scholarly journals Factor V11 deficiency: A rare cause of nasal bleeding

2021 ◽  
Vol 2 (3) ◽  
Author(s):  
Georgina G Balyorugulu ◽  
◽  
Richard F Kiritta ◽  
Emmanuela Ambrose ◽  
Erius Tebuka ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Prothrombin Time ◽  
Fresh Frozen Plasma ◽  
Activated Partial Thromboplastin Time ◽  
Factor Vii ◽  
Factor Vii Deficiency ◽  
Fresh Frozen ◽  
Nasal Bleeding ◽  
Frozen Plasma ◽  
Functional Factor

Factor VII deficiency is a rare inherited disorder. Clinically the patient presents with bleeding tendencies. Diagnosis is made by prolonged prothrombin time, normal activated partial thromboplastin time and low functions factor VII assay or factor VII antigen. Therapy involves factor VII concentrates, recombinant factor VII, fresh frozen plasma and fibrinolytic inhibitors. We present a 6 years old boy with nose bleed for six months of whom prothrombin time was prolonged with functional factor VII assay of less than 1% confirming factor VII deficiency. He was managed with fresh frozen plasma, blood transfusion, tranexamic acid. Factor VII deficiency even though rare should be sought out in children presenting with bleeding. Keywords: Factor VII deficiency; coagulation; hemorrhage.

scholarly journals Factor VII Deficiency and Pregnancy: A Case Report and Review of Literature

2021 ◽  
Author(s):  
Minakshi Rohilla ◽  
Rakhi Rai ◽  
Jasmina Ahluwalia ◽  
Pankaj Malhotra ◽  
Vanita Jain
Keyword(s):  
Autosomal Recessive ◽  
English Literature ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Coagulation Disorder ◽  
Review Of Literature ◽  
Factor Vii Deficiency ◽  
First Case ◽  
Fresh Frozen ◽  
Frozen Plasma

Abstract Inherited factor VII deficiency is an autosomal recessive coagulation disorder with broad range of bleeding manifestations. The association between bleeding and absolute factor VII level is poor. Usually, the bleeding is associated with FVII levels of less than 1% of the normal value. Factor VII deficiency is associated with prolongation of prothrombin time only with normal activated partial thromboplastin time. Approximately 66 pregnant women have been reported with factor VII deficiency so far in English literature. We hereby, report 2 cases along with the review of literature of Factor VII deficiency during pregnancy. Our patients were diagnosed to have factor VII deficiency after deranged coagulogram with factor VII level of < 1% and 17.1% respectively, however could be managed by fresh frozen plasma only in first case and fresh frozen plasma & factor VII concentrate in second case successfully. Coagulogram is a simple, easily available, affordable and lifesaving investigation to detect this deficiency in pregnancy.

Homozygous 2bp Deletion in the Human Factor VII Gene: A Non-Lethal Mutation that Is Associated with a Complete Absence of Circulating Factor VII

2000 ◽  
Vol 84 (10) ◽  
pp. 635-637 ◽  
Author(s):  
Pier Mannucci ◽  
P. Jenkins ◽  
Anselm Lee ◽  
Raffaella Coppola ◽  
David Perry ◽  
...  
Keyword(s):  
Human Factor ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Causative Mutation ◽  
Direct Sequence ◽  
Factor Vii Deficiency ◽  
Plasma Factor ◽  
Fresh Frozen ◽  
Direct Sequence Analysis ◽  
Frozen Plasma

SummaryWe report the case of a 5-year-old boy with severe factor VII deficiency. The affected child presented at the age of 8 months and again at 18 months with bleeding from the gastrointestinal tract but the diagnosis of factor VII deficiency was not made until the age of 3 years. He was treated with fresh frozen plasma and subsequently factor VII concentrates and to date remains well. To identify the causative mutation, the factor VII gene was screened by SSCP and direct sequence analysis. A single homozygous 2bp deletion (-CT) mutation was identified in exon 1a removing nucleotides 27/28 (codons 52/53). Both parents, who were first cousins, were heterozygous for the mutation. The mutation located in the prepropeptide of factor VII, results in a complete absence of factor VII in plasma. This case indicates that a complete absence of plasma factor VII is not necessarily a lethal condition.

