In these patients with HPS, gene expression was dissimilar in patient 8, who had atypical radiographic findings of predominantly focal lung disease with lobar pulmonary fibrosis and bronchiectasis, Notably, HPS1 was downregulated in patients with HPS-1 pulmonary fibrosis compared with patients with FPF or URels. The gene discussed is HPS1; the disease is Hermansky-Pudlak syndrome.