Hermansky-Pudlak Syndrome Complicated by Pulmonary Fibrosis: Radiologic-Pathologic Correlation and Review of Pulmonary Complications
نویسندگان
چکیده
منابع مشابه
Hermansky-Pudlak syndrome complicated by pulmonary fibrosis.
A 45-year-old woman from Puerto Rico presented to the emergency department with progressively worsening dyspnea over a 3to 4-day period. She had been diagnosed with type 1 Hermansky-Pudlak syndrome complicated by pulmonary fi brosis 4 years earlier. Previous renal biopsy demonstrated nephrosclerosis secondary to HermanskyPudlak syndrome. A brother also had Hermansky-Pudlak syndrome complicated ...
متن کاملDiffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.
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.
متن کاملClinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation
Pulmonary fibrosis is a progressive, fatal manifestation of Hermansky-Pudlak syndrome (HPS). Some patients with advanced HPS pulmonary fibrosis undergo lung transplantation despite their disease-associated bleeding tendency; others die while awaiting donor organs. The objective of this study is to determine the clinical management and outcomes of a cohort with advanced HPS pulmonary fibrosis wh...
متن کاملAcute exacerbation of combined pulmonary fibrosis and emphysema associated with Hermansky–Pudlak syndrome
A 30-year-old male smoker with congenital amblyopia and oculocutaneous albinism was admitted to our hospital complaining of progressive dyspnea on exertion. Chest computed tomography images revealed diffuse reticular opacities and honeycombing in the bilateral lower lobes with sparing of the subpleural region along with emphysema predominantly in the upper lobes. Lung biopsy specimens showed a ...
متن کاملHermansky-Pudlak syndrome.
We present a rare disease condition Hermansky-Pudlak syndrome in a 33-year-old male. He was born of a consanguineous marriage, had occulo-cutaneous albinism, nystagmus, decreased visual acuity, refractory errors, pulmonary fibrosis and granulomatous inflammation of the colon. In spite of all the classical features of this genetic disorder he was labeled to have disseminated tuberculous infectio...
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ژورنال
عنوان ژورنال: Journal of Clinical Imaging Science
سال: 2014
ISSN: 2156-7514
DOI: 10.4103/2156-7514.143437