Is Sjögren's syndrome involved in the formation of localised nodular amyloidosis?

The case of a patient who presented with Sjögren's syndrome complicated by localised cutaneous nodular amyloidosis is reported. We discuss the possible link between these two diseases.

The Str mouse as a model for incontinentia pigmenti.

Striated mouse has been proposed as a model for incontinentia pigmenti (IP) based on the similarities in genetic predisposition and syntenic gene localisation in mouse. IP is considered an ectodermal dysplasia with all four characteristic structures involved: sweat glands, hair, teeth and nails. Recently mutations have been found in the Nsdhl, encoding an NAD(P)H steroid dehydrogenase-like protein in Str and Bpa mice. We analysed the phenotype of the Str mouse to evaluate the involvement of ectodermally derived tissues. Our results demonstrated that in Str mouse in addition to abnormal coat texture, sweat glands were severely dystrophic or missing. Retinal degeneration and skeletal abnormalities were also found. We conclude that Str mouse is a good model to get new insights in the pathogenesis of ectodermal dysplasias and X linked male lethality in humans.

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.

Prevalence of Sjögren's syndrome in Slovenia.

OBJECTIVE: The aim of our study was to determine the prevalence of Sjögren's syndrome (SS) in Slovenia. METHODS: A total of 889 randomly selected adults were invited to take part in our study. The classification of SS was based on the validated criteria reported by a multicentre study performed in Europe. The participants were asked six simple questions for assessing both ocular and oral involvement. Information on co-morbidities and related treatment was collected at the same time. All participants were subjected to a Schirmer-I test, an unstimulated salivary flow test, as well as serological studies (rheumatoid factor, antinuclear antibodies, anti-Ro/SS-A and anti-La/SS-B antibodies). When indicated, Rose Bengal score, salivary scintigraphy and histopathological investigation of the minor salivary glands were carried out until three out of the six European classification criteria for SS were shown to be negative or until SS was diagnosed. RESULTS: Out of the 889 invited subjects, 332 (37.3%) participated in our study: 183 females, mean age (+/- S.D.) 52.2 +/- 13.7 yr (range 20-84) and 149 males, mean age (+/- S.D.) 56.3 +/- 12.9 yr (range 23-84). After the first visit, 244 of the 332 (73.5%) participants proved to be negative for three out of the six above-mentioned criteria, and were eliminated from further tests. The remaining 88 participants were consecutively subjected to Rose Bengal score, salivary scintigraphy and minor salivary gland biopsy. Fifteen participants refused to perform either one or more of the proposed tests at the second study stage. Two females of the 332 study participants [0.60% (exact 95% CI 0.07%, 2.16%)] fulfilled the criteria for primary SS. CONCLUSIONS: The estimated prevalence of definite SS in Slovenia is 0.60%.

Femur osteomyelitis due to a mixed fungal infection in a previously healthy man.

We describe a previously healthy, 22-year-old man who, after a closed fracture of the femur and subsequent operation, developed chronic osteomyelitis. Within a few days, infected bone fragments, bone, and wound drainage repeatedly yielded three different filamentous fungi: Aspergillus fumigatus, Aspergillus flavus, and Chalara ellisii. Histologic examination of the bone revealed septate hyphae. After sequential necrotomies of the femur and irrigation-suction drainage with added antimycotic therapy, the infection ceased and the fracture healed. This case is unique in that it is the only known instance in which a long bone was affected in an immunocompetent individual, with no evidence of any systemic infection, by a mixed population of two different Aspergillus spp. and the rare filamentous fungus C. ellisii. Environmental factors that could potentiate the infection include blood and edema fluid resulting from the surgical procedure and the presence of the osteosynthetic plate.

Skeletal muscle immune deposits in systemic lupus erythematosus. Correlation with histologic changes, autoantibodies, and clinical involvement.

