Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disease that displays genetic heterogeneity; there are 9 known subtypes. HPS is characterized by oculocutaneous albinism, a platelet storage pool deficiency and resultant bleeding diathesis, and lysosomal accumulation of ceroid lipofuscin. Patients with HPS, specifically those with the genotypes HPS-1, HPS-2, or HPS-4, are predispose...

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by oculocutaneous albinism, bleeding tendency, and lysosomal accumulation of ceroid-like material, with occasional development of interstitial pneumonia (IP). Nine genetically distinct subtypes of HPS are known in humans; IP develops primarily in types 1 and 4. Most reported cases of HPS with IP are type 1, and the...

Background. Significant facilitators and barriers to organ donation and transplantation remain in the general public and even in health professionals. Negative attitudes of HPs have been identified as the most significant barrier to actual ODT. The purpose of this paper was hence to investigate to what extent HPs (physicians and nurses) experience such facilitators and barriers in ODT and to wh...

In this work we describe two instruments for introducing evolutionary behavior into intelligent systems. The first is the hierarchical prioritized structure (HPS) and the second is the participatory learning paradigm (PLP).