Extern conference

31 Jan 2008Jitrada, Chanunya

Sumaetha, Hanchai, ApichayaDr. Kamol (supervisor)

A 14-year-old Thai boy

presented with abdominal pain for 1 day.

Present History

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

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

Present History

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.

Investigation Factor VIII assay = 4% Factor IX assay = 53% Factor VIII inhibitor was negative

He was given cryoprecipitate and analgesics.

Past History

Perinatal history

Term, normal labour, BW 3000 g No perinatal complication.

Family history

No one in his family has Hemophilia disease

Pedigree

14 10

41 40asthmaAcute

leukemia55

Drug history

No allergic reaction to cryoprecipitate, Factor VIII, seafood, any drugs.

Complete vaccination as the protocol Normal growth & development

Physical examination

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

HEENT : no epistaxis, no bleeding per gum, pharynx not injected, tonsil not enlarged, good tympanic membrane

Physical examination

Respiratory : clear, no adventitious sounds

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

Genitourinary system : no CVA tenderness

Nervous system : WNL

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

Rt. Lower quadrant abdominal pain for 1 day History of Rt.illiopsoas hematoma for 1 month Underlying disease : Hemophillia A

Differential diagnosis

1. Rebleeding illiopsoas hematoma

2. Surgical condition : acute appendicitis

Investigation

Ultrasonography

17/1/2008

Hemophilia

Hemophilia

Both Hemophilia A and B are X-linked recessive disorders

Prevalence 1 : 5,000 males 80-85% are hemophilia A

Affected females are rare extreme lyonization the presence of 2 independent mutations

Hemophilia

High rate of spontaneous mutation. 30% of patient is sporadic case No positive family history, hemophilia cannot

be ruled out.

Clinical manifestations

Neonate Neonatal bleeding eg. after circumcision (30%) Intracranial hemorrhage (2 %)

Toddlers, young children Obvious symptoms because of increasing physical

activity 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 Factor Activity, %

Cause of Hemorrhage

Mild >5 Major trauma or surgery

Moderate 1-5 Mild-to-moderate

trauma

Severe <1 Spontaneous, hemarthrosis

Lab Studies

Prolonged aPTT Bleeding times, prothrombin times, and

platelet counts are normal Specific assay for FVIII and FIX

Treatment

Localized treatment Hematoma Wound Mucosal bleeding Hemathrosis

Treatment

Specific treatment Mild case: DDAVP Moderate to severe: factor replacement

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 DurationType 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

Who’s need to work up hemophilia? Patient’s mother, sister and daughter Female who has family history of hemophilia Motherhood relation

Consult hematologist

Counseling

Home care Bleeding precaution Dental hygiene care Factor prophylaxis only in severe cases

Progression

D1 (admit) D2 D3 D4 D5 D6

27/12 28/12 17/1 18/1 19/1 20/1 21/1 22/1

11น 18น

19น 24น

6น 18น 6น 6น

Hct 35 38 34 34 36.5 35

PT 12.9 13.5 13.9 13.8

APTT 67.1 31.5 70.7 35.1

F8 4.1 2.2

F8 inh neg neg

Tx F8 100% (3000 U) 100% 100% 50% 50% 25%

s/s tender

RLQ

tender

RLQ

Mild tender RLQ

Mild tender

Not tender

NPO Regular diet

Progression

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

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