Clinical audit of inherited bleeding disorders in a developing country.
OBJECTIVE
We did a clinical audit to determine the status of coagulation disorders in a hemophilia care center in Pakistan.


METHODS
Fatimid foundation blood bank and hematological diseases center, Lahore.


METHODS
This is a retrospective descriptive study.


METHODS
All patients registered at Lahore center were included. Data was collected using a questionnaire including age, gender, diagnosis, hepatitis and human immune deficiency virus (HIV) status, number of episodes of bleeding, most common site of bleeding, severity of disorder and number of transfusions required to treat the episode.


RESULTS
During the study period, a total of 923 registered patients were reviewed at Lahore center and of these, 408 patients (44.2%) were on regular follow-up. Inherited bleeding disorders identified in these patients included hemophilia A, hemophilia B, vWD, factor VII deficiency, factor V deficiency, factor X deficiency, dysfibrinogenemia, afibrinogenemia, factor XIII deficiency; and platelet function defects. Median age was 17 years with a range of three to 57 years. Median age at diagnosis was one year. There were 329 (80.6%) males and 79 (19.3%) females. The products used in these patients included factor VIII concentrate, fresh frozen plasma, cryoprecipitate, cryosupernatant and platelets. Testing for transmission of viral infections was also done in these patients and one patient (0.2%) was found hepatitis B positive, six patients (1.4%) were hepatitis C positive and two patients (0.49%) were HIV positive.


CONCLUSIONS
Hemophilia A, hemophilia B and vWD are the commonly encountered inherited bleeding disorders in our patients followed by other recessively transmitted disorders with a median age of 17 years and male to female ratio of 4: 1. Most of the patients utilized services available at Fatimid foundation with good clinical results. In Pakistan, non-governmental organizations (NGOs) are trying their best for providing optimal treatment to patients with inherited bleeding disorders. There is a need for government participation to improve the availability of current hemophilia care services.
Sajid
Raihan
R
Department of Pathology and Microbiology, Aga Khan University, Stadium Road, Karachi 74800, Pakistan.
Khalid
Safoorah
S
Mazari
Nazish
N
Azhar
Waleed Bin
WB
Khurshid
Mohammad
M
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D025861:Blood Coagulation Disorders, Inherited / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D054869:Clinical Audit; D003906:Developing Countries; D005260:Female; D006302:Health Services Research; D006801:Humans; D008297:Male; D008875:Middle Aged; D010154:Pakistan; D012737:Sex Factors; D011795:Surveys and Questionnaires; D055815:Young Adult