Transcript Extern conference

Extern conference
31 Jan 2008
Jitrada, Chanunya
Sumaetha, Hanchai, Apichaya
Dr. Kamol (supervisor)
A 14-year-old Thai boy
presented with abdominal pain for 1 day.
Present History
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1 mo PTA, his younger sister hit him on his
back then he suddenly developed abdominal
pain at RLQ.
He was admitted at the Siriraj hospital.
CT abdomen showed that he had Rt.
iliopsoas hematoma size about 5 cm
Present History
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At that time he was given Factor VIII
To raise factor level 100% for 2 days then
50% for 2 days and 25% for 2 days, clinical
improved and discharged.
Present History
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1 d PTA. He suddenly got RLQ abdominal
pain without referred pain.
Characteristic of pain was sustained dull
aching pain which relieved when he bended
forward.
No nausea or vomiting, diarrhea, dysuria,
fever was observed.
Past History

Underlying disease : Hemophilia A (moderate
severity) which was diagnosed when he was
4 years old. He presented with knee joint
swelling for 1 weeks after he fell down.
Past History
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Investigation
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Factor VIII assay = 4%
Factor IX assay = 53%
Factor VIII inhibitor was negative
He was given cryoprecipitate and analgesics.
Perinatal history
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Term, normal labour, BW 3000 g
No perinatal complication.
Family history

No one in his family has Hemophilia disease
Pedigree
Acute
leukemia
55
asthma
40
41
14
10
Drug history
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No allergic reaction to cryoprecipitate, Factor
VIII, seafood, any drugs.
Complete vaccination as the protocol
Normal growth & development
Physical examination
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Vital sign : T 37.5oC, PR 100/min, RR 18/min,
BP 118/78 mmHg

GA : Good consciousness, not pale, no jaundice,
no dry lips, no petechiae or ecchymosis
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HEENT : no epistaxis, no bleeding per gum,
pharynx not injected, tonsil not enlarged, good
tympanic membrane
Physical examination
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Respiratory : clear, no adventitious sounds
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CVS : normal S1S2, no murmur

Abdomen : no abdominal distension,
soft, tender at Rt. Lower quadrant,
no guarding, no rigidity ,
no rebound tenderness,
normal bowel sound
psoas sign +ve
Physical examination
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Genitourinary system : no CVA tenderness

Nervous system : WNL
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Growth : Weight 45 Kg (P50-75)
Height 150 cm. (P25-50)
Tanner’s stage 3
Development : studies in Grade 8
good social relationship
Problem list
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Rt. Lower quadrant abdominal pain for 1 day
History of Rt.illiopsoas hematoma for 1 month
Underlying disease : Hemophillia A
Differential diagnosis
1.
2.
Rebleeding illiopsoas hematoma
Surgical condition : acute appendicitis
Investigation
Ultrasonography
17/1/2008
Hemophilia
Hemophilia
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Both Hemophilia A and B are X-linked
recessive disorders
Prevalence 1 : 5,000 males
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80-85% are hemophilia A
Affected females are rare
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extreme lyonization
the presence of 2 independent mutations
Hemophilia
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High rate of spontaneous mutation.
30% of patient is sporadic case
No positive family history, hemophilia cannot
be ruled out.
Clinical manifestations
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Neonate
 Neonatal bleeding eg. after circumcision (30%)
 Intracranial hemorrhage (2 %)
Toddlers, young children
 Obvious symptoms because of increasing physical
activity
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Easy bruising
Intramuscular hematoma
Hemarthrosis (Hallmark of hemophilia)
Chronic arthropathy is a late complication of recurrent
hemarthrosis in a target joint.
The absence of hemorrhagic manifestations at birth
does not exclude hemophilia.
Classification
Classification
Mild
Factor
Cause of
Activity, % Hemorrhage
>5
Major trauma or
surgery
Moderate
1-5
Mild-tomoderate
trauma
Severe
<1
Spontaneous,
hemarthrosis
Lab Studies
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Prolonged aPTT
Bleeding times, prothrombin times, and
platelet counts are normal
Specific assay for FVIII and FIX
Treatment
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Localized treatment
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Hematoma
Wound
Mucosal bleeding
Hemathrosis
Treatment
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Specific treatment
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Mild case: DDAVP
Moderate to severe: factor replacement
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Factor VIII 250U/vial
FFP 1U/ml
Cryoprecipitate 80-120 U/bag 1 U
In emergency situation and you don’t know type of
hemophilia, you must give FFP only
Replacement Therapy, Dose Calculations
Factor
Half-Life ,
hr
Increase
After 1
U/kg, %
VIII
8-12
2
IX
24
1
Type of hemorhage
Desired level and Duration
Type of hemorhage
Desired level(%)
Duration(day)
Joint and muscle
40-60
1-3
iliopsoas
80-100 then 30-60
1-2 then 3-5
CNS/head
80-100then50
1-7then 8-21
Throat and neck
80-100then50
1-7then 8-14
GI
80-100then50
1-7then 8-14
renal
50
3-5
Deep laceration
50
5-7
Major surgery
Preop 80-100
Postop 60-80
40-60
30-50
1-3
4-6
7-14
Counseling
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Who’s need to work up hemophilia?
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Patient’s mother, sister and daughter
Female who has family history of hemophilia
Motherhood relation
Consult hematologist
Counseling
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Home care
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Bleeding precaution
Dental hygiene care
Factor prophylaxis only in severe cases
Progression
27/12
28/12
Hct
D1 (admit)
D2
D3
D4
D5
D6
17/1
18/1
19/1
20/1
21/1
22/1
6น
50%
25%
11น
18น
35
38
19น
PT
12.9
13.5
13.9
13.8
APTT
67.1
31.5
70.7
35.1
F8
4.1
F8 inh
Tx
s/s
24น
6น
18น
6น
34
34
36.5
35
2.2
neg
neg
F8 100% (3000 U)
tend
er
RLQ
tend
er
RLQ
NPO
100%
100%
50%
Mild tender
RLQ
Mild
tender
Not
tender
Regular
diet
Progression
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His clinical was improved.
He discharged from the hospital.
Follow up iliopsoas hematoma by U/S whole
abdomen which show hematoma size is
smaller than previous
Take home message
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No positive family history, hemophilia cannot be
ruled out. 30% of patient is sporadic case
In emergency situation and you don’t know type of
hemophilia, you must give FFP only
Thank you for your
attention