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 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

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 asthma Acute leukemia 55

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 37.5 o C, 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 S 1 S 2, 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

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 Factor Activity, % Cause of Hemorrhage Mild>5Major trauma or surgery Moderate1-5Mild-to- moderate trauma Severe<1Spontaneous, 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 U/bag 1 U In emergency situation and you don’t know type of hemophilia, you must give FFP only

Replacement Therapy, Dose Calculations FactorHalf-Life, hr Increase After 1 U/kg, % VIII8-122 IX241

Type of hemorhage Desired level and Duration Type of hemorhageDesired level(%)Duration(day) Joint and muscle iliopsoas then then 3-5 CNS/head80-100then501-7then 8-21 Throat and neck80-100then501-7then 8-14 GI80-100then501-7then 8-14 renal503-5 Deep laceration505-7 Major surgeryPreop Postop

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)D2D3D4D5D6 27/1228/1217/118/119/120/121/122/1 11 น 18 น 19 น 24 น 6น6น 18 น 6น6น 6น6น Hct PT APTT F F8 inhneg TxF8 100% (3000 U)100% 50% 25% s/stend er RLQ tend er RLQ Mild tender RLQ Mild tender Not tender NPORegular 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