Vohwinkel syndrome : ichthyosiform variant in a family

Vohwinkel syndrome belongs to the group of hereditary palmoplantar keratoderma, having an autosomal dominant inheritance. In this report, the authors present a case of a four-year-old boy with diffuse scaling over his entire body and transgredient palmoplantar hyperkeratosis with some fissured areas. Family evaluation revealed that his mother and other family members were affected. Based on his clinical findings and on family history, the diagnosis of the ichthyotic Vohwinkel syndrome subtype, characterized by generalized ichthyosis and palmoplantar hyperkeratosis, was established

Nail psoriasis treated with pulsed dye laser

Nail changes are present in about 50% of psoriasis patients and tend to be refractory to conventional treatments. Pulsed dye laser has emerged as an alternative therapy. Our aim is to evaluate the efficacy of pulsed dye laser in nail psoriasis and the impact of treatment on quality of life. Fourteen patients were treated in monthly sessions for three months. The outcome assesment was made by the Nail Psoriasis Severity Index (NAPSI). The median improvement in the scores of the overall NAPSI, nail bed NAPSI, and nail matrix NAPSI were 44.2% (P = 0.002), 50% (P = 0.033) and 65.1% (P = 0.024), respectively

A description of ICNIRP’S independent, best practice system of guidance on the protection of people and the environment from exposure to non-ionizing radiation

In this statement, the International Commission on Non-Ionizing Radiation Protection (ICNIRP) presents its structure, its activities, and general approach to providing guidance on NIR protection. The statement highlights ICNIRP’s independence and presents the principle and requirements of no commercial or other vested interests. ICNIRP’s funding arrangements and collaboration with other advisory bodies and radiation protection authorities are also described. The statement also presents the types of guidance documents that are produced by ICNIRP and the general approach in assessing scientific evidence

Postmortem acinar autolysis in rat sublingual gland: a morphometric study

ABSTRACT OBJECTIVE: To analyze and to quantify morphological acinar postmortem changes in rat sublingual glands (SLG). MATERIAL AND METHODSs: Fifty rats were divided into two groups of 25 animals each. Group I was used for morphological and morphometric evaluations and group II for the determination of gland density and processed gland volume. Acinar autolytic changes were studied at 0 (control group), 3, 6, 12 and 24 h postmortem periods. The morphometric analysis of the volume density (Vv) and total volume (Vt) of intact (ia) and autolyzed (aa) acini was performed under light microscopy using a Zeiss II integration grid with 100 symmetrically distributed points. RESULTS: Morphologically, temporal progressive nuclear alterations and gradual loss of the structural architecture of acinar cells were found. Regarding quantitative results, both the Vvaa and the Vvia showed statistically significant differences among all postmortem periods (p<0.05). Vvaa increased from 0.42% at 0 h to 75.84% at 24 h postmortem and Vvia decreased from 71.16% to 0% over the same period. For Vtaa and Vtia, no statistically significant differences occurred between 12-24 h and 0-3 h (p&gt;0.05), respectively. Vtaa increased from 0.18 mm³ at 0 h to 38.17 mm³ at 12 h, while Vtia showed a decrease from 33.47 mm³ to 0 mm³ between 3-24 h postmortem. Data concerning Vtaa were adjusted by two-variable linear regression, obtaining the equation: y=-3.54 + 3.38x (r²=0.90). The Vtaa growth rate calculated by this equation was 3.38 mm³/h between 0-12 h. CONCLUSION: Acinar autolysis on rat SLG demonstrated the most significant signs during the first 6 h postmortem and was widely spread through the gland at 12 h

Syndrome in question

Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmen-tation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness

Estudo radiográfico e histológico do reparo de defeito ósseo perene em calvária de rato após o tratamento com material de enxerto orgânico bovino poroso

