Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematologic condition which could be revealed by deep venous thrombosis. It could be fatal unless correctly treated.
Case report: We report here the case of a 28 year-old male with no medical history who was admitted to the emergency room for severe abdominal pain. Computerized Tomography angiography (CT) scan revealed portal vein thrombosis. Laboratory findings showed pancytopenia with severe regenerative normocytic anemia resulting in PNH. Because of the lack of Eculizumab, treatment was first based on curative anticoagulation until bone marrow transplant, with no success.
Conclusion: PNH remains a severe disease with bad prognosis unless treated with Eculizumab.
Background: Congenital Vascular malformation relatively rare and extremely varied clinical presentations. The purpose of our study was to present our initial experience of embolization in a series of 26 patients with congenital vascular malformation to assess retrospectively the results and the complications of ethanol and coils embolization treatment of these patients.
Methods: Retrospective trial, the study group consisted of 26 patients with congenital vascular malformations. Transcatheter arterial embolization by ethanol or coils were performed, Therapeutic outcomes were established by evaluating the clinical outcome of symptoms and signs, as well as the degree of devascularization at follow-up angiography.
Results: Between November 2014 and March 2018, 26 consecutive patients (3 male, 23 female) at Alshifa Hospital - Cardiac Catheterization Center with congenital vascular malformations in the body and extremities underwent staged ethanol or coils embolization. The mean age of the patients was 25 years (age range, 6– 59 years). Ethanol embolization was administrated in 16 patients, coil embolization in 9 patients and graft stent in one patient. The side effect such as pain, pulsation, and bruit in most of the patients were obtained. The reduction of redness, swelling, and warmth was achieved in all of the patients, According to the angiographic findings, congenital vascular malformation were devascularized 100% in 12 patients, 50% to 99% in 11 patients, less than 50% in 3 patients. The most common complications were reversible skin necrosis.
Conclusion: Transcatheter embolization by ethanol or coils has proved efficacious and safe in the treatment of congenital vascular malformation of the body and extremities but with acceptable risk of complications.
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