Literature DB >> 17852222

Medical and social aspects of the life course for adults with a skeletal dysplasia: a review of current knowledge.

Sue Thompson1, Tom Shakespeare, Michael J Wright.   

Abstract

PURPOSE: The paper examines the general literature and available research evidence on medical, health and social aspects of life for adults with skeletal dysplasia conditions causing profound short stature.
METHOD: The paper reports on a literature review using available medical, psychological and social sources.
RESULTS: There is a dearth of methodologically sound research evidence in this field, and this is particularly marked in areas such as transition to adulthood, ageing and medical, surgical and health experiences.
CONCLUSIONS: There are serious gaps in the available literature and research evidence is sparse and often based on biased samples of limited numbers. This means that it is difficult to get information beyond the anecdotal in assessing the health and social needs of this group of people, and in particular to define needs that are currently unmet. It also limits the scope of advice and information available to health professionals and others in the field who offer support to adults with the conditions and parents of newly-diagnosed babies and young children.

Entities:  

Mesh:

Year:  2008        PMID: 17852222     DOI: 10.1080/09638280701192857

Source DB:  PubMed          Journal:  Disabil Rehabil        ISSN: 0963-8288            Impact factor:   3.033


  7 in total

1.  Inhibiting the integrated stress response pathway prevents aberrant chondrocyte differentiation thereby alleviating chondrodysplasia.

Authors:  Cheng Wang; Zhijia Tan; Ben Niu; Kwok Yeung Tsang; Andrew Tai; Wilson C W Chan; Rebecca L K Lo; Keith K H Leung; Nelson W F Dung; Nobuyuki Itoh; Michael Q Zhang; Danny Chan; Kathryn Song Eng Cheah
Journal:  Elife       Date:  2018-07-19       Impact factor: 8.140

Review 2.  International Consensus Statement on the diagnosis, multidisciplinary management and lifelong care of individuals with achondroplasia.

Authors:  Ravi Savarirayan; Penny Ireland; Melita Irving; Dominic Thompson; Inês Alves; Wagner A R Baratela; James Betts; Michael B Bober; Silvio Boero; Jenna Briddell; Jeffrey Campbell; Philippe M Campeau; Patricia Carl-Innig; Moira S Cheung; Martyn Cobourne; Valérie Cormier-Daire; Muriel Deladure-Molla; Mariana Del Pino; Heather Elphick; Virginia Fano; Brigitte Fauroux; Jonathan Gibbins; Mari L Groves; Lars Hagenäs; Therese Hannon; Julie Hoover-Fong; Morrys Kaisermann; Antonio Leiva-Gea; Juan Llerena; William Mackenzie; Kenneth Martin; Fabio Mazzoleni; Sharon McDonnell; Maria Costanza Meazzini; Josef Milerad; Klaus Mohnike; Geert R Mortier; Amaka Offiah; Keiichi Ozono; John A Phillips; Steven Powell; Yosha Prasad; Cathleen Raggio; Pablo Rosselli; Judith Rossiter; Angelo Selicorni; Marco Sessa; Mary Theroux; Matthew Thomas; Laura Trespedi; David Tunkel; Colin Wallis; Michael Wright; Natsuo Yasui; Svein Otto Fredwall
Journal:  Nat Rev Endocrinol       Date:  2021-11-26       Impact factor: 47.564

3.  Towards an ICF-based self-report questionnaire for people with skeletal dysplasia to study health, functioning, disability and accessibility.

Authors:  Heidi Anttila; Susanna Tallqvist; Minna Muñoz; Sanna Leppäjoki-Tiistola; Outi Mäkitie; Sinikka Hiekkala
Journal:  Orphanet J Rare Dis       Date:  2021-05-22       Impact factor: 4.123

Review 4.  Current knowledge of medical complications in adults with achondroplasia: A scoping review.

Authors:  Svein O Fredwall; Grethe Maanum; Heidi Johansen; Hildegun Snekkevik; Ravi Savarirayan; Ingeborg B Lidal
Journal:  Clin Genet       Date:  2019-04-22       Impact factor: 4.438

5.  Development of the Screening Tool for Everyday Mobility and Symptoms (STEMS) for skeletal dysplasia.

Authors:  Penelope J Ireland; Ravi Savarirayan; Tash Pocovi; Tracy Tate; Marie Coussens; Louise Tofts; Craig Munns; Verity Pacey
Journal:  Orphanet J Rare Dis       Date:  2021-01-21       Impact factor: 4.123

6.  Optimising care and follow-up of adults with achondroplasia.

Authors:  Svein Fredwall; Yana Allum; Moeenaldeen AlSayed; Inês Alves; Tawfeg Ben-Omran; Silvio Boero; Valerie Cormier-Daire; Encarna Guillen-Navarro; Melita Irving; Christian Lampe; Mohamad Maghnie; Klaus Mohnike; Geert Mortier; Sérgio B Sousa; Michael Wright
Journal:  Orphanet J Rare Dis       Date:  2022-08-20       Impact factor: 4.303

Review 7.  Optimal management of complications associated with achondroplasia.

Authors:  Penny J Ireland; Verity Pacey; Andreas Zankl; Priya Edwards; Leanne M Johnston; Ravi Savarirayan
Journal:  Appl Clin Genet       Date:  2014-06-24
  7 in total

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