scholarly journals Manajemen Perioperatif pada Perdarahan akibat Atonia Uteri

2020 ◽  
Vol 3 (1) ◽  
pp. 47-58
Author(s):  
Budi Yulianto Sarim
Keyword(s):  
Acute Lung Injury ◽  
Blood Transfusion ◽  
Lung Injury ◽  
Post Partum ◽  
Fresh Frozen Plasma ◽  
Uterine Atony ◽  
Factor Vii ◽  
First Choice ◽  
Fresh Frozen ◽  
Frozen Plasma

Perdarahan obstetri merupakan penyebab utama kematian maternal dan perinatal. Atonia uteri merupakan penyebab tersering perdarahan postpartum. Perdarahan post partum adalah perdarahan lebih dari 500 cc setelah bayi lahir pervaginam atau lebih dari 1.000 ml setelah persalinan abdominal atau jumlah perdarahan lebih dari normal dan telah menyebabkan perubahan tanda vital. Penyebab atonia uteri adalah overdistensi uterus, kelelahan otot miometrium, plasenta letak rendah, toksin bakteri (korioamnionitis, endomiometritis, septikemia), hipoksia akibat hipoperfusi atau uterus couvelaire pada solusio plasenta dan hipotermia akibat resusitasi masif. Manajemen atonia uteri dapat berupa non farmakologi, farmakologi dan pembedahan menurut algoritma Varatharajan yaitu “HAEMOSTASIS”.Manejemen perioperatif atoni uteri terdiri dari terapi O2, monitoring noninvasif, pemasangan jalur intra vena dengan menggunakan kateter intravena yang besar dan resusitasi cairan. Tehnik anestesi tergantung keadaan klinis dan rencana tindakan berikutnya oleh dokter kandungan. Pilihan pertama transfusi darah adalah transfusi sel darah merah, platelet, fresh frozen plasma, kriopresipitat, faktor VII dan fibrinogen sintetis (RiaSTAP), Transfusi masif adalah pemberian transfusi darah sebanyak volume darah pasien dalam waktu 24 jam atau lebih dari 7 % berat badan ideal dewasa. Komplikasi yang dapat terjadi pada transfusi masif adalah hipotermi, hipokalsemia, hipomagnesemia, hiperkalemia, asidosis/ alkalosis, koagulopati dilusional, transfusion related acute lung injury (TRALI) Perioperative Management in Bleeding cause by Uterine Atony Abstract Obstetric bleeding is a major cause of maternal and perinatal death. Uterine atony is the most common cause of postpartum hemorrhage. Post partum hemorrhage is bleeding more than 500 cc after the baby is vaginal labor or more than 1,000 ml after abdominal labor or the amount of bleeding is more than normal and has caused changes in vital signs. The causes of uterine atony are uterine overdistence, myometrial muscle fatigue, low lying placenta, bacterial toxin (chorioamnionitis, endomyometritis, septicemia), hypoxia due to hypoperfusion or uterine couvelaire in placental abruption and hypothermia due to massive resuscitation. Management of uterine atony can be in the form of non pharmacology, pharmacology and surgery according to the Varatharajan algorithm is "HAEMOSTASIS". Anesthesia management consists of O2 therapy, noninvasive monitoring, installation of intravenous lines using a large intravenous catheter and fluid resuscitation. Anesthesia techniques depend on clinical conditions and subsequent action plans by the obstetrician. The first choice of blood transfusion is transfusion of red blood cells, platelets, fresh frozen plasma, cryoprecipitate, factor VII and synthetic fibrinogen (RiaSTAP), massive transfusion is the administration of blood transfusion as much as the patient's blood volume within 24 hours or more than 7% of the ideal adult body weight . Complications that can occur in massive transfusions are hypothermia, hypocalcemia, hypomagnesemia, hyperkalemia, acidosis / alkalosis, dilutional coagulopathy, transfusion related acute lung injury (TRALI).

