Abstract Vitiligo is an autoimmune disease of the skin that results in localized or disseminated white macules. One common feature of several existing classification protocols is the distribution of the disease into two main subtypes, non-segmental vitiligo (NSV) and segmental vitiligo (SV). SV is characterized by depigmentation spreading within one or more skin segments while NSV is widespread. Several clinical-epidemiological observations suggest that SV has distinct autoimmune pathophysiology compared to NSV. Furthermore, the clinical distribution pattern of SV lesions closely resembles other melanocyte mosaicism diseases. These observations led us to hypothesize that SV is caused by a localized autoimmune reaction targeting epidermal mosaicism melanocytes. Here, we proposed examples of experimental approaches to assess mosaicism in SV patients.

OBJECTIVES: To assess the craniofacial skeletal growth in pediatric hematopoietic stem-cell transplantation (HSCT) survivors in comparison with age-sex matched-paired controls. METHODS: A case-controlled retrospective comparison of the craniofacial growth in 25 HSCT children and 25 matched-paired controls was conducted. Craniofacial growth was quantitatively assessed by linear and angular measurements in panoramic radiographic images using ImageJ¯. Stature growth and body weight were obtained through physical examination. Cancer diagnosis, myeloablative conditioning, and HSCT were retrieved from medical records. RESULTS: Patients aged 12.2 years (±3.8; 16 male, 9 female). Radiographic images were obtained on an average of 2.43 (±2.0) years after HSCT. The main malignant diagnosis was acute lymphoblastic leukemia (56%), followed by acute myeloid leukemia (36%) and myelodysplastic syndromes (8%). Total body irradiation was associated with chemotherapy at 80%. Mean age at transplantation was 10 (±4.7) years. HSCT survivors showed reduced a vertical growth of the mandibular ramus (p=0.003). This persisted among individuals below 12 years of age (p=0.017). The HSCT group showed delayed dental eruption, though there was no statistically significant difference (p=0.3668). The HSCT group showed stature deficit, increased weight, and body mass index (Z-score stature: -0.28; Z-score weight: 0.38, respectively). CONCLUSIONS: Pediatric HSCT has decreased vertical craniofacial growth compared to their matched controls. There might be an association between reduced craniofacial vertical growth and reduced estature growth. Further studies to quantitatively investigate the impact of different myeloablative regimens in craniofacial skeletal growth and development.

OBJECTIVE: Hematopoietic stem-cell transplantation (HSCT) childhood survivors of hematologic malignancies are prone to develop late osteopenia and osteoporosis. The purpose of this retrospective study was to quantitatively and qualitatively assess bone mineral density (BMD) in HSCT childhood survivors and to compare the effectiveness of both qualitative and quantitative assessment methods. METHODS: DESIGN BMD assessment using panoramic radiographs of childhood HSCT survivors aged 3.69-18.88 years using two radiomorphometric indexes. Case-control double-blinded comparison of panoramic radiographic images from childhood HSCT survivors and age- and sex-matched healthy controls. Quantitative assessment was performed by measuring the cortical bone width bilaterally at the mental foramen level. Qualitative assessment was performed using the mandibular cortical index bilaterally on all panoramic images. RESULTS: Radiographs were taken 6.59-83.95 months after bone marrow transplantation [median±SD=25.92±24.9 months]. Fifty-two panoramic radiographic images were analyzed: 21 from HSCT survivors and 31 from healthy controls aged 3.69-25.1 years [mean±SD=11.89±5.28 years]. The mandibular cortical bone width was 17% smaller in childhood HSCT survivors than in healthy controls (case group: 2.420, control group: 3.307; p=0.00617). Qualitative analysis revealed an increased frequency of severe mandibular cortical erosion in childhood HSCT survivors, although no significant difference was observed (case group: 1.540, control group: 1.490; p=0.32). The interobserver agreement was 85% (Kappa index). CONCLUSIONS: HSCT childhood survivors exhibit quantitative and qualitative mandibular bone impairments. Further studies are needed to establish an association between mandibular cortical bone impairment and osteoporosis.

