Professional background

Dr Sian Hughes is a consultant pathologist at UCLH and an honorary associate professor at University College London (UCL). She has trained and worked in several of the major London teaching hospitals.

Dr Hughes graduated in medicine in 1989 from King's College Hospital School of Medicine and Dentistry (London) and completed an MSc in experimental pathology with Distinction at the Royal Postgraduate Medical School (London) before undertaking her PhD at United Medical and Dental Schools (UMDS, London) in molecular biology investigating the role of growth factors in cardiovascular disease.

Dr Hughes trained in pathology at Guy's and St Thomas' Hospital (London) and undertook her training in dermatopathology at the internationally recognised St John's Institute of Dermatology. Dr Hughes was a clinical senior lecturer/honorary consultant in cardiovascular pathology at St George's Hospital Medical School (London) prior to her appointment at UCLH in 2002.

Dr Hughes provides essential cancer diagnostic services for dermatopathology as well as the reporting of inflammatory dermatoses and skin biopsies with an infectious aetiology from the Hospital for Tropical Diseases. In addition, to her clinical and academic practice, Dr Hughes is involved with the teaching and training of doctors pursuing a career in pathology, acting as an educational supervisor. She also takes an active role in mentoring and teaching the next generation of doctors: Dr Hughes is a personal tutor for UCL and provides lectures in cardiac disease to undergraduate MBBS students.

Research interests

Dr Hughes' main clinical and research interests are dermatopathology and cardiac pathology. She has published both book chapters and peer reviewed articles in the fields of cardiac and skin disease. Sian has also reviewed 'Atherosclerosis' for the NHS A-Z of Treatments providing up to date and accurate information for the public.

