Since Iran has a high rate of RBDs with life-threatening bleeding, molecular studies can be used for carrier detection and, therefore, prevention of the further expansion of these disorders and their fatal consequence.

Intracranial hemorrhage (ICH) is a medical emergency. In congenital bleeding disorders, ICH is a devastating presentation accompanied with a high rate of morbidity and mortality. The prevalence of ICH is highly variable among congenital bleeding disorders, with the highest incidence observed in factor (F) XIII deficiency (FXIIID) (∼30%). This life-threatening presentation is less common in afibrinogenemia, FVIII, FIX, FVII, and FX deficiencies, and is rare in severe FV and FII deficiencies, type 3 von Willebrand disease and inherited platelet function disorders (IPFDs). In FXIIID, this diathesis most often occurs after trauma in children, whereas spontaneous ICH is more frequent in adults. About 15% of patients with FXIIID and ICH die; the bleeding causes 80% of deaths in this coagulopathy. Although in FXIIID, the bleed most commonly is intraparenchymal (> 90%), epidural, subdural, and subarachnoid hemorrhages also have been reported, albeit rarely. As this life-threatening bleeding causes neurological complications, early diagnosis can prevent further expansion of the hematoma and secondary damage. Neuroimaging plays a crucial role in the diagnosis of ICH, but signs and symptoms in patients with severe FXIIID should trigger replacement therapy even before establishment of the diagnosis. Although a high dose of FXIII concentrate can reduce the rate of morbidity and mortality of ICH in FXIIID, it may occasionally trigger inhibitor development, thus complicating ICH management and future prophylaxis. Nevertheless, replacement therapy is the mainstay of treatment for ICH in FXIIID. Neurosurgery is performed in patients with FXIIID and epidural hematoma and a hemorrhage diameter exceeding 2 cm or a volume of ICH is more than 30 cm. Contact sports are not recommended in people with FXIIID as they can elicit ICH. However, a considerable number of safe sports and activities have been suggested to have more benefits than dangers for patients with congenital bleeding disorders, and are hence suitable for these patients.

Introduction: Satureja khozestanica grows mainly in the southwest part of Iran as a native plant. This edible herb contains various compounds including the S. Khuzestanica essential oil (SKEO) and monoterpene known as Carvacrol. Previous studies have shown the anti-inflammatory effects of S. Khuzestanica without mentioning the exact mechanism of its function. Given that prostaglandin synthesis, which is one of the main mediators of inflammation, is regulated by the cyclooxygenase-2 (COX2) gene, the present study investigated the effects of SKEO and Carvacrol on the expression of the COX2 gene in the stimulated-J774A.1 macrophage cell line. Methods: To this end, fresh aerial parts of the plant were processed to prepare SKEO. Then, different doses of SKEO and Carvacrol (i.e., 0.004%, 0.008%, and 0.016% v/v) were used to treat with the lipopolysaccharides (LPS)-stimulated cell line for eight hours. After RNA extraction, the real-time polymerase chain reaction technique was applied for gene expression analysis. Results: In the LPS-stimulated J774A.1 macrophage cell line, COX2 gene expression reduced significantly in a dose-dependent manner (0.004%, 0.008%, and 0.016%, P = 0.024, P = 0.021, and P = 0.013 v/v of SKEO, respectively) by SKEO, and the effect of Carvacrol was less powerful (0.008% and 0.016%, P = 0.027 and P = 0.038 v/v, respectively) compared to SKEO. Finally, the comparison between SKEO and Carvacrol showed higher significant inhibitory effects of SKEO on COX2 gene expression in comparison with Carvacrol in 0.004% v/v concentration (P = 0.037). Conclusion: In general, SKEO significantly reduced COX2 gene expression, thus it can be suggested that its anti-inflammatory effect may result from the inhibition of the synthesis of this pro-inflammatory gene.

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