Over the last few years, various bone graft materials of bovine origin to be used in oromaxillofacial surgeries have entered the market. In the present study, we determined the capacity of a block organic bone graft material (Gen-ox, Baumer SA, Brazil) prepared from bovine cancellous bone to promote the repair of critical size bone injuries in rat calvaria. A transosseous defect measuring approximately 8mm in diameter was performed with a surgical trephine in the parietal bone of 25 rats. In 15 animals, the defects were filled with a block of graft material measuring 8mm in diameter and soaked in the animal's own blood, and in the other 10 animals the defects were only filled with blood clots. The calvariae of rats receiving the material were collected 1, 3 and 6 months after surgery, and those of animals receiving the blood clots were collected immediately and 6 months after surgery. During surgery, the graft material was found to be of easy handling and to adapt perfectly to the receptor bed after soaking in blood. The results showed that, in most animals treated, the material was slowly resorbed and served as a space filling and maintenance material, favoring angiogenesis, cell migration and adhesion, and bone neoformation from the borders of the lesion. However, a foreign body-type granulomatous reaction, with the presence of numerous giant cells preventing local bone neoformation, was observed in two animals of the 1-month subgroup and in one animal of the 3-month subgroup. These cases were interpreted as resulting from the absence of demineralization and the lack of removal of potential antigen factors during production of the biomaterial. We conclude that, with improvement in the quality control of the material production, block organic bone matrix will become a good alternative for bone defect repair in the oromaxillofacial region due to its high osteoconductive capacity.Nos últimos anos, vários materiais de origem bovina para enxerto ósseo têm sido lançados no comércio para serem utilizados em cirurgias bucomaxilofaciais. Na presente pesquisa avaliamos a capacidade de um material de enxerto ósseo orgânico em bloco (Gen-ox, Baumer SA - Brasil) preparado à partir de osso medular bovino, em promover a reparação de lesões ósseas de tamanho crítico em calvária de ratos. Uma lesão trans-óssea de aproximadamente 8mm de diâmetro foi realizada com trefina cirúrgica nos ossos parietais de 25 ratos, sendo que em 15 os defeitos foram preenchidos com bloco de 8mm de diâmetro do material de enxerto, embebida com sangue do próprio animal e em 10 somente com coágulo sanguíneo. As calvárias dos ratos tratados com o material foram coletadas após 1, 3 e 6 meses após as cirurgias e dos tratados com coágulo sanguíneo, imediatamente após as cirurgias e decorridos 6 meses. Durante as cirurgias verificamos que esse material enxertado é de fácil manuseio e se adapta perfeitamente ao leito receptor após sua embebição com sangue. Os resultados obtidos mostraram que na maioria dos casos tratados, o material foi reabsorvido lentamente e serviu como material de preenchimento e mantenedora de espaço, favorecendo a angiogênese, migração e adesão celular e a neoformação óssea à partir das bordas da lesão. No entanto, em 2 casos no subgrupo tratado - 1 mês e 1 caso no subgrupo tratado - 3 meses, ocorreu a presença de reação granulomatosa tipo corpo estranho com a presença de inúmeras células gigantes, que inibiu a neoformação óssea local. Esses casos foram interpretados como decorrentes de falhas na desmineralização e na retirada de potenciais fatores antigênicos durante a produção do biomaterial. Concluímos que com um aprimoramento do controle de qualidade na linha de produção do material, a matriz orgânica medular em bloco poderá ser uma boa alternativa para ser usada no reparo de defeitos ósseos na região buco-maxilofacial, devido a sua alta capacidade osteocondutora

Estudo estereológico comparativo entre os ameloblastos de secreção e de maturação em incisivos de ratos

