Hermansky-Pudlak syndrome 6 protein (HPS6) has originally been identified as a subunit of the BLOC-2 protein complex that is involved in the biogenesis of lysosome-related organelles. Here, we demonstrate that HPS6 directly interacts with the dynactin p150(Glued) subunit of the dynein-dynactin motor complex and acts as cargo adaptor for the retrograde motor to mediate the transport of lysosomes...

Ultrasound is extremely beneficial in the evaluation of acute pediatric abdominal disease, such as HPS, intussusception, and acute appendicitis. As techniques and equipment improve, its role in the evaluation of infants and children continues to increase.

PURPOSE The processes used to revise the 2008 Basel Statements on the future of hospital pharmacy are summarized, and the revised statements are presented. METHODS The process for revising the Basel Statements followed an approach similar to that used during their initial development. The Hospital Pharmacy Section (HPS) of the International Pharmaceutical Federation (FIP) revised the 2008 FIP...

Different studies of the treatment of trichotillomania (TTM) have used varying standards to determine the proportion of patients who obtain clinically meaningful benefits, but there is little information on the similarity of results yielded by these methods or on their comparative validity. Data from a stepped-care (Step 1: Web-based self-help; Step 2: Individual behavior therapy; N = 60) treat...

The recessive mutation at the pale ear (ep) locus on mouse chromosome 19 was found to be the homologue of human Hermansky-Pudlak syndrome (HPS). A positional cloning strategy using yeast artificial chromosomes spanning the HPS locus was used to identify the HPS gene and its murine counterpart. These genes and their predicted proteins are highly conserved at the nucleotide and amino acid levels....

Hermansky-Pudlak syndrome (HPS) is a rare disorder characterised by oculocutaneous albinism, a bleeding tendency, and lipofuscinosis. This retrospective study reviews the clinical history and haematological features of 23 cases of HPS. Information was gathered from patient notes and by direct interview. Thirteen of the 23 children were of Turkish origin, 12 being members of four kindreds from t...

Hermansky Pudlak syndrome type 2 (HPS2) is a rare disorder associated with mutations in the Adaptor Protein 3 (AP-3) complex, which is involved in sorting transmembrane proteins to lysosomes and related organelles. We now report 2 unrelated subjects with HPS2 who show a characteristic clinical phenotype of oculocutaneous albinism, platelet and T-lymphocyte dysfunction and neutropenia. The subje...

BACKGROUND As efforts to address patient safety (PS) in health professional (HP) education increase, it is important to understand new HPs' perspectives on their own PS competence at entry to practice. This study examines the self-reported PS competence of newly registered nurses, pharmacists and physicians. METHODS A cross-sectional survey of 4496 new graduates in medicine (1779), nursing (2...

In 2003, after three pilot projects successfully implemented WHO's Health-Promoting Schools (HPS) concept, officials in Zhejiang Province, China, expanded to additional 51 schools (93,000 students and their families and 6800 school personnel). Each school identified a health issue to begin HPS development, followed by conceptual orientation, resource mobilization, teacher training, surveys, int...