A Case of Congenital Vitamin K Dependent Coaguation Factor Defeciency.
- Author:
Cheol Hong KIM
1
;
Seong Hoon KIM
;
Ju Eun LEE
;
Sang Wook NAM
;
Young Tak LIM
Author Information
1. Department of Pediatrics, College of Medicine, Pusan National University, Pusan, Korea.
- Publication Type:Case Report
- Keywords:
Congenital vitamin K dependent coagulation factors
- MeSH:
Administration, Intravenous;
Anticoagulants;
Blood Coagulation;
Blood Coagulation Factors;
Contusions;
Eating;
Hemorrhage;
Humans;
Infant;
Liver Diseases;
Male;
Palate, Hard;
Parents;
Partial Thromboplastin Time;
Parturition;
Plasma;
Prothrombin Time;
Reference Values;
Siblings;
Vitamin K 1;
Vitamin K*;
Vitamins*
- From:Korean Journal of Pediatric Hematology-Oncology
1998;5(1):177-181
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital combined deficiency of vitamin K dependent coagulation factors is a rare coagulation disorder. We experienced a 20-month old boy who was found to have a congenital vitamin K dependent coagulation factor defeciency. He presented with continuous bleeding on lacerated hard palate and had a history of numerous hemorrhagic episodes with multiple bruises after birth. Laboratory finding showed prolonged prothrombin time and partial thromboplastin time. Blood coagulation work-up showed marked decreased activities of the coagulation factors II, VII, IX, X and the natural anticoagulants proteins C and S. Assay of coagulation factors in the parents and sibling were with the normal range. There's no evidence of malabsorption, liver disease or ingestion of a coumarin compound. Response to intravenous administration of vitamin K1 was not significant but transfusion of fresh frozen plasma corrected prothrombin time and partial thromboplastin time. We reported a case of congenital combined deficiency of vitamin K dependent coagulation factors.