FREQUENCY OF HAEMOPHILIA AND BLEEDING PARAMETERS OF PERSON’S WITH BLEEDING DISORDERS IN SOUTH EAST NIGERIA

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ABSTRACT

A significant number of people living with haemophilia are reported to be either under-diagnosed or mismanaged in most developing countries. This may results in increase in disease related morbidity and mortality in childhood. The aim of this study is to evaluate the frequency of haemophilia and bleeding parameters of those with bleeding disorders in South East Nigeria. Fifty consecutive consenting persons with bleeding disorder that met inclusion criteria were recruited from the four tertiary hospitals in South East Nigeria. Blood samples were collected for full blood count, coagulation screening test and Factor VIII assays. Data was analyzed using the graph pad prism version 6. Results obtained from this study showed that 2% of subjects with bleeding disorder has haemophilia and they are within mild range, the most common bleeding symptoms is gastrointestinal bleeding (23.4%) and the most common bleeding score of four (32.4%) was obtained from subjects with bleeding disorders in South East Nigeria. Furthermore, this study also showed that thrombocytopaenia (68%) was found in subjects with bleeding disorders. These results showed that haemophilia may not be under-diagnosed as earlier suggested by other authors. However, it may be necessary to screen for Factor VIII in cases of bleeding when indicated. Further studies may be needed to determine the likely causes of thrombocytopaenia in bleeding disorders in our environment. 

TABLE OF CONTENTS

Title Page
Table of Contents
List of Tables
List of Figures
Glossary
Abstract

CHAPTER ONE
Introduction
Justification for the study
Aim and objectives

CHAPTER TWO
Literature Review
Types and clinical severity of haemophilia
Clinical manifestation of haemophilia
Diagnosis
Treatment
Complications
Condensed version of the bleeding assessment tool

CHAPTER THREE
Study design/ methods
Study Area
Ethical clearance
Sample size
Inclusion criteria
Exclusion criteria
Equipment
Sample collection
Haematologic parameters
Coagulation screening test
Correction studies
Specific factor assay
Data Analysis

CHAPTER FOUR
Results

CHAPTER FIVE
Discussion
Conclusion and Recommendation
References

Appendix

ABSTRACT
A significant number of people living with haemophilia are reported to be either under-diagnosed or mismanaged in most developing countries. This may results in increase in disease related morbidity and mortality in childhood. The aim of this study is to evaluate the frequency of haemophilia and bleeding parameters of those with bleeding disorders in South East Nigeria. Fifty consecutive consenting persons with bleeding disorder that met inclusion criteria were recruited from the four tertiary hospitals in South East Nigeria. Blood samples were collected for full blood count, coagulation screening test and Factor VIII assays. Data was analyzed using the graph pad prism version 6. Results obtained from this study showed that 2% of subjects with bleeding disorder has haemophilia and they are within mild range, the most common bleeding symptoms is gastrointestinal bleeding (23.4%) and the most common bleeding score of four (32.4%) was obtained from subjects with bleeding disorders in South East Nigeria. Furthermore, this study also showed that thrombocytopaenia (68%) was found in subjects with bleeding disorders. These results showed that haemophilia may not be under-diagnosed as earlier suggested by other authors. However, it may be necessary to screen for Factor VIII in cases of bleeding when indicated. Further studies may be needed to determine the likely causes of thrombocytopaenia in bleeding disorders in our environment.

CHAPTER ONE
INTRODUCTION
Bleeding disorder affects 1 in 1000 men and women globally (Skinner 2012) with haemophilia being the most well-known inherited bleeding disorder, although it is relatively rare and affect mostly males. Platelet disorder is the most common cause of bleeding disorder and usually acquired rather than inherited, but the most common inherited bleeding disorder in America is vonWillebrand disease and it affects both male and female (America Society of Haematology 2013).
 World Federation of Haemophilia (WFH) estimates that the prevalence of haemophilia worldwide is 105 and 28 per million males of haemophilia A and B respectively, with a global population of 6 billion this would give a prevalence of about 315,000 and 84,000 persons living with haemophilia (PLWH) A and B respectively (O’Mahony et al 2005). Data collected among 84 countries showed that about 115,000 persons has haemophilia which means that about 70% of PLWH remain undiagnosed and untreated mostly in developing countries including Nigeria (O’Mahony et al 2005). Study in Indian reported 14, 718 persons with bleeding disorder and 78.7% of them have haemophilia A (WFH 2013). Prevalence of haemophilia in Senegal in West Africa showed 2.3 per 100,000 male births (Diop et al 2014), while the incidence of haemophilia A and vonWillebrand vWD in South West Nigeria is 1.64% (Fakunle et al 2007).
International society of thrombosis and haemostasis, observed that majority of persons with haemophilia in the world has not been diagnosed because of lack of diagnostic capabilities, no access to proper care, no economic means of managing those persons and little or no available factor VIII replacement therapy (Stonebraker et al 2012). In Nigeria there are paucity of data on the prevalence and characterization of haemophilia but based on WFH it is estimated that there are 9,000 to 12,000 PLWH in Nigeria (WFH 2013), the question is how of those PLWH has being diagnosed in Nigeria?
JUSTIFICATION FOR THE STUDY
PLWH are reported to be either under-diagnosed or mismanaged in most developing countries like Nigeria, resulting in increase in disease related morbidity and mortality in childhood, for those who survive into adulthood it is often complicated with chronic pain and crippling disabilities requiring lifelong care (O’Mahony et al 2005 and Stonebraker et al 2012). These may affect their quality of life, socio-economic status and their ability to contribute positively to the society. Therefore, this study aims at evaluating the frequency of haemophilia and bleeding parameters of person’s with bleeding disorders in South East Nigeria, with the view to generate the much needed data to facilitate and improve the health care given to those people with bleeding disorders. Results generated from this study will provide feedback into characterization of causes of bleeding disorders which may quality of life, life expectancy and reduce complications arising from haemophilia and other causes of bleeding disorder.
AIM AND OBJECTIVES
AIM
The study aims at evaluating the frequency of haemophilia and bleeding parameters of person’s with bleeding disorders in South East Nigeria.
 SPECIFIC OBJECTIVES  
1.     To determine the frequency of haemophilia among person’s with bleeding disorder in South East Nigeria.
2.     To determine the bleeding symptoms of person’s with bleeding disorder in South East Nigeria.

3.     To determine the bleeding scores of person’s with bleeding disorder in South East Nigeria......

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