Incontinentia pigmenti presenting as hypodontia in a 3-year-old girl: a case report

<p>Abstract</p> <p>Introduction</p> <p>Incontinentia pigmenti or Bloch-Sulzberger syndrome is a rare X-linked dominant disease that mainly affects the skin, eyes, hair, central nervous system and teeth. The disease is predominant among women. Although dermatologic manifestations are among the most important aspects for the diagnosis of the syndrome, they are less damaging to the patient and do not require treatment. However, oral involvement characterized by hypodontia of deciduous and permanent teeth is important for the diagnosis and treatment of the patient.</p> <p>Case presentation</p> <p>We report the case of a 3-year-old girl with ophthalmologic and neurologic disturbances, cutaneous manifestations and hypodontia. Since the patient did not present more damaging manifestations such as neurologic and/or ophthalmologic problems, her most severe complications were related to dental anomalies. The importance of integrated dental treatment, which combines pediatric dentistry, orthodontics and conventional prosthesis, is emphasized.</p> <p>Conclusion</p> <p>Hypodontia is a frequent finding in incontinentia pigmenti, and dentists should be aware of this condition in order to help with the diagnosis.</p

Numerical study of circulation on the inner Amazon Shelf

Author Posting. © Springer, 2008. This is the author's version of the work. It is posted here by permission of Springer for personal use, not for redistribution. The definitive version was published in Ocean Dynamics 58 (2008): 187-198, doi:10.1007/s10236-008-0139-4.We studied the circulation on the coastal domain of the Amazon Shelf by applying the hydrodynamic module of the Estuarine and Coastal Ocean Model and Sediment Transport - ECOMSED. The first barotropic experiment aimed to explain the major bathymetric effects on tides and those generated by anisotropy in sediment distribution. We analyzed the continental shelf response of barotropic tides under realistic bottom stress parametrization (Cd), considering sediment granulometry obtained from a faciologic map, where river mud deposits and reworked sediments areas are well distinguished, among others classes of sediments. Very low Cd values were set in the fluid mud regions off the Amapa coast (1.0 10-4 ), in contrast to values around 3:5 10-3 for coarser sediment regions off the Para coast. Three-dimensional experiments represented the Amazon River discharge and trade winds, combined to barotropic tide influences and induced vertical mixing. The quasi-resonant response of the Amazon Shelf to the M2 tide act on the local hydrodynamics by increasing tidal admittance, along with tidal forcing at the shelf break and extensive fluid mud regions. Harmonic analysis of modeled currents agreed well with analysis of the AMASSEDS observational data set. Tidal-induced vertical shear provided strong homogenization of threshold waters, which are subject to a kind of hydraulic control due to the topographic steepness. Ahead of the hydraulic jump, the low-salinity plume is disconnected from the bottom and acquires negative vorticity, turning southeastward. Tides act as a generator mechanism and topography, via hydraulic control, as a maintainer mechanism for the low-salinity frontal zone positioning. Tidally induced southeastward plume fate is overwhelmed by northwestward trade winds so that, along with background circulation, probably play the most important role on the plume fate and variability over the Amazon Shelf

Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1.

In 2008, we published the first set of guidelines for standardizing research in autophagy. Since then, this topic has received increasing attention, and many scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Thus, it is important to formulate on a regular basis updated guidelines for monitoring autophagy in different organisms. Despite numerous reviews, there continues to be confusion regarding acceptable methods to evaluate autophagy, especially in multicellular eukaryotes. Here, we present a set of guidelines for investigators to select and interpret methods to examine autophagy and related processes, and for reviewers to provide realistic and reasonable critiques of reports that are focused on these processes. These guidelines are not meant to be a dogmatic set of rules, because the appropriateness of any assay largely depends on the question being asked and the system being used. Moreover, no individual assay is perfect for every situation, calling for the use of multiple techniques to properly monitor autophagy in each experimental setting. Finally, several core components of the autophagy machinery have been implicated in distinct autophagic processes (canonical and noncanonical autophagy), implying that genetic approaches to block autophagy should rely on targeting two or more autophagy-related genes that ideally participate in distinct steps of the pathway. Along similar lines, because multiple proteins involved in autophagy also regulate other cellular pathways including apoptosis, not all of them can be used as a specific marker for bona fide autophagic responses. Here, we critically discuss current methods of assessing autophagy and the information they can, or cannot, provide. Our ultimate goal is to encourage intellectual and technical innovation in the field

