7 yrs, male sympyomatic for 5 days Multiple episodes of hematemesis and melena history of echymosis at i.m. site prolonged bleed after cirumcision Elder male sibling having similar complaints Another male sibling died at the age of 9 days due to intracranial bleed(CT proven) Investigation: PT 21.6 (13.5) INR 1.84 aPTT 42.2 (28) Platelets 4.5 lacs Hb 5 TLC 25000 (80 percent neutrophils) Urea 70 Creat 1.8 Albumin 3.9 Globulin 1.2 LFT normal



Probably case of hemophilia. With ARF

Likely to be hematological disorder ?Hemophilia factor correction study

Hemophilia. Bleeding can occur in any part of body. 2% neonates with H. can sustain ICH. Target joints m.c. ankle. 85% Cases are H. A ( factor viii Deficiency), rest 10-15% H.B. The specific assay for factors viii &ix will confirm dx. Treat as severe hemophilia with factor viii concentrate.

Why PT is deranged in hemophilia? And how will you explain deranged KFT in hemophilia?

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Is there is any h/o fever.what about urine output,BP.

No fever Urine output is more than 1 ml/kg/hr

Haemophelic child

Hemophilia with aki

Old hemophilia With ? ARF (HUS)

Severe hemophilia probably



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