Pulmonary, gastrointestinal and liver vascular malformations in Rendu-Osler disease.

Hereditary hemorrhagic telangiectasia (HHT) is an angiodysplastic disorder transmitted in autosomal dominant fashion. This case report describes a 46-year-old woman with multiple arteriovenous malformations that eventually caused multiorgan failure. The diagnosis was made in the early childhood based on frequently recurring episodes of severe epistaxis. Telangiectases were found in the skin, the visible mucosal linings, gastrointestinal and bronchial mucosa and liver. Episodes of severe hemoptysis necessitated partial resection of the right lung middle lobe at the age of 22. Liver dysfunction associated with the primary disease was diagnosed by the characteristic clinical picture, laboratory findings and instrumental examinations including abdominal ultrasonography, upper endoscopy, liver CT venography.

Antifungal prophylaxis with low doses fluconazole in patients with hematological malignancies.

INTRODUCTION: The incidence of mycotic infections in immunocompromised patients has reached a 20-fold rise for the last two decades. AIM: The goal of the study was to evaluate fluconazole efficacy as antifungal prophylaxis in patients with hematological malignancies. MATERIAL AND METHODS: Sixty nine patients with hematological malignancies and neutrophil count less than 1.0 x 10(9)/L received fluconazole orally at a dose of 150 mg every other day. Fluconazole was discontinued when a neutrophil count above 1.5 x 10(9)/L was maintained. Duration of neutropenic periods, afebrile cycles, and incidence of mycotic infections were assessed. The same variables were observed in a control group of 41 patients who did not receive antifungal prophylactic therapy. RESULTS: Both groups were similar in the mean duration of neutropenic period but the afebrile cycles in the patients receiving antifungal prophylaxis were longer by 5 days compared to these in the patients without prophylaxis. The mycotic infections had lower incidence in the patients on antifungal prophylaxis and the difference was statistically significant (p < 0.01). The superficial mycotic infections composed the majority of the clinically and/or microbiologically verified infections. These were presented by oropharyngeal infection (61%), esophageal (22%), and single cases of skin, genital or rectal infections. Candida albicans was isolated in 85% of the cases. The adverse reactions of fluconazole therapy were mild, transient, and easily manageable. No signs of liver and renal dysfunction were observed. CONCLUSION: Lower dose of fluconazole, 150 mg every other day p.o., has the same antifungal effect as a dose of 200 mg/day p.o. in neutropenic patients with hematological malignancies which lowers the cost of treatment.

[Minkowski-Chauffard disease in 1 family]. / Morbus Minkowski-Chauffard s opisanie na edna familiia.

A check up of literature has been made concerning the congenital microspherocytic hemolytic anemia, reporting of some new data about the pathogenesis of the disease. One family is described with CMHA--one father and his two daughters, the two children of his first daughter and the only child of the second daughter, hence confirming the familial character of the disease. All patients have been splenectomized. After the splenectomy they are practically healthy, without hemolytic crises, supporting the statement that splenectomy is the treatment of choice in CMHA therapy.