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Hamman-rich syndrome (alveolar capillary block syndrome; diffuse pulmonary fibrosis syndrome; rheumatoid lung syndrome) 5


Hamman-Rich Syndrome (Alveolar Capillary Block Syndrome; Diffuse Pulmonary Fibrosis Syndrome; Rheumatoid Lung Syndrome) 414j96e 414j96e 414j96e 414j96e 414j96e 5

General: Etiology unknown; insidious onset with progressive exertional dyspnea; association with rheumatoid arthritis or scleroderma; autosomal recessive; occurs between age 40 and 50 years.

Ocular: Xerophthalmia; keratomalacia; retinal venous congestion and engorgement; ischemic retinopathy; cystic macular changes.

Clinical: Cyanosis; dyspnea; cough; weight loss; clubbing of fingers; high sodium and chloride concentrations in sweat; heart failure.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Hamman L, Rich RR. Acute diffuse interstitial fibrosis of lungs. Bull Johns Hopkins Hosp 1944; 74:177.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

Spalter HF. Cystic fibrosis and the eye. J Pediatr Ophthalmol 1971; 8:6.




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