{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T12:07:56Z","timestamp":1779797276943,"version":"3.53.1"},"reference-count":10,"publisher":"Scientific Scholar","content-domain":{"domain":["ijmr.org.in"],"crossmark-restriction":true},"short-container-title":["Indian J Med Res"],"abstract":"<jats:sec>\n                    <jats:title>Background and objectives<\/jats:title>\n                    <jats:p>Congenital fibrinogen disorders encompass both quantitative and qualitative abnormalities of fibrinogen, characterised by variable bleeding or thrombotic tendencies. Due to their rarity and varied presentations, delays in diagnosis are common, which can significantly impact patient outcomes. This study was conducted to describe the clinical and demographic profiles of patients with congenital quantitative fibrinogen disorders over a 20-year period (2003-2024) at a tertiary care centre.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective analysis was conducted using laboratory records to identify 17 patients with quantitative fibrinogen disorders, characterised by prolonged prothrombin time (PT), activated partial thromboplastin time (APTT), and undetectable or low fibrinogen levels. Only 10 out of 17 patients could be contacted for follow up. The clinical presentation, including scoring of bleeding symptoms using the International Society on Thrombosis and Haemostasis - Bleeding Assessment Tool (ISTH-BAT), as well as demographic variables such as age of onset, first visit to our hospital, and diagnostic delays, were recorded. Treatment and follow up data were recorded.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Majority of patients showed symptoms in the neonatal period, with umbilical bleeding as the most common initial manifestation, followed by post-traumatic and cutaneous bleeding. Despite early symptom onset, a median diagnostic delay of four years was observed. Seven cases were under regular follow up with on-demand therapy. Three patients died during follow up. Most of the cases received fresh frozen plasma.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation and conclusions<\/jats:title>\n                    <jats:p>This study describes the clinical profile of quantitative fibrinogen disorders and emphasises the importance of increased awareness, early diagnosis, and standardised management in these patients. Given the significant risk of bleeding, proactive measures such as patient education and close monitoring are crucial.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.25259\/ijmr_2494_2025","type":"journal-article","created":{"date-parts":[[2026,5,17]],"date-time":"2026-05-17T12:14:34Z","timestamp":1779020074000},"page":"687-691","update-policy":"https:\/\/doi.org\/10.25259\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Quantitative fibrinogen disorders: A retrospective study from a tertiary care centre in India"],"prefix":"10.25259","volume":"163","author":[{"given":"Mili","family":"Jain","sequence":"first","affiliation":[{"name":"Department of Pathology, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Himadri","family":"Shukla","sequence":"additional","affiliation":[{"name":"Department of Pathology, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rashmi","family":"Kushwaha","sequence":"additional","affiliation":[{"name":"Department of Pathology, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjay","family":"Mishra","sequence":"additional","affiliation":[{"name":"Department of Pathology, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geeta","family":"Yadav","sequence":"additional","affiliation":[{"name":"Department of Pathology, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nishant","family":"Verma","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10894","published-online":{"date-parts":[[2026,5,16]]},"reference":[{"key":"10.25259\/IJMR_2494_2025\/ref-1","doi-asserted-by":"publisher","first-page":"304","DOI":"10.1111\/bjh.13058","article-title":"Br J Haematol","volume":"167","author":"Mumford","year":"2014"},{"key":"10.25259\/IJMR_2494_2025\/ref-2","doi-asserted-by":"publisher","first-page":"1938","DOI":"10.1111\/j.1538-7836.2012.04844.x","article-title":"Classification of rare bleeding disorders (RBDs) based on the association between coagulant factor activity and clinical bleeding severity","volume":"10","author":"Peyvandi","year":"2012","journal-title":"J Thromb Haemost."},{"key":"10.25259\/IJMR_2494_2025\/ref-3","doi-asserted-by":"publisher","first-page":"3127","DOI":"10.1182\/blood.2020009472","article-title":"Clinical phenotype, fibrinogen supplementation, and health-related quality of life in patients with afibrinogenemia","volume":"137","author":"Casini","year":"2021","journal-title":"Blood."},{"key":"10.25259\/IJMR_2494_2025\/ref-4","doi-asserted-by":"publisher","first-page":"2140","DOI":"10.3390\/diagnostics11112140","article-title":"Congenital afibrinogenemia and hypofibrinogenemia: Laboratory and genetic testing in rare bleeding disorders with life-threatening clinical manifestations and challenging management","volume":"11","author":"Simurda","year":"2021","journal-title":"Diagnostics (Basel)."},{"key":"10.25259\/IJMR_2494_2025\/ref-5","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1111\/hae.12368","article-title":"Rare coagulation factor deficiencies: A countrywide screening data from India","volume":"20","author":"Shetty","year":"2014","journal-title":"Haemophilia."},{"key":"10.25259\/IJMR_2494_2025\/ref-6","doi-asserted-by":"publisher","first-page":"611","DOI":"10.1111\/hae.12143","article-title":"Molecular basis of quantitative fibrinogen disorders in 27 patients from India","volume":"19","author":"Sumitha","year":"2013","journal-title":"Haemophilia."},{"key":"10.25259\/IJMR_2494_2025\/ref-7","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1111\/j.1538-7836.2012.04653.x","article-title":"Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: Results from the European network of rare bleeding disorders","volume":"10","author":"Peyvandi","year":"2012","journal-title":"J Thromb Haemost."},{"key":"10.25259\/IJMR_2494_2025\/ref-8","first-page":"2063","article-title":"ISTH-BAT\/SSC joint VWF and Perinatal\/Pediatric Hemostasis Subcommittees Working Group","volume":"8","author":"Rodeghiero","year":"2010","journal-title":"ISTH-BAT\/SSC bleeding assessment tool: Astandardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost."},{"key":"10.25259\/IJMR_2494_2025\/ref-9","doi-asserted-by":"publisher","first-page":"831","DOI":"10.1111\/hae.12503","article-title":"Normal range of bleeding scores for the ISTH-BAT: Adult and pediatric data from the merging project","volume":"20","author":"Elbatarny","year":"2014","journal-title":"Haemophilia."},{"key":"10.25259\/IJMR_2494_2025\/ref-10","doi-asserted-by":"publisher","first-page":"e195","DOI":"10.1111\/j.1365-2516.2011.02730.x","article-title":"Correlating clinical manifestations with factor levels in rare bleeding disorders: A report from Southern India","volume":"18","author":"Viswabandya","year":"2012","journal-title":"Haemophilia."}],"container-title":["The Indian Journal of Medical Research"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/ijmr.org.in\/quantitative-fibrinogen-disorders-a-retrospective-study-from-a-tertiary-care-centre-in-india\/","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/ijmr.org.in\/quantitative-fibrinogen-disorders-a-retrospective-study-from-a-tertiary-care-centre-in-india\/","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T11:54:12Z","timestamp":1779796452000},"score":1,"resource":{"primary":{"URL":"http:\/\/ijmr.org.in\/quantitative-fibrinogen-disorders-a-retrospective-study-from-a-tertiary-care-centre-in-india\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,16]]},"references-count":10,"alternative-id":["10.25259\/IJMR_2494_2025"],"URL":"https:\/\/doi.org\/10.25259\/ijmr_2494_2025","relation":{},"ISSN":["0971-5916"],"issn-type":[{"value":"0971-5916","type":"print"}],"subject":[],"published":{"date-parts":[[2026,5,16]]},"article-number":"687"}}