Rivista e Edizione

Volume 9 (2022): Edizione 1 (January 2022)

Volume 8 (2021): Edizione 1 (January 2021)

Volume 7 (2020): Edizione 1 (January 2020)

Volume 6 (2019): Edizione 2 (January 2019)

Volume 6 (2019): Edizione 1 (January 2019)

Volume 5 (2018): Edizione 1 (April 2018)

Volume 4 (2017): Edizione 1 (January 2017)

Volume 3 (2016): Edizione 2 (July 2016)

Volume 3 (2016): Edizione 1 (January 2016)

Volume 2 (2015): Edizione 2 (July 2015)

Volume 2 (2015): Edizione 1 (March 2015)

Volume 1 (2014): Edizione 3 (October 2014)

Volume 1 (2013): Edizione 2 (May 2013)

Volume 1 (2013): Edizione 1 (January 2013)

Dettagli della rivista
Formato
Rivista
eISSN
2055-3390
Pubblicato per la prima volta
22 Dec 2017
Periodo di pubblicazione
1 volta all'anno
Lingue
Inglese

Cerca

Volume 2 (2015): Edizione 2 (July 2015)

Dettagli della rivista
Formato
Rivista
eISSN
2055-3390
Pubblicato per la prima volta
22 Dec 2017
Periodo di pubblicazione
1 volta all'anno
Lingue
Inglese

Cerca

13 Articoli

Kd50: Introduction

Accesso libero

Celebrating 50 years of haemophilia care at the Royal Free Hospital

Pubblicato online: 21 Apr 2018
Pagine: 1 - 2

Astratto

Abstract

In recent decades, few disorders have benefited more from scientific research than haemophilia. Those living with the condition have witnessed improved standards of care resulting in dramatic improvements in quality of life. The Katharine Dormandy Haemophilia Centre and Thrombosis Unit has made a proud contribution to this improvement in care.

Parole chiave

  • haemophilia
  • quality of life
  • Katharine Dormandy
  • home care

Kd50: My Story

Accesso libero

The road to cloning of factor VIII and the recombinant era

Pubblicato online: 21 Apr 2018
Pagine: 3 - 4

Astratto

Abstract

Use of recombinant clotting factor now represents the standard of care in haemophilia across the developed world. But the recombinant era would not have occurred without the isolation from plasma of factor VIII in sufficient quantities to allow characterisation and cloning. Much of this development work occurred at the Royal Free Hospital.

Parole chiave

  • haemophilia
  • factor VIII
  • cloning
Accesso libero

Blood borne infections and haemophilia: the worst of times

Pubblicato online: 21 Apr 2018
Pagine: 5 - 7

Astratto

Abstract

The HIV and hepatitis C epidemics of the 1980s represent the darkest days in the history of modern haemophilia care. The haemophilia centre at the Royal Free Hospital was at the forefront of research into the natural history of both diseases. This work led directly to the widespread use of recombinant products, as well as the establishment of combined haemophilia clinics with hepatologists and HIV physicians.

Parole chiave

  • HIV
  • hepatitis C
  • haemophilia
  • Katharine Dormandy
Accesso libero

Diagnosis and management of inhibitors: the need for professional collaboration

Pubblicato online: 21 Apr 2018
Pagine: 8 - 10

Astratto

Abstract

Advances in the understanding of haemophilia require effective collaboration, both at the national and international levels. Such collaborations are currently playing a major role in elucidating the natural history of acquired haemophilia, and also in clarifying the issue of product-related inhibitor development in previously untreated patients with haemophilia.

Parole chiave

  • inhibitor
  • haemophilia
  • Katharine Dormandy
Accesso libero

International databases open the door to improved care for rare bleeding disorders

Pubblicato online: 21 Apr 2018
Pagine: 11 - 12

Astratto

Abstract

The development of registries through international collaboration has facilitated better understanding of the rare bleeding disorders. Such work has shown that rare bleeding disorders are heterogeneous and need to be studied singularly, and that heterozygous patients may bleed. There is a need to understand the minimum plasma coagulant activity level to prevent spontaneous bleeding. Moreover, due to the low prevalence of rare bleeding disorders, the management of this patient population remains a challenge. Data collection on clinical history, efficacy and side effects of treatment needs to be harmonised.

