Albinism -
Hermansky-Pudlak syndrome or abbreted HPS) is an extremely rare autosomal recessive disorder which results in oculocutaneous albinism (decreased pigmentation), bleeding problems due to a platelet abnormality (platelet storage pool defect), and storage of an abnormal fat. Ref Davidson 23rd edition pg 1311
Hair bulb for tyrosine positivity test is useful only v/hen the child is 5 years old
Is associated with platelets abnormality especially with aspirin
Complete decussation is invariable in all albinism
Is associated with thrombosis under general anaesthesia
Miscellaneous