1. John
Martinelli,
MSIII,
SGUSOM
DATE:
10/6/13
Pediatrics,
Case
3:
Heart
Transplant,
Immunosuppression
Identifying
Data:
A.A.
is
a
pleasant,
English
speaking,
3-­‐year-­‐old
African-­‐American
boy
who
presented
to
the
SBMC
ED
with
his
mother
and
father
on
the
evening
of
September
24,
2013.
He
was
subsequently
admitted
to
the
pediatric
step
down
unit
on
this
same
visit.
DOB:
6/26/10
Chief
Complaint:
A.A.’s
parents
reported
recent
swelling
of
his
left
eyelid
and
A.A.
pointing
to
his
left
eye
complaining
“my
eye
hurts”.
History
of
Present
Illness:
Beginning
four
days
prior
to
A.A.’s
ED
visit
and
admission,
he
was
observed
to
have
increasing
redness,
swelling,
pain,
and
tenderness
of
his
left
upper
eyelid.
This
continued
to
progress,
which
prompted
his
parents
to
seek
medical
care.
Upon
presentation
to
the
ED,
the
left
upper
lid
was
significantly
erythematous,
edematous,
as
well
as
tender
to
touch.
He
had
not
noticed
a
change
in
vision
or
diplopia.
There
was
no
evidence
of
extra-­‐ocular
muscle
restriction,
paresis,
paralysis,
or
nystagmus.
The
eye
did
not
appear
exophthalmic.
There
was
no
anisocoria
noted.
Conjunctival
injection
was
not
evident.
There
was
no
history
of
recent
sick
contacts,
travel,
change
in
environment,
trauma,
or
insect
bites
reported.
Prior
to
presentation,
he
was
found
to
have
a
temperature
of
101
at
home
and
101.5
recorded
in
the
ED.
He
did
not
have
any
recent
nausea,
vomiting,
diarrhea,
chest
pain,
shortness
of
breath,
or
signs
of
respiratory
distress.
Considering
A.A.’s
increasing
temperature
combined
with
his
ocular
signs
and
symptoms,
it
was
decided
to
admit
for
further
treatment
as
a
precaution
against
progressive
orbital
involvement.
Past
Medical
History:
A.A.
was
born
at
term
via
uncomplicated
cesarean
section
at
8lbs,
0oz
at
Columbia
University
with
a
prenatal
diagnosis
of
left
heart
hypoplasia.
He
was
immediately
admitted
to
the
NICU
and
underwent
his
first
cardiac
surgery
at
5
days
of
age.
Post-­‐operatively,
he
remained
in
the
NICU
for
approximately
one
month.
He
has
an
extensive
cardiac
history
and
required
a
second
procedure
at
6
½
months
of
age.
In
December
of
2011,
he
received
a
successful
allogeneic
heart
transplant.
He
currently
has
no
signs
or
symptoms
of
rejection,
and
on
September
6,
2013
he
underwent
his
annual
heart
biopsy,
which
confirmed
no
rejection.
Subsequent
to
A.A.’s
heart
transplant,
he
did
require
temporary
speech
and
language
therapy;
however,
he
has
progressed
to
a
point
whereby
these
services
have
been
discontinued.
Currently
there
are
no
concerns
with
respect
to
reaching
his
developmental
milestones.
At
the
time
of
his
recent
biopsy,
he
was
diagnosed
with
idiopathic
autoimmune
hemolytic
anemia
for
which
he
required
a
blood
transfusion.
His
current
hemoglobin
is
now
close
to
normal
levels
and
he
continues
to
be
monitored.
He
was
also
found
to
be
neutropenic
during
this
biopsy
visit,
which
has
persisted
to
date.
He
has
had
no
other
serious
or
significant
illnesses
and
his
immunization
history
is
documented
and
up
to
date.

2. Family
History:
A.A.’s
mother
developed
gestational
diabetes
during
her
pregnancy
that
subsequently
resolved
post-­‐
partum.
Maternal
and
paternal
grandparents
history
is
significant
for
DM
Type
II,
with
his
maternal
grandfather
also
being
recently
diagnosed
with
colon
cancer.
A.A.’s
parents
health
history
is
unremarkable.
Social
History:
A.A.
lives
at
home
with
his
mother,
father,
and
three
older
siblings.
A
5-­‐year-­‐old
sister,
14-­‐year-­‐old
sister,
and
a
19-­‐year-­‐old
brother.
Either
his
mother
or
father
is
always
present
at
home.
He
does
occasionally
attend
pre-­‐school
and
according
to
his
parents,
he
is
doing
well
and
they
have
no
concerns
at
this
time.
