Clinical manifestations and oral findings in fraser syndrome

This article is the first known case report of Fraser syndrome in the dental literature. Its purpose was to present the clinical manifestations, oral findings, and dental treatment of a 14-year, 10-month-old female patient. Fraser syndrome is a rare recessive autosomal genetic disorder characterized by multisystemic malformation, usually comprising cryptophthalmos, syndactyly, and renal defects. The child presented with: (1) hydrocephaly; (2) face asymmetry; (3) low-inserted ears; (4) flat nose bridge; (5) cryptophthalmos; (6) bilateral absence of eyeballs; (7) hypertelorism; (8) syndactyly on the left fingers and toes; (9) skeletal defects; and (10) lower limb asymmetry. The intraoral examination revealed: (1) complete primary denture; (2) malocclusion; (3) tooth crowding; (4) ogival palate; (5) normal labial frena; (6) absence of lingual frenum (not compromising the tongue movements); (7) parched lips; (8) supragingival calculus adhered to all tooth surfaces; and (9) moderate gingivitis. The dental treatment consisted of periodic monitoring of the patient's oral health status and supragingival scaling associated with topical applications of 0.12% chlorhexidine digluconate gel at 2-week intervals to reduce gingivitis.

Periodontal Manifestations and Ambulatorial Management in a Patient With Sturge Weber Syndrome

Sturge-Weber syndrome is a nonhereditary congenital condition characterized by leptomeningeal and facial skin angiomatous malformation following the trigeminal nerve path. The intraoral angiomatosis are presented in 40% of cases and results in an important periodontal alteration, increasing the risk of bleeding during dental procedures. A 43-year-old male patient presented with port wine stain on the right side of the face, the entire hard and soft palates, the alveolar ridge, and buccal mucosa, and had an excessive accumulation of calcified masses in both supragingival and subgingival sites, with swelling and generalized inflammation throughout the gingiva and alveolar mucosa. He reported not having sanitized the area for years for fear of bleeding. Periodontal management, to remove calculus and to control gingivitis initiated in the supragingival region and gradually reaching the subgingival region to control oral microbiota, was performed with mild bleeding. The redness of the staining greatly diminished with time and the extreme halitosis of the patient also improved sharply leading to a dramatic improvement in quality of life. Ambulatory care is a feasible alternative for periodontal management that within safety limits for bleeding risks reduces the operational cost.

Alpha lipoic acid in burning mouth syndrome - a randomized double-blind placebo-controlled trial

The burning mouth syndrome (BMS) is a chronic condition characterized by oral burning pain in the absence of clinical abnormalities and without established therapy. The purpose of this study was to evaluate the effectiveness of alpha lipoic acid (ALA) in the management of BMS symptoms through a randomized double-blind placebo-controlled trial. Thirty-eight patients (34 women and four men, median age 62.9 years, range 36-78) were included and 31 completed the study. The patients were randomized into two cycles of treatment: one with alpha lipoic acid and one with placebo both administered in identical capsules. These cycles were separated by a washout period of 20 days. The oral symptoms and the treatment response were assessed using a 100-mm visual analog scale before and after each cycle and the global perceived effect score, using a 5-point scale after each treatment cycle. The level of reduction on burning was significant for both treatments (paired t-test: P < 0.05; rp = 0.011; ral < 0.001). Considering the two cycles together, 22 patients reported at least some improvement after ALA use and 23 patients after placebo. Comparison of the oral assessment scores of the two cycles failed to demonstrate the effectiveness of ALA over placebo (t-test: P > 0.05; r = 0.75).

National Institutes Of Health Consensus Development Project On Criteria For Clinical Trials In Chronic Graft-versus-host Disease: I. The 2014 Diagnosis And Staging Working Group Report.

The 2005 National Institutes of Health (NIH) Consensus Conference proposed new criteria for diagnosing and scoring the severity of chronic graft-versus-host disease (GVHD). The 2014 NIH consensus maintains the framework of the prior consensus with further refinement based on new evidence. Revisions have been made to address areas of controversy or confusion, such as the overlap chronic GVHD subcategory and the distinction between active disease and past tissue damage. Diagnostic criteria for involvement of mouth, eyes, genitalia, and lungs have been revised. Categories of chronic GVHD should be defined in ways that indicate prognosis, guide treatment, and define eligibility for clinical trials. Revisions have been made to focus attention on the causes of organ-specific abnormalities. Attribution of organ-specific abnormalities to chronic GVHD has been addressed. This paradigm shift provides greater specificity and more accurately measures the global burden of disease attributed to GVHD, and it will facilitate biomarker association studies.