Ameloblastos são células de origem epitelial, que apresentam funções de síntese, secreção e maturação do esmalte dental. Assim sendo, ameloblastos passam por mudanças morfológicas durante o seu curso de desenvolvimento. Desse modo, cinco ratos adultos Wistar foram utilizados para avaliar a morfologia de ameloblastos da fase secretora e em maturação, usando de métodos estereológicos em microscopia eletrônica de transmissão. Os dados foram analisados pela análise de variância a um critério (ANOVA). Os resultados mostraram que da fase de secreção para a de maturação da amelogênese: a) ocorreu uma redução de 23% no volume absoluto nuclear e o volume absoluto citoplasmático não mostrou diferença estatisticamente significante (p>;0.05); b) o volume total e a superfície total do retículo endoplasmático rugoso (RER) diminuíram 74% e 90%, respectivamente; c) o volume total e a superfície total das mitocôndrias aumentaram 742% e 384%, respectivamente; d) a relação superfície-volume do RER e das mitocôndrias diminuíram 59% e 42%, respectivamente; e) os pequenos grânulos de secreção, dispostos principalmente na área apical, desapareceram junto com o Processo de Tomes e apareceram os lisossomos e vacúolos digestivos principalmente na área supranuclear. Portanto, apesar de ocorrer uma significante adaptação morfológica durante a modulação da fase secretora para a de maturação do desenvolvimento do esmalte, o volume citoplasmático do ameloblasto permanece inalterado.The ameloblasts both in secretion phase and in smooth-ended ameloblasts in maturation phase were studied using stereologic methods in transmission electron microscopy (TEM). From secretion to maturation phase of amelogenesis, the nucleus volume decreased 23% and cytoplasm volume did not show significant changes; the total volume and surface of the rough endoplasmic reticulum (RER) decreased 74% and 90%, respectively, and of the mitochondria increased 742% and 384%, respectively; the surface-to-volume ratio for RER and mitochondria decrease 59% and 42%, respectively; and the predominantly apical secretory granules disappeared joined at Tomes process and lysosomes and phagic vacuoles have appeared principly in supranuclear cytoplasm. Although significant morphologic variation occurs from the secretory to the maturation phase of the ameloblasts, their cytoplasm volume remains unaltered

Spontaneous Periodontitis Development in Diabetic Rats Involves an Unrestricted Expression of Inflammatory Cytokines and Tissue Destructive Factors in the Absence of Major Changes in Commensal Oral Microbiota

Diabetes mellitus is a heterogeneous group of disorders, in which hyperglycemia is a main feature. The objective was to evaluate the involvement of RAGE, inflammatory cytokines, and metalloproteinases in spontaneous periodontitis triggered by diabetes induction. Immunohistochemical procedures for MMP-2, MMP-9, TNF-α, IL-1β, IL-6, RANKL, and RAGE were performed in rats after 1, 3, 6, 9, and 12 months of diabetes induction. Total DNA was extracted from paraffin-embedded tissues and evaluated by Real-TimePCR for 16S total bacterial load and specific periodontopathogens. Our data did not demonstrate differences in microbiological patterns between groups. In diabetic groups, an increase in RAGE-positive cells was detected at 6, 9, and 12 months, while TNF-alpha-stained cells were more prevalent at 6 and 12 months. In experimental groups, IL-β-positive cells were increased after 12 months, IL-6 stained cells were increased at 9 and 12 months, and RANKL-positive cells at 9 months. Diabetes resulted in widespread expression of RAGE, followed by expression of proinflammatory mediators, without major alterations in oral microbial profile. The pervasive expression of cytokines suggests that spontaneous periodontitis development may be independent of microbial stimulation and may be triggered by diabetes-driven imbalance of homeostasis

Spontaneous Periodontitis Development in Diabetic Rats Involves an Unrestricted Expression of Inflammatory Cytokines and Tissue Destructive Factors in the Absence of Major Changes in Commensal Oral Microbiota

Diabetes mellitus is a heterogeneous group of disorders, in which hyperglycemia is a main feature. The objective was to evaluate the involvement of RAGE, inflammatory cytokines, and metalloproteinases in spontaneous periodontitis triggered by diabetes induction. Immunohistochemical procedures for MMP-2, MMP-9, TNF-α, IL-1β, IL-6, RANKL, and RAGE were performed in rats after 1, 3, 6, 9, and 12 months of diabetes induction. Total DNA was extracted from paraffin-embedded tissues and evaluated by Real-TimePCR for 16S total bacterial load and specific periodontopathogens. Our data did not demonstrate differences in microbiological patterns between groups. In diabetic groups, an increase in RAGE-positive cells was detected at 6, 9, and 12 months, while TNF-alpha-stained cells were more prevalent at 6 and 12 months. In experimental groups, IL-β-positive cells were increased after 12 months, IL-6 stained cells were increased at 9 and 12 months, and RANKL-positive cells at 9 months. Diabetes resulted in widespread expression of RAGE, followed by expression of proinflammatory mediators, without major alterations in oral microbial profile. The pervasive expression of cytokines suggests that spontaneous periodontitis development may be independent of microbial stimulation and may be triggered by diabetes-driven imbalance of homeostasis