ABSTRACT
BACKGROUND: Factor VII deficiency is a rare inherited bleeding disorder that has similar clinical presentation to hemophilia. CASE REPORT: A 7-year-old male child of African origin experienced recurrent nasal bleeding since 3 years of age and recurrent swelling of the joints that was remarkable at the age of 5-6 years. He received multiple blood transfusions and has been managed as a patient with hemophilia until he presented to our facility. Reviewed evaluation of the patient revealed abnormal prothrombin and normal activated partial thromboplastin time, FVII analysis showed activity level of less than 1%, and the diagnosis of FVII deficiency was made. The patient was treated with fresh frozen plasma, vitamin K injection, and tranexamic tablets. CONCLUSION: Even though factor VII deficiency is an extremely rare bleeding disorder, it does occur in our setting. This case highlights the need for clinicians to consider this condition when faced with challenging patients presenting with bleeding disorders.
Subject(s)
Factor VII Deficiency , Hemophilia A , Male , Humans , Child , Child, Preschool , Factor VII Deficiency/complications , Factor VII Deficiency/diagnosis , Factor VII Deficiency/genetics , Hemorrhage/etiology , Hemorrhage/therapy , PlasmaABSTRACT
INTRODUCTION: Kawasaki disease is a common vasculitis of unknown etiology that occurs mainly in preschool children. It manifests as a self-limited acute febrile illness with other features including extremity changes, cervical lymphadenopathy, oropharyngeal changes, truncal rash, and conjunctivitis. Intestinal involvement is not uncommon, with abdominal pain and vomiting being the most frequently reported symptoms. Intussusception has been described as a manifestation or complication of this disease, but few reports exist in literature. CASE PRESENTATION: A 7-month-old boy of Asian origin who presented with vomiting and passage of bloody mucoid stool was diagnosed with intussusception that was successfully reduced during emergency laparotomy. The baby was discharged home post-surgery following clinical improvement. He was readmitted on the fourth postoperative day with fever, irritability, and diarrhea. He was investigated and treated for presumed intraabdominal sepsis with multiple antibiotics with no improvement. He gradually developed the mucocutaneous features of Kawasaki disease and was treated with intravenous immunoglobulin and aspirin with good outcome. CONCLUSION: Intussusception and Kawasaki disease both commonly occur in children less than 2 years old. It is important to include Kawasaki disease as a differential diagnosis in children of this age who present with an acute febrile illness and gastrointestinal symptoms. A common underlying pathologic process could be contributing to both conditions.
