haemophilia in orthopaedics
DESCRIPTION
HAEMOPHILIA IN ORTHOPAEDICS. PRESENTER: DR. LEMAYIAN DISCUSSANT: DR.KARANU. B.O. MALE 35YRS OLD DISTRICT OF ORIGIN: MARSABIT DATE OF ADMISSION: 2/3/2012 DATE OF DISCHARGE: 21/8/2012. Referral from Marsabit District Hospital as a known haemophiliac - PowerPoint PPT PresentationTRANSCRIPT
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HAEMOPHILIA IN ORTHOPAEDICSPRESENTER: DR. LEMAYIANDISCUSSANT: DR.KARANU
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B.O. MALE 35YRS OLD DISTRICT OF ORIGIN: MARSABIT DATE OF ADMISSION: 2/3/2012 DATE OF DISCHARGE: 21/8/2012
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Referral from Marsabit District Hospital as a known haemophiliac
1/12 h/o septic wound dorsal aspect of right foot
Involved in an RTA 1/12 prior sustaining degloving injury to the dorsal aspect of the right foot
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Reason for refferal
Surgical debridement done twice at the hospital resulted in excessive bleeding
FURTHER MANAGEMENT
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PMHx/PSHx
Excessive bleeding during circumcision Recurrent swelling of the knee joints since he
was 5yrs old Occurred with trivial falls and while playing Small cuts and bruises that would bleed for
long Admitted severally to Marsabit D.H. with DX of
BLEEDING DISORDER Multiple whole blood transfusions Progressive joint deformation esp RT. Worsened
in 1997 resulting in pathological fusion
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FSHx
1st born in a family of 5 siblings(2 other brothers and 2 sisters)
Both brothers are also known haemophiliacs
DX made at KNH in one of the younger brothers in 1990 when he was referred with similar severe bleeding disorder
Patient works as a shop attendant in Marsabit
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Maternal Side-9 of his mother’s relatives had died due to excessive bleeding post circumcision
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EXAMINATION UPON ADMISSION FGC , wasted, Mildly pale BP-109/67mmHg, PR-60/minMSS:Fused right knee in fixed extension, valgus
deformityWasted LL musculature bilaterallyNecrotic wound on the dorsal aspect of right
footL knee also deformed in fixed flexionNo signs of any soft tissue swellingsSensation was intactOTHER SYSTEMS: Essentially normal
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INVX AT ADMISSION
CBC- Hb 9.38g/dl -WBC 5.23; Neutrophil 50% -platelets 294 Coagulation profile -PT test 19s -PTI 73.7% -INR 1.36
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-APTT test >120s -APTT control 30s U/E/CR-normal XRAY L KNEE-complete joint fusion -subchondral cysts -disuse osteopaenia
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SUBSEQUENT TESTS
p24-Reactive CD4+-205 cells/microlitre Viral load-? Substitution TestsNormal plasma+ pt’s plasma(1:1)—39.5sFVIII def plasma+ pt’s plasma(1:1)—75.1sFIX def plasma + pt’s plasma(1:1)—50.4sDEFINITIVE DX: FVIII def (Haemophilia A)
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Progressively:
Sepsis worsened despite several surgical debridements
Metartarsals became exposed Nutritionist involvement—nutritional
supplementation Haematologist review Initial Decision-midfoot amputation
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In view of worsening and ascending infection (as well as patient preference) decision made to do BKA
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Pre-Op blood workup
CBC- Hb 7.38g/dl; WBC 4.47; Neutrophils- 44.3%; Platelets- 320
U/E/CR-normal(Na+ 131; k+ 3.52; Urea 2.3 mmol/l)
GXM 4 Units of cryoprecipitate
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Transfused 2 units pre-op, 1 unit intra-op and 1 unit post-op
BKA done on 13/8/12 Post-op medication:i. i.v. tramadol 50mg BDii. i.v ceftriaxone 1g BD Discharged through both orthopaedic
and haematology clinics
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