Inflammation of the kidneys filtering units, called glomerulonephritis, is responsible for about 10-20 percent of the extensive kidney damage patients experience, although the affected pathway is common to many forms of progressive kidney damage
It is typically supplied as a lyophilized (freeze-dried) powder that must be reconstituted with bacteriostatic water before use
Genetic predisposition plays a central role in breaking immune tolerance to self-antigens
If vomiting occurs within an hour of administration, redose with another full dose
Etiology Genetics COL5A1 or COL5A2 mutation in 40-50% less common mutations identified below Associated conditions Connective tissue disorder characterized by Classification Berlin Classification (1988) - revised Types I - XI exist Villefranche Classification (1998) Presentation Symptoms double-jointedness easily damaged, bruised, & stretchy skin easy scarring & poor wound healing increased joint mobility, joint popping, early arthritis chronic musculoskeletal pain (50%) Physical exam a score of 5 or more on 9-point Beighton-Horan scale defines joint hypermobility
Manufactured in USA facilities using pharmaceutical-grade synthesis protocols and verified through independent laboratory analysis