None
of
his
family
members
smoke
and
there
is
no
smoking
at
home.
There
are
no
pets
at
home.
He
is
able
to
tolerate
an
adult
diet,
however,
he
must
avoid
grapefruit
and
pomegranate
juice
due
to
possible
alterations
in
metabolizing
his
medication.
Medications:
Current:
Poly-­‐Vi-­‐Sol
Drops:
1ml,
Daily
Amlodipine:
1.5mg,
Daily
Ferrous
Sulfate:
15mg,
BID
Lansoprazole
3mg/ml
oral
suspension:
5ml,
Daily
Prednisone
5mg/ml
oral
solution:
3ml,
Daily
Tacrolimus
1mg
oral
capsule:
4mg,
Oral,
Daily,
AM
+
4.5mg,
Oral,
Bedtime.
Upon
Admission:
D5NS
1,000ml:
25ml/hr,
IV
NS
0.9%
Flush
3ml:
2ml,
IV
Push
Zosyn:
1480mg,
29.6ml,
IV
Piggyback,
Q8H
Vancomycin:
225mg,
45ml,
45ml/hr,
IV
Piggyback,
Q6H
Allergies:
NKA,
NKDA
Review
of
Systems
(upon
admission):
General:
Unremarkable,
except
as
documented
in
history
of
present
illness.
Skin:
Unremarkable.
Eye:
As
documented
in
history
of
present
illness,
left
eyelid
erythema,
edema,
pain,
tenderness.
HENT:
Unremarkable.
Normocephalic.
Cardiovascular:
As
documented
in
past
medical
history,
successful
heart
transplant
(2011).
Pulmonary:
Unremarkable.
Lymphatics:
Unremarkable.
Gastrointestinal:
Unremarkable.
Genitourinary:
Unremarkable.
Musculoskeletal:
Unremarkable.
Neurologic:
Unremarkable.
Hematologic:
As
documented
in
past
medical
history,
hemolytic
anemia,
neutropenia.
Endocrine:
Unremarkable.
Psychiatric:
Alert
and
oriented.
Appropriate
affect.
2

3. Physical
Exam
(upon
admission):
Vitals:
BP:
110/76
Weight:
14.8
kg
HR:
113
Height:
96
cm
RR:
24
BMI:
16.1
Pulse
Ox:
100%
(RA)
T:
99.3
General:
Vertical
midline
surgical
scar
evident
at
sternum,
supine,
awakening
from
sleep.
Skin:
Unremarkable.
Eye:
PERRLA
(-­‐)APD.
EOM’s
full
without
restriction
OU,
(-­‐)diplopia,
orthophoric.
Confrontation
visual
fields
full
OU.
(+)Red
reflex
OU.
Upper
lid
OS
with
localized
pre-­‐tarsal
grade
II+
erythema,
II+
edema,
and
tenderness
to
palpation
5/10.
Anterior
segment
(penlight):
(-­‐)Conjunctival
or
episcleral
injection/congestion
OU.
Clear
corneal
reflex
OU.
HENT:
Unremarkable.
Normocephalic.
Cardiovascular:
Regular
rate,
rhythm,
no
murmurs,
adequate
peripheral
perfusion,
no
edema.
Pulmonary:
Clear
to
auscultation
with
equal
breath
sounds
bilaterally.
Gastrointestinal:
Abdomen
non-­‐distended,
soft,
and
non-­‐tender
in
all
quadrants.
Genitourinary:
Deferred
physical
exam.
Musculoskeletal:
Adequate
equal
tone
and
strength
of
upper
and
lower
extremities.
Neurologic:
CN
II
–
XII
intact.
Speech
and
language
intact.
Normal
sensation
upper
and
lower
extremities.
DTR
normal
response
bilaterally.
Lymphatics:
No
lymphadenopathy
at
neck,
axilla,
groin.
Endocrine:
No
evidence
of
goiter,
myxedema,
tremor,
exophthalmos.
Psychiatric:
Alert
and
oriented.
Appropriate
affect.
Labs
(upon
admission):
WBC:
2.9
Na:
136
K:
5.2
Gl:
95
Hgb:
10.6
Cl:
102
HCO3:
22
Ca:
8.9
Platelets:
299
BUN:
18
Cr:
0.56
ALT:
39
Tbili:
0.8
ESR:
10
AST:
40
Tprotein:
7.0
CRP:
7.66
ALP:
705
Albumin:
3.8
Discussion/Differential
Diagnosis:
A.A.
is
a
3-­‐year-­‐old
boy
with
a
history
of
successful
allogeneic
heart
transplantation
performed
in
December
2011
due
to
congenital
hypoplastic
left
heart
syndrome
(HLHS).
