lungs, intestine, kidneys, and heart. The major complication of most forms of the disorder is pulmonary fibrosis, which typically exhibits in patients ages 40–50 years. This is a fatal complication seen in many forms of HPS, and is the usual cause of death from the disorder. HPS patients who develop pulmonary fibrosis typically have type 1 or type 4. Research HPS is one of the rare lung diseases currently being studied by The Rare Lung Diseases Consortium (RLDC). The RLDC is part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR), of the National Center for Advancing Translational Sciences (NCATS). The RLDC is dedicated to developing new diagnostics and therapeutics for patients with rare lung diseases, through collaboration between the NIH, patient organizations and clinical investigators. Eponym It is named for František Heřmanský (1916–1980) and Pavel Pudlák (1927–1993). See also Biogenesis of lysosome-related organelles complex 1 List of cutaneous conditions References External links GeneReviews/NCBI/NIH/UW entry on Hermansky-Pudlak Syndrome Disturbances of human pigmentation Syndromes affecting the skin Amino acid metabolism disorders Autosomal recessive disorders Rare syndromes Coagulopathies IUIS-PID table 3 immunodeficiencies Syndromes affecting blood Syndromes affecting the lung