Table Comparing Abetalipoproteinemia, Familial Hypobetalipoproteinemia and Chylomicron Retention Disease


 

 

Abeta

FHBL
Heterozygous
(one mutation)

FHBL
Homozygous
(two mutations)

 

CMRD

Failure to thrive

Yes

Rarely

Yes

Yes

Acanthocytes

Yes

Rarely

Some

Some

Fat malabsorption

Yes

Some

Yes

Yes

Fat soluble vitamin deficiency

 

Yes

 

Some

 

Yes

 

Yes

Neurological symptoms

Reduced or may be eliminated with vitamin supplementation

Reduced or may be eliminated with vitamin supplementation

Reduced or may be eliminated with vitamin supplementation

Reduced or may be eliminated with vitamin supplementation

Eye disorders

Reduced or may be eliminated with vitamin supplementation

Reduced or may be eliminated with vitamin supplementation

Reduced or may be eliminated with vitamin supplementation

Reduced or may be eliminated with vitamin supplementation

Fat accumulation in liver

 

Some

 

Yes

 

Yes

 

No

Fat accumulation in small intestine

 

Yes

 

No

 

No

 

Yes

Gene location

 

4q22-q24

2p24
3p22-p21.1
+ others

2p24
3p22-p21.1
+ others

 

5q31.1

Inheritance

Autosomal Recessive

Autosomal Dominant

Autosomal Dominant

Autosomal Recessive

Abeta = Abetalipoproteinemia
FHBL = Familial Hypobetalipoproteinemia
CMRD = Chylomicron Retention Disease

 

What is Abetalipoproteinemia?

Familial Hypobetalipoproteinemia

Chylomicron Retention Disease




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