Clinically, there are two main types of LAL-D: Wolman disease, which is rapidly progressive in infants and symptom onset later in life, also known as cholesteryl ester storage disease (CESD), which occurs in paediatrics and adults.1,2
LAL-D is a genetic hereditary disorder that affects fat metabolism.1,2
It is characterised by the storage of some specific lipids (cholesterol esters and triglycerides) in the majority of organs and tissues, due to an absence or deficiency of the lysosomal enzyme lysosomal acid lipase.1,2
LAL-D is a genetic hereditary disorder that affects fat metabolism.1,2
It is characterised by the storage of some specific lipids (cholesterol esters and triglycerides) in the majority of organs and tissues, due to an absence or deficiency of the lysosomal enzyme lysosomal acid lipase.1,2
The clinical onset of Wolman disease occurs in the first few weeks of life and affects multiple organs.1,2 This is a severe disease and affected infants can experience gastrointestinal abnormalities, progressive malnutrition, hepatosplenomegaly and steatorrhea.1,2
A low-fat diet is an important and integral part of patient management.3,4 Regular dietary adjustments are needed in order to ensure that growth is optimal and fat consumption should be consistent with recommended goals.3 Please talk to your child’s treating physician to obtain the appropriate dietary advice.
A low-fat diet is an important and integral part of patient management.3,4 Regular dietary adjustments are needed in order to ensure that growth is optimal and fat consumption should be consistent with recommended goals.3 Please talk to your child’s treating physician to obtain the appropriate dietary advice.
A simple guide to macronutrients
There are 3 types of macronutrients in our food: Carbohydrates, Fats, Proteins. Foods are a combination of macros, for example chicken is a protein but it also contains fat.
Tips for maintaining your LAL-D diet
Every time you do eat, always include a source of quality protein such as skinless chicken, fish, egg whites, beans, lentils. Protein is more satisfying than carbohydrates and will give...
The LAL-D friendly diet for adults
We should follow a low-fat diet (20 g fat per day) and encourage the consumption of healthy foods. It is important to opt for foods that are high in fibre such as fruit, vegetables...
CESD, cholesteryl ester storage disease; LAL-D, lysosomal acid lipase deficiency.