Publications

  • 'Expression of the Fibroblast Frowth Factor (FGF) and Fibroblast Frowth Factor Receptor (FGFR) Multigene Families in Normal and Atherosclerotic Human Arteries'. In Fibroblast Growth Factor in the Cardiovascular System. Ed: Cuevas P, I.Holzapfel Verlag 2002. Hughes SE. Cardiac stem cells. J Pathol, (Annual Review Issue on “Stem Cells in Pathology and Pathobiology”) 2002;197:468-478.
  • Hughes SE. Detection of apoptosis by in situ markers for DNA strand breaks in the failing human heart. Fact or epiphenomenon? J Pathol 2003;201:181-6.Hughes SE. The pathology of hypertrophic cardiomyopathy. Histopathology 2004;44:412-27.
  • Murphy RT, Mogensen J, Shaw A, Kubo T, Hughes S, McKenna WJ. Novel mutation in cardiac troponin I in recessive idiopathic dilated cardiomyopathy. Lancet 2004;363:371-372.
  • Hughes SE, McKenna WJ. New insights into the pathology of inherited cardiomyopathy. Heart 2005;91:257-264.Murphy RT, Mogensen J, McGarry K, Bahl A, Evans A, Osman E, Syrris P, Gorman G, Farrell M, Holton JL, Hanna MG, Hughes S, Elliott PM, MacRae CA, McKenna WJ. Adenosine monophosphate-activated protein kinase disease mimicks hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome. J Am Coll Cardiol 2005;45:922-930.
  • Arno G, Kaski JC, Smith DA, Akiyu JP, Hughes SE, Baboonian C. Matrix metalloproteinase-9 expression is associated with presence of Chlamydia pneumoniae in coronary atherosclerotic plaques. Heart 2005;91:521-525.
  • Chen-Scarabelli C, Hughes SE, Landon G, Rowley P, Allebban Z, Lawson N, Saravolatz, Latchman D, Scarabelli T. A case of fatal ephedra intake associated with lipofucsin accumulation, caspase activation and cleavage of myofibrillary proteins. Eur J Heart Failure 2005;7:927-930. 
  • Norman N, Simpson M, Mogensen J, Shaw A, McKoy G, Rowland E, Hughes S, Crosby A, McKenna WJ A novel desmoplakin mutation causes arrhythmogenic left ventricular cardiomyopathy. Circulation 2005 112:636-42.
  • Zhang JC, Hughes SE. Role of the ephrin and Eph receptor tyrosine kinase (RTK) families in angiogenesis and development of the cardiovascular system. J Pathol 2006 208:453-461.
  • Hughes SE. Heart muscle disease. MEDICINE 2006;34:207-209.Al-Mahdawi S, Pinto RM, Varshney D, Lawrence L, Lowrie MB, Hughes S, Webster Z, Blake J, Cooper JM, King R, Pook MA. GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology. Genomics 2006 88:580-590.
  • Hughes SE and Hall PA. Editorial - The fibroblast growth factor and receptor multigene families. J Pathol 1993;170:219-221.Hughes SE, Crossman D, Hall PA. Expression of basic and acidic fibroblast growth factors and their receptor in normal and atherosclerotic human arteries. Cardiovasc Res 1993;27:1214-1219.
  • Hughes SE and Hall PA. Commentary - Overview of the fibroblast growth factor and receptor families: complexity, functional diversity, and implications for future cardiovascular research. Cardiovasc Res 1993;27:1199-1203.
  • Hughes SE and Hall PA. Immunolocalization of fibroblast growth factor receptor 1 and its ligands in human tissues. Lab Invest 1993;71:173-182.Hughes SE. Functional characterization of the spontaneously transformed human umbilical vein endothelial cell line ECV304: use in an in vitro model of angiogenesis. Exp Cell Res 1996;225:171-185.
  • Hughes SE. Localisation and differential expression of the fibroblast growth factor receptor (FGFR) multigene family in normal and atherosclerotic arteries. Cardiovasc Res 1996;32:557-569.
  • Hughes SE. Differential expression of the fibroblast growth factor receptor (FGFR) multigene family in normal human adult tissues. J Histochem Cytochem 1997;45:1005-1019.
  • Hughes SE. Heart muscle disease. MEDICINE 2002;30:89-91.Chapter 5: ‘The Role of the Pathologist in the Diagnosis of Cardiomyopathy . A Personal View’. In Progress in Pathology Volume 7. Eds: Kirkham N, Shepherd NA. Cambridge University Press 2007. 
  • Syrris P, Ward D, Asimaki A, Evans A, Sen-Chowdhry S, Hughes SE, McKenna WJ. Desmoglein-2 mutations in arrhythmogenic right ventricular cardiomyopathy: a genotype-phenotype characterization of familial disease. Eur Heart J 2007;28:581-8 
  • Suri A, Moon JC, Dhamrait SS, Hughes S, Holdright D. Cardiac amyloid by cardiovascular magnetic resonance. Heart 2007:93:1496
  • Tsang VT, Kilner PJ, Hsia TY, Hughes S, Yacoub M. Interruption of the aorta with multilobulated arch aneurysms: a new clinicopathologic entity. J Thorac CardiovascSurg 2007;133:1092-3.
  • Ffolkes LV, Brull D, S Krywawych, Hayward M, Hughes SE. Aortic stenosis in cardiovascular ochronosis. J Clin Pathol 2007;60:92-93.Behr
  • ER, Dalageorgou C, Christiansen M, Syrris P, Hughes S, Tome Esteban MT, Rowland E, Jeffry S, McKenna WJ. Sudden arrhythmic death syndrome: familial evaluation identifies inheritable heart disease in the majority of families. Eur Heart J 2008;29:1670-80.
  • Sen-Chowdhry S, Syrris P, Prasad SK, Hughes SE, Merrifield R, Ward D, Pennell DJ, McKenna WJ. Left-dominant arrhythmogenic cardiomyopathy: an underrecognized clinical entity. J Am Coll Cardiol 2008:52:2175-87.#
  • Hughes C, McEwan JR, Longair I, Hughes S, Cohen H, Machin S, Scully M. Cardiac involvement in acute thrombotic thrombocytopenic purpura:association with troponin T and IgG antibodies to ADAMTS 13. J Thromb Haemost 2009 7;529-36.
  • Rudiger A, Dyson A, Felsmann K, Carre JE, Taylor V, Hughes S, Clatworthy I, Protti A, Pellerin D, Lemm J, Klaus RA, Bauer M, Singer M. Early functional and transcriptomic changes in the myocardium predict outcome in a long-term rat model of sepsis. Clinical Science 2013;124:391-401.
  • Nandagudi A, Jury EC, Alonzi D, Butters TD, Hughes S, Isenberg DA. Heart failure in a woman with SLE, antiphospholipid syndrome and Fabry’s disease. Lupus 2013;10:1070-1076.
  • Chapter 9: ‘Valvular Heart Disease’ and Chapter 11:‘Cardiomyopathies’. In Cardiac Pathology. A Guide to Current Practice, Ed: Suvarna, SK. Springer-Verlag 2013.Primary eccrine carcinoma treated with Mohs micrographic surgery and adjuvant radiotherapy. Chandrakumar A, Veitch D, Proctor I, Hughes S, Perrett CM. Clinical and Experimental Dermatology 2014.
  • A rare colocalization of lichen planus and vitiligo. Veitch D, Kravvas G, Hughes S, Bunker C. Case Reports in Dermatological Medicine;Volume 2015:1-3.
  • Another face of demodicosis. Noy ML, Hughes S, Bunker CB. Clinical and Experimental Dermatology 2016;41:958-959.Localized genital bullous pemphigoid. Mounsey SJ, Heelan K, Hughes S, Fawcett H, Bunker CB. Clinical and Experimental Dermatology; 2018;43:810-812.
  • Platelet-derived transforming growth factor-?1 promotes keratinocyte proliferation in cutaneous wound healing. Chong DWL, Trinder S, Labelle M, Rodriguez-Justo M, Hughes S, Holmes AM, Scotton CJ, Porter JC. J Tiss Eng Regen Med; 2020;14:645-649 .
  • Long- term adaptive versus maladaptive remodelling of the pulmonary autograft after the Ross operation. Yacoub MH, Tsang V, Sarathchandra P, Jensen H, Hughes S, Latif N. Eur J Cardiothoracic Surg; 2020;57:977-985.