Michaela Wilhelmi

Background: Factor XIII (FXIII) deficiency increases bleeding risk and delays wound healing, yet its recognition, diagnosis, and treatment remain challenging in the complex clinical routine. Objective: To determine real-world diagnosis and management strategies of acquired FXIII deficiency. Methods: Adult patients undergoing FXIII activity testing (diagnostic phase) and FXIII supplementation (treatment phase) for acquired FXIII deficiency were consecutively enrolled in this retrospective data collection. Treatment outcomes were analyzed in patients with postsurgical bleeding and wound healing disorders (WHD). Results: A total of 255 patients were included in the diagnostic cohort, of whom 84% were diagnosed with clinically relevant FXIII deficiency and 77% received FXIII supplementation. Median FXIII activity in patients with a clinically relevant deficiency was 42.5%, compared to 74.0% in those without. Most patients with FXIII activity levels below 60% were considered to have a clinically relevant deficiency. The treatment cohort comprised 96 postsurgical bleeding cases and 78 WHD cases. Median FXIII activity levels were similarly reduced in both cohorts (44.0% vs 48.0%). Conventional coagulation tests remained mainly within reference ranges for most patients, highlighting the diagnostic gap. Persistent infection was the leading comorbidity, affecting 33% of patients in both cohorts. Conclusion: In this real-world setting, FXIII supplementation was most frequently initiated at FXIII activity levels below 60%-70%. These findings suggest using this range as a clinical reference point for therapeutic intervention, while bearing in mind the exploratory nature of the data. Routine FXIII testing should be considered in patients with unexplained bleeding or impaired wound healing, particularly perioperatively, after major trauma, or with persistent infections.

Background: The role of factor XIII (FXIII) in trauma-induced coagulopathy (TIC) is not fully understood. Methods: We evaluated FXIII supplementation in severely injured patients with persistent bleeding. This was a retrospective case series analysis. Results: Twenty-four patients received FXIII concentrate within 24 h of admission for bleeding that continued after transfusion of > 6 U red blood cells (RBCs); control patients (n = 27) did not receive FXIII concentrate. Both study groups were similar regarding injury severity score and global coagulation tests, but FXIII activity levels were significantly higher and lactate levels significantly lower in the control group, respectively. The differences in FXIII activity between the groups could be attributed to a more severe trauma-induced coagulopathy in FXIII-deficient patients, as demonstrated by lower fibrinogen and higher lactate levels. The median dose of FXIII concentrate within 24 h of admission was 2500 IU (IQR: 1250–4375). Median 24-h transfusion of RBCs (primary study endpoint) was significantly higher in the FXIII group versus controls (10.0 U, IQR 5–14 U vs. 2, IQR 0–6 U; p < 0.01). Subsequently, while patients were in the intensive care unit, there was no statistically significant difference regarding RBC transfusion anymore and the overall clinical outcomes were similar in both patient groups. Conclusions: The substitution of FXIII in patients who were more seriously compromised due to higher lactate levels and who presented with initially more severe bleedings than patients in the control group, resulted in a comparable transfusion necessity after 24 h. Thus, we guess that the substitution of FXIII in severely injured patients with ongoing bleeding might have an impact on their clinical outcome.

Introduction. On rare occasions, a pneumonectomy needs to be performed after a severe polytrauma. Statistically, this procedure increases the mortality rate to 50. One option to reduce this high rate could be an organ transplantation if a matching organ can be found. However, the current literature lacks any documentation of such a case. One reason for this stems from the fact that regulations for organ transplantation are very restricted and rarely allow exceptions. In addition, the chance for survival of polytraumatized patients in need of organs in the acute phase of the therapy is often quite unsure. Case Presentation. In this paper, we present the case of an 18-year-old patient who was involved in a serious motorcycle accident. His injury severity score was 29, but he suffered from severe bleeding in his lung which made a pneumonectomy necessary. The procedure led to a severe deterioration of his overall condition. An ECMO therapy was initiated, which effectively only slowed the aggravation. Therefore, he was transferred to our clinic where he was stabilized temporarily. A few days later, his situation turned worse. Since he had no other chance of survival, he was scheduled for a lung transplantation and was assigned an organ. After the transplantation, he stabilized quickly and recovered almost completely. Discussion. In this report, we want to show that an exception to the rules for organ transplantation might make sense on rare occasions. However, to increase the chance for any success, patients must be transferred to highly specialized clinics capable of treating patients with severe conditions. This might be the only chance for those patients to survive.

Introduction. Pelvic and lumbar spine injuries are very common especially in multiple trauma patients. The usual mechanism in young patients leading to pelvic fractures is a high-energy trauma such as traffic accidents. In elderly patients, low energy traumas are causal for such injuries. Compared to the high number of patients with pelvic or lumbar spine injuries, cerebral fat embolism is a quite rare finding but it needs to be considered to not misinterpret the radiological findings. Case. We present the case of a 41-year-old patient, who got hit and trapped in the lumbar region by a hydraulic arm in a car repair shop. The patient was primarily admitted to a level II trauma center. The radiological and clinical examinations revealed an open pelvic type C injury in terms of a spinopelvic dissociation, dislocation of the left hip joint, rupture of the mesentery of the rectum and colon sigmoideum, and a complex injury to the left ureter. Additionally, CT scan showed fluid with higher density than cerebro spinal fluid (CSF) in the lateral ventricles indicating an intracranial bleeding. After an immediate surgery to stabilize the patient, he was admitted to a level I trauma center. The reanalysis of the existing CT datasets combined with a new head CT leads to the conclusion that the high density fluid in the lateral ventricles is not a intracranial bleeding but rather fat deriving from the complex pelvic and lumbar spine fracture into the CSF system. Therefore, an immediate operation was performed to stabilize the spinopelvic dissociation and to close the injured dural sheath. Additionally, a ventricle drainage has been placed, which confirmed the diagnosis of intrathecal fat embolism. Afterwards, complex plastic surgery was necessary to restore the soft tissue damage. Conclusions. Intrathecal fat embolism in muliple trauma patients is a rare condition, which should be considered in patients with complex spine or pelvic injuries. It is important to distinguish this rare condition from intracranial bleedings, which are much more common because the consequent therapeutic strategy is quite different. In case of intrathecal fat embolism, a ventricle drainage system should be placed immediately, and the underlying spine or pelvic injuries need to be stabilized combined with closure of the dural sheath to prevent continuous fat embolism and meningeal infection.