LEGEND

Pathop

Diagno

Manifes

Complic

Medical

Nursing

Can bleed
with bad

Can bleed
with bad
Injury (ex.
surgery,
shots)

Rare
Hemarthrosi

Impaired p
mobility r/t j

Plasmapheresi
concurrent
immunosuppre
ve therapy

ve therapy

GEND:

athophysiology

Diagnostic Finding

anifestations

omplication

edical Management

ursing Management

Ine
prote
fac

prote
fac
defi

eed
bad

Mi
Hemo
6%-2

eed
bad
(ex.
ery,
s)

Hemo
6%-2
factor

Altered
Pain

e
hrosis

Pain
comp
seco
hema

red physical
y r/t joint pain

eresis or
rrent
uppressi
rapy

rapy

Infusion
frozen
(repla
the

the

Acti
proth
com
conce

com
conce

Predisposing F
Age: Toddler
diagnosis
Genetics: X-Li
Disease
Sex: Male

Sex: Male

Amnioce
or chori
villus sam

nt

nt

villus sam

Ineffective
protection r/t
factor VIII

protection r/t
factor VIII
deficiency

Mild
emophilia
6%-25%

emophilia
6%-25%
actor level

tered comfort:
Pain r/t nerve

Pain r/t nerve

compression
secondary to
hemarthrosis

He was given
Factor VIII
transfusion 25

transfusion 25
IU once daily fo
the next four
days.

usion of fresh
ozen plasma
replacement
therapy)

therapy)

Activated
prothrombin
complex
oncentrates

complex
oncentrates

Conc

osing Factor:
Toddler age
agnosis
cs: X-Linked
isease
ex: Male

Fat

ex: Male

Fat

iocentesis
chorionic
s sampling

s sampling

Change
Ge

Ge

Hemophilia A
(Factor VIII)

Hitting lef
elbow to th
wall

Pain

He

Left elbow

given
VIII
n 250

Left elbow
decreased
ROM

Long-term

n 250
aily for
four
.

Long-term
Damag
CBC

Activated
Partial
Thromboplast
Time (APTT) Te
72.5 sec

Time (APTT) Te
72.5 sec
(prolonged)

Prothrombin
Time (PT) Tes
9.9 sec
(normal)

oncept Map on

Father

Father

Fo
the

Spontaneos ge
DNA Form
Chro

anges in f8
Gene

Chro

Gene

hilia A
VIII)

Clottin

Clottin
Facto
Test
(Facto
Assay

Mode
Hemop
1%-5%

g left
to the
all

Hemop
1%-5%
leve
2.4

Hemarthrosis

elbow

Swelling

elbow
ased
OM

Swelling

term Joint

Can

term Joint
amage

Can
with
Inj

Severe An

ted
al
plastin
TT) Test
sec

Severe An

TT) Test
sec
ged)

mbin
) Test
ec
al)

p on A Patient w
BY:
Cabarrubias
Navarro
Powers

Powers

Formation of
the Son's DNA
(XhY)

(XhY)

os genetic mutatio
Formation on the X
Chromosome

Chromosome

Cha

otting

otting
actor
Test
Factor
ssay)

Fibrino
Test

Moderate
emophilia
-5% factor

emophilia
-5% factor
level:
2.4%

Cu
he
fo

sis

ling

he
fo
wit
trau
an i

ling

Can bleed

par

pr

Can bleed
with Slight
Injury

e Anemia

pr
hem

Com
S

e Anemia

Com
S

nt with Hemoph

as

Mothe

of
NA

Mothe

tation on
he X

Changes in f9
Gene

Gene
Hemophilia B
(Factor IX)

brinogen
Test

Easy bruising

Cutaneous
hematoma
formation

hematoma
formation
with minor
trauma (e.g.,
an injection)

Pain or
paralysis due
to the
pressure of

pressure of
hematomas on
nerves
Compartment
Syndrome

Compartment
Syndrome

mophilia

other

Preci
E

other

Son's DN
the X-Chro
with the

with the

*The X-Link
will be pas
to the

aB
)

to the
generat
majority c
Fem

ising

Us
Hemar

Hemar

Internal
Bleeding

GI bleeding,
with
hematemesi
(vomiting

on

hematemesi
(vomiting
blood)

nt
occult blood

nt
occult blood
in the stools

gastric pain
abdominal p

gastric pain
abdominal p

Severe Inte
Bleedin

Bleedin

Precipitating Factors:
Environment
Trauma

s DNA Carries
-Chromosome
the disease.

the disease.

X-Linked disease
be passed down
to the next

to the next
neration, the
rity carriers are
Female.

Severe
Hemoph
Less than

Less than
factor le

Usual
emarthrosis

emarthrosis

al
ng

Bleedin
the gum
prolon
bleed

ding,
h
mesis
ting

prolon
bleed
following
injuries

mesis
ting
d)

ood

Aminoc
ac

ood
ools

pain, or
nal pain

pain, or
nal pain

e Internal
eding

eding

evere
mophilia
than 1%

than 1%
or level

Spontaneou
hematuria o
epistaxis

hematuria o
epistaxis

eding from
gums and
rolonged
bleeding

rolonged
bleeding
owing minor
ries or cuts

minocaproic
acid

neous
ria or
axis

Can ble
even mi
injury

ria or
axis

Desmop

n bleed
n minor
njury

smopressin