alexa Demographics And Clinical Data Of Hemophilia Patients Presented In A Tertiary Care Hospital Of Pakistan
ISSN: 2155-9864

Journal of Blood Disorders & Transfusion
Open Access

Like us on:
OMICS International organises 3000+ Global Conferenceseries Events every year across USA, Europe & Asia with support from 1000 more scientific Societies and Publishes 700+ Open Access Journals which contains over 50000 eminent personalities, reputed scientists as editorial board members.

Open Access Journals gaining more Readers and Citations

700 Journals and 15,000,000 Readers Each Journal is getting 25,000+ Readers

This Readership is 10 times more when compared to other Subscription Journals (Source: Google Analytics)

Share This Page

Additional Info

Loading
Loading Please wait..
 

International Conference on Hematology and Oncology
June 29-July 01, 2017 Bangkok, Thailand

Arifa Khalid, Syed Nasir Abbas Bukhari, Safia Khan, Saadia Anwar, Mahwish Faizan, Usman Wasti
The Children’s Hospital & The Institute of Child Health, Pakistan
Posters & Accepted Abstracts: J Blood Disord Transfus
DOI: 10.4172/2155-9864-C1-026
Abstract
Background: Hemophilia is a rare X-linked recessive congenital bleeding disorder and has been classified as Hemophilia A (deficiency of Factor VIII), and Hemophilia B (Deficiency of factor IX). The overall incidence is 1 per 10,000 male live births with 80% cases of Hemophilia A. The characteristic phenotype is the bleeding tendency and most of the patients present with easy bruising, prolonged post traumatic bleeds and hemarthrosis. The severity of bleeding in Hemophilia correlates with the level of factor deficiency. Late diagnosis and improper management can lead to lifelong complication of chronic arthropathy. Therefore timely and proper factor replacement therapy can prevent complications and improve quality of life. Objective: The study was conducted to analyze the clinical data of disease in a developing country with limited resources so that we can predict how we can prevent morbidity and improve the quality of life in our set up. Methods: The population of the research was patients of Hemophilia, who were reported, in last one year, in Children’s Hospital & Institute of Child Health, Lahore. A sample of 55 patients of Hemophilia A and B were reviewed retrospectively. The data was collected irrespective of any discrimination based on demographic factors and following factors were recorded: Clinical features, age at first presentation, age at diagnosis, severity of disease, frequency of admission, factor replacement, and life threatening bleed. Blood complete picture, bleeding time, PT/APTT, mixing studies and factor assays were noted. Results: Among these 55 patients all were male. Around 93% of the patients were diagnosed as Hemophilia A and approximately 7% as Hemophilia B. Most of the patients diagnosed after 1 year of age (96.4%) although approximately 62% were symptomatic before 1 year of age. About 49% of them were having moderate disease, mild disease was noted in 18%, and rest were with severe disease. Family history was positive in 51% and in all the patients who presented before 6 months of age had severe hemophilia. APTT was prolonged in all the patients with the mean value of >1 minute. Severity of disease and family history has significant relation at p<0.005, while relation between severity of disease and the age at diagnosis is significant but compromised at p=0.05, i.e., critical line. In this research, post circumcision bleed (45.5%) was observed prominently as primary symptom followed by bruises and epistaxis, respectively. However, life threatening bleed was reported in 9% and about 66% developed hemarthrosis. Only 2 patients had APTT done before circumcision. Most of the patients were on demand factor replacement therapy i.e.74.6% and only 5.4% of them were on primary prophylaxis. Conclusion: On the basis of early presentation and family history, we can predict the severity of the disease. Since family history has strong correlation with severity of disease, therefore performing PT/APTT before circumcision will be helpful and cost effective as screening and early diagnosis of Hemophilia. Early diagnosis, adequate and timely prophylaxis can prevent complications like hemarthrosis leading to chronic arthropathy and severe life threatening bleeds. It can help to improve the quality of life and decrease the morbidity and mortality in a developing country.
image PDF   |   image HTML
 

Relevant Topics

Peer Reviewed Journals
 
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2017-18
 
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

Agri & Aquaculture Journals

Dr. Krish

[email protected]

1-702-714-7001Extn: 9040

Biochemistry Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Business & Management Journals

Ronald

[email protected]

1-702-714-7001Extn: 9042

Chemistry Journals

Gabriel Shaw

[email protected]

1-702-714-7001Extn: 9040

Clinical Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Engineering Journals

James Franklin

[email protected]

1-702-714-7001Extn: 9042

Food & Nutrition Journals

Katie Wilson

[email protected]

1-702-714-7001Extn: 9042

General Science

Andrea Jason

[email protected]

1-702-714-7001Extn: 9043

Genetics & Molecular Biology Journals

Anna Melissa

[email protected]

1-702-714-7001Extn: 9006

Immunology & Microbiology Journals

David Gorantl

[email protected]

1-702-714-7001Extn: 9014

Materials Science Journals

Rachle Green

[email protected]

1-702-714-7001Extn: 9039

Nursing & Health Care Journals

Stephanie Skinner

[email protected]

1-702-714-7001Extn: 9039

Medical Journals

Nimmi Anna

[email protected]

1-702-714-7001Extn: 9038

Neuroscience & Psychology Journals

Nathan T

[email protected]

1-702-714-7001Extn: 9041

Pharmaceutical Sciences Journals

Ann Jose

[email protected]

1-702-714-7001Extn: 9007

Social & Political Science Journals

Steve Harry

[email protected]

1-702-714-7001Extn: 9042

 
© 2008- 2017 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version
adwords