A B Mehta

Summary

Affiliation: Royal Free Hospital
Country: UK

Publications

  1. doi Fabry disease: a review of current management strategies
    A Mehta
    Lysosomal Storage Disorders Unit, Department of Academic Haematology, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    QJM 103:641-59. 2010
  2. doi Enzyme replacement therapy with agalsidase alfa in patients with Fabry's disease: an analysis of registry data
    A Mehta
    University College London, London, UK
    Lancet 374:1986-96. 2009
  3. ncbi Fabry disease defined: baseline clinical manifestations of 366 patients in the Fabry Outcome Survey
    A Mehta
    University College London, London, UK
    Eur J Clin Invest 34:236-42. 2004
  4. doi Therapeutic goals in the treatment of Fabry disease
    Atul Mehta
    The Royal Free Hospital, University College London School of Medicine, London, United Kingdom
    Genet Med 12:713-20. 2010
  5. ncbi Fabry disease--whom to treat and when
    Atul Mehta
    Royal Free Hospital, Royal Free and University College School of Medicine, University College London, London, United Kingdom
    Clin Ther 30:S43-4. 2008
  6. doi Gaucher disease: unmet treatment needs
    Atul Mehta
    Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital and University College Medical School, London, UK
    Acta Paediatr Suppl 97:83-7. 2008
  7. ncbi Anderson-Fabry disease: developments in diagnosis and treatment
    A B Mehta
    Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital and University College Medical School, London, UK
    Int J Clin Pharmacol Ther 47:S66-74. 2009
  8. doi Fabry disease: beyond men
    Atul Mehta
    Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital and University College Medical School, London, UK
    Acta Paediatr Suppl 97:31-2. 2008
  9. ncbi Natural history of the cerebrovascular complications of Fabry disease
    A Mehta
    Department of Haematology, Royal Free Hospital, London, UK
    Acta Paediatr Suppl 94:24-7; discussion 9-10. 2005
  10. ncbi Access to expensive drugs in the NHS: myths and realities for cancer patients
    A B Mehta
    Department of Haematology, Royal Free Hospital and Royal Free and University College London School of Medicine, London, UK
    Int J Clin Pract 61:2126-9. 2007

Detail Information

Publications56

  1. doi Fabry disease: a review of current management strategies
    A Mehta
    Lysosomal Storage Disorders Unit, Department of Academic Haematology, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    QJM 103:641-59. 2010
    ..We highlight a need to develop comprehensive international guidelines to optimize ERT and adjunctive therapy in patients with Fabry disease, including females and children...
  2. doi Enzyme replacement therapy with agalsidase alfa in patients with Fabry's disease: an analysis of registry data
    A Mehta
    University College London, London, UK
    Lancet 374:1986-96. 2009
    ..We analysed 5-year treatment with agalsidase alfa enzyme replacement therapy in patients with Fabry's disease who were enrolled in the Fabry Outcome Survey observational database (FOS)...
  3. ncbi Fabry disease defined: baseline clinical manifestations of 366 patients in the Fabry Outcome Survey
    A Mehta
    University College London, London, UK
    Eur J Clin Invest 34:236-42. 2004
    ..This paper presents the first analysis of the FOS database and provides essential baseline data against which the effects of enzyme replacement can be measured...
  4. doi Therapeutic goals in the treatment of Fabry disease
    Atul Mehta
    The Royal Free Hospital, University College London School of Medicine, London, United Kingdom
    Genet Med 12:713-20. 2010
    ..Fabry disease is X-linked, but women are often symptomatic and may be as severely affected as men...
  5. ncbi Fabry disease--whom to treat and when
    Atul Mehta
    Royal Free Hospital, Royal Free and University College School of Medicine, University College London, London, United Kingdom
    Clin Ther 30:S43-4. 2008
  6. doi Gaucher disease: unmet treatment needs
    Atul Mehta
    Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital and University College Medical School, London, UK
    Acta Paediatr Suppl 97:83-7. 2008
    ..Potential new treatments for Gaucher disease include small molecules, which may penetrate tissues that are not accessible by ERT...
