CLINICAL SEVERITY AND HEMATOLOGICAL PARAMETERS INVESTIGATION OF HEMOPHILIA A PATIENTS FROM SOME CITIES OF SINDH, PAKISTAN

S. H. NAQVI, K. H. LASHARI, Z. A. PALH, M. A. QURESHI, F. MEMON

Abstract


Hemophilia A is a serious inherited as an X-linked bleeding disorder, which is caused by a deficiency or complete absence of factor VIII activity. In the developing countries it is reported that around 80% of thepatients were affected with hemophilia. In Pakistan the disease was seen in all parts of the country because marriages in blood relatives. The aim of this study to collect the data of Hemophillia A and to give basic awareness to the society about Hemophiliacs by involving government to provide diagnostic and health care facilities. This study was also designed for investigation, correlation of some necessary and related blood parameters from Hemophilia A patients in some cities of Sindh, Pakistan. 28 hemophilia A families were selected from Hyderabad and its adjacent areas. A questionnaire (22 questions) was filled for the collection of complete history of patients. On the basis of collected information about hemophilia A patients family charts/ pedigrees were drawn. Spreading of hemophilia A also categories and observed on the basis of marriages within or outside families, ethnic and different age groups and occurrence in different cities. The Coagulation, hematological and biochemical parameters were also assessed to determine the correlation with hemophilia A patients. It was noted that 71% marriages were within family and belonged to four different ethnic groups. Seven different cities Hyderabad and its adjacent areas were also observed the average of Hyderabad patients were higher as compared to other cities. The reported data in this study exhibited some correlation and some variation in the blood parameters results in different age group, ethnic groups etc.


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