An unusual form of Naxos disease and its improvement by adjuvant low-dose colchicine therapy.

We evaluated a female patient with an unusual form of Naxos disease, who presented with central cyanosis and clubbing, simulating congenital heart disease. Adjuvant low-dose colchicine therapy (0.5 mg once daily) showed positive effects and has been continued for six months. Colchicine has anti-inflammatory and anti-fibrotic properties. It inhibits mitosis by disrupting tubulin assembly and enhances cellular apoptosis. Follow-up showed improvement in the patient's clinical status, with a dramatic disappearance of the electrical storm and reductions in cyanosis and palmoplantar hyperkeratosis. Low-dose colchicine may be safe and effective in patients with Naxos disease and may reduce related complications.

Multiple angiomatous mitral valve cysts leading to floppy mitral valve syndrome.

We report an extremely rare case of cystic-tumor like formations that originated from the mitral valve tissue affected by verrucous endocarditis, leading to floppy mitral valve syndrome. These cystic tumoral formations were discovered during two-dimensional echocardiographic examination of a 46 year-old woman with cardiac symptoms of palpitation, dyspnea, and exertional angina pectoris. Multiple cysts were attached to the anterior mitral leaflet, resulting in pansystolic pseudoparachute-like floppy mitral valve prolapse, and severe mitral regurgitation. The patient underwent prosthetic mitral valve replacement following removal of the mitral valve and multiple cystic-tumoral formations. She had an uneventful postoperative course. Histological diagnosis was diffuse angiomatous cystic development of vasculatory tumor-like structures due to verrucous endocarditis.

[New discoveries in the mechanisms of apoptosis and cell survival and novel potential therapeutic strategies]. / Hücrede apoptoz ve sagkalim mekanizmalarinin kesfedilmesi ve yeni potansiyel tedavi stratejileri.

New discoveries in the mechanisms of apoptosis and cell survival have been a major breakthrough in biological sciences in recent years of the new millennium. Apoptosis is genetically programmed cell death in any nucleated cells of the organism. This type of cell death occurs through different mechanisms from those seen in necrosis and complement lysis of any cell, without affecting the neighboring cells. In the nature, apoptosis and cell survival are very important not only for the elimination of cells that acquire unstable features, become useless and detrimental to the organism, but also for the mechanisms of numerous biological events and disorders seen during the lifespan of many organisms--from the embryological period to death. The discovery of mechanisms of apoptosis and cell survival has enabled the development of new therapeutic strategies in heart diseases, cancers, neurodegenerative diseases, AIDS, and many disorders. Combination of opportunities afforded by degenerative medicine with those of new therapeutic approaches of regenerative medicine such as stem-cell therapy and somatic cell nuclear transfer will possibly introduce new horizons and rational therapeutic approaches in the foreseeable future.

In vitro susceptibility of yeasts isolated from patients in intensive care units to fluconazole and amphotericin B during a 3-year period.

Fungal infections have increased dramatically in recent years and candidemia is a major risk factor for morbidity and mortality in intensive care units (ICUs). Candidemia has been considered to be a nosocomial infection that is strongly associated with neutropenia, recent surgery or presence of intravascular lines, and previous colonization is an independent risk factor. We evaluated the in vitro efficacy of fluconazole and amphotericin B against yeasts isolated from various clinical specimens of colonized or infected patients treated in the ICUs of the Institute of Cardiology, Istanbul University. A total of 1397 ICU patients were treated at the Institute of Cardiology between January 2000 and December 2002. A total of 117 yeasts isolated from 97 patients were included in this study. These ICU patients were hospitalized for a mean of 29 days. All yeasts were identified by conventional methods and using the API (20C AUX, ID 32C) system (Bio Meriéux, France). Susceptibility to fluconazole and amphotericin B was evaluated using the E-test (AB Biodisk, Solna, Sweden). The most commonly isolated yeast was Candida albicans (72.6%), followed by Candida tropicalis (16.2%), Candida kefyr, Candida krusei, Candida parapsilosis, Trichosporon mucoides and Geotrichum spp. Fluconazole and amphotericin B MIC90 values were 0.75 microg/ml; 0.19 microg/ml and 1 microg/ml; 0.38 microg/ml for C. albicans and C. tropicalis, respectively. All Geotrichum spp. were found to be susceptible-dose dependent (SDD) (MIC=16-32 microg/ml) to fluconazole. Two C. albicans, two C. tropicalis, one C. krusei and one Geotrichum spp. had a MIC value of > or = 0.38 microg/ml for amphotericin B. The rate of colonization was 3.36% (47/1397). Only 10 (0.71%) patients out of a total of 1397 developed candidemia during the period of the investigation. Of these, 7 (70%) were caused by non-albicans Candida spp.