PubMed Health⌕ Search

Biomedical subjects

A Kansky

Publications and source records attributed to A Kansky.

At least 19 recordsLinked to original sources

Increase in contact allergy to fragrances: patch-test results 1989-1998.

We report the results of patch tests with fragrance-mix as a part of the standard series carried out over the last 10 years (1989-1998) during routine testing of 6129 patients in our department. 5.9% of the total number of patients who were patch tested were positive to fragrance mix. The sex ratio was 2.3:1 with a female predominance. In 1989-1993, the frequency of contact sensitivity to fragrance mix was 3.9% (4.9% for females and 2.1% for males). This rate rose both in female and male patients during the observed period of time and attained 8.9% (females) and 4.1% (males) in 1994-1998; the overall frequency in 1994-98 was 7.5%. This rising trend, which was statistically significant, might be the consequence of an increased use of cosmetics and toiletries containing fragrances in our population.

Allergens↗

Cathepsin D expression in early cutaneous malignant melanoma.

BACKGROUND: The aspartic proteinase cathepsin D is believed to be associated with proteolytic processes leading to the invasion and seeding of tumor cells. An association between cathepsin D tissue concentration and aggressiveness of tumors has been detected in different cancer types, as well as in metastatic melanoma. METHODS: The concentration of cathepsin D was measured immunoradiometrically (ELSA-CATH-D kit, CIS Bio International) in the cytosols of 51 primary cutaneous melanomas (with Breslow index < 4 mm) to estimate the tissue concentrations of cathepsin D in early cutaneous melanoma. RESULTS: A significantly elevated concentration of cathepsin D was measured in the tumor cytosols as compared to adjacent normal tissue (44.2 vs. 14.7 pmol/mg of total protein, P < 0.001). CONCLUSIONS: Our results indicate that cathepsin D is expressed at high levels by melanoma cells. The extremely high expression of cathepsin D in two of our patients, with later progression of the disease over a 42-month follow-up period, suggests a possible correlation between the cathepsin D tissue concentration and the prognosis of primary cutaneous malignant melanoma.

Adult↗

Mutation of a type II keratin gene (K6a) in pachyonychia congenita.

Pachyonychia congenita (PC) is a rare autosomal dominant condition characterized by multiple ectodermal abnormalities. Patients with Jadassohn-Lewandowsky Syndrome (MIM #167200; PC-1) have nail defects (onchyogryposis), palmoplantar hyperkeratosis, follicular hyperkeratosis and oral leukokeratosis. Those with the rarer Jackson-Lawler Syndrome (MIM #167210; PC-2) lack oral involvement but have natal teeth and cutaneous cysts. Ultra-structural studies have identified abnormal keratin tonofilaments and linkage to the keratin gene cluster on chromosome 17 has been found in PC families. Keratins are the major structural proteins of the epidermis and associated appendages and the nail, hair follicle, palm, sole and tongue are the main sites of constitutive K6, K16 and K17 expression. Furthermore, mutations in K16 and K17 have recently been identified in some PC patients. Although we did not detect K16 or K17 mutations in PC families from Slovenia, we have found a heterozygous deletion in a K6 isoform (K6a) in the affected members of one family. This 3 bp deletion (AAC) in exon 1 of K6a removes a highly conserved asparagine residue (delta N170) from position 8 of the 1A helical domain (delta N8). This is the first K6a mutation to be described and this heterozygous K6a deletion is sufficient to explain the pathology observed in this PC-1 family.

Amino Acid Sequence↗

Hereditary palmoplantar keratoderma, type papulosa, in Croatia.

BACKGROUND: Hereditary palmoplantar keratoderma (HPPK), type papulosa, is rare, and epidemiologic data are sporadic and inconsistent. An epidemiologic population study of this disease has not been performed previously. OBJECTIVE: We performed a large population study on prevalence of HPPK, type papulosa, in Croatia. METHODS: The data were collected from medical records of dermatology departments throughout Croatia; 14 patients and their relatives were examined. Histopathologic studies were performed in 11 of these 14 patients. RESULTS: Fifty-five patients were identified and the prevalence was 1.17 per 100,000 inhabitants. All 55 patients belonged to 20 different families. An autosomal dominant mode of inheritance was confirmed in 13 families. All 14 patients examined by the authors had both palmar and plantar lesions; the volar aspects of fingers were also involved. Thickened nails were observed in four patients, and no significant skin lesions were found elsewhere. CONCLUSION: HPPK, type papulosa, is rare, and its prevalence in Croatia is about four times lower than HPPK, Unna-Thost type. It should be considered a distinct entity.

