Acquired-transient factor X deficiency associated with anticardiolipin antibodies in a child with extensive burns.
The article presents the case of an 18-month-old boy with major scald burns complicated by acquired F-X deficiency. On the 15th day of hospitalization, the patient developed sepsis and fever. He also exhibited bruxism, especially during the febrile episodes, which his permanent teeth to luxate and become mobile. Pedodontists decided that all the child's teeth should be extracted to ensure proper development of the jaw with growth. Twelve hours later, he developed a leukemoid reaction, which was attributed to infection with another aerobic organism or development of anaerobic bacteremia after teeth extraction. Twenty-four hours after the extractions, the burn wounds began oozing and there was extensive gingival bleeding and epistaxis. Coagulation parameters were assessed immediately. Disseminated intravascular coagulation was detected initially and was successfully treated with fresh-frozen plasma transfusions, but bleeding from the burn wounds and nasal/oral mucous membranes continued. Further testing revealed the diagnosis of acquired isolated F-X deficiency linked with antiphospholipid antibodies. Treatment with plasmapheresis, steroids, and intravenous immunoglobulin was successful. Hypertrophic scar formation was the only issue during 7 months of follow-up.
Department of General Surgery, Medical Faculty, Başkent University, Ankara, Turkey.
Mehmet
M
Haberal
Ozgür
O
Basaran
Ayşe E
AE
Sakallioğlu
Emine
E
Kesik
Bülent
B
Alioğlu
Namik
N
Ozbek
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D017153:Antibodies, Anticardiolipin / Q000097:blood*; D001803:Blood Transfusion; D002012:Bruxism / Q000150:complications / Q000601:surgery; D002056:Burns / Q000150:complications* / Q000628:therapy; D004211:Disseminated Intravascular Coagulation / Q000209:etiology / Q000628:therapy; D005171:Factor X Deficiency / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D005334:Fever / Q000209:etiology / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D008297:Male; D010956:Plasmapheresis; D018805:Sepsis / Q000209:etiology / Q000628:therapy; D014081:Tooth Extraction / Q000009:adverse effects
D000305:Adrenal Cortex Hormones; D017153:Antibodies, Anticardiolipin; D016756:Immunoglobulins, Intravenous