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Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder

Fibrinogen is a precursor of fibrin, which acts as a procoagulant plasma soluble protein. It is involved in blood viscosity and clot stability to help in the recovery of damaged blood vessels. We describe the case of a preterm newborn who presented with abdominal distension and manifestations of ble...

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Autores principales: Acevedo Viales, Karol, Valverde Muñoz, Kathia, Gutiérrez Valverde, Daniela
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7845748/
https://www.ncbi.nlm.nih.gov/pubmed/33532157
http://dx.doi.org/10.7759/cureus.12397
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author Acevedo Viales, Karol
Valverde Muñoz, Kathia
Gutiérrez Valverde, Daniela
author_facet Acevedo Viales, Karol
Valverde Muñoz, Kathia
Gutiérrez Valverde, Daniela
author_sort Acevedo Viales, Karol
collection PubMed
description Fibrinogen is a precursor of fibrin, which acts as a procoagulant plasma soluble protein. It is involved in blood viscosity and clot stability to help in the recovery of damaged blood vessels. We describe the case of a preterm newborn who presented with abdominal distension and manifestations of bleeding in venipuncture sites. In the initial laboratories, prolonged coagulation times were observed with a decreased concentration of fibrinogen. This newborn had transfusion support, with recovery in factor levels and a subsequent decrease in them. Based on this trend and ruling out other possible causes of hypofibrinogenemia, such as liver disease, sepsis, or disseminated intravascular coagulation, the diagnosis of congenital hypofibrinogenemia was made. This case report includes the diagnostic and therapeutic approach of an unusual hemorrhagic presentation in the newborn, highlighting the need for transfusion and dynamic fibrinogen replacement to prevent complications and seek rapid improvement in symptoms.
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spelling pubmed-78457482021-02-01 Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder Acevedo Viales, Karol Valverde Muñoz, Kathia Gutiérrez Valverde, Daniela Cureus Pediatrics Fibrinogen is a precursor of fibrin, which acts as a procoagulant plasma soluble protein. It is involved in blood viscosity and clot stability to help in the recovery of damaged blood vessels. We describe the case of a preterm newborn who presented with abdominal distension and manifestations of bleeding in venipuncture sites. In the initial laboratories, prolonged coagulation times were observed with a decreased concentration of fibrinogen. This newborn had transfusion support, with recovery in factor levels and a subsequent decrease in them. Based on this trend and ruling out other possible causes of hypofibrinogenemia, such as liver disease, sepsis, or disseminated intravascular coagulation, the diagnosis of congenital hypofibrinogenemia was made. This case report includes the diagnostic and therapeutic approach of an unusual hemorrhagic presentation in the newborn, highlighting the need for transfusion and dynamic fibrinogen replacement to prevent complications and seek rapid improvement in symptoms. Cureus 2020-12-31 /pmc/articles/PMC7845748/ /pubmed/33532157 http://dx.doi.org/10.7759/cureus.12397 Text en Copyright © 2020, Acevedo Viales et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pediatrics
Acevedo Viales, Karol
Valverde Muñoz, Kathia
Gutiérrez Valverde, Daniela
Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder
title Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder
title_full Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder
title_fullStr Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder
title_full_unstemmed Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder
title_short Congenital Hypofibrinogenemia: Presentation of a Rare Coagulation Disorder
title_sort congenital hypofibrinogenemia: presentation of a rare coagulation disorder
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7845748/
https://www.ncbi.nlm.nih.gov/pubmed/33532157
http://dx.doi.org/10.7759/cureus.12397
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