gomez-Cardero, Primitivo / Alvarez-Roman, Maria Teresa / Jimenez-Yuste, Victor

Haemophilia : the official journal of the World Federation of Hemophilia

2024 Volume 30, Issue 2, Page(s) 286–294

Abstract: Introduction: People with haemophilia (PWH) not administered primary haematological prophylaxis since childhood, that is, those treated haematologically on demand or not treated at all, often experience the degeneration of the ankles, leading to pain ... ...

Abstract	Introduction: People with haemophilia (PWH) not administered primary haematological prophylaxis since childhood, that is, those treated haematologically on demand or not treated at all, often experience the degeneration of the ankles, leading to pain and functional impairment. Aim: To analyse the outcomes and complications of arthroscopic ankle surgery performed on PWH. Methods: For this narrative review of the literature, a search was conducted in PubMed on 2, December 2023, using the keywords "haemophilia", "ankle" and "arthroscopy". Of the 29 articles identified, 15 specifically related to ankle arthroscopy in PWH were selected (inclusion criterion). The remaining articles did not meet this requirement (exclusion criterion) and were therefore eliminated. Results: Arthroscopic procedures (arthroscopic synovectomy, debridement and arthrodesis of the ankle) are increasingly used in the surgical treatment of haemophilic ankle arthropathy. Although arthroscopic ankle surgery offers good outcomes in patients with haemophilia, the procedure is not free of complications, which range from 7.9% for arthroscopic ankle debridement to 13.1% in arthroscopic ankle synovectomy and 17.8% in arthroscopic ankle arthrodesis, respectively. The non-union rate of arthroscopic ankle arthrodesis is 7.1% (2/28). Conclusion: Although arthroscopic interventions in the haemophilic ankle (synovectomy, debridement, arthrodesis) offer good functional outcomes, they are associated with a non-negligible rate of complications. Arthroscopic ankle surgery in PWH is major surgery and should be treated as such.
MeSH term(s)	Humans ; Child ; Hemophilia A/complications ; Ankle ; Hemarthrosis/complications ; Arthroscopy/adverse effects ; Arthroscopy/methods ; Ankle Joint ; Arthritis/complications ; Arthrodesis/adverse effects ; Treatment Outcome
Language	English
Publishing date	2024-02-20
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	1229713-6
ISSN	1365-2516 ; 1351-8216 ; 1355-0691
ISSN (online)	1365-2516
ISSN	1351-8216 ; 1355-0691
DOI	10.1111/hae.14967
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Zs.A 4249: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Hemostatic cover in orthopedic surgery.

Álvarez Román, María Teresa

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis

2023 Volume 34, Issue S1, Page(s) S22–S25

Abstract: Bleeding into joints, known as hemarthrosis, is the most common kind of bleeding experienced by patients with hemophilia. Repeat bleeds into the same joint lead to the so-called hemophilic arthropathy. Patients with this condition tend to require surgery ...

Abstract	Bleeding into joints, known as hemarthrosis, is the most common kind of bleeding experienced by patients with hemophilia. Repeat bleeds into the same joint lead to the so-called hemophilic arthropathy. Patients with this condition tend to require surgery earlier and most frequently than the general population. Successful hemostasis is essential to carry out such procedures. Thanks to the advances made in the treatment of hemophilia, most surgical techniques can be performed safely and reliably. The present review shall focus on the international recommendations related to the performance of these surgical procedures. We shall be examining the available treatments, including the way they should be administered as well as the requirements regarding the postoperative period and the subsequent rehabilitation program.
MeSH term(s)	Humans ; Hemophilia A/complications ; Hemophilia A/surgery ; Hemostatics/therapeutic use ; Hemarthrosis/surgery ; Hemorrhage ; Hemostasis ; Orthopedic Procedures
Chemical Substances	Hemostatics
Language	English
Publishing date	2023-05-31
Publishing country	England
Document type	Review ; Journal Article
ZDB-ID	1033551-1
ISSN	1473-5733 ; 0957-5235
ISSN (online)	1473-5733
ISSN	0957-5235
DOI	10.1097/MBC.0000000000001201
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Zs.A 2920: Show issues

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Article ; Online: Long-term impact of primary prophylaxis on joint status in patients with severe hemophilia A.

Meijón Ortigueira, María Del Mar / Álvarez-Román, María Teresa / De La Corte Rodríguez, Hortensia / Butta Coll, Nora / Jiménez-Yuste, Víctor

Research and practice in thrombosis and haemostasis

2023 Volume 7, Issue 1, Page(s) 100005

Abstract: Background: Primary prophylaxis with factor VIII concentrates is the therapeutic gold standard for severe hemophilia A. Although this approach will change substantially with the use of nonsubstitutive therapies, the long-term effects of primary ... ...

