Professor Nick Bishop

Professor Nick BishopDepartment of Oncology & Metabolism
Academic Unit of Child Health
Sheffield Children's NHS Foundation Trust
Sheffield Children's Hospital
Room 1, Damer Street Building
Sheffield S10 2TH

Office: Room 3

Tel: +44 (0)114 226 0716
Email: n.j.bishop@sheffield.ac.uk

Secretary:
Gillian Griffiths
Email: g.l.griffiths@sheffield.ac.uk
Tel: 0114 215 9048/226 0716

Professor of Paediatric Bone Disease

The UK's only Professor of Paediatric Bone Disease. Trained in Manchester (clinical), Cambridge (MRC and Wellcome Fellowships) and Montreal(visiting Professor at McGill). Appointed to Chair in Sheffield in 1998. Clinical research group focuses on treatment of childhood bone fragility and rare bone diseases; basic science group on early life events and skeletal development, as well as pathophysiology of childhood bone diseases.

Research

Our research aims to improve outcomes for children with inherited and acquired bone diseases and understand better the factors contributing to fracture in apparently healthy children.

We are working with colleagues locally and nationally to determine the phenotype-genotype relationships in early onset bone fragility disorders, and to develop a pipeline to bring forward new therapies in these disorders.

Our previous clinical studies of the use of bisphosphonates in osteogenesis imperfecta (OI) showed that bone mass increases in a dose-responsive manner in moderate-severely affected children without a clear reduction in fracture risk; and that mildly affected children can reduce fracture risk by approximately 50% when taking the oral bisphosphonate risedronate. We recruited the first UK patient to the Amgen-funded study of Denosumab in OI. We participated in the global studies of asfotase alfa, a recombinant enzyme replacement therapy for hypophosphatasia (HPP), leading in Europe initially with children travelling from other EU countries to participate.

We have developed a new biomarker to assess the acute skeletal response to mechanical stimulation that is now being used in prospective studies of children with fracture and OI.

In collaboration with colleagues nationally, we are assessing the effects of antenatal vitamin D supplementation on skeletal development and health; we have developed a model system of antenatal vitamin D depletion in which we are assessing the postnatal response to mechanical loading.

Internationally, we are developing the European Reference Network for Rare Bone Diseases that will deliver improved health care outcomes for patients with skeletal disorders such as OI and HPP, translating our research into clinical practice.

Principal funding sources

  • MRC
  • NIHR
  • Arthritis Research Campaign
  • Sir Halley Stewart Trust
  • Amgen
  • Alexion Pharma

Representative Publications

Bishop N, Harrison R, Ahmed F, Shaw N, Eastell R, Campbell M, Knowles E, Hill C, Hall C, Chapman S, Sprigg A, Rigby A.  A Randomised Controlled Dose-Ranging Study of Risedronate in Children with Moderate and Severe Osteogenesis Imperfecta.  J Bone Miner Res. 2010 Jan;25(1):32-40

Arundel P, Offiah A, Bishop NJ Evolution of the radiographic appearance of the metaphyses over the first year of life in type V osteogenesis imperfecta: clues to pathogenesis. J Bone Miner Res. 2011 Apr;26(4):894-8. DOI: 10.1002/jbmr.258

Parker MJ, Deshpande C, Rankin J, Wilson LC, Balasubramanian M, Hall CM, Wagner BE, Pollitt R, Dalton A, Bishop NJ. Type 1 collagenopathy presenting with a Russell-Silver phenotype. Am J Med Genet A. 2011 Jun;155(6):1414-8. doi: 10.1002/ajmg.a.33998.

Whyte MP, Greenberg CR, Salman NJ, Bober MB, McAlister WH, Wenkert D, Van Sickle BJ, Simmons JH, Edgar TS, Bauer ML, Hamdan MA, Bishop N, Lutz RE, McGinn M, Craig S, Moore JN, Taylor JW, Cleveland RH, Cranley WR, Lim R, Thacher TD, Mayhew JE, Millan JL, Skrinar AM, Crine P, Landy H. Enzyme Replacement For Life-Threatening Hypophosphatasia. N Engl J Med. 2012 Mar 8;366(10):904-13.