  7. ncbi Anderson-Fabry disease: developments in diagnosis and treatment
    A B Mehta
    Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital and University College Medical School, London, UK
    Int J Clin Pharmacol Ther 47:S66-74. 2009
    ..This review provides an overview of current issues in the diagnosis and treatment of patients with Fabry disease and considers what may lie ahead in this rapidly evolving therapeutic area...
  8. doi Fabry disease: beyond men
    Atul Mehta
    Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital and University College Medical School, London, UK
    Acta Paediatr Suppl 97:31-2. 2008
  9. ncbi Natural history of the cerebrovascular complications of Fabry disease
    A Mehta
    Department of Haematology, Royal Free Hospital, London, UK
    Acta Paediatr Suppl 94:24-7; discussion 9-10. 2005
    ..Furthermore, the database has demonstrated that significant renal or cardiac disease often co-exists with cerebrovascular disease, and may predispose patients with Fabry disease to neurological disability and stroke...
  10. ncbi Access to expensive drugs in the NHS: myths and realities for cancer patients
    A B Mehta
    Department of Haematology, Royal Free Hospital and Royal Free and University College London School of Medicine, London, UK
    Int J Clin Pract 61:2126-9. 2007
    ..Patient support organisation (Myeloma UK) and Hospital Myeloma Clinic...
  11. doi Natural course of Fabry disease: changing pattern of causes of death in FOS - Fabry Outcome Survey
    A Mehta
    University College London, London, UK
    J Med Genet 46:548-52. 2009
    ..This paper provides an update on the first analysis of FOS data...
  12. ncbi Fabry disease and the skin: data from FOS, the Fabry outcome survey
    C H Orteu
    Department of Dermatology, Royal Free Hospital, London NW3 2QG, UK
    Br J Dermatol 157:331-7. 2007
    ..Fabry disease (also known as Anderson-Fabry disease) is a rare, X-linked lysosomal storage disorder that is characterized by accumulation of globotriaosylceramide throughout a range of tissues in the body...
  13. ncbi Autologous plasma activates Akt/protein kinase B and enhances basal survival and resistance to DNA damage-induced apoptosis in B-chronic lymphocytic leukaemia cells
    R G Wickremasinghe
    Department of Haematology, Royal Free and University College School of Medicine, London, UK
    Br J Haematol 114:608-15. 2001
    ..Pharmacological blockade of this pathway may have potential in the development of novel therapeutic strategies for B-CLL treatment...
  14. ncbi Effects of enzyme replacement therapy on the cardiomyopathy of Anderson-Fabry disease: a randomised, double-blind, placebo-controlled clinical trial of agalsidase alfa
    D A Hughes
    Department of Academic Haematology, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK
    Heart 94:153-8. 2008
    ..The present study was designed to assess the safety and efficacy of enzyme replacement therapy with agalsidase alfa on the cardiac manifestations of Anderson-Fabry disease...
  15. ncbi Effective treatment of an elderly patient with Gaucher's disease and Parkinsonism: a case report of 24 months' oral substrate reduction therapy with miglustat
    D A Hughes
    Royal Free and University College Medical School, Rowland Hill Street, London, UK
    Parkinsonism Relat Disord 13:365-8. 2007
    ..Oral miglustat should be considered for the treatment of patients with type I GD and concurrent movement disorders who are unsuitable for ERT...
  16. doi Enhanced differentiation of osteoclasts from mononuclear precursors in patients with Gaucher disease
    M Reed
    Lysosomal Storage Disorders Unit, Department of Haematology, Royal Free Hospital and University College, London, UK
    Blood Cells Mol Dis 51:185-94. 2013
    ..Elucidation of the underlying mechanisms of these changes will suggest rational therapies for the most disabling aspect of this condition. ..
