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[Recurrent small-bowel intussusceptions revealing a disseminated BCG infection related to severe combined immunodeficiency].
[severe combined immunodeficiency]
Infant
small
-
bowel
intussusceptions
,
most
of
the
time
idiopathic
,
may
exceptionally
reveal
a
severe
digestive
disease
.
We
report
the
case
of
a
4
-
month
-old
infant
who
presented
multiple
and
simultaneous
ileal
intussusceptions
associated
with
severe
acute
gastroenteritis
.
Initially
,
the
infant
showed
a
protein-losing
enteropathy
with
a
clear
alteration
of
the
general
state
of
health
and
undocumented
fever
,
resistant
to
broad
-spectrum
antibiotic
therapy
.
Skin
and
splenic
nodules
associated
with
hepatosplenomegaly
and
pancytopenia
set
in
progressively
.
The
etiologic
evaluation
led
to
the
diagnosis
of
a
Mycobacterium
bovis
BCG
infection
related
to
severe
combined
immune
deficiency
.
The
treatment
consisted
in
anti-
tuberculosis
quadruple
therapy
in
association
with
immunoglobin
supplementation
.
Secondarily
,
the
patient
underwent
gene
therapy
in
a
clinical
trial
.
An
early
BCG
vaccine
in
the
first
weeks
of
life
,
before
the
outbreak
of
infection
revealing
the
immune
deficiency
,
is
a
risk
factor
in
triggering
a
disseminated
BCG
infection
in
immunodepressed
infants
.
This
clinical
case
is
the
first
reported
of
severe
combined
immune
deficiency
revealed
by
small
-
bowel
intussusceptions
related
to
a
disseminated
BCG
infection
.