Parole chiave

  • rare bleeding disorders
  • haemophilia
  • Katharine Dormandy
  • databases
Accesso libero

Supporting outreach programmes for haemophilia diagnostics

Pubblicato online: 21 Apr 2018
Pagine: 13 - 13

Astratto

Abstract

The Katharine Dormandy haemophilia centre has played an enormous role over many years in providing outreach programmes to centres in developing countries. Doing so offers the potential to improve patient care by enhancing understanding of the need for accurate diagnosis and management.

Parole chiave

  • laboratory
  • outreach
  • Katharine Dormandy

Kd50: Comprehensive Care

Accesso libero

The modern challenges of haemophilia care in childhood and adolescence

Pubblicato online: 21 Apr 2018
Pagine: 14 - 16

Astratto

Abstract

Modern haemophilia care based on good diagnosis and effective prophylaxis has allowed boys born with haemophilia to grow up leading essentially normal lives. Nevertheless, there remain challenges notably those posed by inhibitors and patient expectations. There is now a significant cohort of men in their 30s, 40s and 50s who have been looked after extremely well but many of whom now have significant ankle arthropathy because they played football, even when advised not to do so at a time when prophylaxis was limited or started late. The imminent era of longer-acting clotting factors and gene therapy will also impact on future patient expectations. Meeting and managing these challenges will be much enhanced by the development and maintenance of good relationships between the patient and the haemophilia team.

Parole chiave

  • family
  • haemophilia
  • Katharine Dormandy
  • inhibitors
Accesso libero

Musculoskeletal care in haemophilia: the importance of the joint team approach

Pubblicato online: 21 Apr 2018
Pagine: 17 - 19

Astratto

Abstract

The multidisciplinary team approach to providing comprehensive care is the cornerstone of the management of musculoskeletal issues in haemophilia. At the Royal Free the team takes a holistic approach to musculoskeletal disease management that is not just about assessment of a painful knee or having an operation. It recognises that patients sit on a spectrum of musculoskeletal disease severity that is dependent on age and the type of treatments available and the issues that may have been experienced with those treatments. The patient’s own goals sit at the heart of the team’s approach.

Parole chiave

  • orthopaedic
  • haemophilia
  • Katharine Dormandy
  • physiotherapy
  • joints
Accesso libero

Gynaecological and obstetric care for women with inherited bleeding disorders

Pubblicato online: 21 Apr 2018
Pagine: 20 - 21

Astratto

Abstract

The issue of women with bleeding disorders was first reported by Professor von Willebrand in 1926, but it is only from around the mid 1990s that the issue has been fully recognised. Much of this is due to the vision and hard work of Professor Christine Lee and colleagues at the Katharine Dormandy Haemophilia Centre. This work has led to better diagnosis, better quality care and improved quality of life for women with bleeding disorders.

Parole chiave

  • women
  • haemophilia
  • Katharine Dormandy
Accesso libero

An approach that puts the family at the centre of haemophilia care

Pubblicato online: 21 Apr 2018
Pagine: 22 - 23

Astratto

Abstract

Staff at the Katharine Dormandy Haemophilia Centre pioneered a systemic family therapy model for haemophilia, in which reviews combined medical care and family counselling. That approach has now been extended to specialised joint clinics such as in orthopaedics, women’s and genetic counselling. This multidisciplinary team approach enables specialist clinicians to focus on what they do best while the family therapy team manages the psychological, practical and family issues, and supports patients to make difficult decisions regarding their care.