scholarly journals MENORRHAGIA;

2013 ◽  
Vol 20 (05) ◽  
pp. 846-848
Author(s):  
FARZANA ZIA ◽  
SHAHZEEM BHAYANI ◽  
RUBINA HUSSAIN
Keyword(s):  
Autosomal Recessive ◽  
Past History ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Severe Anemia ◽  
Factor Vii Deficiency ◽  
Fresh Frozen ◽  
Life Threatening ◽  
Frozen Plasma

Factor VII deficiency is a rare, autosomal recessive coagulopathy that becomes symptomatic in the form of a hemorrhagicsyndrome characterized by severe life threatening bleeding. This may present in young women as severe anemia due to bleeding pervaginum. We report one such case of a 12 year old girl who presented at the gynecology outpatient department with complaints of severemenorrhagia at menarche. Her past history was consistent with episodes of profuse epistaxis and bleeding from gums. Her completeblood count showed severe anemia. Upon further investigation her prothrombin time was prolonged but her APTT was normal which wasindicative of Factor VII deficiency and was confirmed by serum assays of Factor VII. It is important to diagnose this disorder earlier in orderto avoid long term complications especially in women who may suffer from severe life threatening hemorrhage during menses orrecurrent miscarriages during pregnancy. Therefore, our patient was transfused with packed cells and fresh frozen plasma immediatelyand started on low dose oestrogen and progesterone pill along with tranexemic acid to control her symptoms.

scholarly journals DIFFERENCES IN CHANGES OF HEMOGLOBIN BETWEEN 6-12 HOURS AND 12-14 HOURS AFTER TRANSFUSION

2018 ◽  
Vol 24 (2) ◽  
pp. 108
Author(s):  
Rosita Linda ◽  
Devita Ninda
Keyword(s):  
Blood Transfusion ◽  
Secondary Data ◽  
Hemoglobin Concentration ◽  
Fresh Frozen Plasma ◽  
Blood Components ◽  
Blood Products ◽  
Fresh Frozen ◽  
Frozen Plasma ◽  
Hemoglobin Measurement ◽  
Baseline Hemoglobin

Each year more than 41,000 blood donations are needed every day and 30 million blood components are transfused. Blood products that can be transfused include Packed Red Cells (PRC), Whole Blood (WB), Thrombocyte Concentrate (TC), Fresh Frozen Plasma (FFP). Monitoring Hemoglobin (Hb) after transfusion is essential for assessing the success of a transfusion. The time factor after transfusion for Hemoglobin (Hb) examination needs to be established, analyze to judge the success of a blood transfusion which is performed. The aim of this study was to analyze the differences in changes of hemoglobin between 6-12 hours, and 12-24 hours after-transfusion. This study was retrospective observational using secondary data. The subjects were patients who received PRC, and WBC transfusion. At 6-12, and 12-24 hours after-transfusion, hemoglobin, RBC, and hematocrit were measured. Then the data were analyzed by unpaired t-test. The collected data included the results of the Hb pre-transfusion, 6-12, and 12-24 hours after-transfusion. The subjects of this study were 98 people. The administration of transfusion increased by 10-30% in hemoglobin concentration at 6-12 hours after-transfusion. While at 12-24 hours after-transfusion, hemoglobin after-transfusion increased 15-37% from the baseline. Hemoglobin values were not different at any of the defined after-transfusion times (p = 0.76 (p>0.05)). Hemoglobin values were not different at 6-12 hours, and 12-24 hours after-transfusion.    Keywords: Hemoglobin, measurement, after-transfusion 

Comparison of the coagulation potential of lyophilized blood plasma virus-inactivated by two different methods.

