Factor XIII Deficiency

For infants that present with intracranial hemorrhage in the setting of suspected abusive head trauma (AHT), the standard recommendation is to perform an evaluation for a bleeding disorder. Factor XIII (FXIII) deficiency is a rare congenital bleeding disorder associated with intracranial hemorrhages in infancy, though testing for FXIII is not commonly included in the initial hemostatic evaluation. The current pediatric literature recognizes that trauma, especially traumatic brain injury, may induce coagulopathy in children, though FXIII is often overlooked as having a role in pediatric trauma-induced coagulopathy. We report an infant that presented with suspected AHT in whom laboratory workup revealed a decreased FXIII level, which was later determined to be caused by consumption in the setting of trauma induced coagulopathy, rather than a congenital disorder. Within the Child Abuse Pediatrics Research Network (CAPNET) database, 85 out of 569 (15 %) children had FXIII testing, 3 of those tested (3.5 %) had absent FXIII activity on qualitative testing, and 2 (2.4 %) children had activity levels below 30 % on quantitative testing. In this article we review the literature on the pathophysiology and treatment of low FXIII in the setting of trauma. This case and literature review demonstrate that FXIII consumption should be considered in the setting of pediatric AHT.

Abnormal bleeding due to low fibrinogen (Fib) and coagulation factor XIII (FXIII) levels after lumbar vertebral surgery is exceedingly rare. Excessive bleeding is also associated with secondary hyperfibrinolysis. This report presents a case of abnormal incision bleeding caused by coagulation factor XIII deficiency (FXIIID) and secondary hyperfibrinolysis in a state of low fibrinogen after lumbar vertebral surgery.

A middle-aged woman experienced prolonged incision and excessive bleeding after lumbar vertebral surgery.

Combined with coagulation factors, coagulation function tests, and thromboelastography, the patient clinical presentation supported the diagnosis of FXIIID and secondary hyperfibrinolysis in a hypofibrinogenemic state.

Cryoprecipitat, Fresh Frozen Plasma, Fibrinogen Concentrate, Leukocyte-depleted Red Blood Cells, Hemostatic (Carbazochrome Sodium Sulfonate; Hemocoagulase Bothrops Atrox for Injection; Tranexamic Acid).

After approximately a month of replacement therapy and symptom treatment, the patient coagulation function significantly improved, and the incision healed without any hemorrhage during follow-up.

Abnormal postoperative bleeding may indicate coagulation and fibrinolysis disorders that require a full set of coagulation tests, particularly coagulation factors. Given the current lack of a comprehensive approach to detect coagulation and fibrinolysis functions, a more comprehensive understanding of hematology is imperative. The current treatment for FXIIID involves replacement therapy, which requires supplementation with both Fib and FXIII to achieve effective hemostasis.

The aim of this study was to identify clinical factors associated with progressive facial swelling after orthognathic surgery. Patients diagnosed with jaw deformities and undergoing orthognathic surgery were retrospectively evaluated, and those with surgical site infection, Le Fort I osteotomy, or genioplasty only were excluded. Facial swelling volume was calculated by comparing facial volume preoperatively and three days postoperatively using 3D images and image analysis software (VECTRA H2). FXIII was measured within three days after surgery in only patients with unexplained postoperative bleeding or hematoma. The correlation between facial swelling volume and clinical factors was statistically analyzed. Facial swelling volume was examined in 78 patients. Univariate analysis showed a significant difference between facial swelling volume (mean = 41.6 cm³) and operation time (mean = 209.3 min, r = 0.283, p = 0.012), ΔHb level (mean = 1.18 g/dL, r = 0.235, p = 0.039), as well as decreased factor XIII activity (mean = 75.3%, p = 0.012). Multivariate analysis showed a significant difference between facial swelling volume and FXIII deficiency (standard error = 6.44, p = 0.031).Progressive facial swelling immediately after orthognathic surgery may be due to factor XIII deficiency.