vonwillebrand disease type 1 case
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Transcript of vonwillebrand disease type 1 case
CASE HISTORY
BY
DR. SIDDQUE AKBAR SATTI
MBBS,FCPSCONSULTANT PHYSICIAN
CAPITAL HOSPITAL ISLAMABAD
PAKISTAN
CASE PRESENTATION
Demographic Details
Mr. XYZ , 38yr old Male, Dental technician, Resident of
Rawalpindi, is admitted through E.R on 10- 12 -2012.
PRESENTING COMPLAINTS;-
Black colored stools......20 days
Shortness of breath ,,,,,,,5 days
HISTORY OF PRESENT ILLNESS;-
Patient was in usual state of health when he
started suffering from passing black colored stools of
soft consistency ,with frequency 1-2 times per day. There
was no history of haematemesis or fresh blood in stools.
There is also history of continuous NSAIDS use a month
before. No history of pain epigastrum ,heart burn ,nausea
or dyspepsia like feeling. Later on with the passage of
time patient start suffering from easy fatigability even
after milder exertion and also shortness of breath
on exertion which was of gradual onset ,progressive and
increased to such extent at time of presentation that
patient cant walk for 10 steps without rest. During this
period and also for about last 2 months he was also
suffering from small mucosal bleed from gums, that had
also increased in amount and frequency at time of
presentation. No other history of bruising of the skin nor
history of bleeding from other site.
PAST MEDICAL HISTORY;-
Not significant
PAST SURGICAL HISTORY;-
No history of any surgical procedure
FAMILY HISTORY;-
Patient has 2 brothers and 2 sisters
Father is diabetic and hypertensive
Mother was also diabetic and died of sudden
cardiac arrest
SOCIOECONOMIC HISTORY;-
Lab technician by profession with monthly
income of about 30,000 a month
lives in well lighted an ventilated home
PERSONAL HISTORY;-
Cigarette smoker.
No history of addiction to any drug or alcohol
Drug history
History of transfusion of whole blood for Anemia one
month back
SYSTEMIC INQUIRY
NAD
EXAMINATION
GPE
A young man lying comfortably in his bed, well oriented in
time , place and person.
VITALS:
Pulse : 90bpm, regular
BP : 130/70mmHg
Temp : 98.6 oF
RR : 16/min
Jaundice: -ve
Cyanosis: -ve
Koilonychia: -ve
Clubbing: -ve
Leuconychia: -ve
Ankle edema: -ve
Thyroid: Not enlarged
Lymhnodes Not palpable
CONT…(GENERAL PHYSICAL EXAMINATION)
Pallor: +ve
Bruises on Right thigh
CVS:- (Unremarkable)
CNS:- (Unremarkable)
GIT:- (Unremarkable)
RESPIRATORY SYSTEM:-
(Unremarkable)
SYSTEMIC EXAMINATIONS
D/D:-1. CLD
2. GASTRITIS
3.Coagulation disorder
INVESTIGATIONS CBC;-
Hb...............3.8 g/dl
TLC.............9400/cmm
RBCs............1.99 million /cmm
Hct................14%
MCV.............70.4 fl (77-96 fl)
MCH.............19.1 pg (26-32 pg)
MCHC...........27.1 gm/dl (32-36 gm/dl)
Platelets............480,000/cmm
PT;-
PT....................15 sec
Control............13 sec
INR.................1.20
APTT;-
APPT....................65 sec
Control............ 33 sec
BT.......................>15 min (Normal 2-11 min)
CT.......................05 min 13 sec (Normal 4-11min)
Hess test..............Negative
STOOL FOR OCCULT BLOOD ...........
Positive
Hepatic profile
S.Bilirubin...........0.7mg/dl
ALT....................38 U/L
GGT....................33 U/L
ALP ....................139U/L
HBsAg.......................Negative
HCV antibody............Positive
HCV PCR Qualitative.......Detected
USG ABDOMEN;-
Fatty liver
No evidence of CLD & Portal
hypertension
H.PYLORI ANTIBODY;-
IgM.....................Non reactive
IgG.....................Reactive
UPPER GI ENDOSCOPY;-
Esophagus normal
Multiple erosions at pre pyloric ,fundus,and lesser
curvature of stomach.
Small ulcler at anterior wall of 1st part of duodenum
and multiple duodenal erosions
Oozing of Blood ++
No oesophageal varices
VON WILLEBRAND AG FACTOR;-
.................7.9% (normal range 50-160)
Results are suggestive of type 1 von willebrand disease
Solubility test for factor X111.............Negative
TC-RBC LABELED STUDY FOR GI
BLEED;-
Scan appearance is suggestive of active bleeding from
stomache and proximal duodenum,moving through the
small intestine.
FINAL DIAGNOSIS
After investigations diagnosis
of vonwillebrand
disease type 1 was made.
TREATMENT
Inf. Esomeprazole 40mg ................ I/V BD
Inj Transamic acid 500mg................ I/V TDS
Transfusion of Red cell concetrate........ 40 pints
Transfusion of cryoprecipitates ……… 126 pints
H.Pylori eradication therapy
Humate P (Anti hemophilic Factor/von
Willebrand Factor Complex)
Loading dose of 40-75 units/kg IV, then 40-60
units/kg q8-12hr for 3 d.
Endoscopic APC(Argon Plasma Coagulation) was done
and bleeding was stopped .
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