Background: Neonatal bleeding disorders can be quite challenging in neonatal intensive care units (NICUs) and are often linked to primary or secondary hemostatic defects. In Egypt, the high prevalence of rare inherited bleeding disorders (RIBDs) is attributed to consanguinity and close community marriages. We aimed to evaluate the bleeding phenotype of a neonatal cohort and to assess the usefulness of the International Society for Thrombosis and Hemostasis bleeding assessment tool (ISTH BAT) in identifying neonates with potential RIBDs.

Methods: A prospective research was conducted over one year, including all neonates (n = 61) with any bleeding symptom/s admitted to Cairo University Hospital NICU. Clinical evaluations, laboratory testing (CBC, PT, aPTT, INR, TT, fibrinogen, factor VII), and ISTH BAT scoring were performed.

Results: Upper gastrointestinal tract (36.1%) and pulmonary (19.7%) hemorrhages were the most common bleeding sites. Our cohort was separated into 2 groups: healthy(n=10) and unhealthy(n=51). An abnormal ISTH BAT score (≥3) has been found in 42.6% and was significantly higher among septic neonates (p-value = 0.013). Among the healthy group, 7 had vitamin K deficiency bleeding (VKDB) and 3 had afibrinogenemia, the latter showing the highest ISTH BAT scores (7–8).

Conclusion: VKDB and sepsis were the commonest causes of bleeding in healthy and unhealthy neonates, respectively. Bleeding due to afibrinogenemi are presented 4.9% and 30% of the whole cohort and healthy neonates, respectively. The ISTHBAT can be a useful screening tool for assessing significant bleeding in neonates, nonetheless identifying those with RIBDs.