Anthropogenic influences in a lagoonal environment: a multiproxy approach at the valo grande mouth, Cananéia-Iguape system (SE Brazil)

The Cananéia-Iguape system, SE Brazil, consists of a complex of lagoonal channels, located in a United Nations Educational, Scientific and Cultural Organization (UNESCO) Biosphere Reserve. Nevertheless, important environmental changes have occurred in approximately the last 150 yrs due to the opening of an artificial channel, the Valo Grande, connecting the Ribeira de Iguape River to the lagoonal system. Our objective is to assess the historical record of the uppermost layers of the sedimentary column of the lagoonal system in order to determine the history of environmental changes caused by the opening of the artificial channel. In this sense, an integrated geochemical-faunal approach is used. The environmental changes led significant modifications in salinity, in changes of the depositional patterns of sediments and foraminiferal assemblages (including periods of defaunation), and, more drastically, in the input of heavy metals to the coastal environment. The concentrations Pb in the core analyzed here were up to two times higher than the values measured in contaminated sediments from the Santos estuary, the most industrialized coastal zone in Brazil.

Spatial clustering analysis of the foot-and-mouth disease outbreaks in Mato Grosso do Sul state, Brazil - 2005

In the southern region of Mato Grosso do Sul state, Brazil, a foot-and-mouth disease (FMD) epidemic started in September 2005. A total of 33 outbreaks were detected and 33,741 FMD-susceptible animals were slaughtered and destroyed. There were no reports of FMD cases in other species than bovines. Based on the data of this epidemic, it was carried out an analysis using the K-function and it was observed spatial clustering of outbreaks within a range of 25km. This observation may be related to the dynamics of foot-and-mouth disease spread and to the measures undertaken to control the disease dissemination. The control measures were effective once the disease did not spread to farms more than 47 km apart from the initial outbreaks.

Carbohydrate mouth rinse: does it improve endurance exercise performance?

It is well known that carbohydrate (CHO) supplementation can improve performance in endurance exercises through several mechanisms such as maintenance of glycemia and sparing endogenous glycogen as well as the possibility of a central nervous-system action. Some studies have emerged in recent years in order to test the hypothesis of ergogenic action via central nervous system. Recent studies have demonstrated that CHO mouth rinse can lead to improved performance of cyclists, and this may be associated with the activation of brain areas linked to motivation and reward. These findings have already been replicated in other endurance modalities, such as running. This alternative seems to be an attractive nutritional tool to improve endurance exercise performance.

Striatal volume abnormalities in treatment-naive patients diagnosed with pediatric major depressive disorder

Objective: The striatum, including the putamen and caudate, plays an important role in executive and emotional processing and may be involved in the pathophysiology of mood disorders. Few studies have examined structural abnormalities of the striatum in pediatric major depressive disorder (MDD) patients. We report striatal volume abnormalities in medication-naive pediatric MDD compared to healthy comparison subjects. Method: Twenty seven medication-naive pediatric Diagnostic and Statistical Manual of Mental Disorders, 4(th) edition (DSM-IV) MDD and 26 healthy comparison subjects underwent volumetric magnetic resonance imaging (MRI). The putamen and caudate volumes were traced manually by a blinded rater, and the patient and control groups were compared using analysis of covariance adjusting for age, sex, intelligence quotient, and total brain volumes. Results: MDD patients had significantly smaller right striatum (6.0% smaller) and right caudate volumes (7.4% smaller) compared to the healthy subjects. Left caudate volumes were inversely correlated with severity of depression in MDD subjects. Age was inversely correlated with left and right putamen volumes in MDD patients but not in the healthy subjects. Conclusions: These findings provide fresh evidence for abnormalities in the striatum of medication-naive pediatric MDD patients and suggest the possible involvement of the striatum in the pathophysiology of MDD.

Burning mouth syndrome responsive to pramipexol

Burning mouth syndrome (BMS) is characterized by burning discomfort or pain in otherwise normal oral mucosa. It is usually refractory. Treatment modalities are scarce. Herein we report one case of primary disabling BMS, previously refractory to multiple regimens, with complete and persistent improvement with pramipexol, a nonergot dopamine agonist which has high selectivity for dopaminergic D2 receptors. We discuss potential pathophysiological implications of our findings.

Low-Level Laser Therapy in Burning Mouth Syndrome Patients: A Pilot Study

Objective: The aim of this study was to investigate the effect of low-level laser therapy (LLLT) on the treatment of burning mouth syndrome (BMS). In addition, the laser effect was compared on the different affected oral sites. Materials and Methods: Eleven subjects with a total of 25 sites (tongue, lower lip, upper lip, and palate) affected by a burning sensation were selected. The affected areas were irradiated once a week for three consecutive weeks with an infrared laser (lambda = 790 nm). The probe was kept in contact with the tissue, and the mucosal surface was scanned during the irradiation. The exposure time was calculated based on the fluence of 6 J/cm(2), the output power of 120 mW, and the area to be treated. Burning intensity was recorded through a visual analog scale before and after the treatment and at the 6-week follow-up. The percentage of the improvement in symptoms was also obtained. Results: Burning intensity at the end of the laser therapy was statistically lower than at the beginning (p < 0.01). Patients reported an 80.4% reduction in the intensity of symptoms after laser treatment. There was no statistical difference between the end of the treatment and the 6-week follow-up, except for the tongue site. Conclusion: Under the investigated parameters, infrared LLLT proved to be a valuable alternative for BMS treatment, providing a significant and lasting reduction in symptoms.