Skeletal muscle biopsy and autopsy samples of 132 SLE patients were studied by immunofluorescence and light microscopic techniques. Immune deposits were compared to histologic abnormalities and clinical and serologic findings. Immune deposits with a mainly granular pattern were observed at different locations in 49 patients (37%). They correlated significantly (p<0.001) with inflammatory myopathy, demonstrated in 33(25%), with noninflammatory myopathy which occurred in 38 (29%) vasculitis, observed in 13(10%), and with noninflammatory vasculopathy which was noted in 10 patients (8%). The correlation of muscle immune deposits with anti-DNA antibodies was significant at p= 0.016. Anti-Sm and anti-U1 RNP antibodies were associated significantly with the intensity of immune deposits. Furthermore, a correlation of immune deposits with increased creatine phosphokinase and myopathic electromyogram, but not with evidence of clinical involvement, was shown. A key pathogenetic role of immune deposits in the development of skeletal muscle histologic abnormalities in SLE was demonstrated.

Langerhans and other immunocompetent cells in vocal cord epithelial hyperplastic lesions of patients with chronic laryngitis.

The aim of the study was to evaluate the intraepithelial and stromal density of Langerhans cells and lymphoid infiltrate in different stages of carcinogenesis in vocal cord biopsies of 24 randomly selected patients with chronic laryngitis. The Langerhans and lymphoid cells were counted using immunolabelling with antibodies against CD1a, S100, CD3, CD20, and CD68 on paraffin-embedded sections of 24 archival laryngeal vocal cord mucosa biopsy specimens, 6 classified as simple, 7 as abnormal, and 11 as atypical epithelial hyperplasia. Results were statistically evaluated using the Kruskal-Wallis and Wilcoxon sign rank tests. The mean number of Langerhans cells and T lymphocytes per mm2 of cross-sectioned epithelium was found to increase from simple to atypical hyperplasia. There were statistically significant differences in Langerhans cell density between atypical hyperplasia and each of the other 2 grades, simple and abnormal hyperplasia, with p < 0.05. Our study suggests the involvement of immune mechanisms, particularly cell mediated, during laryngeal carcinogenesis and the possibility that the assessment of Langerhans cell density might be of prognostic significance.

[Psychiatry--where are we and where are we going?]. / Psihijatrija--gdje smo sada i kamo nam je ici.

The article gives a short review of the historical, ideological, theoretical and organizational development of the concept of protection and improvement of mental health (within a psychiatric context) both in Croatia and the rest of the world with special emphasis on the problems and possibilities which arise from the present situation in Croatia. It also explains the necessity to analyze the Law on Health Care and Health Insurance from a psychiatric point of view, the need to link the areas of psychiatry (preventive and clinical work) which deal with primary protection, the need to define the criteria and standards regarding the organization and reorganization of psychiatric clinics and general hospitals, the need to analyze and reorganize the study of psychiatry, the need to establish national and regional coordination boards and hold elections for national and regional coordinators and the need to establish the Centre for the Protected and Improvement of Mental Health.

[Utilization of findings from computerized tomography of the brain in psychiatric diagnosis]. / Primjena nalaza kompjutorizirane tomografije mozga u psihijatrijskoj dijagnostici.

The application and critical evaluation of brain-computed tomography (CT) to psychiatry are presented primarily in relation to the cerebral atrophies and organic brain syndromes. It can be observed, that clinical diagnosis of brain atrophy is frequently made only upon findings of diffuse cortical atrophy. The attention is directed to the non-consistent CT scan measures and diagnostic criteria of brain atrophy on CT scan due to non-consistent methodologies of sulcus and ventricular measurements. The linear and planimetric measurements and difficulties in exact analysis of cerebral atrophy on CT scanning are discussed. The nosology, clinical criteria and etiology of chronic organic brain syndromes, the clinical conditions and diseases connected with diffuse cerebral atrophy noticeable on CT scan are reported. The conclusion is, that diffuse cerebral atrophy on CT scan without clinical symptoms and criteria of cerebral atrophy cannot be considered as basis for making psychiatric diagnosis, respectively, that such finding needs other exact clinical, psychiatric and psychological examinations.