Parole chiave

  • haemophilia
  • Katharine Dormandy
  • home treatment
  • family therapy
Accesso libero

Into the future with novel emerging therapies for haemophilia

Pubblicato online: 21 Apr 2018
Pagine: 24 - 25

Astratto

Abstract

Patients with haemophilia have benefited hugely from the advances in basic science achieved over the past 50 years. The introduction of clotting factor concentrates opened the door to home treatment and the subsequent availability of plasma and then recombinant products heralded the prophylaxis era. As clinicians and patients begin to manage the introduction of longer-acting factor concentrates, there are also many novel therapies and approaches in development, many of which offer the potential to transform the lives for individuals with inherited bleeding disorders and their families.

Parole chiave

  • haemophilia
  • Katharine Dormandy
  • clotting factor
  • novel therapies
Accesso libero

Gene therapy for haemophilia: a very modern success story

Pubblicato online: 21 Apr 2018
Pagine: 26 - 28

Astratto

Abstract

AAV-mediated gene therapy has the potential to be paradigm shifting as a treatment for haemophilia. A single administration of AAV vector can result in safe and consistent long-term expression of transgene (>5 years), reduction in spontaneous bleeding episodes, reduction in clotting factor usage and an improvement in quality of life. There is huge commercial interest in this approach, with the expectation that an AAV gene therapy product for haemophilia B will be licensed by 2020.

Parole chiave

  • gene therapy
  • AAV
  • haemophilia
  • Katharine Dormandy
Accesso libero

Literature Review: Transition From Paediatric to Adult Services in Haemophilia

Pubblicato online: 24 Jan 2019
Pagine: 29 - 51

Astratto

Abstract

The need to prepare children and young people with haemophilia for long-term care in the adult sector has long been accepted and is reflected in many national health care policies. Ideally, transition should be an individualised age- and development-appropriate process, through which each young person is empowered to self-manage, with support from their families and multidisciplinary team (MDT). It is widely recognised that young people who are not engaged in self-management frequently become non-adherent to therapy. For those with haemophilia, this can have a life-long impact on joint health and quality of life. In practice, anecdotal reports from individuals suggest that the transition process is often poorly planned, leaving them feeling lost and unsupported by the healthcare professionals who, until this point, have been core members of their support network. Rarely are the views of young people or their families sought regarding the outcome of their transition process. For those with less severe disease or rarer bleeding disorders, and for girls at the menarche, transition is often not addressed at all. This literature review forms the first part of a project that seeks to identify the outcome measures that can be used to drive a nurse-led initiative improvement in transition for young people with bleeding disorders, through changes in both working practices among health professionals and in the underpinning system, which enables them to put such changes into practice.

Parole chiave

  • transition
  • haemophilia
  • self-management
  • literature review
13 Articoli

Kd50: Introduction

Accesso libero

Celebrating 50 years of haemophilia care at the Royal Free Hospital

Pubblicato online: 21 Apr 2018
Pagine: 1 - 2

Astratto

Abstract

In recent decades, few disorders have benefited more from scientific research than haemophilia. Those living with the condition have witnessed improved standards of care resulting in dramatic improvements in quality of life. The Katharine Dormandy Haemophilia Centre and Thrombosis Unit has made a proud contribution to this improvement in care.

Parole chiave

  • haemophilia
  • quality of life
  • Katharine Dormandy
  • home care

Kd50: My Story

Accesso libero

The road to cloning of factor VIII and the recombinant era

Pubblicato online: 21 Apr 2018
Pagine: 3 - 4

Astratto

Abstract

Use of recombinant clotting factor now represents the standard of care in haemophilia across the developed world. But the recombinant era would not have occurred without the isolation from plasma of factor VIII in sufficient quantities to allow characterisation and cloning. Much of this development work occurred at the Royal Free Hospital.

