fibrodysplasia ossificans progressiva en · NOUN
Pronunciation
- /ˌfaɪbɹoʊdɪsˈpleɪʒ(i)ə ɒˈsɪfɪkænz pɹəˈɡɹɛsɪvə/ (General-American)
- /-ˈpleɪʒə-/ (Received-Pronunciation)
- /ˌfaɪbɹəʊdɪsˈpleɪzɪə ɒˈsɪfɪkɑːnz pɹəˈɡɹɛsɪvə/ (Received-Pronunciation)
- (General-American) audio
- /-pɹoʊ-/ (General-American)
- /-ˈpleɪziə-/ (General-American)
Etymology
PIE word *h₃ésth₁ From English fibrodysplasia, and borrowed from New Latin ossificans + Latin prōgressīvā, literally “abnormal development of fibrous tissue with ossification that is progressive”: * Fibrodysplasia is derived from fibro- (prefix meaning ‘fibre; fibrous’) (from Latin fibra (“fibre; filament”), ultimately from Proto-Indo-European *bʰeyd- (“to split”)) + dysplasia (“abnormal development of cells or tissue”) (from dys- (prefix meaning ‘abnormal; disease’) + -plasia (suffix meaning ‘formation; growth’)). * Ossificans is from Latin ossīs (a plural form of ossum (“bone”), ultimately from Proto-Indo-European *h₃ésth₁ (“bone”)) + -ficāns (the present active participle of -ficō (suffix forming chiefly causative or factitive verbs)). * Prōgressīvā is the ablative feminine singular of prōgressīvus (“progressive”), from prōgressus (“advanced, proceeded; developed, progressed”) (the perfect active participle of prōgredior (“to advance, proceed; (figurative) to develop, progress”), from prō- (prefix denoting a forward direction) + gradior (“to step; to walk; to advance”) (ultimately from Proto-Indo-European *gʰredʰ- (“to go; to walk”))) + -īvus (suffix forming adjectives). The disease was originally termed myositis ossificans progressiva in 1868. The use of fibrodysplasia in place of myositis was suggested by K. H. Bauer and W. Bode in 1940, and adopted by the American medical geneticist Victor A. McKusick (1921–2008) in his work Heritable Disorders of Connective Tissue (1956).
Meanings
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(qualifier:medical genetics, uncountable) A very rare, disabling genetic disorder of the connective tissue in which fibrous tissue (including ligaments, muscles, and tendons) ossifies (“transforms into bone”) when damaged.
This year that show [Grey's Anatomy] featured a story about a patient with fibrodysplasia ossificans progressiva, a disorder in which damaged muscles and connective tissue turn to bone, forming, essentially, a second skeleton.
2007 June 18, Ginia Bellafante, “TV review: ‘Heartland’: Doctor, give me the news, and sew me up pretty”, in The New York Times, New York, N.Y.: The New York Times Company, →ISSN, →OCLC, archived from the original on 26 Jan 2021:A mother of a one-year-old girl with an illness that turns muscle into bone has urged the government to do more to help research into rare conditions. Lexi Robins, of Hemel Hempstead has the very rare condition Fibrodysplasia Ossificans Progressiva (FOP).
2022 July 7, Alex Pope, “Hemel Hempstead Mother’s Funding Plea for Daughter’s Rare Disease”, in BBC News, archived from the original on 11 Dec 2023:Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. We mapped FOP to chromosome 2q23-24 by linkage analysis and identified an identical heterozygous mutation (617G → A; R206H) in the glycine-serine (GS) activation domain of ACVR1, a BMP type I receptor, in all affected individuals examined.
2006 April 23, Eileen M. Shore [et al.], “A Recurrent Mutation in the BMP Type I Receptor ACVR1 Causes Inherited and Sporadic Fibrodysplasia Ossificans Progressiva”, in Nature Genetics, volume 38, New York, N.Y.: Nature Portfolio, published 1 May 2006, →DOI, →ISSN, →OCLC, abstract, page 525:The cardinal manifestations of fibrodysplasia ossificans progressiva are microdactyly and progressive ossification of fascia, [aponeuroses], and other fibrous structures related to muscles.
1956, Victor A[lmon] McKusick, “Concluding Comments”, in Heritable Disorders of Connective Tissue, St. Louis, Mo.: The C. V. Mosby Company, →OCLC, pages 194–195:
Synonyms
myositis ossificans progressiva · Münchmeyer disease · stone man syndrome · FOP
Translations (25)
pt fibrodisplasia ossificante progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · eu fibrodisplasia osifikatzaile progresibo (genetic disorder in which fibrous tissue ossifies when damaged) · fi fibrodysplasia ossificans progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · li fibrodysplasia ossificans progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · ru фибродисплази́я оссифици́рующая прогресси́рующая (genetic disorder in which fibrous tissue ossifies when damaged) · tr fibrodisplazi ossifikans progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · es fibrodisplasia osificante progresiva (genetic disorder in which fibrous tissue ossifies when damaged) · pl postępujące kostniejące zapalenie mięśni (genetic disorder in which fibrous tissue ossifies when damaged) · de Fibrodysplasia ossificans progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · vi hội chứng người hoá đá (genetic disorder in which fibrous tissue ossifies when damaged) · it fibrodisplasia ossificante progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · ca fibrodisplàsia ossificant progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · ja 進行性骨化性線維異形成症 (genetic disorder in which fibrous tissue ossifies when damaged) · mk прогресивна осифирачка фибродисплазија (genetic disorder in which fibrous tissue ossifies when damaged) · cs fibrodysplasia ossificans progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · ar خلل التنسج الليفي المعظم المترقي (genetic disorder in which fibrous tissue ossifies when damaged) · ro fibrodisplazie osificantă progresivă (genetic disorder in which fibrous tissue ossifies when damaged) · eo progresa ostiĝa fibrodisplazio (genetic disorder in which fibrous tissue ossifies when damaged) · lv progresējošā osificējošā fibrodisplāzija (genetic disorder in which fibrous tissue ossifies when damaged) · he תסמונת FOP (genetic disorder in which fibrous tissue ossifies when damaged) · ko 진행성 골화성 섬유이형성증 (genetic disorder in which fibrous tissue ossifies when damaged) · fa فیبرودیسپلازی استخوانیشونده پیشرونده (genetic disorder in which fibrous tissue ossifies when damaged) · cmn 進行性骨化性纖維發育不良 /进行性骨化性纤维发育不良 (genetic disorder in which fibrous tissue ossifies when damaged) · nl fibrodysplasia ossificans progressiva (genetic disorder in which fibrous tissue ossifies when damaged) · ml ഫൈബ്രോഡിസ്പ്ലാസിയ ഓസിഫിക്കൻസ് പ്രോഗ്രെസ്സിവ (genetic disorder in which fibrous tissue ossifies when damaged)
wikipedia: Harry Raymond Eastlack · wikipedia: Mütter Museum · wikipedia: Julius Springer · wikipedia: Theodor von Dusch · wikipedia: Victor A. McKusick