Revista medico-chirurgicală̆ a Societă̆ţ̜ii de Medici ş̧i Naturaliş̧ti din Iaş̧i
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Rev Med Chir Soc Med Nat Iasi · Oct 2002
Case Reports[Osler--Rendu Disease: an example of a family clustering in a genetic disorder].
The case of a 75 years old woman with hereditary hemorrhagic telangiectasia (HHT) is presented. This condition is an autosomal dominant mucocutaneous and visceral fibrovascular dysplasia in which telangiectasia, arteriovenous malformations and aneurysms may be widely distributed throughout the cardiovascular system. It is usually recognized as a "triad" of telangiectasia, recurrent epistaxis and a family history of the disorder.
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The Tumor Lysis Syndrome (TLS) is a constellation of metabolic disturbances due to the rapid tumoral cell destruction, either spontaneous or induced by cytoreduction therapy, which manifests as an acute, subacute or chronic renal insufficiency. Hyperuricemia is the hallmark of the TLS. The therapy consists in management of hydro-electrolytic and metabolic disturbances, treatment of hyperuricemia (Allopurinol and a new therapeutical agent, Uricase), hemodialysis. If promptly supported during the TLS, a number of these patients have an excellent probability of long-term remission.