Parole chiave

  • haemophilia
  • factor VIII
  • cloning
Accesso libero

Blood borne infections and haemophilia: the worst of times

Pubblicato online: 21 Apr 2018
Pagine: 5 - 7

Astratto

Abstract

The HIV and hepatitis C epidemics of the 1980s represent the darkest days in the history of modern haemophilia care. The haemophilia centre at the Royal Free Hospital was at the forefront of research into the natural history of both diseases. This work led directly to the widespread use of recombinant products, as well as the establishment of combined haemophilia clinics with hepatologists and HIV physicians.

Parole chiave

  • HIV
  • hepatitis C
  • haemophilia
  • Katharine Dormandy
Accesso libero

Diagnosis and management of inhibitors: the need for professional collaboration

Pubblicato online: 21 Apr 2018
Pagine: 8 - 10

Astratto

Abstract

Advances in the understanding of haemophilia require effective collaboration, both at the national and international levels. Such collaborations are currently playing a major role in elucidating the natural history of acquired haemophilia, and also in clarifying the issue of product-related inhibitor development in previously untreated patients with haemophilia.

Parole chiave

  • inhibitor
  • haemophilia
  • Katharine Dormandy
Accesso libero

International databases open the door to improved care for rare bleeding disorders

Pubblicato online: 21 Apr 2018
Pagine: 11 - 12

Astratto

Abstract

The development of registries through international collaboration has facilitated better understanding of the rare bleeding disorders. Such work has shown that rare bleeding disorders are heterogeneous and need to be studied singularly, and that heterozygous patients may bleed. There is a need to understand the minimum plasma coagulant activity level to prevent spontaneous bleeding. Moreover, due to the low prevalence of rare bleeding disorders, the management of this patient population remains a challenge. Data collection on clinical history, efficacy and side effects of treatment needs to be harmonised.

Parole chiave

  • rare bleeding disorders
  • haemophilia
  • Katharine Dormandy
  • databases
Accesso libero

Supporting outreach programmes for haemophilia diagnostics

Pubblicato online: 21 Apr 2018
Pagine: 13 - 13

Astratto

Abstract

The Katharine Dormandy haemophilia centre has played an enormous role over many years in providing outreach programmes to centres in developing countries. Doing so offers the potential to improve patient care by enhancing understanding of the need for accurate diagnosis and management.

Parole chiave

  • laboratory
  • outreach
  • Katharine Dormandy

Kd50: Comprehensive Care

Accesso libero

The modern challenges of haemophilia care in childhood and adolescence

Pubblicato online: 21 Apr 2018
Pagine: 14 - 16

Astratto

Abstract

Modern haemophilia care based on good diagnosis and effective prophylaxis has allowed boys born with haemophilia to grow up leading essentially normal lives. Nevertheless, there remain challenges notably those posed by inhibitors and patient expectations. There is now a significant cohort of men in their 30s, 40s and 50s who have been looked after extremely well but many of whom now have significant ankle arthropathy because they played football, even when advised not to do so at a time when prophylaxis was limited or started late. The imminent era of longer-acting clotting factors and gene therapy will also impact on future patient expectations. Meeting and managing these challenges will be much enhanced by the development and maintenance of good relationships between the patient and the haemophilia team.

Parole chiave

  • family
  • haemophilia
  • Katharine Dormandy
  • inhibitors
Accesso libero

Musculoskeletal care in haemophilia: the importance of the joint team approach

Pubblicato online: 21 Apr 2018
Pagine: 17 - 19

Astratto

Abstract

The multidisciplinary team approach to providing comprehensive care is the cornerstone of the management of musculoskeletal issues in haemophilia. At the Royal Free the team takes a holistic approach to musculoskeletal disease management that is not just about assessment of a painful knee or having an operation. It recognises that patients sit on a spectrum of musculoskeletal disease severity that is dependent on age and the type of treatments available and the issues that may have been experienced with those treatments. The patient’s own goals sit at the heart of the team’s approach.