Atuação de enfermeiras nas ações de controle do câncer de mama em oito unidades de saúde do Ceará, Brasil Nurses’ performance in breast cancer control actions in eight healthcare units located in Ceará, Brazil

OBJETIVOS: analisar a atuação de enfermeiras nas ações de controle do câncer de mama, em Unidades Primárias de Saúde do Ceará, Brasil. MÉTODOS: trata-se de um estudo de caráter descritivo, em que se utilizou a observação participante, como técnica na coleta dos dados. Na observação foram seguidos os passos do Instrumento de Reconhecimento da Qualidade (IRQ) do Projeto de Qualidade (PROQUALI), que apresenta os critérios mínimos de qualidade para a atuação da enfermeira no controle do câncer de mama. A amostra foi constituída por 13 enfermeiras, de seis municípios, de uma microrregião de saúde do Ceará, Baturité. RESULTADOS: 23% das enfermeiras tiveram o cuidado de lavar as mão antes de realizar o exame clínico das mamas (ECM), enquanto 38% só o fizeram após o procedimento; 31% explicaram o ECM às usuárias; 62% cobriram a mulher adequadamente; 54% realizaram a inspeção; 100% realizaram a palpação e 69% realizaram a expressão; somente 30,8% das enfermeiras orientaram as mulheres quanto ao auto-exame das mamas. CONCLUSÕES: o estudo evidenciou a necessidade de capacitação das enfermeiras para oferecerem as ações de controle do câncer de mama com um melhor padrão de qualidade.<br>OBJECTIVES: to analyze nurses'performance in breast cancer control in Primary Healthcare Units located in Ceará, Brazil. METHODS: it is a descriptive study where participant observation was used as a data collection technique. Observation consisted of following the steps of the Quality Assurance Recognition Tool from the Quality Project (PROQUALI) comprising minimal quality criteria for nurses'performance in breast cancer control. The sample encompassed 13 nurses of six cities of a Healthcare micro-region of Ceará, Baturité. RESULTS: 23% of the nurses were careful enough to wash their hands before performing clinical examination of the breast (BCE), while 38% only washed their hands upon completing it; 31% explained what clinical breast examination was all about (BCE), 62% covered the women adequately; 54% accomplished the inspection; 100% performed node seeking palpation and 69% realized expression and only 30,8% of the nurses oriented the women to perform breast self-examination. CONCLUSIONS: the study indicated the need for training the nurses providing breast cancer control according to approved quality standards

Platelet Counts, Acute Kidney Injury, and Mortality after Coronary Artery Bypass Grafting Surgery.

BACKGROUND: Cardiac surgery requiring cardiopulmonary bypass is associated with platelet activation. Because platelets are increasingly recognized as important effectors of ischemia and end-organ inflammatory injury, the authors explored whether postoperative nadir platelet counts are associated with acute kidney injury (AKI) and mortality after coronary artery bypass grafting (CABG) surgery. METHODS: The authors evaluated 4,217 adult patients who underwent CABG surgery. Postoperative nadir platelet counts were defined as the lowest in-hospital values and were used as a continuous predictor of postoperative AKI and mortality. Nadir values in the lowest 10th percentile were also used as a categorical predictor. Multivariable logistic regression and Cox proportional hazard models examined the association between postoperative platelet counts, postoperative AKI, and mortality. RESULTS: The median postoperative nadir platelet count was 121 × 10/l. The incidence of postoperative AKI was 54%, including 9.5% (215 patients) and 3.4% (76 patients) who experienced stages II and III AKI, respectively. For every 30 × 10/l decrease in platelet counts, the risk for postoperative AKI increased by 14% (adjusted odds ratio, 1.14; 95% CI, 1.09 to 1.20; P < 0.0001). Patients with platelet counts in the lowest 10th percentile were three times more likely to progress to a higher severity of postoperative AKI (adjusted proportional odds ratio, 3.04; 95% CI, 2.26 to 4.07; P < 0.0001) and had associated increased risk for mortality immediately after surgery (adjusted hazard ratio, 5.46; 95% CI, 3.79 to 7.89; P < 0.0001). CONCLUSION: The authors found a significant association between postoperative nadir platelet counts and AKI and short-term mortality after CABG surgery