  17. doi A randomised, double-blind, placebo-controlled, crossover study to assess the efficacy and safety of three dosing schedules of agalsidase alfa enzyme replacement therapy for Fabry disease
    D A Hughes
    Department of Haematology, Royal Free Campus, University College London, Rowland Hill Street, London NW3 2PF, UK
    Mol Genet Metab 109:269-75. 2013
    ....
  18. doi Late-onset Fabry disease associated with angiokeratoma of Fordyce and multiple cherry angiomas
    V Hogarth
    Royal Free Hospital, London, UK
    Clin Exp Dermatol 36:506-8. 2011
    ..It also raises the question of whether the presence of multiple cherry angiomas in patients with cardiac disease should raise the possible diagnosis of FD...
  19. ncbi Extracellular matrix turnover and disease severity in Anderson-Fabry disease
    J S Shah
    The Heart Hospital, UCL, London, UK
    J Inherit Metab Dis 30:88-95. 2007
    ..Patients with AFD develop progressive left ventricular (LV) remodelling and heart failure. We hypothesized that altered extracellular matrix (ECM) turnover contributes to the pathophysiology of cardiac disease in AFD...
  20. pmc A phase I clinical trial of recombinant interleukin 2 following high dose chemo-radiotherapy for haematological malignancy: applicability to the elimination of minimal residual disease
    D J Gottlieb
    Department of Haematology, Royal Free Hospital, London, UK
    Br J Cancer 60:610-5. 1989
    ..We conclude that IL2 may be used in minimal residual haematological malignancy, and by producing anti-neoplastic effector cells has the potential, as yet unproven, to prolong disease-free survival of patients entering remission...
  21. ncbi A multicentre, open, non-comparative phase II study of a combination of fludarabine phosphate, cytarabine and granulocyte colony-stimulating factor in relapsed and refractory acute myeloid leukaemia and de novo refractory anaemia with excess of blasts in
    G Jackson
    Department of Haematology, Royal Victoria Infirmary, Newcastle upon Tyne, UK
    Br J Haematol 112:127-37. 2001
    ..It is therefore an important advance in developing new treatment options for these patients...
  22. ncbi Heterogeneity of VH-JH gene rearrangement patterns: an insight into the biology of B cell precursor ALL
    I Moreira
    Haematology Department, Royal Free and University College School of Medicine, London, UK
    Leukemia 15:1527-36. 2001
    ..Finally, in our cohort of patients, the presence of related or unrelated IGH clones did not influence overall survival...
  23. ncbi Intravenous enzyme replacement therapy: better in home or hospital?
    A Milligan
    Lysosomal Storage Disorders Unit, Royal Free Hospital, London, UK
    Br J Nurs 15:330-3. 2006
    ..Only 4 (9%) patients chose to continue receiving infusions in hospital. The majority of patients with Fabry disease and Gaucher disease found home-based therapy to be more convenient and less stressful than hospital-based therapy...
  24. doi Diagnosing Gaucher disease: an on-going need for increased awareness amongst haematologists
    A S Thomas
    Lysosomal Storage Disorders Unit, Department of Academic Haematology, Royal Free Hospital and University College Medical School, London, UK
    Blood Cells Mol Dis 50:212-7. 2013
    ....
  25. doi Long-term outcomes of liver transplantation in type 1 Gaucher disease
    R M Ayto
    Department of Haematology, University College London Medical School, London, UK
    Am J Transplant 10:1934-9. 2010
    ..All four patients have had excellent outcomes from liver transplantation for up to 10 years postprocedure with no evidence of Gaucher-related pathology in the graft...
  26. pmc Fludarabine phosphate for the treatment of low grade lymphoid malignancy
    J S Whelan
    ICRF Department of Medical Oncology, St Bartholomew s Hospital, London, UK
    Br J Cancer 64:120-3. 1991
    ..No other significant toxicity was reported in a total of 164 cycles of Fludarabine. This agent is active in advanced low grade lymphoid malignancy. Further studies are required to assess its role in newly diagnosed patients...
