Clinical feature Associated with HPS subtype No of patients with clinical feature
restrictive lung disease 1, 2, 4 127
variable pigment dilution 1, 2, 4, 7-9 3
decreased visual acuity 1-4, 9 11
nystagmus 1-9 1
hemorrhage, prolonged bleeding, easy bruising 1-8 7
granulomatous colitis 1, 4 1
conductive hearing loss 2 1
neutropenia, immunodeficiency 2 1
dysplastic acetubulae 2 0
poor balance 2 0
hypercholesterolemia 5 8
Table 1: Overview of clinical features of HPS with associated subtype in our IPF cohort.