BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuCongenital fibrinogen deficiencyAfibrinogenemia; Hypofibrinogenemia; Dysfibrinogenemia; Factor I deficiencyCongenital fibrinogen deficiency is a very rare, inherited blood disorder in which the blood does not clot normally. It affects a protein called fibrinogen. This protein is needed for the blood to clot. Causes This disease is due to a deficiency of the amount or function of fibrinogen. How fibrinogen is affected depends on how the genes that determine how it is formed are inherited:When the abnormal gene is passed down from both parents, a person will have a complete lack of fibrinogen (afibrinogenemia). When the abnormal gene is passed down from one parent, a person will have either a reduced level of fibrinogen (hypofibrinogenemia) or a problem with the function of fibrinogen (dysfibrinogenemia). Sometimes, these two fibrinogen problems can occur in the same person. Symptoms People with a complete lack of fibrinogen may have any of the following bleeding symptoms:Bruising easily BruisingA bruise is an area of skin discoloration. A bruise occurs when small blood vessels break and leak their contents into the soft tissue beneath the s...Read Article Now Book Mark Article Bleeding from the umbilical cord just after birth Bleeding in the mucous membranes Bleeding in the brain (very rare) Bleeding in the joints Heavy bleeding after injury or surgery Nosebleeds that do not stop easily People with a reduced level of fibrinogen bleed less often and the bleeding is not as severe when compared to people with no fibrinogen at all. Those with a problem with the function of fibrinogen often don't have symptoms. Exams and Tests If your health care provider suspects this problem, you will have lab tests to confirm the type and severity of the disorder.Tests include:Bleeding time Bleeding timeBleeding time is a medical test that measures how fast small blood vessels in the skin stop bleeding. Because it is difficult to perform correctly, i...Read Article Now Book Mark Article Fibrinogen test and reptilase time to check fibrin level and quality Fibrinogen testFibrinogen is a protein produced by the liver. This protein helps stop bleeding by helping blood clots to form. A blood test can be done to tell ho...Read Article Now Book Mark Article Partial thromboplastin time (PTT) Partial thromboplastin timePartial thromboplastin time (PTT) is a blood test that looks at how long it takes for blood to clot. It can help tell if you have a bleeding problem...Read Article Now Book Mark Article Prothrombin time (PT) Prothrombin timeProthrombin time (PT) is a blood test that measures the time it takes for the liquid portion (plasma) of your blood to clot. It measures the functio...Read Article Now Book Mark Article Thrombin time Treatment The following treatments can be used for bleeding episodes or to prepare for surgery:Cryoprecipitate (a blood product containing concentrated fibrinogen and other clotting factors) Fibrinogen (RiaSTAP) Plasma (the liquid portion of the blood containing clotting factors) People with this condition should get the hepatitis B vaccine. Having many transfusions raises your risk of getting hepatitis.Hepatitis B vaccineAll content below is taken in its entirety from the CDC Hepatitis B Vaccine Information Statement (VIS): www. cdc. gov/vaccines/hcp/vis/vis-statement...Read Article Now Book Mark Article Outlook (Prognosis) Excessive bleeding is common with this condition. These episodes may be severe, or even fatal. Bleeding in the brain is a leading cause of death in people with this disorder. Possible Complications Complications may include:Blood clots with treatment Development of antibodies (inhibitors) to fibrinogen with treatment Gastrointestinal bleeding Gastrointestinal bleedingGastrointestinal (GI) bleeding refers to any bleeding that starts in the gastrointestinal tract. Bleeding may come from any site along the GI tract, ...Read Article Now Book Mark Article Miscarriage MiscarriageA miscarriage is the spontaneous loss of a fetus before the 20th week of pregnancy. Pregnancy losses after the 20th week are called stillbirths. Mi...Read Article Now Book Mark Article Rupture of the spleen Slow healing of wounds When to Contact a Medical Professional Contact your provider or seek emergency care if you have excessive bleeding.Tell your surgeon before you have surgery if you know or suspect you have a bleeding disorder. Prevention This is an inherited condition. There is no known prevention.Open ReferencesReferencesGailani D, Benjamin TF, Wheeler AP. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 135.Ragni MV. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 165.AllVideoImagesTogRelated Information Blood clots(Condition)Fibrinogen blood test(Medical Test)Gastrointestinal bleeding(Symptoms) Review Date: 2/2/2023 Reviewed By: Mark Levin, MD, Hematologist and Oncologist, Monsey, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. 