CLINICAL PROFILE OF HAEMOPHILIA PATIENTS OF UPPER ASSAM- A HOSPITAL-BASED STUDY

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CLINICAL PROFILE OF HAEMOPHILIA PATIENTS OF UPPER ASSAM- A HOSPITAL-BASED STUDY

Journal Title: Journal of Evolution of Medical and Dental Sciences - Year 2017, Vol 6, Issue 37

Abstract

BACKGROUND Haemophilia is a rare condition where patients present with abnormal bleeding and other complications of bleeding due to genetic or acquired deficiency of coagulation factor VIII (Haemophilia A) or Factor IX (Haemophilia B). Aims and Objectives- We have studied the clinical profile of haemophilia patients who have come to our tertiary medical centre for hospitalisation with some complication. MATERIALS AND METHODS All patients who were admitted in the Department of Medicine and Paediatrics with Haemophilia were included in the study. Detail clinical and family history was taken followed by physical examination and necessary investigations. Factor replacement was done using our institutional protocol, which is based on the Significant Resource Constraint Guidelines of Haemophilia Federation India. RESULTS A total of 79 haemophilia patients were studied with 94.9% males and 4 females; 74.7% were below the age of 20 years and 78.5% were known haemophilia patients, whereas 21.5% were newly diagnosed; 64.6% patients had a definite family history; 79.7% were factor VIII deficient and 20.3% were factor IX deficient; 47 (74.6%) patients had severe factor VIII deficiency, 12 (19%) patients had moderate factor VIII deficiency and 4 (6.4%) patients had mild factor VIII deficiency; 11 (68.7%) patients had severe factor IX deficiency. Out of the 57 patients who presented with joint bleed 38 (67%) had knee joint involvement, 16 (28%) had ankle joint involvement, 13 (22.8%) had wrist involvement, 8 (14%) had elbow involvement and 3 (5.3%) had shoulder joint bleed. Out of the 19 cases of intramuscular bleed, 13 (68.4%) patients had psoas bleed; 43 (75.4%) patients had multiple joint involvement; 7 (12.3%) patients both joint bleed and intramuscular bleed; 68.4% patients had joint deformity. Out of the four females with mild-to-moderate factor VIII deficiency, two had joint bleed and two had uncontrolled menstrual bleed. CONCLUSION Most of our patients were below 20 years (74.7%) and haemophilia A (79.7%). One-third of them did not have a family history. Most of them had a severe factor deficiency and knee joint was the most common joint to bleed. Two-third of the patients had joint deformity.

Authors and Affiliations

Anupam Dutta, Taniya Sarkar Dutta, Pranoy Dey

Keywords

Haemophilia A Haemophilia B Haemarthrosis Factor Deficiency VIII and IX.

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EP ID EP210724
DOI 10.14260/Jemds/2017/645
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