Wolman's Disease
Wolman's disease
or
Wolman’s syndrom
is a rare (named after
Moshe Wolman
) , inborn
metabolic
disorder
(
lipidosis
) in which
infants
develop
hepatosplenomegaly
,
calcification
of the
adrenal glands
, and
foam
cells
in the
bone marrow
and other
tissues
. Inclusion
bodies
of
lipid
droplets can be seen in the circulating
white
cells
. After a few weeks when the child is mentally bright and alert it manifests in the first few weeks of life with failure to thrive, diarrhoea, vomiting. Positive Babinski's
reflex
.
Death
in first months of
life
. Both
sexes
affected. Inheritance is
autosomal
recessive
.
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