title: First living-related liver transplant to cure factor VII deficiency.
abstract: Congenital factor VII deficiency is an autosomal recessive serious disorder of blood coagulation with wide genotypic and phenotypic variations. The clinical presentation can vary from asymptomatic patients to patients with major bleedings in severe deficiency (factor VII <1%). Investigations show prolonged PT and low factor VII. Treatment modalities include FFP and repeated recombinant factor VII infusions. We hereby report the first successful LRLT for factor VII deficiency in an infant, the first-ever youngest baby reported worldwide. A six-month-old male child presented with easy bruisability, ecchymotic patches, hematuria, and convulsions. CT of the head showed subdural hemorrhage, which was treated conservatively. He had markedly increased PT (120 s) with normal platelets, and aPTT with factor VII level <1%. Despite the treatment by rFVIIa administration weekly, which was very expensive, he still had repeated life-threatening bleeding episodes. LRLT was performed with mother as the donor, whose factor VII level was 57%. A factor VII infusion plan for pre-, intra- and postoperative periods was formulated and TEG followed. Postoperatively, his factor VII started increasing from third day and was 38% on 24th day with PT <14 s. He had uneventful intraoperative and postoperative courses. LT is a safe and definite cure for factor VII deficiency.
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Liver Transplantation, Medanta the Medicity, Gurgaon, Haryana, India.
authors.forename: Neelam
authors.initials: N
authors.lastname: Mohan
authors.affiliation: Department of Pediatric Gastroenterology, Hepatology and Liver Transplantation, Medanta the Medicity, Gurgaon, Haryana, India.
authors.forename: Sakshi
authors.initials: S
authors.lastname: Karkra
authors.affiliation: Institute of Liver Transplant and Regenerative Medicine, Medanta the Medicity, Gurgaon, Haryana, India.
authors.forename: Anu S
authors.initials: AS
authors.lastname: Jolly
authors.affiliation: Institute of Liver Transplant and Regenerative Medicine, Medanta the Medicity, Gurgaon, Haryana, India.
authors.forename: Vijay
authors.initials: V
authors.lastname: Vohra
authors.affiliation: Institute of Liver Transplant and Regenerative Medicine, Medanta the Medicity, Gurgaon, Haryana, India.
authors.forename: Ravi
authors.initials: R
authors.lastname: Mohanka
authors.affiliation: Institute of Liver Transplant and Regenerative Medicine, Medanta the Medicity, Gurgaon, Haryana, India.
authors.forename: Amit
authors.initials: A
authors.lastname: Rastogi
authors.affiliation: Institute of Liver Transplant and Regenerative Medicine, Medanta the Medicity, Gurgaon, Haryana, India.
authors.forename: A S
authors.initials: AS
authors.lastname: Soin
mesh_terms: D005168:Factor VII Deficiency / Q000601:surgery*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male
keywords: factor VII; liver transplantation; pediatric living donor; rfactorVII; thromboelastogram