  27. ncbi Hearing improvement in patients with Fabry disease treated with agalsidase alfa
    D Hajioff
    Department of Otolaryngology, Royal Free Hospital, London, UK
    Acta Paediatr Suppl 92:28-30; discussion 27. 2003
    ..To describe the nature and prevalence of hearing loss in Fabry disease, and its response to enzyme replacement therapy (ERT) with agalsidase alfa...
  28. ncbi Hearing loss in Fabry disease: the effect of agalsidase alfa replacement therapy
    D Hajioff
    Department of Otolaryngology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
    J Inherit Metab Dis 26:787-94. 2003
    ..alpha-Galactosidase A replacement therapy with agalsidase alfa appears to reverse the hearing deterioration in these patients. This improvement is gradual, however, suggesting the need for long-term enzyme replacement therapy...
  29. ncbi Multiple myeloma and human immunodeficiency virus-1 (HIV-1) infection
    T T Yee
    Department of Haematology, Royal Free and University College Medical School, University College, London, United Kingdom
    Am J Hematol 66:123-5. 2001
    ....
  30. ncbi Caspase 8 activation independent of Fas (CD95/APO-1) signaling may mediate killing of B-chronic lymphocytic leukemia cells by cytotoxic drugs or gamma radiation
    D T Jones
    Department of Hematology, Royal Free and University College Medical School, Royal Free Campus, London, United Kingdom
    Blood 98:2800-7. 2001
    ..However, Fas-independent activation of caspase 8 may play a crucial role in the regulation of apoptosis in these cells...
  31. ncbi Multiple myeloma: causes and consequences of delay in diagnosis
    C C Kariyawasan
    Department of Academic Haematology, Royal Free Hospital and University College Medical School, London NW3 2QG, UK
    QJM 100:635-40. 2007
    ..Myeloma is a serious and usually fatal haematological malignancy with reported mortality of 10-20% within the first 2 months of presentation. Symptoms are non-specific, and patients thus present to a range of medical practitioners...
  32. ncbi Vascular complications of Fabry disease: enzyme replacement and other therapies
    D A Hughes
    Department of Academic Haematology, Royal Free and University College Medical School, London, UK
    Acta Paediatr Suppl 94:28-33; discussion 9-10. 2005
    ..Preliminary evidence suggests that ERT may have beneficial effects on the vascular component of this multisystem disease...
  33. ncbi New developments in the management of Anderson-Fabry disease
    A Mehta
    Department of Haematology, Royal Free Hospital, London
    QJM 95:647-53. 2002
    ..Treatment was formerly entirely symptomatic, but enzyme replacement therapy has recently been licensed and management is evolving from genetic counselling and palliative care to early diagnosis and active intervention...
  34. doi The impact of dose escalation and resistance modulation in older patients with acute myeloid leukaemia and high risk myelodysplastic syndrome: the results of the LRF AML14 trial
    Alan K Burnett
    Department of Haematology, School of Medicine, Cardiff University Heath Park, Cardiff, UK
    Br J Haematol 145:318-32. 2009
    ..In conclusion, these four interventions have not improved outcomes in older patients. New agents need to be explored and novel trial designs are required to maximise prospects of achieving timely progress...
  35. doi Molecular analysis of novel PROP1 mutations associated with combined pituitary hormone deficiency (CPHD)
    D Kelberman
    Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, Institute of Child Health, London, UK
    Clin Endocrinol (Oxf) 70:96-103. 2009
    ..We have ascertained three pedigrees with PROP1 mutations from a large cohort of patients with variable degrees of CPHD who were screened for mutations in PROP1...
  36. ncbi Case 3. Fabry disease
    S Eisman
    Department of Dermatology and Haematology, Royal Free Hospital, London, UK
    Clin Exp Dermatol 28:569-70. 2003
  37. ncbi Gastrointestinal symptoms in 342 patients with Fabry disease: prevalence and response to enzyme replacement therapy
    Bjoern Hoffmann
    Department of General Pediatrics, University Children s Hospital, Heinrich Heine University, Duesseldorf, Germany
    Clin Gastroenterol Hepatol 5:1447-53. 2007
    ....
