[Perioperative management for fracture in a child with homozygous congenital protein C deficiency].
Rinsho Ketsueki
; 65(3): 164-168, 2024.
Article
em Ja
| MEDLINE
| ID: mdl-38569860
ABSTRACT
Congenital protein C (PC) deficiency is one type of hereditary thrombosis. Patients with hereditary thrombosis are at high risk for thrombosis in the perioperative period, but a standard management strategy has not been established. Here we report a case of perioperative management of a fracture in a child with homozygous congenital PC deficiency. The patient was a 3-year-old boy who was diagnosed with congenital PC deficiency at birth. He sustained a traumatic supracondylar fracture of the right humerus and underwent emergency surgery. To prepare for open surgery for fixation of the fracture, warfarin was discontinued, and an activated PC (APC) concentrate was used in combination with vitamin K antagonism. However, warfarin was administered during the scheduled nail extraction because the operation was minimally invasive. No thrombotic or bleeding complications occurred in either operation. In emergency surgery in patients with congenital PC deficiency, the combination of vitamin K and APC concentrate is considered a maintenance option for PC deficiency. Postoperative PT-INR control was difficult in our patient due to the administration of vitamin K and withdrawal of warfarin, and this issue must be addressed in the future. Further case experience is desirable to standardize perioperative management.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Trombose
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Deficiência de Proteína C
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Fraturas Ósseas
Limite:
Child, preschool
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Humans
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Male
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Newborn
Idioma:
Ja
Ano de publicação:
2024
Tipo de documento:
Article