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Deficiência congénita de FXIII no sul da Tunísia

Deficiência congénita de FXIII no sul da Tunísia


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About the Book

A deficiência de factor XIII congénito é uma coagulopatia rara cuja prevalência no mundo é de cerca de um caso por 2 milhões de habitantes. Este estudo permitiu-nos enumerar os casos de deficiência de factor XIII no sul da Tunísia e avaliar os aspectos epidemiológicos, diagnósticos e terapêuticos. O nosso estudo é retrospectivo, cobrindo um período de 20 anos e incluindo 30 doentes. Foi realizado um teste padrão de hemostasia e um ensaio de factor XIII em todos os nossos pacientes. A proporção de sexo foi de 0,67. A maioria dos pacientes era de Sidi Bouzid. A idade média de início dos sintomas foi de 1,4 anos. A idade média no diagnóstico foi de 8,8 anos. 26 pacientes foram diagnosticados após uma síndrome hemorrágica e 4 após uma investigação familiar. Todos tinham níveis de factor XIII zero, medidos pela técnica de solubilidade do coágulo. Todos os doentes receberam terapia de substituição com plasma fresco congelado. A maioria dos casos eram de casamentos consanguíneos.Por conseguinte, as investigações familiares são de interesse para se obter um melhor perfil epidemiológico e molecular.


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Product Details
  • ISBN-13: 9786205914649
  • Publisher: KS Omniscriptum Publishing
  • Publisher Imprint: Edicoes Nosso Conhecimento
  • Height: 229 mm
  • No of Pages: 88
  • Spine Width: 5 mm
  • Width: 152 mm
  • ISBN-10: 6205914646
  • Publisher Date: 20 Apr 2023
  • Binding: Paperback
  • Language: Portuguese
  • Returnable: N
  • Weight: 190 gr


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