  38. ncbi Clinical manifestations of Fabry disease in children: data from the Fabry Outcome Survey
    Uma Ramaswami
    Department of Paediatric Endocrinology, Diabetes and Metabolism, Addenbrooke s Hospital, Cambridge, UK
    Acta Paediatr 95:86-92. 2006
    ..This produces progressive lysosomal accumulation of globotriaosylceramide throughout the body, leading to organ failure and premature death...
  39. ncbi Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey
    Ales Linhart
    Second Department of Internal Medicine, First Faculty of Medicine, Charles University, Prague, Czech Republic
    Eur Heart J 28:1228-35. 2007
    ..The aim of this study was to determine the prevalence and characteristics of cardiac disease in AFD patients...
  40. ncbi Agalsidase alfa: specific treatment for Fabry disease
    Atul Mehta
    Royal Free Hospital, London NW3 2QG
    Hosp Med 63:347-50. 2002
    ..The recent availability of enzyme-replacement therapy with agalsidase alfa offers specific treatment for this serious, progressive condition...
  41. ncbi Middelheim Fabry Study (MiFaS): a retrospective Belgian study on the prevalence of Fabry disease in young patients with cryptogenic stroke
    Raf Brouns
    Department of Neurology and Memory Clinic, ZNA Middelheim General Hospital, Antwerp, Belgium
    Clin Neurol Neurosurg 109:479-84. 2007
    ..To assess the prevalence of Fabry disease in young patients with cryptogenic stroke...
  42. ncbi Nature and prevalence of pain in Fabry disease and its response to enzyme replacement therapy--a retrospective analysis from the Fabry Outcome Survey
    Bjoern Hoffmann
    University Children s Hospital, Heinrich Heine University Duesseldorf, Germany
    Clin J Pain 23:535-42. 2007
    ..Fabry disease is a multisystemic life-threatening lysosomal storage disorder caused by deficiency of alpha-galactosidase A. Symptoms of the disease may occur in different organs including kidney, heart, and the nervous system...
  43. doi Enzyme replacement therapy in Fabry disease: comparison of agalsidase alfa and agalsidase beta
    Atul Mehta
    Mol Genet Metab 95:114-5. 2008
  44. doi The binary endocardial appearance is a poor discriminator of Anderson-Fabry disease from familial hypertrophic cardiomyopathy
    Stavros Kounas
    Heart Hospital, University College London, London, United Kingdom
    J Am Coll Cardiol 51:2058-61. 2008
    ..We compared the frequency of a binary endocardial appearance in patients with hypertrophic cardiomyopathy (HCM) and Anderson-Fabry disease (AFD)...
  45. ncbi Home therapy for lysosomal storage disorders
    Derryalynn A Hughes
    Department of Academic Haematology, Royal Free Hospital and University College Medical School, London, UK
    Br J Nurs 16:1384, 1386-9. 2007
    ..Home therapy requires a well organized and regulated community infrastructure, individual assessments of patient suitability and protocols for management of possible infusion associated reactions...
  46. ncbi Thirty-four novel mutations of the GLA gene in 121 patients with Fabry disease
    Ellen Schäfer
    Institute of Human Genetics, University Hospital Eppendorf, Hamburg, Germany
    Hum Mutat 25:412. 2005
    ..6%), deletions (17.8%) or insertions/duplications (5.6%) of a few nucleotides, and complex rearrangements including larger deletions (2.2%). GLA mutations were identified in 82 (97.6%) of the 84 unrelated male patients...
  47. ncbi Enzyme replacement in Fabry disease: pharmacokinetics and pharmacodynamics of agalsidase alpha in children and adolescents
    Markus Ries
    Developmental and Metabolic Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 3D03, 9000 Rockville Pike, Bethesda, MD 20892 1260, USA
    J Clin Pharmacol 47:1222-30. 2007
    ..Except for clearance in younger patients, agalsidase alpha appears to have comparable pharmacokinetic and pharmacodynamic profiles in pediatric and adult Fabry patients of both genders...