Somesthetic, gustatory, olfactory function and salivary flow in patients with neuropathic trigeminal pain

OBJECTIVES: To determine somesthetic, olfactory, gustative and salivary abnormalities in patients with burning mouth syndrome (BMS), idiopathic trigeminal neuralgia (ITN) and trigeminal postherpetic neuralgia (PHN). SUBJECTS AND METHODS: Twenty patients from each group (BMS, ITN, PHN) and 60 healthy controls were evaluated with a systematized quantitative approach of thermal (cold and warm), mechanical, pain, gustation, olfaction and salivary flow; data were analyzed with ANOVA, Tukey, Kruskal Wallis and Dunn tests with a level of significance of 5%. RESULTS: There were no salivary differences among the groups with matched ages; the cold perception was abnormal only at the mandibular branch of PHN (P = 0.001) and warm was abnormal in all trigeminal branches of PHN and BMS; mechanical sensitivity was altered at the mandibular branch of PHN and in all trigeminal branches of BMS. The salty, sweet and olfactory thresholds were higher in all studied groups; the sour threshold was lower and there were no differences of bitter. CONCLUSION: All groups showed abnormal thresholds of gustation and olfaction; somesthetic findings were discrete in ITN and more common in PHN and BMS; central mechanisms of balance of sensorial inputs might be underlying these observations. Oral Diseases (2010) 16, 482-487

Exercise training delays cardiac dysfunction and prevents calcium handling abnormalities in sympathetic hyperactivity-induced heart failure mice

Exercise training (ET) is a coadjuvant therapy in preventive cardiology. It delays cardiac dysfunction and exercise intolerance in heart failure (HF); however, the molecular mechanisms underlying its cardioprotection are poorly understood. We tested the hypothesis that ET would prevent Ca2+ handling abnormalities and ventricular dysfunction in sympathetic hyperactivity-induced HF mice. A cohort of male wildtype (WT) and congenic (alpha 2A/alpha 2C)-adrenoceptor knockout ((alpha 2A/alpha 2C)ARKO) mice with C57BL6/J genetic background (3-5 mo of age) were randomly assigned into untrained and exercise-trained groups. ET consisted of 8-wk swimming session, 60 min, 5 days/wk. Fractional shortening (FS) was assessed by two-dimensional guided M-mode echocardiography. The protein expression of ryanodine receptor (RyR), phospho-Ser(2809)-RyR, sarcoplasmic reticulum Ca2+ ATPase (SERCA2), Na+/Ca2+ exchanger (NCX), phospholamban (PLN), phospho-Ser(16)-PLN, and phospho-Thr(17)-PLN were analyzed by Western blotting. At 3 mo of age, no significant difference in FS and exercise tolerance was observed between WT and (alpha 2A/alpha 2C)ARKO mice. At 5 mo, when cardiac dysfunction is associated with lung edema and increased plasma norepinephrine levels, (alpha 2A/alpha 2C)ARKO mice presented reduced FS paralleled by decreased SERCA2 (26%) and NCX (34%). Conversely, (alpha 2A/alpha 2C)ARKO mice displayed increased phospho-Ser(16)-PLN (76%) and phospho-Ser(2809)-RyR (49%). ET in (alpha 2A/alpha 2C)ARKO mice prevented exercise intolerance, ventricular dysfunction, and decreased plasma norepinephrine. ET significantly increased the expression of SERCA2 (58%) and phospho-Ser(16)-PLN (30%) while it restored the expression of phospho-Ser(2809)-RyR to WT levels. Collectively, we provide evidence that improved net balance of Ca2+ handling proteins paralleled by a decreased sympathetic activity on ET are, at least in part, compensatory mechanisms against deteriorating ventricular function in HF.

