Werlhof's Secondary Syndrome

Werlhof's disease primary syndrome (CHD). - glycosaminoglycan storage disease (GAG). According to the mechanism of elimination: hypophosphate, (autosomal recessive inheritance type), occurs against the background of insufficient activity of the enzyme N-acetylglucosamine-N-acyltransferase, which leads to disruption of the production of the main acid in the serum responsible for the binding of glycosaminoglycans to chondroitin sulfate. This is accompanied by the deposition of cholesterol on the surface of the mucous membrane of the intestine, spleen, lungs and liver; damage to joints (tendency to epiphyseal fractures), nervous system and many internal organs. The younger the patient, the greater the likelihood of a malignant course of the disease.