Abstract
Clinical and histological studies were done on three patients (7-month-old girl, 1-year and 4-month old boy, 8-year-old boy) with total albinism. In cases of total albinism, it has been considered that the skin and hair were white and that no pigmentation occurred during the life span. Ophthalmological examination, photo test of skin, tyrosinase activity test of the hair bulb, dopa test and electron micoscope examination of the skin were done. Tyrosinase activity of the hair bulb was considered to be most useful for diagnosing the tyrosinase type of albinism. One child was diagnosed as being tyrosinase-negative type and the other two were the positive type. Due to the photosensitivity of the skin and eyes, the three patients had to be protected from ultraviolet rays by the use of ointment and eye glasses. The patient with the tyrosinase-negative type could not open her eyes in the presence of sun light. The hair and iris in the two of the tyrosinase-positive type have attained color, but the eyes should be protected, at best during childhood.