Parole chiave

  • orthopaedic
  • haemophilia
  • Katharine Dormandy
  • physiotherapy
  • joints
Accesso libero

Gynaecological and obstetric care for women with inherited bleeding disorders

Pubblicato online: 21 Apr 2018
Pagine: 20 - 21

Astratto

Abstract

The issue of women with bleeding disorders was first reported by Professor von Willebrand in 1926, but it is only from around the mid 1990s that the issue has been fully recognised. Much of this is due to the vision and hard work of Professor Christine Lee and colleagues at the Katharine Dormandy Haemophilia Centre. This work has led to better diagnosis, better quality care and improved quality of life for women with bleeding disorders.

Parole chiave

  • women
  • haemophilia
  • Katharine Dormandy
Accesso libero

An approach that puts the family at the centre of haemophilia care

Pubblicato online: 21 Apr 2018
Pagine: 22 - 23

Astratto

Abstract

Staff at the Katharine Dormandy Haemophilia Centre pioneered a systemic family therapy model for haemophilia, in which reviews combined medical care and family counselling. That approach has now been extended to specialised joint clinics such as in orthopaedics, women’s and genetic counselling. This multidisciplinary team approach enables specialist clinicians to focus on what they do best while the family therapy team manages the psychological, practical and family issues, and supports patients to make difficult decisions regarding their care.

Parole chiave

  • haemophilia
  • Katharine Dormandy
  • home treatment
  • family therapy
Accesso libero

Into the future with novel emerging therapies for haemophilia

Pubblicato online: 21 Apr 2018
Pagine: 24 - 25

Astratto

Abstract

Patients with haemophilia have benefited hugely from the advances in basic science achieved over the past 50 years. The introduction of clotting factor concentrates opened the door to home treatment and the subsequent availability of plasma and then recombinant products heralded the prophylaxis era. As clinicians and patients begin to manage the introduction of longer-acting factor concentrates, there are also many novel therapies and approaches in development, many of which offer the potential to transform the lives for individuals with inherited bleeding disorders and their families.

Parole chiave

  • haemophilia
  • Katharine Dormandy
  • clotting factor
  • novel therapies
Accesso libero

Gene therapy for haemophilia: a very modern success story

Pubblicato online: 21 Apr 2018
Pagine: 26 - 28

Astratto

Abstract

AAV-mediated gene therapy has the potential to be paradigm shifting as a treatment for haemophilia. A single administration of AAV vector can result in safe and consistent long-term expression of transgene (>5 years), reduction in spontaneous bleeding episodes, reduction in clotting factor usage and an improvement in quality of life. There is huge commercial interest in this approach, with the expectation that an AAV gene therapy product for haemophilia B will be licensed by 2020.

Parole chiave

  • gene therapy
  • AAV
  • haemophilia
  • Katharine Dormandy
Accesso libero

Literature Review: Transition From Paediatric to Adult Services in Haemophilia

Pubblicato online: 24 Jan 2019
Pagine: 29 - 51

Astratto

Abstract

The need to prepare children and young people with haemophilia for long-term care in the adult sector has long been accepted and is reflected in many national health care policies. Ideally, transition should be an individualised age- and development-appropriate process, through which each young person is empowered to self-manage, with support from their families and multidisciplinary team (MDT). It is widely recognised that young people who are not engaged in self-management frequently become non-adherent to therapy. For those with haemophilia, this can have a life-long impact on joint health and quality of life. In practice, anecdotal reports from individuals suggest that the transition process is often poorly planned, leaving them feeling lost and unsupported by the healthcare professionals who, until this point, have been core members of their support network. Rarely are the views of young people or their families sought regarding the outcome of their transition process. For those with less severe disease or rarer bleeding disorders, and for girls at the menarche, transition is often not addressed at all. This literature review forms the first part of a project that seeks to identify the outcome measures that can be used to drive a nurse-led initiative improvement in transition for young people with bleeding disorders, through changes in both working practices among health professionals and in the underpinning system, which enables them to put such changes into practice.

Parole chiave

  • transition
  • haemophilia
  • self-management
  • literature review

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