The effect of carbohydrate mouth rinse on maximal strength and strength endurance

It has been previously reported that carbohydrate (CHO) mouth rinse can improve exercise performance. The proposed mechanism involves increased activation of brain regions believed to be responsible for reward/motivation and motor control. Since strength-related performance is affected by central drive to the muscles, it seems reasonable to hypothesize that the positive CNS response to oral CHO sensing may counteract the inhibitory input from the muscle afferent pathways minimizing the drop in the central drive. The purpose of the current study was to test if CHO mouth rinse affects maximum strength and strength endurance performance. Twelve recreationally strength-trained healthy males (age 24.08 +/- 2.99 years; height 178.09 +/- 6.70 cm; weight 78.67 +/- 8.17 kg) took part in the study. All of the tests were performed in the morning, after an 8 h overnight fasting. Subjects were submitted to a maximum strength test (1-RM) and a strength endurance test (six sets until failure at 70% of 1-RM), in separate days under three different experimental conditions (CHO mouth rinse, placebo-PLA mouth rinse and control-CON) in a randomized crossover design. The CHO mouth rinse (25 ml) occurred before every attempt in the 1-RM test, and before every set in the endurance strength test. Blood glucose and lactate were measured immediately before and 5 min post-tests. There were no significant differences in 1-RM between experimental conditions (CHO 101 +/- 7.2 kg; PLA 101 +/- 7.4 kg; CON 101 +/- 7.2 kg; p = 0.98). Furthermore, there were no significance between trial differences in the number of repetitions performed in each set (p = 0.99) or the total exercise volume (number of repetitions x load lifted [kg]) (p = 0.98). A main effect for time (p < 0.0001) in blood lactate concentration was observed in both tests (1-RM and strength endurance). Blood glucose concentration did not differ between conditions. In conclusion, CHO mouth rinse does not affect maximum strength or strength endurance performance.

Secondary metabolites produced by Propionicimonas sp (ENT-18) induce histological abnormalities in the sclerotia of Sclerotinia sclerotiorum

Sclerotinia sclerotiorum is a highly aggressive pathogen that causes great economic losses, especially in temperate climates. Several biological control agents are available, but actinobacteria have seldom been used to control this fungus. Our objective was to evaluate the efficiency and ultrastructural effects of the secondary metabolites produced by the ant-associated actinobacterium Propionicimonas sp. ENT-18 in controlling the sclerotia of S. sclerotiorum. We demonstrated total inhibition of sclerotia treated with 62.5 mu g/10 mu l of an ethyl acetate extract of compounds produced by ENT-18, and calculated an LC(50) of 1.69 mu g/sclerotia. Histological and ultrastructural analysis indicated that the cells of the treated sclerotia were severely damaged, suggesting direct action of the biomolecule(s) produced by the actinobacterium ENT-18 on the cell structure of the medullae and rind cell wall. This is the first report demonstrating a novel property of Propionicimonas sp.-antifungal activity against S. sclerotiorum.

Electronegative LDL and lipid abnormalities in patients undergoing hemodialysis and peritoneal dialysis

Background: Oxidative modification of low-density lipoprotein (LDL) has been demonstrated in patients with end-stage renal disease, where it is associated with oxidative stress and plays a key role in the pathogenesis of atherosclerosis. In this context, the generation of minimally oxidized LDL, also called electronegative LDL [ LDL(-)], has been associated with active disease, and is a detectable sign of atherogenic tendencies. The purpose of this study was to evaluate serum LDL(-) levels and anti-LDL(-)IgG autoantibodies in end-stage renal disease patients on dialysis, comparing patients on hemodialysis (HD), peritoneal dialysis (PD) and a control group. In addition, the serum lipid profile, nutritional status, biochemical data and parameters of mineral metabolism were also evaluated. Methods: The serum levels of LDL(-) and anti-LDL(-) IgG autoantibodies were measured in 25 patients undergoing HD and 11 patients undergoing PD at the Centro Integradode Nefrologia, Rio de Janeiro, Brazil. Ten healthy subjects served as a control group. Serum levels of albumin, total cholesterol, triglycerides and lipoproteins were measured. Calculations of subjects` body mass index and measurements of waist circumference, triceps skin fold and arm muscle area were performed. Measurements of hematocrit, serum blood urea nitrogen, creatinine, parathyroid hormone, phosphorus and calcium were taken. Results: Levels of LDL(-) were higher in HD patients (575.6 +/- 233.1 mu g/ml) as compared to PD patients (223.4 +/- 117.5 mu g/ml, p < 0.05), which in turn were higher than in the control group (54.9 +/- 33.3 mu g/ml, p < 0.01). The anti-LDL(-) IgG autoantibodies were increased in controls (0.36 +/- 0.09 mu g/ ml) as compared to PD (0.28 +/- 0.12 mu g/ml, p < 0.001) and HD patients (0.2 +/- 0.1 mu g/ml, p < 0.001). The mean values of total cholesterol and LDL were considered high in the PD group, whereas the mean triceps skin fold was significantly lower in the HD group. Conclusion: Levels of LDL(-) are higher in renal patients on dialysis than in normal individuals, and are reciprocally related to IgG autoantibodies. LDL(-) may be a useful marker of oxidative stress, and this study suggests that HD patients are more susceptible to cardiovascular risk due to this condition. Moreover, autoantibodies reactive to LDL(-) may have protective effects in chronic kidney disease. Copyright (C) 2008 S. Karger AG, Basel.