Gastrointestinal Bleeding in Congenital Bleeding Disorders

Abstract

Gastrointestinal bleeding (GIB) is serious, intractable, and potentially life-threatening condition. There is considerable heterogeneity in GIB phenotypes among congenital bleeding disorders (CBDs), making GIB difficult to manage. Although GIB is rarely encountered in CBDs, its severity in some patients makes the need for a comprehensive and precise assessment of underlying factors and management approaches imperative. Initial evaluation of GIB begins with assessment of hematological status; GIB should be ruled out in patients with chronic anemia, and in presentations that include hematemesis, hematochezia, or melena. High-risk patients with recurrent GIB require urgent interventions such as replacement therapy for treatment of coagulation factor deficiency (CFD). However, the best management strategy for CFD-related bleeding remains controversial. While several investigations have identified CBDs as potential risk factors for GIB, research has focused on assessing the risks for individual factor deficiencies and other CBDs. This review highlights recent findings on the prevalence, management strategies, and alternative therapies of GIB related to CFDs, and platelet disorders.

Editorial Compilation XII

“…This issue of STH continues with the theme of bleeding, with a review from Samii et al on gastrointestinal bleeding in congenital bleeding disorders. 4 Gastrointestinal bleeding is a serious, intractable, and potentially life-threatening condition. There is considerable heterogeneity in gastrointestinal bleeding phenotypes among congenital bleeding disorders, making gastrointestinal bleeding difficult to manage.…”

Gastrointestinal bleeding in children: diagnostic approach

“…Bleeding from vitamin K deficiency should be considered in infants with maternal exposure to antiepileptic drugs that affect vitamin K, dysbiosis from antibiotic exposure, cholestasis, short bowel syndrome, or failure to receive perinatal vitamin K prophylaxis [ 10 , 11 ]. Vitamin K deficiency is easily corrected by the intramuscular or intravenous administration of vitamin K. Failure to correct bleeding should raise suspicion for congenital bleeding disorders, such as clotting factor deficiencies or von Willebrand’s disease [ 24 ].…”