The characterisation of pulmonary function in patients with mucopolysaccharidoses IVA: A longitudinal analysis

Autor: Johnny J. Kenth, Gabrielle Thompson, Catherine Fullwood, Stuart Wilkinson, Simon Jones, I.A. Bruce

Publikováno v: Molecular Genetics and Metabolism Reports, Vol 20, Iss , Pp - (2019)

Introduction: Mucopolysaccharidosis (MPS) type IVA is a rare, autosomal recessive lysosomal storage disease causing substrate accumulation in various organs and tissues. MPS IVA is associated with both obstructive and restrictive airway disease, with

Externí odkaz: https://doaj.org/article/82320e84a5b5411d8a54301f7ca6fad7

Data in support of the longitudinal characterization of pulmonary function in children with Mucopolysaccharidoses IVA

Autor: Iain A. Bruce, Gabrielle Thompson, Simon Jones, Stuart Wilkinson, Johnny J. Kenth

Publikováno v: Data in Brief, Vol 27, Iss, Pp-(2019)
Data in Brief

Mucopolysaccharidoses type IVA (Morquio disease) is a rare, autosomal recessive lysosomal storage disease that causes both obstructive and restrictive airway pathology, with respiratory failure being the primary cause of death. This article provides

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::399c68de30604d82d4c6e80cc94c871b
https://doi.org/10.1016/j.dib.2019.104756

Novel approach for tracheal resection in Morquio a syndrome with end-stage critical airway obstruction: a UK case series.

Autor: Kenth J; Department of Paediatric Anaesthesia, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester, UK. jkenth@me.com.; Divisions of Infection, Immunity and Respiratory Medicine, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. jkenth@me.com., Maughan E; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.; UCL Great Ormond Street Institute of Child Health, UCL Great Ormond Street Institute of Child Health, Great Ormond Street Hospital for Children, UK., Butler CR; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.; UCL Great Ormond Street Institute of Child Health, UCL Great Ormond Street Institute of Child Health, Great Ormond Street Hospital for Children, UK., Gabrie J; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK., Rouhani M; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK., Silver B; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK., Ogunbiyi OK; Great Ormond Street Hospital for Children, NHS Foundation Trust Research Histopathology Service, London, UK., Wilkinson S; Department of Paediatric Respiratory Medicine, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester, UK., Nandi R; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK., Walker R; Department of Paediatric Anaesthesia, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester, UK., Muthialu N; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.; UCL Great Ormond Street Institute of Child Health, UCL Great Ormond Street Institute of Child Health, Great Ormond Street Hospital for Children, UK., Jones S; Divisions of Infection, Immunity and Respiratory Medicine, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.; The Willink Metabolic Unit, Manchester Centre for Genomic Medicine, Manchester University NHS Foundation Trust, Manchester, UK., Hewitt R; National Paediatric Tracheal Service, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK., Bruce IA; Divisions of Infection, Immunity and Respiratory Medicine, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.; Paediatric ENT Department, Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.

Publikováno v: Orphanet journal of rare diseases [Orphanet J Rare Dis] 2024 Jul 22; Vol. 19 (1), pp. 274. Date of Electronic Publication: 2024 Jul 22.

A novel case of adult, acute epiglottitis successfully treated with noninvasive ventilation.

Autor: Kenth J; From the *City Hospital, Birmingham; and †the Department of Anaesthesia, Barnet and Chase Farm Hospital, London, United Kingdom., Mumtaz T

Publikováno v: A & A case reports [A A Case Rep] 2014 Mar 01; Vol. 2 (5), pp. 55-6.