Publications
204 results found
Bartram J, Old J, Phelan L, et al., 2012, Novel Haemoglobin Variant [β42 Phe → Cys]: Hb Little Venice, 54th Annual Meeting and Exposition of the American-Society-of-Hematology (ASH), Publisher: AMER SOC HEMATOLOGY, ISSN: 0006-4971
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- Citations: 1
Uttenthal BJ, Layton DM, Vyse TJ, et al., 2012, Clinical problem-solving. The wolf at the door., N Engl J Med, Vol: 366, Pages: 2216-2221
Uttenthal BJ, Layton DM, Vyse TJ, et al., 2012, The Wolf at the Door, NEW ENGLAND JOURNAL OF MEDICINE, Vol: 366, Pages: 2216-2221, ISSN: 0028-4793
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- Citations: 11
Zhang EG, Regan F, Layton M, et al., 2011, Managing the difficult case of fetal anemia, JOURNAL OF MATERNAL-FETAL & NEONATAL MEDICINE, Vol: 24, Pages: 1498-1503, ISSN: 1476-7058
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- Citations: 2
Gilmore A, Cho G, Howard J, et al., 2011, Feasibility and benefit of hydroxycarbamide as a long-term treatment for sickle cell disease patients: Results from the North West London Sickle Cell Disease Registry, AMERICAN JOURNAL OF HEMATOLOGY, Vol: 86, Pages: 958-961, ISSN: 0361-8609
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- Citations: 10
Tso ACY, Roper DR, Wong CL, et al., 2011, Splenic infarction in a patient with sickle cell trait and hereditary spherocytosis, AMERICAN JOURNAL OF HEMATOLOGY, Vol: 86, Pages: 695-696, ISSN: 0361-8609
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- Citations: 5
Chase AR, Sohal M, Howard J, et al., 2010, Pregnancy outcomes in sickle cell disease: a retrospective cohort study from two tertiary centres in the UK., Obstet Med, Vol: 3, Pages: 110-112, ISSN: 1753-495X
The objective of this retrospective cohort study from two tertiary centres in the UK was to describe the pregnancy outcomes of women with sickle cell disease (SCD) who booked at these centres between 2004 and 2008, and to compare this with historical data. The study population comprised 122 singleton pregnancies in women with SCD: homozygous sickle cell disease 64, sickle cell haemoglobin C disease 45, sickle b plus thalassaemia 11, sickle cell haemoglobin E disease 1 and sickle cell delta disease 1 from 2004 to 2008 managed in the joint haematology/obstetric antenatal clinics in two tertiary teaching hospitals. The main outcome measures were the frequency of sickle cell crises and obstetric complications. Age and gestation at booking were 18-43 years (mean 29.7) and 9-36 weeks gestation (mean 17.3), respectively. Complications of SCD occurred in 25% of pregnancies. Fifty-four percent of women had induction of labour and 39% were delivered by emergency caesarean section. Thirty-three percent had a postpartum haemorrhage. Nineteen percent of women delivered before 37 completed weeks. Birth weight below 2500 g occurred in 20% of singleton pregnancies. Three neonates developed transient complications related to maternal opiate exposure postnatally. Three intrauterine deaths occurred at 24, 29 and 34 weeks. Two of these had congenital defects, and the other severe intrauterine growth restriction. No maternal deaths occurred. Successful pregnancy outcomes can be achieved in SCD. There has been an improvement in fetal and maternal morbidity and mortality compared with historical data. Pregnancy in women with SCD remains high risk. Early access to antenatal care and to expertise in SCD is essential. A matched control population from the same time period and prospective data collection is needed to address confounders such as ethnicity and deprivation.
