Bassen-Kornzweig Syndrome

Introduction: Bassen-Kornzweig Syndrome

Description of Bassen-Kornzweig Syndrome

Bassen-Kornzweig Syndrome (medical condition): A rare disorder involving abnormalities in fat metabolism. The...more »

See also:

Abetalipoproteinemia:
  »Introduction: Abetalipoproteinemia
  »Symptoms of Abetalipoproteinemia
  »Treatments for Abetalipoproteinemia

Bassen-Kornzweig Syndrome: A rare inherited fat metabolism disorder.

Bassen-Kornzweig Syndrome: A disorder of lipid metabolism inherited as an autosomal recessive trait characterized by the near absence of APOLIPOPROTEINS B and apoB-containing lipoproteins in plasma. Microsomal triglyceride transfer protein is deficient or absent in enterocytes. Clinical and laboratory findings include acanthocytosis, hypocholesterolemia, peripheral neuropathy, posterior column degeneration, ataxia, and steatorrhea. Intellectual abilities may also be impaired. (Menkes, Textbook of Child Neurology, 5th ed, p118; Curr Opin Lipidol 1994 Apr;5(2):81-6)
Source: Diseases Database

Bassen-Kornzweig Syndrome: Related Topics

These medical condition or symptom topics may be relevant to medical information for Bassen-Kornzweig Syndrome:

• Bassen
• Kornzweig
• SYN
• Abetalipoproteinemia (2 causes)

Bassen-Kornzweig Syndrome: Rare Disease

Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Bassen-Kornzweig Syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Bassen-Kornzweig Syndrome, or a subtype of Bassen-Kornzweig Syndrome, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Bassen-Kornzweig Syndrome as a Disease

Bassen-Kornzweig Syndrome: Another name for Abetalipoproteinemia (or close medical condition association).
  »Introduction: Abetalipoproteinemia
  »Symptoms of Abetalipoproteinemia
  »Treatments for Abetalipoproteinemia

Bassen-Kornzweig Syndrome: Related Diseases

Bassen-Kornzweig Syndrome: Bassen-Kornzweig Syndrome is listed as a type of (or associated with) the following medical conditions in our database:

• Cholesterol metabolism conditions
• Lipid metabolism conditions
• Metabolic conditions
• Blood conditions
• Congenital conditions - metabolic disorders

Symptoms of Bassen-Kornzweig Syndrome (Abetalipoproteinemia)

Some of the symptoms of Bassen-Kornzweig Syndrome incude:

• Absence of apoprotein B-100
• Acanthocytosis
• Low/absent serum betalipoproteins
• Hypercholesterolemia
• Steatorrhea

See full list of 14 symptoms of Bassen-Kornzweig Syndrome (Abetalipoproteinemia)

Treatments for Bassen-Kornzweig Syndrome (Abetalipoproteinemia)

Treatments for Bassen-Kornzweig Syndrome (Abetalipoproteinemia) include:

• Vitamin A + Vitamin E
• Vitamin E - possibly used for treatment of related Vitamin E deficiency

Read more about treatments for Bassen-Kornzweig Syndrome

Treatment of Bassen-Kornzweig Syndrome: For more treatment information about Bassen-Kornzweig Syndrome, see treatment of Abetalipoproteinemia (Bassen-Kornzweig Syndrome)

Bassen-Kornzweig Syndrome: Related Disease Topics

These medical disease topics may be related to Bassen-Kornzweig Syndrome:

• autosomal recessive disorder
• familial abetalipoproteinemia
• trigylcerides
• dyspraxia
• mental retardation
• reinitis pigmentosa
• scoliosis
• muscle weakness
• acanthocytes
• triglycerides

Terms associated with Bassen-Kornzweig Syndrome:

Terms Similar to Bassen-Kornzweig Syndrome:

• Betalipoprotein deficiency disease
• Abetalipoproteinaemia
• Abetalipoproteinemia
• ApoB deficiency

Source: Diseases Database

• Low-density beta lipoprotein deficiency
• Microsomaltriglyceride transfer protein deficiency
• Abetalipoproteinemia
• ABL
• Acanthocytosis

Source - NIH

The following terms can be used for Bassen-Kornzweig Syndrome

• abetalipoproteinemia

Source: CRISP

External links related to: Bassen-Kornzweig Syndrome

• abetalipoproteinemia
• OMIM - ABETALIPOPROTEINEMIA; ABL
• NORD - National Organization for Rare Disorders, Inc.

Source: Diseases Database

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