To test whether active pneumococci immunization can alleviate inflammation and improve cholesterol metabolism in lysosomal lipid storage diseases and associated metabolic disorders.

Growing evidence describes the central role of oxidized low density lipoproteins (oxLDL) in diseases related to lipid metabolism. Moreover, oxLDL has been shown to be involved in pathological processes such as an inappropriate inflammatory response, disturbance of cholesterol metabolism and dysfunction of the lysosomal apparatus. Relevantly, it has been shown that immunizing mice with Streptococcus pneumoniae results in higher serum titers of anti-oxLDL IgM antibodies due to molecular mimicry.

Inclusion Criteria:

• Patients of following diseases (characterized by lysosomal lipid storage or associated metabolic pathology)

  • Familial hypercholesterolemia,
  • NPB,
  • NPC
  • Partial lipodystrophy (PPARg mutations and laminin A/C mutations)

• Intention to be treated and participate to the treatment - Written informed consent

Exclusion Criteria:

• Medical conditions that may interfere with the study procedures: cancer Hodgkin lymphoma (all related to immune cells); autoimmune diseases; immune deficiency; splenectomy syndrome
• Alcohol abuse (quantitative limit >20g/day for men and >10g for women)
• Illiteracy
• Patients younger than 10 years.
• Any condition which in the opinion of the (co-) investigator might interfere with the evaluation of the study objectives.