Niemann-Pick Disease

Niemann-Pick syndrome

Lipidoses (Lipoidosis, Lipodystrophy) lipoclastic-pseudoxanthomum hamartomatosis **Niemann-Pick syndrome** is a hereditary disease of adults and children from the group of monogenic defects of lipid metabolism. The basis of all clinical manifestations is the insufficiency of lysosomal enzymatic systems that ensure the utilization of a number of lipids, including neutral fats,