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. 1994 Jun;49(6):617–618. doi: 10.1136/thx.49.6.617

Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.

S P Reynolds 1, B H Davies 1, A R Gibbs 1
PMCID: PMC474969  PMID: 8016804

Abstract

The Hermansky-Pudlak syndrome consists of albinism, platelet function defect, pigment laden macrophases and, on occasions, pulmonary fibrosis. The clinical course and postmortem findings of a patient with pulmonary fibrosis which mimicked cryptogenic fibrosing alveolitis are reported. Histological examination revealed a chronic inflammatory infiltrate of pigment laden microphages.

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Selected References

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

  1. BEDNAR B., HERMANSKY F., LOJDA Z. VASCULAR PSEUDOHEMOPHILIA ASSOCIATED WITH CEROID PIGMENTOPHAGIA IN ALBINOS. Am J Pathol. 1964 Aug;45:283–294. [PMC free article] [PubMed] [Google Scholar]
  2. Davies B. H., Tuddenham E. G. Familial pulmonary fibrosis associated with oculocutaneous albinism and platelet function defect. A new syndrome. Q J Med. 1976 Apr;45(178):219–232. [PubMed] [Google Scholar]
  3. DePinho R. A., Kaplan K. L. The Hermansky-Pudlak syndrome. Report of three cases and review of pathophysiology and management considerations. Medicine (Baltimore) 1985 May;64(3):192–202. [PubMed] [Google Scholar]
  4. Garay S. M., Gardella J. E., Fazzini E. P., Goldring R. M. Hermansky-Pudlak syndrome. Pulmonary manifestations of a ceroid storage disorder. Am J Med. 1979 May;66(5):737–747. doi: 10.1016/0002-9343(79)91111-2. [DOI] [PubMed] [Google Scholar]
  5. HERMANSKY F., PUDLAK P. Albinism associated with hemorrhagic diathesis and unusual pigmented reticular cells in the bone marrow: report of two cases with histochemical studies. Blood. 1959 Feb;14(2):162–169. [PubMed] [Google Scholar]
  6. Schinella R. A., Greco M. A., Cobert B. L., Denmark L. W., Cox R. P. Hermansky-Pudlak syndrome with granulomatous colitis. Ann Intern Med. 1980 Jan;92(1):20–23. doi: 10.7326/0003-4819-92-1-20. [DOI] [PubMed] [Google Scholar]
  7. Takahashi A., Yokoyama T. Hermansky-Pudlak syndrome with special reference to lysosomal dysfunction. A case report and review of the literature. Virchows Arch A Pathol Anat Histopathol. 1984;402(3):247–258. doi: 10.1007/BF00695079. [DOI] [PubMed] [Google Scholar]
  8. White D. A., Smith G. J., Cooper J. A., Jr, Glickstein M., Rankin J. A. Hermansky-pudlak syndrome and interstitial lung disease: report of a case with lavage findings. Am Rev Respir Dis. 1984 Jul;130(1):138–141. doi: 10.1164/arrd.1984.130.1.138. [DOI] [PubMed] [Google Scholar]

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