This paper aims to study antepartum and postpartum bleeding manifestations of patients with factor VII (FVII) deficiency, their management, and feto-maternal outcomes, to establish danger signs and management protocols. We describe a case series of nine pregnancies in four patients with FVII deficiency diagnosed at a tertiary care referral center in India between 2012 and 2023. Out of nine pregnancies, six had cesarean deliveries, two had vaginal deliveries, and one had dilatation and curettage for unwanted pregnancies. One out of nine pregnancies (11.11%) with an unknown FVII deficiency had antepartum hemorrhage (abruption) necessitating multiple transfusions, ICU stay, and neonatal loss. Three patients with no prior history of obstetric hemorrhage were diagnosed with severe deficiencies and received prophylactic recombinant FVII preoperatively, averting the potential loss of lives. In patients with no history of bleeding, no hemorrhage was reported with or without prophylaxis while 33.33% of hemorrhage was reported in patients with a history of bleeding. Factors like the history of bleeding, FVII levels, mode of delivery, and other risk factors for hemorrhage should all be considered to predict the risk of bleeding in delivery. Cesarean is a surgical procedure, and prophylactic use of recombinant FVII concentrate (rFVIIa) should be considered.
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http://dx.doi.org/10.7759/cureus.64079 | DOI Listing |
Cureus
December 2024
Department of Surgery, King Abdulaziz Specialist Hospital, Taif, SAU.
Congenital factor VII (FVII) deficiency is a rare coagulation disorder that increases the risk of bleeding complications during surgery. Although laparoscopic sleeve gastrectomy (LSG) is the most common metabolic bariatric surgery (MBS), it is rarely performed in patients with congenital coagulation disorders such as FVII deficiency, due to the high risk of intraoperative and postoperative bleeding. We report the case of a 57-year-old female with class II obesity (BMI 37.
View Article and Find Full Text PDFBlood Coagul Fibrinolysis
December 2024
Department of Hematology, The Second Affiliated Hospital, Chongqing Medical University, Jiangnan, Chongqing, China.
Background: Congenital factor VII (FVII) deficiency is a genetic disorder characterized by decreased FVII activity, which sometimes leads to fatal bleeding. Numerous variants have been found in FVII deficiency, but mutations vary among patients. Each mutation deserves further exploration for each patient at risk of bleeding.
View Article and Find Full Text PDFLife (Basel)
November 2024
Onco-Hematology, Cell and Gene Therapy and Bone Marrow Transplant Clinic Area, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
Inherited factor VII deficiency is the most common rare bleeding disorder, affecting about 1/500,000 individuals without gender predilection. Most of the patients with FVII 20-50% are asymptomatic, but post-traumatic or post-surgical bleeding may often occur since there is not an exact correlation between FVII plasma levels and the bleeding phenotype. We enrolled 19 children and adolescents with FVII levels of 20-35% and 33 controls.
View Article and Find Full Text PDFItal J Pediatr
January 2025
Pediatric Hematology and Oncology, SBU Sisli Hamidiye Etfal Training and Research Hospital, Istanbul, Turkey.
Background: Rare bleeding disorders (RBDs) include fibrinogen (Factor I), prothrombin (Factor II), Factor V(FV), combined Factor V and Factor VIII, Factor VII, Factor X, Factor XI, Factor XII, and Factor XIII deficiencies. This group accounts for 3-5% of all factor deficiencies. Different symptoms may occur, ranging from mild or moderate bleeding to serious and life-threatening bleeding, which may not be related to the factor level.
View Article and Find Full Text PDFJ Pediatr Hematol Oncol
December 2024
Pediatrics Clinic, Bakirkoy Dr. Sadi Konuk Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.
Background: Rare factor deficiency (RFD) is characterized by a deficiency of factor (F)I, FII, FV, FVII, FX, FXI, FXII, FXIII, or a combined deficiency of FV+FVIII or vitamin K-dependent factors. The prevalence of RFD ranges from 1/1,000,000 to 3,000,000. Combined deficiencies of vitamin K-related factors have been described in 30 families worldwide, and these patients can present with a wide range of clinical symptoms, from mucocutaneous bleeding to life-threatening symptoms such as central nervous system and gastrointestinal bleeding.
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