[Guidelines for diagnosis, therapy and follow up of Anderson-Fabry disease]. / Smjernice za dijagnostiku, lijecenje i pracenje Anderson-Fabryjeve bolesti.

Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the α-galactosidase A (α-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations. Estimates of the incidence range from one per 40,000 to 60,000 in males, and 1:117,000 in the general population. Pain is usually the first symptom and is present in 60%-80% of affected children, as well as gastrointestinal disturbances, ophthalmologic abnormalities and hearing loss. Renal failure, hypertrophic cardiomyopathy, or stroke as the presenting symptom may also be found even as isolated symptoms of the disease. Life expectancy is reduced by approximately 20 years in males and 10-15 years in females, therefore enzyme replacement therapy should be introduced in patients of any age and either sex, who meet treatment criteria for Anderson-Fabry disease.

[Guidelines for the diagnosis and treatment of psoriasis]. / Smjernice za dijagnostiku i lijecenje vulgarne psorijaze.

Croatian dermatovenerological society of the Croatian Medical Association formed the working group which consists of leading experts for psoriasis in Croatia. After a critical analysis of relevant scientific papers, the working group has developed guidelines for the diagnosis and treatment of psoriasis.

[Antisynthetase syndrome - a case report]. / Antisintetaza sindrom - prikaz bolesnice.

Twenty-eight year-old woman with predominant signs of polymyositis, pulmonary interstitium involvement and with positive anti-Jo1 antibodies was suspected for antisynthetase syndrome. Over the next three months sores and ulcerations have appeared at the fingertips. In the later course of the disease clinical picture of mixed connective tissue disease associated with interstitial lung disease, with a dominant picture of systemic sclerosis have emerged. She was treated with glucocorticoides and immunosuppressive therapy. Patient condition was mostly stable, without significant progression of lung lesions. Early diagnosis and treatment antisynthetase syndrome significantly contributes to more favorable course and outcome of disease. A prerequisite for that are well-defined diagnostic criteria and an appropriate choice of treatment.

Guidelines for diagnosis, therapy and follow up of Anderson-Fabry disease.

Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the alpha-galactosidase A (alpha-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations. Estimates of the incidence range from one per 40,000 to 60,000 in males, and 1:117,000 in the general population. Pain is usually the first symptom and is present in 60%-80% of affected children, as well as gastrointestinal disturbances, ophthalmologic abnormalities and hearing loss. Renal failure, hypertrophic cardiomyopathy, or stroke as the presenting symptom may also be found even as isolated symptoms of the disease. Life expectancy is reduced by approximately 20 years in males and 10-15 years in females, therefore enzyme replacement therapy should be introduced in patients of any age and either sex, who meet treatment criteria for Anderson-Fabry disease.

Historical development of local therapy of psoriasis vulgaris.

Throughout the history people have used various remedies for the treatment of psoriasis. Some of them were bizarre, some ineffective and harmful, and some have survived until today and are still in use. Salicylic acid, anthralin, coal tar, corticosteroids, vitamin D3 analogues, retinoids must be mentioned as remarkably effective ones. Phototherapy and laser therapy are both promising techniques, as well as immunomodulators. Underlying mechanisms of psoriasis and mode of action of therapeutics that have been elucidated over the time allowed for the development of more potent and promising therapy of psoriasis.

Epidemiology of dermatomycosis in the eastern Croatia--today and yesterday.

The aim of our investigation was to compare the distribution of dermatomycosis species in Eastern Croatia between two different periods: first period from 1997-2001 year, and second period from 1986-88 year. The outpatients from Department of Dermatovenerology University Hospital "Osijek" with confirmed diagnosis infection. Tinea, were selected on the basis o age, gender, localization and dermatomycosis species. During the first period (1997-2001) among 75,691 outpatients Tinea infection was confirmed in 558 (0.73%), while in the second period among 47,832 outpatients there were 126 (0.26%) cases with Tinea, what showed significant increase of fungal infections among population this region. According the age and gender in both periods predominant population were under of the age 16(40.14%: 41.26%), and female population was predominant (58.60% and 57.14%) in comparison to males (41.39% and 42.85%). The most frequent localization of lesions in period I were cutis glabrae (47.31%), palms and soles (31.36%), capitis (17.38%) and unguis (9.31%) and isolated species were as followed: Trichophyton (39.06%), Microsporum (31.72%) and Candida (28.13%) species. In period II the most frequent localization were palms and soles (40.47%), cutis glabrae (36.50%), capitis (12.69%) and unguis (10.31%). The isolated species in this period were: Trichophyton (80.15%), Candida (12.69%) and Microsporum (4.76%) species. From the data collected during two different periods we can observe 1) increase of fungal infection generally in our region; 2) significant changes in causative species (increase of Microsporum and Candida species infection, but Trichophyton spp still remain the first causative agent); and 3) changes in the localization of lesions.