Certain Matrix Metalloproteinases Are Potential Biomarkers for PF in Rare Genetic Disorder, Study Suggests

Increased activity of certain matrix metalloproteinases (MMPs), enzymes that regulate cell behavior, may explain the risk for developing aggressive pulmonary fibrosis in people with Hermansky-Pudlak syndrome (HPS), a rare genetic disorder, according to new research. The study highlights MMP-2 and MMP-9. The findings suggest that these unique…

Lung Transplant Can Benefit Those with PF-Linked Hermansky-Pudlak Syndrome, Study Reports

A lung transplant can benefit those whose pulmonary fibrosis (PF) stems from Hermansky-Pudlak syndrome (HPS), a study indicate. The study, “Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation,” was published in the journal PLOS One. HPS is a rare…

Researchers Start a Study of Lung Fibrosis in Patients With Hermansky-Pudlak Syndrome

Researchers from two Southern U.S. universities and a federal healthcare agency have begun a study of pulmonary fibrosis in people with the rare disease Hermansky-Pudlak syndrome (HPS). The objective of the observational clinical trial is to learn why those with HPS develop lung fibrosis, or tissue scarring. The team from …