Abstract	Background: Primary prophylaxis with factor VIII concentrates is the therapeutic gold standard for severe hemophilia A. Although this approach will change substantially with the use of nonsubstitutive therapies, the long-term effects of primary prophylaxis remain unclear. We present information on joint health with tailored primary prophylaxis in a consecutive series at a single center. Methods: We retrospectively analyzed 60 patients who did not develop early inhibitors. The annual bleeding rate and annual joint bleeding rate, prophylaxis characteristics, physical activity, adherence, and development of inhibitors were compared between those with and without joint involvement at the end of follow-up. Joint involvement was defined as a Hemophilia Joint Health Score or Hemophilia Early Arthropathy Detection with an ultrasound score ≥1. Results: Among 60 patients with median follow-up of 113 ± 6 months after starting prophylaxis, 76.7% had no joint involvement at the end of the follow-up. Those without joint involvement started prophylaxis at a younger median age (1 [IQR 1-1] year vs 3 [IQR 2-4.3] years). They also had lower annual joint bleeding rate (0.0 [IQR 0-0.2] vs 0.2 [IQR 0.1-0.5]), were more often physically active (70% vs 50%), and had lower trough factor VIII levels. Adherence to treatment was not significantly different between groups. Conclusion: Initiation of primary prophylaxis at a younger age was the main factor associated with long-term preservation of joint status in patients with severe hemophilia A.
Language	English
Publishing date	2023-01-12
Publishing country	United States
Document type	Journal Article
ISSN	2475-0379
ISSN (online)	2475-0379
DOI	10.1016/j.rpth.2022.100005
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Article ; Online: Long-term outcomes of prophylaxis with a recombinant factor VIII Fc or recombinant factor IX Fc in patients with hemophilia previously treated on demand.

Álvarez-Román, María-Teresa / Shapiro, Amy D / Ragni, Margaret V / Palmborg, Helena / Bystrická, Linda / Szamosi, Johan / Casiano, Sandra / Chambost, Hervé

Research and practice in thrombosis and haemostasis

2023 Volume 7, Issue 6, Page(s) 102163

Abstract: Background: Prophylactic factor replacement therapy is recommended over on-demand treatment for preserving long-term joint health in hemophilia. Extended half-life products, including efmoroctocog alfa/eftrenonacog alfa (recombinant factor VIII [FVIII]/ ... ...

Abstract	Background: Prophylactic factor replacement therapy is recommended over on-demand treatment for preserving long-term joint health in hemophilia. Extended half-life products, including efmoroctocog alfa/eftrenonacog alfa (recombinant factor VIII [FVIII]/FIX Fc fusion proteins; herein rFVIIIFc/rFIXFc), have the potential to reduce treatment burden with less frequent administration and improve bleed prevention. Objectives: We report post hoc data from patients with hemophilia A or B (HA/HB) who switched from prestudy on-demand FVIII/FIX to rFVIIIFc/rFIXFc prophylaxis at the start of A-LONG/B-LONG or start of/during ASPIRE/B-YOND phase 3 studies. Methods: Patients with ≥6 months rFVIIIFc/rFIXFc prophylaxis were enrolled. Treatment exposure, dosing, annualized bleeding rates, joint health, and health-related quality of life (HRQoL) outcomes were assessed. Results were also stratified by age. Results: Sixty-seven patients with HA and 50 with HB were analyzed; ≥60% were from regions outside Europe/North America, predominately those aged 12 to ‍25 years. No subjects returned to on-demand treatment postswitch.After switch to rFVIIIFc/rFIXFc prophylaxis, median annualized bleeding rates were reduced and sustained at low levels with stable factor usage across age groups (median treatment duration: 4.8/3.6 years). HRQoL outcomes improved for all ages; most pronounced changes were in the sports and leisure and physical health domains. After switch to rFVIIIFc prophylaxis, total modified Hemophilia Joint Health Score and joints with pain decreased in 64.6% and 29.2% of patients with HA. Insufficient data from patients with HB limited joint health evaluation of rFIXFc. Conclusions: Findings add to existing evidence and demonstrate the clinical and HRQoL benefits of switching patients from on-demand treatment to rFVIIIFc/rFIXFc prophylaxis.
Language	English
Publishing date	2023-08-09
Publishing country	United States
Document type	Journal Article
ISSN	2475-0379
ISSN (online)	2475-0379
DOI	10.1016/j.rpth.2023.102163
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