Clinical profile of hemophilia patients in Jodhpur Region

Background: Hemophilia is widely distributed all over the world, but little is known about its clinical profile in resource-limited regions. An insight into its clinical spectrum will help in the formulation of policies to improve the situation in these areas. Aims: To study the clinical profile of...

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Main Authors: Vikas Payal, Pramod Sharma, Vishnu Goyal, Rakesh Jora, Manish Parakh, Deepika Payal
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2016-01-01
Series:Asian Journal of Transfusion Science
Subjects:
Online Access:http://www.ajts.org/article.asp?issn=0973-6247;year=2016;volume=10;issue=1;spage=101;epage=104;aulast=Payal
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author Vikas Payal
Pramod Sharma
Vishnu Goyal
Rakesh Jora
Manish Parakh
Deepika Payal
author_facet Vikas Payal
Pramod Sharma
Vishnu Goyal
Rakesh Jora
Manish Parakh
Deepika Payal
author_sort Vikas Payal
collection DOAJ
description Background: Hemophilia is widely distributed all over the world, but little is known about its clinical profile in resource-limited regions. An insight into its clinical spectrum will help in the formulation of policies to improve the situation in these areas. Aims: To study the clinical profile of hemophiliacs (age <18 years) in Jodhpur region and screen them for transfusion-transmitted infections. Materials and Methods: A cross-sectional study conducted in the Department of Pediatrics, Umaid Hospital, Dr. S. N. Medical College, Jodhpur, over a period of 12 months. Result: Out of a total of 56 cases enrolled, 51 (91%) cases were diagnosed as hemophilia A while 5 (9%) were diagnosed as hemophilia B. Positive family history was found in 26 (46%) cases. According to their factor levels, 25 (44%) cases had severe disease, 20 (36%) had moderate disease, and 11 (20%) had mild disease. The mean age of onset of symptoms and diagnosis was 1.73 ± 1.43 and 3.87 ± 3.84 years, respectively. First clinical presentation was posttraumatic bleed in 20 (36%), gum bleeds in 17 (30%), epistaxis in 4 (7%), joint bleeds in 4 (7%), skin bleeds in 4 (7%), and circumcision bleed in 3 (5%) cases. Knee joint was the predominant joint affected by hemarthrosis in 38 (68%), followed by ankle in 29 (52%), elbow in 20 (36%), and hip joint in 7 (13%) cases. All patients had a negative screening test for transfusion-transmitted infections. Conclusion: Occurrence of posttraumatic bleeds and gum bleeds in an otherwise normal child should warn the clinician for evaluation of hemophilia.
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spelling doaj.art-d4d1229069424489845454bd3ff9cd9a2022-12-22T02:28:47ZengWolters Kluwer Medknow PublicationsAsian Journal of Transfusion Science0973-62471998-35652016-01-0110110110410.4103/0973-6247.164269Clinical profile of hemophilia patients in Jodhpur RegionVikas PayalPramod SharmaVishnu GoyalRakesh JoraManish ParakhDeepika PayalBackground: Hemophilia is widely distributed all over the world, but little is known about its clinical profile in resource-limited regions. An insight into its clinical spectrum will help in the formulation of policies to improve the situation in these areas. Aims: To study the clinical profile of hemophiliacs (age <18 years) in Jodhpur region and screen them for transfusion-transmitted infections. Materials and Methods: A cross-sectional study conducted in the Department of Pediatrics, Umaid Hospital, Dr. S. N. Medical College, Jodhpur, over a period of 12 months. Result: Out of a total of 56 cases enrolled, 51 (91%) cases were diagnosed as hemophilia A while 5 (9%) were diagnosed as hemophilia B. Positive family history was found in 26 (46%) cases. According to their factor levels, 25 (44%) cases had severe disease, 20 (36%) had moderate disease, and 11 (20%) had mild disease. The mean age of onset of symptoms and diagnosis was 1.73 ± 1.43 and 3.87 ± 3.84 years, respectively. First clinical presentation was posttraumatic bleed in 20 (36%), gum bleeds in 17 (30%), epistaxis in 4 (7%), joint bleeds in 4 (7%), skin bleeds in 4 (7%), and circumcision bleed in 3 (5%) cases. Knee joint was the predominant joint affected by hemarthrosis in 38 (68%), followed by ankle in 29 (52%), elbow in 20 (36%), and hip joint in 7 (13%) cases. All patients had a negative screening test for transfusion-transmitted infections. Conclusion: Occurrence of posttraumatic bleeds and gum bleeds in an otherwise normal child should warn the clinician for evaluation of hemophilia.http://www.ajts.org/article.asp?issn=0973-6247;year=2016;volume=10;issue=1;spage=101;epage=104;aulast=PayalClinical manifestationhemarthrosishemophilia
spellingShingle Vikas Payal
Pramod Sharma
Vishnu Goyal
Rakesh Jora
Manish Parakh
Deepika Payal
Clinical profile of hemophilia patients in Jodhpur Region
Asian Journal of Transfusion Science
Clinical manifestation
hemarthrosis
hemophilia
title Clinical profile of hemophilia patients in Jodhpur Region
title_full Clinical profile of hemophilia patients in Jodhpur Region
title_fullStr Clinical profile of hemophilia patients in Jodhpur Region
title_full_unstemmed Clinical profile of hemophilia patients in Jodhpur Region
title_short Clinical profile of hemophilia patients in Jodhpur Region
title_sort clinical profile of hemophilia patients in jodhpur region
topic Clinical manifestation
hemarthrosis
hemophilia
url http://www.ajts.org/article.asp?issn=0973-6247;year=2016;volume=10;issue=1;spage=101;epage=104;aulast=Payal
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