HLHS
can
be
described
as
a
malformation
leading
to
a
diminutive
left
ventricle
incapable
of
providing
adequate
systemic
3

4. circulation.
His
post-­‐operative
course
has
continued
to
prove
positive
with
respect
to
rejection,
cardiopulmonary
failure,
infection,
as
well
as
numerous
additional
possible
complications.
At
the
time
of
his
most
recent
heart
biopsy
in
September
2013,
A.A.
reportedly
presented
with
significantly
decreased
hemoglobin
and
hematocrit
levels,
along
with
additional
laboratory
indicators
pointing
to
autoimmune
hemolytic
anemia.
The
etiology
of
the
autoimmune
mechanism
was
not
determined,
however,
a
drug
trigger
is
suspected.
He
was
given
a
single
blood
transfusion
with
an
appropriate
response.
Understanding
the
need
for
immunomodulation
post-­‐transplant
creates
an
additional
area
of
concern
regarding
infection,
be
it
nosocomial,
community,
or
atypical
opportunistic
infection.
His
current
immunosuppressive
therapy
includes
prednisone
in
addition
to
tacrolimus.
Tacrolimus
is
a
macrolide
that
has
been
found
to
reduce
IL-­‐2
cytokine
levels
produced
by
T-­‐Cells,
thereby
assisting
in
the
prevention
of
organ
rejection.
In
addition,
although
rare,
several
studies
have
shown
a
correlation
between
tacrolimus
and
neutropenia
post
transplant.
There
have
also
been
reports
of
neutropenia
being
associated
with
prolonged
prednisone
therapy.
A.A.’s
WBC
count
of
2.9
is
perhaps
indicative
of
tacrolimus
and/or
prednisone
associated
neutropenia.
His
labs
on
this
current
admission
show
a
Hgb
of
10.6
(age
normal
10.5
–
12.7)
suggesting
recovery
from
his
hemolytic
anemia.
However,
of
note,
both
his
ESR
and
CRP
levels
were
elevated
at
10
and
7.66
respectively
(age
normal
ESR
1
–
8,
CRP
0
–
1.2),
perhaps
signifying
persistent
autoimmune
activity.
Also
on
this
admission,
his
ALP
level
was
found
to
be
markedly
elevated
at
705
(age
normal
115
–
391),
which
may
be
interpreted
as
a
normal
variant.
However,
elevated
ALP
in
the
presence
of
normal
liver
function
tests
(ALT/AST/Tbili/Tprotein/Albumin)
as
shown
in
this
case
has
been
recognized
in
various
pathologies
including
bone,
cardiac
failure,
as
well
as
prolonged
corticosteroid
therapy.
This
ALP
elevation
may
therefore
be
representative
of
his
cardiac
history
and/or
current
prednisone
therapy.
Considering
A.A.’s
transplant
history
requiring
ongoing
immunomodulation
therapy,
and
concurrent
with
persistent
neutropenia,
the
predilection
for
acute,
chronic,
or
progressive
complicated
infection
must
constantly
be
assessed.
Therefore,
his
presentation
to
the
ED
with
ocular
signs
and
symptoms
characteristic
of
pre-­‐septal
cellulitis
required
more
aggressive
in-­‐patient
care
versus
typical
outpatient
oral
pharmacotherapy.
Assessment:
1. S/P
Allogeneic
Heart
Transplantation
(December
2011),
successful
post-­‐operative
course.
2. Idiopathic
Autoimmune
Hemolytic
Anemia
(September
2013),
s/p
single
blood
transfusion
with
recovering
hemoglobin
and
hematocrit
levels.
3. Persistent
Idiopathic
Neutropenia
(current
admission).
4. Pre-­‐Septal
Cellulitis
OS
upper
lid
(current
admission),
(-­‐)
evidence
of
orbital
progression/orbital
cellulitis.
Plan:
1. Continue
care
with
cardiac
team
at
Columbia
University.
2. Monitor
CBC
at
Columbia
for
continued
improvement
of
hemoglobin,
hematocrit.
3. Advised
cardiac
team
at
Columbia
of
persistent
diminished
WBC
count/neutropenia
and
increased
infection
risk.
?Consider
future
therapy
modification.
4. Admitted
to
pediatric
step-­‐down
unit
at
SBMC.
Efficacious
response
to
IV
antibiotic
therapy
with
significant
resolution
of
cellulitis,
no
progression
to
orbital
cellulitis.
Discharged
on
9/26/13
with
transition
to
PO
clindamycin
and
omnicef
for
10
days.
Advised
to
return
to
ED
if
signs
or
symptoms
return.
4