Cholesterol emulsion and supplementary food for the treatment of Smith-Lemli-Opitz syndrome
The Smith-Lemli-Opitz syndrome is a heredi-tary autosomal recessive malformation syn-drome based on a metabolic disorder of cho-lesterol biosynthesis. As a result, there is a deficiency of cholesterol and at the same time increase of 7-dehydrocholesterol (7-DHC), resulting in the affected patients with many health impairments.
The current standard therapy consists in the administration of cholesterol in water or oil, for example as a suspension. Due to the low solu-bility of cholesterol in both liquids, a sediment are often formed, whereby only a small part of the amount of cholesterol administered to the patient can be ingested and absorbed, while the majority of the cholesterol is excreted un-used.
Therefore, there is a further need of cholester-ol-containing compositions that can be used for an optimized intake of cholesterol.
The present technology offer relates to edible emulsions of particular composition and their use in medicine. Especially, for the treatment of diseases associated with a decreased cho-lesterol level, such as the Smith-Lemli-Opitz syndrome. Further, a process for producing such emulsions has been developed.
Advantages of the invention
- Emulsions can be prepared to contain a defined amount of cholesterol.
- Properties of the emulsions can be adapted to the application according to the respective patient requirements.
- Lowering the 7-DHC level.
- High storage stability with cooling.
Patent application filed in Germany.