Costa J, Nolasco M, Silva G, et al., 2010, GATA-1 MEDIATES PIGM TRANSCRIPTION IN IGD AND NORMAL ERYTHROID TISSUES, Publisher: FERRATA STORTI FOUNDATION, Pages: 33-34, ISSN: 0390-6078
Costa J, Nolasco M, Silva G, et al., 2010, GATA-1 MEDIATES PIGM TRANSCRIPTION IN IGD AND NORMAL ERYTHROID TISSUES, 15th Annual Meeting of the European-Hematology-Association, Publisher: FERRATA STORTI FOUNDATION, Pages: 33-34, ISSN: 0390-6078
Khalique Z, Pavlu J, Lefroy D, et al., 2010, Erythrocytapheresis in the prevention of recurrent myocardial infarction in sickle cell disease, AMERICAN JOURNAL OF HEMATOLOGY, Vol: 85, Pages: 91-91, ISSN: 0361-8609
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- Citations: 5
Georgiou E, Layton M, Karadimitris A, 2009, Inherited GPI Deficiency: A Disorder of Histone Hypoacetylation, BIRTH DEFECTS RESEARCH PART C-EMBRYO TODAY-REVIEWS, Vol: 87, Pages: 327-334, ISSN: 1542-975X
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- Citations: 2
Cabrita IZ, Abubakar M, Layton DM, et al., 2009, Survival of patients with pulmonary hypertension and sickle cell disease in the UK, EUROPEAN HEART JOURNAL, Vol: 30, Pages: 258-259, ISSN: 0195-668X
Cabrita IZ, Ruisanchez C, Layton M, et al., 2009, Cardiovascular complications in sickle cell disease:echocardiographic features, EUROPEAN HEART JOURNAL, Vol: 30, Pages: 57-57, ISSN: 0195-668X
Almeida A, Layton M, Karadimitris A, 2009, Inherited glycosylphosphatidyl inositol deficiency: A treatable CDG, BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE, Vol: 1792, Pages: 874-880, ISSN: 0925-4439
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- Citations: 32
Almeida A, Layton M, Karadimitris A, 2009, Inherited GPI Deficiency, ENZYMES, VOL 26: GLYCOSYLPHOSPHATIDYLINOSITOL (GPI) ANCHORING OF PROTEINS, Editors: Menon, Orlean, Kinoshita, Tamanoi, Publisher: ELSEVIER ACADEMIC PRESS INC, Pages: 357-373, ISBN: 978-0-12-374963-5
Costa JS, Nolasco M, Almeida AM, et al., 2008, GATA-1 Enhances the Transcription of <i>PIGM</i> and Is Responsible for Increased GPI Expression in Erythrocytes in Inherited Glycosylphosphatidylinositol Deficiency: A Model for Tissue-Specific Housekeeping Gene Transcription Regulation, 50th Annual Meeting of the American-Society-of-Hematology, Publisher: AMER SOC HEMATOLOGY, Pages: 1181-1181, ISSN: 0006-4971
Bhavnani M, Braithwaite J, Burthem J, et al., 2008, British Society for Haematology, slide session, Annual Scientific Meeting, Bournemouth, 2007, INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Vol: 30, Pages: 95-104, ISSN: 1751-5521
McDonald V, Benjamin S, Bevan D, et al., 2008, Thrombotic thrombocytopenic purpura precipitated by acute pancreatitis, 48th Annual Scientific Meeting of the British-Society-for-Haematology, Publisher: WILEY-BLACKWELL, Pages: 78-79, ISSN: 0007-1048
Sohal M, Davies JO, Howard J, et al., 2008, Pregnancy in sickle cell disease (SCD): experience in a single tertiary centre, 48th Annual Scientific Meeting of the British-Society-for-Haematology, Publisher: BLACKWELL PUBLISHING, Pages: 111-111, ISSN: 0007-1048
Smith TG, Brooks JT, Balanos GM, et al., 2008, Mutation of the von Hippel-Lindau gene alters human cardiopulmonary physiology, INTEGRATION IN RESPIRATORY CONTROL: FROM GENES TO SYSTEMS, Vol: 605, Pages: 51-56, ISSN: 0065-2598
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- Citations: 16
Smith TG, Brooks JT, Balanos GM, et al., 2008, Mutation of the von Hippel-Lindau gene alters human cardiopulmonary physiology, 10th Oxford Conference on Modeling and Control of Breathing, Pages: 51-56
Intracellular responses to hypoxia are coordinated by the von Hippel-Lindau - hypoxia-inducible factor (VHL-HIF) transcriptional system. This study investigated the potential role of the VHL-HIF pathway in human systems-level physiology. Patients diagnosed with Chuvash polycythaemia, a rare disorder in which VHL signalling is specifically impaired, were studied during acute hypoxia and hypercapnia. Subjects breathed through a mouthpiece and ventilation was measured while pulmonary vascular tone was assessed echocardiographically. The patients were found to have elevated basal ventilation and pulmonary vascular tone, and ventilatory, pulmonary vasoconstrictive and heart rate responses to acute hypoxia were greatly increased, as were heart rate responses to hypercapnia. The patients also had abnormal pulmonary function on spirometry. This study's findings demonstrate that the VHL-HIF signalling pathway, which is so central to intracellular oxygen sensing, also regulates the organ systems upon which cellular oxygen delivery ultimately depends.
