Dear Lactnetters - Would anyone know if it is possible for a mother with
Abetalipoproteinemia to breastfeed? It is a rare autosomal recessive disease
which includes malabsorption of fat and cobblestone mucosa in the intestinal
tract. In looking for studies I found:

Lipid composition and lipolytic activities in milk from a patient with
homozygous familial hypobetalipoproteinemia.

Am J Clin Nutr 1987 Apr;45(4):730-6
Wang CS, Illingworth DR.

We have investigated the composition of breast milk from a patient with
abetalipoproteinemia. Activity level and specific activity of lipoprotein
lipase in milk samples obtained at 0.5 and 3 mo postpartum were higher than
those found in normal milk; activity of bile salt-activated lipase was found
to be higher in the milk at 6 mo postpartum than in normal milk but average
specific activity of this enzyme in milk samples was not increased. Except
for higher protein content of patient's milk, there was no apparent
abnormality in the protein pattern as determined by SDS-polyacrylamide gel
electrophoresis. Lipid analysis indicated a shift in the molecular weight
distribution of triglycerides, which favored lower molecular weight forms
and reflected an increase in medium-chain and a major decrease in long-chain
essential fatty acids (omega 6 and omega 3) in milk triglycerides.

and this:

Am J Clin Nutr 1985 Jan;41(1):121-8

Milk and plasma lipid composition in a lactating patient with type I
hyperlipoproteinemia.

Steiner G, Myher JJ, Kuksis A.

This report describes studies on the plasma and milk lipid composition of a
patient with primary Type I hyperlipoproteinemia who had been followed
through her second pregnancy. Post-partum she lactated, supplying milk for
assay. It was abnormal in the low content of its total lipid and in the
bizarre composition of its fatty acids. The proportion of long chain fatty
acids was unusually low, and that of medium chain fatty acids unusually
high. Furthermore, the fatty acids of the patient's milk differed greatly
from those of her plasma triglycerides. This was in marked contrast to
normal nursing mothers' milk, in which the fatty acid composition is
comparable to that of plasma triglycerides. The patient's milk fatty acids
were shorter in chain length and deficient in essential fatty acids. During
the time of lactation, the patient remained hyperlipidemic and her
post-heparin plasma had no lipolytic activity. These data and the
differences between the plasma and milk fatty acids suggested that in the
patient the circulating triglyceride fatty acids did not enter the mammary
gland. Without preformed fatty acids entering it from plasma or adipose
tissue, the lactating breast apparently synthesized fatty acids de novo.
These newly synthesized fatty acids were of medium, rather than long chain
length. This accounted for the abundance of medium chain length
triglycerides in the patient's milk. The studies suggested that the deficit
of lipoprotein lipase in Type I hyperlipoproteinemia extended to the mammary
gland.

PMID: 3966415 [PubMed - indexed for MEDLINE]


The baby might or might not be affected.  I would appreciate any thoughts.
       Sincerely, Toby Gish RN, IBCLC  (Haifa, Israel)


I

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