论文部分内容阅读
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.
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 as eries of patients. Participants Sixteen p atients with HPS-1 and 14patients with HPS-3were studied. Methods Complete eye examination, including best-corrected visual acuity and photographs and photographic gradi of iris transillumini-nation and macular transparency using a previously established-grading system. Results Snellen visual acuity was 20/160 -2in the HPS-1group and 20/125 + 2in the HPS-3group (P = 0.017) .Iris grading was substantially significant for less translucence in the HPS-3patients. The HPS-3patients also ten ten to have less transparent maculas, but the Differences was not s tatistically signifi-cant. Confidence Patients with HPS-3have less severe ophthalmic manifestations than patients with HPS -1. Ophthalmologists treating patient s with albinism should conditionside HPS in their differential diagnosis even in the case of mild iris and macular hypopigment ation.