Coagulation profiles and factor XIII activity in pediatric patients with severe motor and intellectual disability: a multicenter exploratory study.

Severe motor and intellectual disabilities (SMID) are a heterogeneous group of disorders characterized by profound motor and cognitive impairment. Although routine coagulation tests are often normal, immobility, use of antiseizure medication (ASM), and poor nutrition may affect hemostasis. However, global coagulation potential in pediatric SMID patients remains unclear. This study evaluated coagulation function in 28 children with Oshima class 1 SMID. Routine coagulation tests and clot waveform analysis (CWA), including clotting time (CT), maximum coagulation velocity (|min1|), and maximum coagulation acceleration (|min2|) were performed. Factor XIII activity (FXIII:C) was measured. Associations with ASM number, valproic acid (VPA) use, BMI z-score, and total number of administered medications were examined. Multivariable regression was adjusted for age, BMI z-score, and home mechanical ventilation. Prothrombin and activated partial thromboplastin times were largely normal. CWA showed prolonged CT in 7 of 28 patients and decreased |min1| and |min2| values in 4 and 7 patients, respectively. CT was not associated with the number of ASMs used, VPA use, BMI z-score, or total medications after adjustment. In contrast, lower FXIII:C remained associated with the total number of administered medications after adjustment. Further studies are required to clarify this association and its clinical significance.