Inhaled vasodilator therapy for pulmonary hypertension may decrease the systemic side effects commonly observed with systemic administration. Inhaled medications only reach ventilated areas of the lung so local vasodilation may improve ventilation/perfusion matching and oxygenation. We compared the effects of intravenous versus aerosolized treprostinil on pulmonary and systemic hemodynamics in ...

Inhaled vasodilator therapy for pulmonary hypertension may decrease the systemic side effects commonly observed with systemic administration. Inhaled medications only reach ventilated areas of the lung, so local vasodilation may improve ventilation-perfusion matching and oxygenation. We compared the effects of intravenous vs. aerosolized treprostinil on pulmonary and systemic hemodynamics in an...

......................................... Received: 06-12-13 Accepted: 21-07-14 Abstract Systemic sclerosis is a multisystem rheumatic autoimmune disorder characterized by fibrosis of the skin and many other organs. The gastrointestinal tract is the second cause of systemic compromise of this disease, but idiopathic portal hypertension is an extremely rare condition in these patients. We descri...

Primary systemic amyloidosis (AL) is an uncommon disease characterized by the extracellular deposition of a protein with a beta-fibrillar structure, consisting of monoclonal immunoglobulin light chains, lambda or kappa (ratio of lambda to kappa, 3:1). In systemic amyloidosis liver involvement is frequent but it rarely has clinical importance. The massive and localized liver deposition of amyloi...

Systemic mastocytosis is a myeloproliferative disorder characterized by extracutaneous involvement of at least one organ. Although rare, infiltration of inflammatory mast cells within the portal vein may lead to obstruction of the sinusoids resulting in non-cirrhotic portal hypertension. We present a patient with known history of systemic mastocytosis with bone marrow involvement presenting wit...

A 28-year-old woman presenting with subacute onset of a tetraplegia is described who was shown to have active systemic lupus erythematosus in association with high circulating anticardiolipin binding and lupus anticoagulant activity. The patient later developed severe symptomatic systemic and pulmonary hypertension and required emergency resuscitation. This case provides further support for an ...

We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed. Systemic vascular alterations caused by deletion of the elastin gene may occur early in individuals with Williams-B...

1 Barst RJ. Experience and reason [editorial]. Chest 2000; 117:2–5 2 Klings ES, Hill NS, Ieong MH, et al. Systemic sclerosisassociated pulmonary hypertension: shortand long-term effects of epoprostenol (prostacyclin). Arthritis Rheum 1999; 42:2638–2645 3 Klings ES, Ieong MH, Simms RW, et al. Long-term intravenous epoprostenol therapy for pulmonary hypertension associated with systemic sclerosis...