fibrodysplasia ossificans progressiva
Language: English
Part of speech: noun
Pronunciation: ipa: /ˌfaɪbɹəʊdɪsˈpleɪzɪə ɒˈsɪfɪkɑːnz pɹəˈɡɹɛsɪvə/
ipa: /-ˈpleɪʒə-/
ipa: /ˌfaɪbɹoʊdɪsˈpleɪʒ(i)ə ɒˈsɪfɪkænz pɹəˈɡɹɛsɪvə/
ipa: /-ˈpleɪziə-/
ipa: /-pɹoʊ-/
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).
Senses: examples: text: The cardinal manifestations of fibrodysplasia ossificans progressiva are microdactyly and progressive ossification of fascia, [aponeuroses], and other fibrous structures related to muscles.
ref: 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:
text: 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.
ref: 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:
text: 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.
ref: 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:
text: 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).
ref: 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:
synonyms: FOP
Münchmeyer disease
myositis ossificans progressiva
stone man syndrome
qualifier: medical genetics
glosses: 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.
wikipedia: Harry Raymond Eastlack
Julius Springer
Mütter Museum
Theodor von Dusch
Victor A. McKusick