Adolescent↗

Isolated cases of palmoplantar keratoderma, Unna-Thost type.

A study on the prevalence of hereditary palmoplantar keratoderma, Unna-Thost type, was carried out in Croatia. Altogether 205 cases were verified, and of these forty-eight were chosen for further studies. Six isolated cases of hereditary palmoplantar keratoderma, Unna-Thost type, were found. The following theories were considered to explain such a phenomenon: spontaneous mutation, reduced gene expression, incomplete penetration of the gene, late onset of the disease, unknown biological father of the proband, and involvement of genes in addition to the autosomal dominant one.

Humans↗

Occurrence of hereditary bullous epidermolyses in Croatia.

To determine the occurrence of hereditary bullous epidermolyses (EB) in Croatia, Yugoslavia, from 1960 to 1987, cases were gathered from the hospital files of dermatologic and pediatric clinics and departments throughout the area. The diagnosis of EB type was made on the basis of clinical features, patients' histories, and light microscopy and electron microscopy findings. Fifty families with 58 patients were registered; 44 patients were examined personally by one of the authors. The most frequent type of EB in Croatia was recessive dystrophic EB Hallopeau-Siemens, occurring in 35 of the 58 individuals. Regional accumulation of cases within the Varazdin area was noted (13 patients). Prevalence of EB in Croatia is 0.956 cases per 100,000 inhabitants. One case of recessive dystrophic EB Hallopeau-Siemens occurred in about every 52,000 live births.

Epidermolysis Bullosa↗

Prevalence of psoriasis in Croatia.

The prevalence of psoriasis in Croatia was studied by the representative samples method. The total number of investigated persons was 8416. The authors detected 131 psoriatics (prevalence -1.55%).

Adolescent↗

A modified photometric quantification of skin surface lipids sampled using the cigarette paper method.

In severe cases of acne conglobata the use of substances which considerably reduce the sebum excretion rate, such as 13-cis-retinoic acid, is reasonable. To monitor the effect of such preparation a noninvasive and accurate method for measuring skin surface lipids (SSLs) is required. A new modified photometric quantification of SSLs sampled using the cigarette paper method based on reaction with phosphovanilic reagent was introduced. Our overall results indicate that a 3-h SSLs assessment measured photometrically is as accurate as the gravimetric technique; it is reproducible in situations with low and high levels of SSLs.

Acne Vulgaris↗

Contact dermatitis in workers processing polyvinyl chloride plastics.

Out of 401 workers employed in a factory producing shoes from polyvinyl chloride (PVC) granulate, 388 were investigated. By patch testing with the standard tests and 4 phthalate compounds, a diagnosis of allergic contact dermatitis was confirmed in 11 of these. Six of them were sensitized to phthalates and 5 to coal tar.

Adult↗

Keratodermia palmoplantaris of the Unna-Thost type in Slovenia.

Keratodermia palmoplantaris of the Unna - Thost type was studied in the population of Slovenia. Altogether 157 patients were recorded: 106 were examined by the authors; for the other 51, anamnestic data were used. Special attention was paid to the accompanying symptoms which are more or less rarely mentioned in the literature: hyperkeratosis of the elbows and knees, transgredience of the hyperkeratosis, hyperkeratotic changes of the nails and changes of the teeth. It is estimated that there are in Slovenia 8.44 PPK UT patients per 100 000 inhabitants.

Adult↗

Erythropoietic protoporphyria in Slovenia. Epidemiologic study.

32 patients with EPP were detected in Slovenia, which gives an incidence of 1.71 cases/100,000 inhabitants. 20 patients had typical clinical symptoms and increased values of laboratory assays, 12 patients had either slightly expressed clinical symptoms or increased values of laboratory tests; these patients were diagnosed as latent cases. The patients belong to 12 families, no blood relationship between these families could be established. In 7 families the latent carriers of this inborn error of the porphyrin metabolism were detected. Almost all patients live in two distinct, rather small geographical areas, one in north-eastern Slovenia and the other near Ljubljana.

Bone Marrow Diseases↗