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Hemophilia, Pediatric

Char M. Witmer, MD, MSCE Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Hemophilia A is factor VIII deficiency, and hemophilia B is factor IX deficiency.

  • Deficiency or absence of factor VIII or factor IX leads to a delay and disruption of blood clotting t...

DIAGNOSIS

HISTORY

  • Family history

    • Familial history of hemophilia in male offspring of female blood relatives is present in only 70% of cases.

  • Excessive bleeding in a male neonate

    • Excessive bleeding with ci...

TREATMENT

GENERAL MEASURES

  • Joint hemorrhage

    • Factor replacement

    • Immobilization: splints, casts, crutches, and/or bed rest (24 to 48 hours)

    • Prolonged immobilization may reduce recovery of joint ROM.

    • Initiati...

ONGOING CARE

COMPLICATIONS

  • Complications of disease:

    • Hemophilic arthropathy: Repeated joint hemorrhages lead to synovial thickening and joint cartilage erosion. Joint space becomes narrowed and eventual...

ADDITIONAL READING

  • Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol.  2008;140(4):378–384. [View Abstract on OvidInsights]

  • Pruthi RK. Hemophilia: a practical approach to ge...

CODES

ICD9

  • 286.0 Congenital factor VIII disorder

  • 286.1 Congenital factor IX disorder

  • 286.2 Congenital factor XI deficiency

ICD10

  • D66 Hereditary factor VIII deficiency

  • D67 Hereditary factor IX deficiency

  • D68....

FAQ

  • Q: Are there any medications contraindicated in a child with hemophilia?

  • A: Aspirin should not be given as it interferes with platelet function. NSAIDs cause a milder effect on platelets and should...

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