Purpose To compare clinically 2diff erent subtypes of Her-mansky-Pudlak syndrome (HPS),type 1(HPS -1)and type 3(HPS -3).Design Cross-sectional study of a s eries of patients.Participants Sixteen p atients with HPS -1and14patients with HPS -3were studied.Methods Complete eye examination,including best-corrected visual acuity and photographs and photographic gradi ng of iris transillumi-nation and macular transparency using a previously estab-lished grading system.Results Snellen visual acuity was20/160-2in the HPS -1group and 20/125+2in the HPS -3group(P=0.017).Iris grading was statistically significant for less translucence i n the HPS -3patients.The HPS -3patients also tended to have less transparent maculas,but the difference was not s tatistically signifi-cant.Conclusions Patients with HPS-3have less severe ophthalmic manifestations than patients with HPS -1.Ophthalmologists treating patient s with albinism shouldconsider HPS in their differential diagnosis even in the case of mild iris and macular hypopigment ation.
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