IgE-mediated anaphylactic reaction to purified and recombinant factor VIII in a patient with severe haemophilia A

We report a 51-year-old patient with severe haemophilia A developing a severe life-threatening anaphylactic reaction to recombinant factor VIII (rFVIII). Anaphylactic reactions are a rare but well-known side effect of FVIII products. The nature of these reactions could not be clarified as previous studies failed to demonstrate a specific IgE response. Here, we could prove a grade 3 anaphlyactic reaction as an IgE-mediated response to rFVIII for the first time by Western blotting.     

Aortic endograft infection: open surgical management with endograft preservation.

INTRODUCTION: We report successful management of aortic endograft infection without graft explantation or extra-anatomic bypass. REPORT: A 66 year-old male who had undergone endovascular repair of an aortic aneurysm presented with abdominal pain and raised inflammatory markers following embolisation of a type-2 'endoleak'. CT scanning revealed a left psoas fluid collection. Endograft infection was diagnosed. Following failure of CT-guided drainage and conservative management, surgical drainage with irrigation drain placement was undertaken with preservation of the endograft. There was no evidence of recurrent infection after follow-up at 30 months. DISCUSSION: Aortic endograft infection may be managed without endograft removal and extra-anatomic bypass.     

Iniencephaly and long-term survival: a rare case report

Introduction Iniencephaly, a neural tube defect involving occiput and inion and combined with rachischisis of the cervical, thoracic spine, and retroflexion position of the head is a very rare congenital abnormality of the fetus–newborn with a 0.1–10 of 10,000 prevalence. This abnormality’s prognosis is thought to be dismal. This abnormality can be associated with other abnormalities such as anencephaly, encephalocele, hydrocephalus, cyclopia, absence of the mandible, cleft lip and palate, cardiovascular disorders, diaphragmatic hernia, renal abnormalities, overgrowth of the arms compared to the legs, and club food and gastrointestinal atresia. Discussion Most of the patients are dead born, and the others die in a few hours. There are only six previously documented long-term survivors. In our case, our patient with iniencephalic signs and findings is still living. She is 2 years old now. We think that this patient presents a mild form of iniencephaly.