  48. ncbi Anemia is a new complication in Fabry disease: data from the Fabry Outcome Survey
    Julia Kleinert
    Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria
    Kidney Int 67:1955-60. 2005
    ..The prevalence and causes of anemia among patients with Fabry disease are unknown...
  49. ncbi Effect of anti-CD20 (rituximab) on resistant thrombocytopenia in autoimmune lymphoproliferative syndrome
    Bridget T Heelan
    Department of Immunology, Royal Free Hospital, Hampstead, London, UK
    Br J Haematol 118:1078-81. 2002
    ..We describe a patient with a defect in the death domain of the FAS molecule who had autoimmune thrombocytopenia resistant to conventional therapy but which responded to a combination of rituximab and vincristine...
  50. ncbi A novel treatment approach for low grade lymphoproliferative disorders using PKC412 (CGP41251), an inhibitor of protein kinase C
    Andres Virchis
    Department of Haematology, The Royal Free and University College School of Medicine, Royal Free Campus, University College, London, UK
    Hematol J 3:131-6. 2002
    ..We have undertaken a single centre, open-label, multi-dose, exploratory Phase II clinical trial of PKC412 in patients with CLL and low grade NHL...
  51. ncbi Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. Evidence for a disease specific abnormality of the myocardial interstitium
    James C C Moon
    Centre for Advanced Magnetic Resonance in Cardiology CAMRIC, Royal Brompton Hospital, London, UK
    Eur Heart J 24:2151-5. 2003
    ..Gadolinium enhanced cardiovascular magnetic resonance can detect focal myocardial fibrosis. We hypothesised that hyperenhancement would be present in AFD...
  52. ncbi Three-dimensional face shape in Fabry disease
    Josanne Cox-Brinkman
    Department of Paediatrics, Academic Medical Center, University Hospital of Amsterdam, Amsterdam, The Netherlands
    Eur J Hum Genet 15:535-42. 2007
    ..However, because discrimination from healthy controls is too low, no key role in the diagnostic process can be expected...
  53. ncbi Gaucher cells demonstrate a distinct macrophage phenotype and resemble alternatively activated macrophages
    Leonie A Boven
    Department of Immunology, Erasmus Medical Center and MS Center ErasMS, Rotterdam, The Netherlands
    Am J Clin Pathol 122:359-69. 2004
    ..Thus, GCs represent a distinctive population of myeloid cells that resemble aamphi but differ from previously described in vitro aamphi...
  54. ncbi Evidence for motor axon depolarization in Fabry disease
    Stella V Tan
    Department of Clinical Neurophysiology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, United Kingdom
    Muscle Nerve 32:548-51. 2005
    ..These findings indicate that the axons were mildly depolarized, probably due to ischemia, and are consistent with the hypothesis that poor nerve perfusion in Fabry disease contributes to axonal damage...
  55. ncbi Prevalence of uncontrolled hypertension in patients with Fabry disease
    Julia Kleinert
    Division of Nephrology and Dialysis, Department of Medicine III, Medical University Vienna, Vienna, Austria
    Am J Hypertens 19:782-7. 2006
    ..The prevalence of uncontrolled hypertension, as well as the effect of enzyme replacement therapy on BP, in patients with Fabry disease is unknown...
  56. pmc Ocular manifestations of Fabry's disease: data from the Fabry Outcome Survey
    Andrea Sodi
    Department of Ophthalmology, University of Florence, Clinica Oculistica, Policlinico di Careggi, Viale Morgagni 85, 50134 Firenze, Italy
    Br J Ophthalmol 91:210-4. 2007
    ..Fabry's disease is an X-linked lysosomal storage disorder characterised by deficient activity of the enzyme alpha-galactosidase A...