Hemophilia B-Case Report. Bijan Keikhaei, Ahvaz Jundishapur University of Medical Sciences. Case Presentation. A 25 years old man admitted in hospital with complaints of abdominal pain , hematemesis , and melena .
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Bijan Keikhaei, Ahvaz Jundishapur University of
He denied Aspirin taking and Alcohol drinking.
The patient’s family history is noteworthy for consanguineous parents (first cousins) and a brother who also suffering from hemophilia B.
Investigation findings were Hb-4gm/dl, MCV: 84Fl,MCH: 27 Pg, ESR-20mm,BUN:28 mg/dl, Cr:1.2 mg/dl
Platelet count-330000/cmm, PT- 14.5 sec (INR-1.2),
APTT- 82.8 sec,
On examination patient is severely anemic,
semi consciousness, pulse rate-140/min, BP- 80/50 mm Hg.
On examination of musculoskeletal system, there was
wasting of thigh and calf muscles and left knee arthropathy.
What is the possible cause of ?What medical intervention would you in evaluating this patient recommend?
Low blood pressure , high pulse rate and low hemoglobin level were used as severity criteria for hemorrhagic shock.
Upper Gastro Intestinal (UGI) Bleeding is one of the most life-threatening complications, and occurs in up to 25% of persons with Hemophilia .
Duodenal ulcer was the main cause of gastrointestinal bleeding, in 22% of cases. Gastritis was responsible for 14% of cases. In 22% of cases the etiology of gastrointestinal
1-Assure adequate airway, breathing, and circulation
2. Attain venous access as quickly as possible.
3. Infuse appropriate FVIII (hemophilia A) or FIX (hemophilia B) at a dose to achieve physiologic levels immediately (50 IU/kg body weight FVIII or 100-120 IU/kg high purity FIX respectively;
4. Obtain CT scan, ultrasound, or other imaging
studies as indicated to ascertain bleeding
5. Request consultation from appropriate physician
7. Monitor FVIII/FIX levels respectively on a
frequent basis to maintain level in the mid
8. Continue with frequent bolus or continuous
clotting factor infusions .
9. Examine the patient following hospitalization.