Nicholas C Harvey, Kassim Javaid, Nicholas Bishop, Stephen Kennedy, Aris T Papageorghiou, Robert Fraser, Saurabh V Gandhi, Inez Schoenmakers, Ann Prentice, Cyrus Cooper. MAVIDOS Maternal Vitamin D Osteoporosis Study: study protocol for a randomized controlled trial. Trials 2012;13:13

Nick Bishop, MD, Silvano Adami, MD, S. Faisal Ahmed, MD, Jordi Antón, MD, Paul Arundel, MD, Christine P. Burren, MD, Jean-Pierre Devogelaer, MD, Thomas Hangartner, PhD, Eva Hosszú, MD, Joseph M. Lane, MD, Roman Lorenc, MD, Outi Mäkitie, MD, Craig F. Munns, MD, Ana Paredes, MD, Helene Pavlov, MD, Horacio Plotkin, MD, Cathleen L. Raggio, MD, Maria Loreto Reyes, MD, Eckhard Schoenau, MD, O. Semler, MD, David O. Sillence MD, Robert D. Steiner, MD.
Randomised, double-blind, placebo-controlled trial of risedronate in children with osteogenesis imperfecta. Lancet 2013 http://dx.doi.org/10.1016/S0140-6736(13)61091-0

Meena Balasubramanian, Michael J. Parker, Ann Dalton, Cecilia Giunta, Uschi Lindert, Luiz C. Peres, Bart E. Wagner, Paul Arundel, Amaka Offiah and Nicholas J. Bishop.
Genotype–phenotype study in type V osteogenesis imperfecta. Clinical Dysmorphology 2013 Jul;22(3):93-101. DOI: 10.1097/MCD.0b013e32836032f0

Balasubramanian M, Wagner BE, Peres LC, Sobey GJ, Parker MJ, Dalton A, Arundel P, Bishop NJ. Ultrastructural and histological findings on examination of skin in osteogenesis imperfecta: a novel study. Clin Dysmorphol. 2015 Apr;24(2):45-54. doi: 10.1097/MCD.0000000000000066.

Balasubramanian M, Pollitt RC, Chandler KE, Mughal MZ, Parker MJ, Dalton A, Arundel P, Offiah AC, Bishop NJ. CRTAP mutation in a patient with Cole-Carpenter syndrome. Am J Med Genet A. 2015 Mar;167(3):587-91. doi: 10.1002/ajmg.a.36916. Epub 2015 Jan 21.

Harrison R, Ward K, Lee E, Razaghi H, Horne C, Bishop NJ. Acute bone response to whole body vibration in healthy pre-pubertal boys. J Musculoskelet Neuronal Interact. 2015 Jun;15(2):112-22.

Balasubramanian M, Cartwright A, Smith K, Arundel P, Bishop NJ. Copy number variants in association with type 1 collagenopathy: Atypical osteogenesis imperfecta. Am J Med Genet A. 2015 Oct 15. doi: 10.1002/ajmg.a.37431.

Whyte MP, Rockman-Greenberg C, Ozono K, Riese R, Moseley S, Melian A, Thompson D, Bishop N, Hofmann C; study ENB-002–08, ENB-003–08, ENB-010–10ENB-011–10 Investigators.
Asfotase Alfa Treatment Improves Survival for Perinatal and Infantile Hypophosphatasia. J Clin Endocrinol Metab. 2015 Nov 3:jc20153462.

Vasanwala RF, Sanghrajka A, Bishop NJ, Högler W. Recurrent Proximal Femur Fractures in a Teenager with Osteogenesis Imperfecta on Continuous Bisphosphonate Therapy: Are we Overtreating? J Bone Miner Res. 2016 Feb 4. doi: 10.1002/jbmr.2805

Cooper C, Harvey NC, Bishop NJ, Kennedy S, Papageorghiou AT, Schoenmakers I, Fraser R, Gandhi SV, Carr A, D'Angelo S, Crozier SR, Moon RJ, Arden NK, Dennison EM, Godfrey KM, Inskip HM, Prentice A, Mughal MZ, Eastell R, Reid DM, Javaid MK; MAVIDOS Study Group.
Maternal gestational vitamin D supplementation and offspring bone health (MAVIDOS): a multicentre, double-blind, randomised placebo-controlled trial. Lancet Diabetes Endocrinol. L 2016 Mar 1. pii: S2213-8587(16)00044-9. doi: 10.1016/S2213-8587(16)00044-9

For a full list of publications please visit myPublications