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. 1981 Jun;36(6):419–423. doi: 10.1136/thx.36.6.419

Cardiopulmonary function in fibrodysplasia ossificans progressiva.

J M Connor, C C Evans, D A Evans
PMCID: PMC471526  PMID: 7314012

Abstract

Cardiopulmonary function was evaluated in 21 patients with fibrodysplasia ossificans progressiva. Neither cardiac enlargement nor failure was observed, but six patients had abnormal electrocardiograms. All had marked restrictive spirometry because of chest wall fixation and depended upon diaphragmatic respiration. The severity of chest restriction was independent of sex, age, duration of disease, and extnet of other physical disability. Progression to chronic respiratory failure was not observed. Chest infection in the presence of diminished pulmonary reserve is the major hazard of life in this rare disease and prophylactic measures should be considered.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bland J. H., Kirschbaum B., O'Connor G. T., Whorton E. Myositis ossificans progressiva. Effect of intravenously given parathyroid extract on urinary excretion of connective tissue components. Arch Intern Med. 1973 Aug;132(2):209–212. doi: 10.1001/archinte.132.2.209. [DOI] [PubMed] [Google Scholar]
  2. Buhain W. J., Rammohan G., Berger H. W. Pulmonary function in myositis ossificans progressiva. Am Rev Respir Dis. 1974 Sep;110(3):333–337. doi: 10.1164/arrd.1974.110.3.333. [DOI] [PubMed] [Google Scholar]
  3. CAMPBELL A. H., MACDONALD C. B. UPPER LOBE FIBROSIS ASSOCIATED WITH ANKYLOSING SPONDYLITIS. Br J Dis Chest. 1965 Apr;59:90–101. doi: 10.1016/s0007-0971(65)80017-1. [DOI] [PubMed] [Google Scholar]
  4. FLETCHER E., MOSS M. S. MYOSITIS OSSIFICANS PROGRESSIVA. Ann Rheum Dis. 1965 May;24:267–272. doi: 10.1136/ard.24.3.267. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Hentzer B., Jacobsen H. H., Asboe-Hansen G. Fibrodysplasia ossificants progressiva. Scand J Rheumatol. 1977;6(3):161–171. doi: 10.3109/03009747709095443. [DOI] [PubMed] [Google Scholar]
  6. Jessamine A. G. Upper lung lobe fibrosis in ankylosing spondylitis. Can Med Assoc J. 1968 Jan 6;98(1):25–29. [PMC free article] [PubMed] [Google Scholar]
  7. LUTWAK L. MYOSITIS OSSIFICANS PROGRESSIVA. MINERAL, METABOLIC AND RADIOACTIVE CALCIUM STUDIES OF THE EFFECTS OF HORMONES. Am J Med. 1964 Aug;37:269–293. doi: 10.1016/0002-9343(64)90011-7. [DOI] [PubMed] [Google Scholar]
  8. Letts R. M. Myositis ossificans progressiva. A report of two cases with chromosome studies. Can Med Assoc J. 1968 Nov 2;99(17):856–862. [PMC free article] [PubMed] [Google Scholar]
  9. Rosenstirn J. A CONTRIBUTION TO THE STUDY OF MYOSITIS OSSIFICANS PROGRESSIVA. Ann Surg. 1918 Nov;68(5):485–520. doi: 10.1097/00000658-191811000-00006. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Russell R. G., Smith R., Bishop M. C., Price D. A. Treatment of myositis ossificans progressiva with a diphosphonate. Lancet. 1972 Jan 1;1(7740):10–11. doi: 10.1016/s0140-6736(72)90004-9. [DOI] [PubMed] [Google Scholar]
  11. SHAFFER A. B., REISER I. Right bundle branch system block in healthy young people. Am Heart J. 1961 Oct;62:487–493. doi: 10.1016/0002-8703(61)90257-5. [DOI] [PubMed] [Google Scholar]
  12. Schroeder H. W., Jr, Zasloff M. The hand and foot malformations in fibrodysplasia ossificans progressiva. Johns Hopkins Med J. 1980 Aug;147(2):73–78. [PubMed] [Google Scholar]
  13. Schönthal H. Atemmechanische Probleme bei der Myositis ossificans progressiva. Beitr Klin Erforsch Tuberk Lungenkr. 1967;135(3):322–324. [PubMed] [Google Scholar]
  14. Smith R. Myositis ossificans progressiva: a review of current problems. Semin Arthritis Rheum. 1975 May;4(4):369–380. doi: 10.1016/0049-0172(75)90018-9. [DOI] [PubMed] [Google Scholar]
  15. Suzuki T., Ishikawa S., Akanuma N., Tsunoda H. Myositis ossificans progressiva with parathyroid hyperplasia and polycystic ovary. Acta Pathol Jpn. 1976 Mar;26(2):251–262. doi: 10.1111/j.1440-1827.1976.tb00878.x. [DOI] [PubMed] [Google Scholar]
  16. Tünte W., Becker P. E., Knorre G. V. Zur Genetik der Myositis ossificans progressiva. Humangenetik. 1967;4(4):320–351. doi: 10.1007/BF00285740. [DOI] [PubMed] [Google Scholar]
  17. Weiss I. W., Fisher L., Phang J. M. Diphosphonate therapy in a patient with myositis ossificans progressiva. Ann Intern Med. 1971 Jun;74(6):933–936. doi: 10.7326/0003-4819-74-6-933. [DOI] [PubMed] [Google Scholar]

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