Title of article :
Ovarian haemorrhage: a rare presentation and diagnostic dilemma in Factor XIII deficiency
Author/Authors :
Alam, Muhammad Matloob Aga Khan University Hospital - Department of Paediatrics and Child Health, Pakistan , Iftikhar, Ahmed Raza Aga Khan University Hospital, Pakistan , Mushtaq, Naureen Aga Khan University Hospital - Department of Paediatrics and Child Health, Pakistan , Fadoo, Zehra Aga Khan University Hospital - Department of Paediatrics and Child Health, Pakistan
Abstract :
Factor XIII (FXIII) deficiency is a rare (autosomal recessive) genetic disorder which is associated with delayed bleeding symptoms that occur hour or days after trauma. Spontaneous rupture of visceral organs due to FXIII deficiency is a rare cause of abdominal pain with grave consequences and can be easily confused with other abdominal pathologies because of normal standard coagulation profile in these patients. We report a 15-yearold girl with spontaneous ovarian haemorrhage and splenic haematoma with FXIII deficiency. She had normal coagulation screen along with normal FXIII screen initially on the 5M urea testing which lead to delay in her diagnosis.
Keywords :
Factor XIII deficiency , Bleeding disorder , Ovarian haemorrhage , Splenic haematoma
Journal title :
Journal of the Pakistan Medical Association (Centre) JPMA
Journal title :
Journal of the Pakistan Medical Association (Centre) JPMA