title: In pursuit of excellence: an integrated care pathway for C1 inhibitor deficiency.
abstract: There are estimated to be approximately 1500 people in the United Kingdom with C1 inhibitor (C1INH) deficiency. At BartsHealth National Health Service (NHS) Trust we manage 133 patients with this condition and we believe that this represents one of the largest cohorts in the United Kingdom. C1INH deficiency may be hereditary or acquired. It is characterized by unpredictable episodic swellings, which may affect any part of the body, but are potentially fatal if they involve the larynx and cause significant morbidity if they involve the viscera. The last few years have seen a revolution in the treatment options that are available for C1 inhibitor deficiency. However, this occurs at a time when there are increased spending restraints in the NHS and the commissioning structure is being overhauled. Integrated care pathways (ICP) are a tool for disseminating best practice, for facilitating clinical audit, enabling multi-disciplinary working and for reducing health-care costs. Here we present an ICP for managing C1 inhibitor deficiency.
authors.affiliation: Department of Immunopathology, Barts Health NHS Trust, London, UK.
authors.forename: A L
authors.initials: AL
authors.lastname: Manson
authors.forename: A
authors.initials: A
authors.lastname: Price
authors.forename: J
authors.initials: J
authors.lastname: Dempster
authors.forename: P
authors.initials: P
authors.lastname: Clinton-Tarestad
authors.forename: C
authors.initials: C
authors.lastname: Greening
authors.forename: R
authors.initials: R
authors.lastname: Enti
authors.forename: S
authors.initials: S
authors.lastname: Hill
authors.forename: S
authors.initials: S
authors.lastname: Grigoriadou
authors.forename: M S
authors.initials: MS
authors.lastname: Buckland
authors.forename: H J
authors.initials: HJ
authors.lastname: Longhurst
mesh_terms: D019090:Case Management; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D050718:Complement C1 Inhibitor Protein; D019091:Critical Pathways; D019468:Disease Management; D019983:Guideline Adherence; D056829:Hereditary Angioedema Types I and II / Q000188:drug therapy* / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D033183:Interdisciplinary Communication; D008501:Medical Records, Problem-Oriented / Q000592:standards*; D010817:Physician-Patient Relations; D017410:Practice Guidelines as Topic; D015995:Prevalence; D006113:United Kingdom
chemical_list: D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human