Hepburn AL, Lampert IA, Boyle JJ, et al., 2007, In vivo evidence for apoptosis in the bone marrow in systemic lupus erythematosus, ANNALS OF THE RHEUMATIC DISEASES, Vol: 66, Pages: 1106-1109, ISSN: 0003-4967
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- Citations: 42
Almeida AM, Murakami Y, Baker A, et al., 2007, Brief report: Targeted therapy for inherited GPI deficiency., NEW ENGLAND JOURNAL OF MEDICINE, Vol: 356, Pages: 1641-1647, ISSN: 0028-4793
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- Citations: 67
Eccersley LRJ, Moule SP, Abrahamson G, et al., 2007, Dysplastic haematopoiesis in untreated chronic lymphocytic leukaemia (CLL), 47th Annual Scientific Meeting of the British-Society-for-Haematology, Publisher: BLACKWELL PUBLISHING, Pages: 21-21, ISSN: 0007-1048
Percy MJ, Crowley LJ, Roper D, et al., 2007, Type I recessive congenital methaemoglobinaemia associated with a triple mutation of NADH-cytochrome b5 reductase, 47th Annual Scientific Meeting of the British-Society-for-Haematology, Publisher: BLACKWELL PUBLISHING, Pages: 86-86, ISSN: 0007-1048
Percy MJ, Crowley LJ, Layton M, et al., 2007, A novel Pro92His, and previously described, Glu255-, double mutation of NADH-cytochrome b5 reductase associated with apparent type I RCM, Experimental Biology 2007 Annual Meeting, Publisher: FEDERATION AMER SOC EXP BIOL, Pages: A1044-A1044, ISSN: 0892-6638
Pavlu J, Ahmed RE, O'Regan DP, et al., 2007, Myocardial infarction in sickle-cell disease, LANCET, Vol: 369, Pages: 246-246, ISSN: 0140-6736
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- Citations: 24
Murakarni Y, Almeida A, Layton M, et al., 2007, A point mutation in an Sp1 binding motif in the promoter of the mannosyltransferase-encoding <i>PIG-M</i> gene causes inherited glycosylphosphatidylinositol deficiency, 21st International Complement Workshop, Publisher: PERGAMON-ELSEVIER SCIENCE LTD, Pages: 218-218, ISSN: 0161-5890
Almeida AM, Murakami Y, Baker A, et al., 2006, Targeted molecular therapy for inherited glycosylphosphatidylinositol deficiency., 48th Annual Meeting of the American-Society-of-Hematology, Publisher: AMER SOC HEMATOLOGY, Pages: 148A-148A, ISSN: 0006-4971
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- Citations: 1
Smith TG, Brooks JT, Balanos GM, et al., 2006, Mutation of von Hippel-Lindau tumour suppressor and human cardiopulmonary physiology, PLOS MEDICINE, Vol: 3, Pages: 1178-1186, ISSN: 1549-1277
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- Citations: 155
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