2021 ◽  
Author(s):  
И.А. Кривов ◽  
А.А. Рагимов ◽  
Э.Л. Салимов
Keyword(s):  
Comparative Evaluation ◽  
Room Temperature ◽  
Coagulation Factors ◽  
Fresh Frozen Plasma ◽  
Activated Partial Thromboplastin Time ◽  
Plasma Virus ◽  
Coagulation Parameters ◽  
Normal Limits ◽  
Fresh Frozen ◽  
Frozen Plasma

Введение. Свежезамороженная плазма (СЗП) — один из самых распространённых компонентов крови, применяемых сегодня в клиниках при оказании медицинской помощи при кровотечениях и тяжёлых коагулопатиях. В отличие от вирусинактивированной замороженной плазмы, сублимированная (лиофилизированная) плазма может храниться при комнатной температуре, и восстановление перед переливанием обычно требует меньших временных затрат. Цель исследования: оценить коагуляционный потенциал лиофилизированной плазмы, полученной из вирусинактивированной плазмы, инактивированной 2 способами: с использованием метиленового синего + видимый свет и рибофлавина + ультрафиолетовое облучение спектра B. Материалы и методы. Проведен анализ 100 образцов иофилизированной плазмы, вирусинактивированной двумя методами. Изучали влияние лиофилизации на уровень факторов свертывания и показатели свертываемости в вирусинактивированной плазме. Для сравнительной оценки в качестве контроля были проанализированы 150 образцов СЗП. Результаты. При использовании обоих технологий инактивации в лиофилизированной вирусинактивированной плазме установлено снижение содержания факторов V и VIII как по отношению к СПЗ, так и по отношению к физиологической норме. Лиофилизация вирусинактивированной плазмы различными методами привела к некоторому увеличению показателей свёртывания крови — протромбинового времени и активированного частичного тромбопластинового времени. Остальные показатели оставались в нормальных пределах. Существенных различий в показателях между образцами плазмы, инактивированной различными методами, выявлено не было. Заключение. По клиническим свойствам вирусинактивированная лиофилизированная плазма может служить альтернативой СЗП, однако для уточнения всесторонних аспектов её применения необходимы дополнительные исследования. Introduction. Fresh frozen plasma (FFP) is one of the most common blood components used today in clinics for medical care of bleeding and severe coagulopathies. Unlike virus-inactivated frozen plasma, sublimated (lyophilized) plasma can be stored at room temperature, and recovery before transfusion usually requires less time. Objectives: to assess the coagulation potential of lyophilized plasma obtained from virus- inactivated plasma inactivated by 2 methods: using methylene blue + visible light and riboflavin + ultraviolet radiation of spectrum B. Materials/Methods. Analysis of 100 samples of lyophilized plasma, virus-inactivated by 2 methods, was carried out. The effect of lyophilization on the level of coagulation factors and coagulation parameters in virus-inactivated plasma was studied. For comparative evaluation, 150 samples of FFP were analyzed as a control. Results. Using both technologies for inactivation of lyophilized virus- inactivated plasma, a decrease in the content of V and VIII factors was found both in relation to the FFP and in relation to the physiological norm. Lyophilization of virus-inactivated plasma by various methods led to a slight increasing in blood coagulation parameters — prothrombin time and activated partial thromboplastin time. The rest of the parameters remained within normal limits. There were no significant differences in parameters between plasma samples inactivated by different methods. Conclusions. According clinical properties, virus- inactivated lyophilized plasma can serve as an alternative to FFP, but more studies are needed to clarify the comprehensive aspects of its use.