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Congenital fibrinogen deficiencyAfibrinogenemia; Hypofibrinogenemia; Dysfibrinogenemia; Factor I deficiencyCongenital fibrinogen deficiency is a very rare, inherited blood disorder in which the blood does not clot normally. It affects a protein called fibrinogen. This protein is needed for the blood to clot. Causes This disease is due to a deficiency of the amount or function of fibrinogen. How fibrinogen is affected depends on how the genes that determine how it is formed are inherited:When the abnormal gene is passed down from both parents, a person will have a complete lack of fibrinogen (afibrinogenemia). When the abnormal gene is passed down from one parent, a person will have either a reduced level of fibrinogen (hypofibrinogenemia) or a problem with the function of fibrinogen (dysfibrinogenemia). Sometimes, these two fibrinogen problems can occur in the same person. Symptoms People with a complete lack of fibrinogen may have any of the following bleeding symptoms:Bruising easily BruisingA bruise is an area of skin discoloration. A bruise occurs when small blood vessels break and leak their contents into the soft tissue beneath the s...Read Article Now Book Mark Article Bleeding from the umbilical cord just after birth Bleeding in the mucous membranes Bleeding in the brain (very rare) Bleeding in the joints Heavy bleeding after injury or surgery Nosebleeds that do not stop easily People with a reduced level of fibrinogen bleed less often and the bleeding is not as severe when compared to people with no fibrinogen at all. Those with a problem with the function of fibrinogen often don't have symptoms. Exams and Tests If your health care provider suspects this problem, you will have lab tests to confirm the type and severity of the disorder.Tests include:Bleeding time Bleeding timeBleeding time is a medical test that measures how fast small blood vessels in the skin stop bleeding. Because it is difficult to perform correctly, i...Read Article Now Book Mark Article Fibrinogen test and reptilase time to check fibrin level and quality Fibrinogen testFibrinogen is a protein produced by the liver. This protein helps stop bleeding by helping blood clots to form. A blood test can be done to tell ho...Read Article Now Book Mark Article Partial thromboplastin time (PTT) Partial thromboplastin timePartial thromboplastin time (PTT) is a blood test that looks at how long it takes for blood to clot. It can help tell if you have a bleeding problem...Read Article Now Book Mark Article Prothrombin time (PT) Prothrombin timeProthrombin time (PT) is a blood test that measures the time it takes for the liquid portion (plasma) of your blood to clot. It measures the functio...Read Article Now Book Mark Article Thrombin time Treatment The following treatments can be used for bleeding episodes or to prepare for surgery:Cryoprecipitate (a blood product containing concentrated fibrinogen and other clotting factors) Fibrinogen (RiaSTAP) Plasma (the liquid portion of the blood containing clotting factors) People with this condition should get the hepatitis B vaccine. Having many transfusions raises your risk of getting hepatitis.Hepatitis B vaccineAll content below is taken in its entirety from the CDC Hepatitis B Vaccine Information Statement (VIS): www. cdc. gov/vaccines/hcp/vis/vis-statement...Read Article Now Book Mark Article Outlook (Prognosis) Excessive bleeding is common with this condition. These episodes may be severe, or even fatal. Bleeding in the brain is a leading cause of death in people with this disorder. Possible Complications Complications may include:Blood clots with treatment Development of antibodies (inhibitors) to fibrinogen with treatment Gastrointestinal bleeding Gastrointestinal bleedingGastrointestinal (GI) bleeding refers to any bleeding that starts in the gastrointestinal tract. Bleeding may come from any site along the GI tract, ...Read Article Now Book Mark Article Miscarriage MiscarriageA miscarriage is the spontaneous loss of a fetus before the 20th week of pregnancy. Pregnancy losses after the 20th week are called stillbirths. Mi...Read Article Now Book Mark Article Rupture of the spleen Slow healing of wounds When to Contact a Medical Professional Contact your provider or seek emergency care if you have excessive bleeding.Tell your surgeon before you have surgery if you know or suspect you have a bleeding disorder. Prevention This is an inherited condition. There is no known prevention.Open ReferencesReferencesGailani D, Benjamin TF, Wheeler AP. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 135.Ragni MV. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 165.