Macrophage activation syndrome in a patient with systemic-onset juvenile idiopathic arthritis initially treated for atypical Kawasaki disease: a case-based review

Systemic-onset juvenile idiopathic arthritis [SoJIA] is the most common rheumatic disorder in children and its presentation can mimic atypical Kawasaki disease. The diagnosis of SoJIA is often challenging and children are often diagnosed and treated for Kawasaki disease initially, especially after an unremitting fever lasting for several days. This fact can delay the treatment of SoJIA as incorrect treatment with intravenous immunoglobulins [IVIG] is being given and this may probably lead to a worse outcome in those individuals. This is a case of a 12-month-old infant who was initially treated for atypical Kawasaki instead of a SoJIA presenting with a macrophage activating syndrome [MAS]. We also present a review of the literature that supports the diagnosis of SoJIA presenting with MAS

Transcatheter aortic valve implantation: acute and 6-month outcomes of the first Lebanese experience and a literature review

Background : Transcatheter aortic valve implantation [TAVI] has recently emerged as a therapeutic alternative for high-risk surgical patients with severe symptomatic aortic valve stenosis and has been shown to improve clinical outcomes and reduce all-cause mortality in the PARTNER Trial. However, there is still no published data on similar outcomes in the Lebanon

Objectives : The aim of the study is to evaluate the immediate and short-term results of transfemoral [TF] and transaortic [TAo] TAVI done in Lebanon with 6 months follow-up

Methods: From July 2012 till March 2015, 10 consecutive high-risk and intermediate risk patients with severe symptomatic AS underwent TAVI using Edwards SAPIEN valve. The mean age was 79.4 +/- 6.9 years, logistic EuroSCORE 12.56 +/- 11.78 and mean STS 5.71 +/- 2.44. Patients were equally distributed among genders [50%]. The mean ejection fraction [EF] was 50.0 +/- 14.9% and mean AV area 0.61 +/- 0.1 cm2; mean aortic valve gradient [mAVG] 45.6 +/- 20.2 mmHg, and AV annulus size 21.8 +/- 1.8 mm

Results: TF approach was performed in 9 patients [90%] and TAo in 1 patient [10%]. All valves [17 size 26 mm and 25 size 23 mm] were implanted successfully. The overall 6-month survival was 80%. Grade I paravalvular aortic regurgitation [AR] was present in 60% of the patients; grade II in 10% while none of the patients developed AR with grade III or VI. The post-procedural mean aortic valve gradient [mAVG] was 9.77 +/- 3.31 mmHg; EF was 52.5 +/- 11.9% and immediate postoperative complications included only one case of newonset arrhythmia [AV Block]. After 6 months, one patient died of cardiac arrest following a massive pulmonary embolism while another patient died from a fatal stroke following infective endocarditis affecting the valve prosthesis

Conclusion: TAVI is a feasible technique for intermediate/high risk AS with high success rate and acceptable complications