Lipoid proteinosis is a rare disease characterized by the accumulation of lipoproteins in various organs and tissues.
Causes
The exact reasons for the development of lipid proteinosis are unknown. It is assumed that the main role is played by a violation of lipoprotein metabolism, as a result of which they accumulate in the body.
Symptoms
The main symptoms of lipid proteinosis:
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enlargement of the liver, spleen, lymph nodes
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yellowness of the skin and mucous membranes
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cough, shortness of breath (with lung damage)
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abdominal pain, diarrhea (with intestinal damage)
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neurological symptoms (headache, weakness, visual disturbances) with damage to the nervous system
Diagnostics
To diagnose lipoid proteinosis, a blood test, CT or MRI of the abdominal organs, and a biopsy of the affected tissue are performed.
Treatment
No specific treatment for lipid proteinosis has been developed. Symptomatic therapy and plasmapheresis are used. In some cases, glucocorticoids and cytostatics are prescribed. The prognosis depends on the degree of organ damage and the timeliness of treatment.