{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,9,27]],"date-time":"2024-09-27T04:29:11Z","timestamp":1727411351752},"reference-count":18,"publisher":"Scientific Scholar","issue":"04","license":[{"start":{"date-parts":[[2022,6,7]],"date-time":"2022-06-07T00:00:00Z","timestamp":1654560000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Lab Physicians"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Background\u2003The objective of this study is to study the prevalence, clinical spectrum, and hematological profile of inherited bleeding disorder with special reference to von Willebrand disease in eastern India.<\/jats:p><jats:p>Materials and Methods\u2003This prospective study was done in a tertiary care center in the eastern part of India over 2 years. Detailed history and clinical findings were noted in a proforma. Laboratory analysis included prothrombin time, activated partial thromboplastin time, bleeding time, and fibrinogen assay along with tests related to specific factor assay.<\/jats:p><jats:p>Results\u2003One hundred and five patients were diagnosed as suffering with the inherited bleeding disorder out of a total of 1,204 patients. The age of patients ranged from 13 days to 35 years. The most common presenting clinical feature was prolonged bleeding after cut (76.19%). Out of 105 patients, 97 patients (92.38%) had coagulation defect, 5 patients (4.76%) had von Willebrand disease (vWD), and 3 patients (2.85%) had platelet defect. Most common coagulation defect was hemophilia A (84 cases), followed by hemophilia B (8 cases). Other rare congenital factor deficiencies were seen in five cases (5.15%). Only platelet defect was Glanzmann's thrombasthenia (GT). The age of vWD patients ranged from 4.5 years to 24 years. Forty percent patients with vWD disease were type 1 followed by 40% of type 2N and 20% of type 3 vWD.<\/jats:p><jats:p>Conclusion\u2003vWD was not so common in eastern India. vWD was present only in 4.76% cases in this study. The most common coagulation defect was hemophilia A (86.59%) in our study. GT was present in only 2.85% cases.<\/jats:p>","DOI":"10.1055\/s-0042-1748827","type":"journal-article","created":{"date-parts":[[2022,6,7]],"date-time":"2022-06-07T23:56:36Z","timestamp":1654646196000},"page":"465-470","source":"Crossref","is-referenced-by-count":1,"title":["Spectrum of Inherited Bleeding Disorder with Special Reference to von Willebrand Disease in Eastern India"],"prefix":"10.25259","volume":"14","author":[{"given":"Nehal","family":"Ahmad","sequence":"additional","affiliation":[{"name":"Department of Pathology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Subhajit","family":"Das","sequence":"additional","affiliation":[{"name":"Divisional Medical Officer, Indian Railway, New Jalpaiguri, West Bengal, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jyoti","family":"Shukla","sequence":"additional","affiliation":[{"name":"Department of Pathology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammad Jaseem","family":"Hassan","sequence":"additional","affiliation":[{"name":"Department of Pathology, J.N. Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10894","published-online":{"date-parts":[[2022,6,7]]},"reference":[{"key":"ref1","first-page":"68","volume-title":"Inherited blood clotting disorders","author":"R Baklaja","year":"2008"},{"issue":"01","key":"ref2","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1111\/j.1365-2516.2009.02127.x","article-title":"A study of variations in the reported haemophilia A prevalence around the world","volume":"16","author":"J S Stonebraker","year":"2010","journal-title":"Haemophilia"},{"key":"ref3","first-page":"126","article-title":"Guideline for the treatment of haemophilia in South Africa","volume":"98","author":"J N Mahlangu","year":"2008","journal-title":"S Afr Med J"},{"issue":"03","key":"ref4","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1111\/j.1365-2516.2004.00894.x","article-title":"The diagnosis of von Willebrand disease: a 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from western India","volume":"121","author":"S Trasi","year":"2005","journal-title":"Indian J Med Res"},{"issue":"03","key":"ref12","doi-asserted-by":"crossref","first-page":"486","DOI":"10.4103\/0377-4929.68287","article-title":"Prevalence and spectrum of von Willebrand disease in Eastern Uttar Pradesh","volume":"53","author":"S Kumar","year":"2010","journal-title":"Indian J Pathol Microbiol"},{"issue":"06","key":"ref13","doi-asserted-by":"crossref","first-page":"E131","DOI":"10.1177\/1076029610391650","article-title":"Congenital bleeding disorders in Karachi, Pakistan","volume":"17","author":"M Borhany","year":"2011","journal-title":"Clin Appl Thromb Hemost"},{"issue":"S 02","key":"ref14","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1046\/j.1365-2516.1999.0050s2071.x","article-title":"Von Willebrand disease in Denmark: demography and treatment","volume":"5","author":"E Scheibel","year":"1999","journal-title":"Haemophilia"},{"issue":"06","key":"ref15","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1007\/BF01695475","article-title":"A study of von Willebrand's disease in Jordan","volume":"64","author":"A S Awidi","year":"1992","journal-title":"Ann Hematol"},{"issue":"04","key":"ref16","first-page":"187","article-title":"[Epidemiology of von Willebrand's disease in the state of Zulia, Venezuela]","volume":"32","author":"M Diez-Ewald","year":"1991","journal-title":"Invest Clin"},{"issue":"02","key":"ref17","first-page":"42","article-title":"Prevalence and clinico-hematologic scenario of von Willebrand disease in a population of Filipinos with bleeding tendency","volume":"16","author":"N G Chuamary","year":"2006","journal-title":"Sysmex J Int"},{"issue":"03","key":"ref18","doi-asserted-by":"crossref","first-page":"1321","DOI":"10.1111\/j.1538-7836.2005.01336.x","article-title":"Relatively high frequency of VWD types 3 and 2 in a cohort of 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