https://ift.tt/2MCgHD3
Κυριακή 26 Αυγούστου 2018
Thoracic endovascular aortic repair with perioperative antibiotic therapy for infected ductus arteriosus aneurysm in an adult
https://ift.tt/2MCgHD3
Retrograde embolization of anterior tibial artery for an iatrogenic arterio-venous fistula causing left lower extremity claudication
https://ift.tt/2P8Fk6R
Urgent Repositioning After Venous Air Embolism During Intracranial Surgery in the Seated Position: A Case Series
https://ift.tt/2LvmWCX
Chordoma: a case series and review of the literature
Chordoma is a rare malignant tumor of the skull base and axial skeleton, with an incidence of less than 0.1/100,000 per year. Patients with advanced chordoma have a poor prognosis due to locoregional recurrenc...
https://ift.tt/2MvSC0m
Tocilizumab for the Treatment of Mevalonate Kinase Deficiency
Mevalonate kinase deficiency (MKD) is a severe autoinflammatory disease caused by recessive mutations in MVK resulting in reduced function of the enzyme mevalonate kinase, involved in the cholesterol/isoprenoid pathway. MKD presents with periodic episodes of severe systemic inflammation, poor quality of life, and life-threatening sequelae if inadequately treated. We report the case of a 12-year-old girl with MKD and severe autoinflammation that was resistant to IL-1 and TNF-α blockade. In view of this, she commenced intravenous tocilizumab (8 mg/kg every 2 weeks), a humanised monoclonal antibody targeting the IL-6 receptor (IL-6R) that binds to membrane and soluble IL-6R, inhibiting IL-6-mediated signaling. She reported immediate cessation of fever and marked improvement in her energy levels following the first infusion; after the fifth dose, she was in complete clinical and serological remission, now sustained for 24 months. This is one of the first reported cases of a child with MKD treated successfully with tocilizumab and adds to the very limited experience of this treatment for MKD. IL-6 blockade could therefore be an important addition to the armamentarium for the treatment of this rare monogenic autoinflammatory disease.
https://ift.tt/2LsRkxK