The bleeding patient

2020 ◽  
pp. 403-428
Author(s):  
Anne Craig ◽  
Anthea Hatfield
Keyword(s):  
Blood Transfusion ◽  
Blood Loss ◽  
Disseminated Intravascular Coagulation ◽  
Oxygen Supply ◽  
Fresh Frozen Plasma ◽  
Blood Transfusions ◽  
Massive Blood Transfusion ◽  
Intravascular Coagulation ◽  
Fresh Frozen ◽  
Frozen Plasma

Part one of this chapter tells you about the physiology of blood and oxygen supply, about anaemia and tissue hypoxia, and the physiology of coagulation. Drugs that interfere with clotting are discussed. Bleeding, coagulation, and platelet disorders are covered as well as disseminated intravascular coagulation. Part two is concerned with bleeding in the recovery room: how to cope with rapid blood loss, managing ongoing blood loss, and how to use clotting profiles to guide treatment. There is also a section covering blood transfusion, blood groups and typing. Massive blood transfusion is clearly described, there are guidelines about when to use fresh frozen plasma, when to use platelets, and when to use cryoprecipitate. The final section of the chapter is about problems with blood transfusions.

Preparation of a Factor VII Concentrate

1979 ◽  
Author(s):  
A.J. MacLeod ◽  
I. Dickson
Keyword(s):  
Specific Activity ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Batch Adsorption ◽  
Citrate Buffer ◽  
Linear Gradient ◽  
Fresh Frozen ◽  
Frozen Plasma ◽  
Phosphate Citrate Buffer

A factor VII concentrate has been prepared from pooled citrated fresh frozen plasma following removal of cryoprecipitate and factors II, IX and X. The method involved batch adsorption on DEAE-Sephadex A-50, fractionation of the subsequent batch eluate by PEG precipitation and passage through a column of DEAE-Sepharose CL-.6B. A phosphate-citrate buffer pH 6.9 was used throughout, this was made 0.2M with NaCl for the batch elution and a 0 - 0.2H NaCl linear gradient was used to elute the components from the column. Factor VII activity was clearly resolved from the bulk of the protein, including caeruloplasmin, and could be recovered as a concentrate at about 20 U FVII/ml with a specific activity of in excess of 1 U FVII/mg of protein and an overall recovery of 40% to 50%

Transfusion of blood products

2016 ◽  
Author(s):  
Alison Smith
Keyword(s):  
Blood Transfusion ◽  
Carrying Capacity ◽  
Blood Cells ◽  
Fresh Frozen Plasma ◽  
Major Haemorrhage ◽  
Blood Products ◽  
Unlimited Supply ◽  
Fresh Frozen ◽  
Oxygen Carrying Capacity ◽  
Frozen Plasma

The transfusion of blood products may be required in the pre- and post-operative periods. However, there are inherent risks associated with blood transfusion, and there is not an unlimited supply of blood donations available. When a patient is anaemic, red blood cells should be transfused to maintain the oxygen-carrying capacity of blood. Blood products, such as platelets and fresh frozen plasma, are transfused to correct a coagulopathy and during major haemorrhage. This chapter reviews the physiology of blood, including ABO compatibility and rhesus status, the main blood products available for transfusion, and transfusion policy, including the treatment of major haemorrhage and the refusal of blood products.

The bleeding patient

2014 ◽  
Author(s):  
Anthea Hatfield
Keyword(s):  
Blood Transfusion ◽  
Blood Loss ◽  
Disseminated Intravascular Coagulation ◽  
Oxygen Supply ◽  
Final Section ◽  
Fresh Frozen Plasma ◽  
Blood Transfusions ◽  
Massive Blood Transfusion ◽  
Fresh Frozen ◽  
Frozen Plasma

Part one of this chapter tells you about the physiology of blood and oxygen supply, about anaemia and tissue hypoxia, and the physiology of coagulation. Drugs that interfere with clotting are discussed. Bleeding, coagulation, and platelet disorders are covered as well as disseminated intravascular coagulation. Part two is concerned with bleeding in the recovery room: how to cope with rapid blood loss, managing ongoing blood loss, and how to use clotting profiles to guide treatment. There is also a section covering blood transfusion, blood groups and typing. Massive blood transfusion is clearly described, there are guidelines about when to use fresh frozen plasma, when to use platelets, and when to use cryoprecipitate. The final section of the chapter is about problems with blood transfusions.

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