Terapevt Arkh
-
The paper describes a case of giant cell arteritis whose leading clinical sign is long-term fever. It discusses current approaches to diagnosing giant cell arteritis.
-
Mesenteric inflammatory veno-occlusive disease (MIVOD) is a rare disease. Its basis is inflammation of the small and medium-sized mesenteric veins, which is generally complicated by thrombosis and intestinal ischemia. Arteries are not therewith involved in the process. ⋯ The lumen of arterial vessels of different diameters was free. No signs of systemic vasculitis and Crohn's disease were found. The postoperative period was uncomplicated.
-
The orphan drug laws enacted firstly in the USA and then in some other countries have given rise to a specific market of drugs for rare diseases and to a drastic increase in the number of proposed drugs. At the same time, the prices for innovative drugs are frequently high, have resulted in debates about their cost effectiveness and the drugs for rare diseases being frequently difficult to be tested for their efficacy due to disease rarity. The article discusses the designs of clinical trials that could enhance their effectiveness, gives examples of standard-design trials, and notes that effectiveness evaluations can be obtained for most diseases, but with a small sample, they will not be very precise, which makes cost-effectiveness analysis difficult. In this connection, a number of mechanisms proposed worldwide to solve the existing situation are discussed.
-
The paper describes a clinical case of Behçet's disease accompanied by oral aphthous elements and external genital sores. The disease occurred after an infectious and inflammatory process in the oropharynx; chronic erythema nodosum and arthritides were successively diagnosed. A set of anamnestic data and clinical and laboratory examinations could establish a correct diagnosis.
-
Hypogammaglobulinemic sprue (HGGS) belongs to orphan (rare) diseases. Its basis is a concurrence of primary immunodeficiency and celiac disease. The clinical picture of HGGS is characterized by recurrent bacterial infection foci, chronic diarrhea, and severe malabsorption syndrome (MAS). ⋯ The disease was characterized by recurrent lung and kidney inflammatory foci concurrent with chronic diarrhea and grade 3 MAS. There was a dramatic reduction in GG, IgG, and IgA levels. Clinical remission and small intestinal mucosal structural recovery occurred due to GFD.