Resumo Objetivo: Avaliar o uso da tocólise em partos prematuros decorrentes de trabalho de parto espontâneo numa amostra brasileira. Métodos Um total de 1.491 mulheres com parto prematuro decorrente de trabalho de parto espontâneo foram avaliadas, considerando a realização de tocólise ou conduta expectante, de acordo com a idade gestacional ao nascimento (< 34 semanas e 34 a 36 þ 6 semanas) e com as drogas prescritas. O estudo ocorreu em 20 hospitais brasileiros, de abril de 2011a julho de 2012. Análises bivariadas foram realizadas para avaliar associações com características sociodemográficas e obstétricas. Foram calculadas as relações de probabilidade comseus respectivos intervalos de confiança (95%) para os desfechos neonatais e maternos. Resultados Um total de 1.491 casos de partos prematuros foram considerados, e a tocólise foi realizada em 342 (23%) casos, dos quais 233 (68,1%) tiveram partos antes das 34 semanas. No grupo da conduta expectante, 73% forampré-termos tardios e com trabalho de parto mais avançado à admissão. As drogas mais utilizadas foram os bloqueadores do canal de cálcio (62.3%), seguidos pelos betamiméticos (33%). No grupo da tocólise houvemais complicações neonatais ematernas (maioria não grave) e um uso de corticosteroides 29 vezes mais frequente que nos casos de conduta expectante. Conclusão A tocólise foi mais favorável nos casos de trabalho de parto inicial e nos partos realizados antes de 34 semanas de gestação, usando preferencialmente bloqueadores do canal de cálcio, comsucesso em realizar altas taxas de corticoterapia. A tocólise esteve associada a maiores taxas de complicações maternas e neonatais, o que pode ser explicado pela diferença basal dos casos à admissão. Entretanto, esses resultados devem acender um alerta em relação ao uso de tocolíticos.

Abstract Objective To evaluate the use of tocolysis in cases of preterm birth due to spontaneous preterm labor in a Brazilian sample. Methods A sample of 1,491 women with preterm birth due to spontaneous preterm labor were assessed, considering treatment with tocolysis or expectant management, according to gestational age at birth (< 34 weeks and 34 to 36 þ 6 weeks) and drugs prescribed. The study took place in 20 Brazilian hospitals from April 2011 to July 2012. Bivariate analyses were conducted to evaluate associations with sociodemographic and obstetric characteristics and odds ratios with their respective 95% confidence intervals were estimated for maternal and neonatal outcomes. Results A total of 1,491 cases of preterm birth were considered. Tocolysis was performed in 342 cases (23%), 233 of which (68.1%) were delivered before 34 weeks. Within the expectant management group, 73% was late preterm and with more advanced labor at the time of admission. The most used drugs were calcium channel blockers (62.3%), followed by betamimetics (33%). Among the subjects in the tocolysis group, there were more neonatal and maternal complications (majority non-severe) and an occurrence of corticosteroid use that was 29 higher than in the expectant management group. Conclusion Tocolysis is favored in cases of earlier labor and also among thosewith less than 34 weeks of gestation, using preferably calcium channel blockers, with success in achieving increased corticosteroid use. Tocolysis, in general, was related to higher maternal and neonatal complication rates, which may be due to the baseline difference between cases at admission. However, these results should raise awareness to tocolysis use.

The aim of this study was to provide an updated review of dental procedures undertaken at the dental unit of the Onco-hematology service of the Instituto da Criança at the Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (ICr/HC-FMUSP). We retrospectively reviewed 565 of 1902 medical and dental records of patients diagnosed with onco-hematological diseases who were seen in a 3-year study (January 2015 to December 2017). We assessed data regarding population characteristics, onco-hematological diagnosis and dental procedures performed. Of the selected medical records, preventive dentistry was the most common procedure undertaken in this population, followed by oral maxillofacial surgeries, restorative dentistry and oral mucositis treatment. The most prevalent malignant diagnosis was acute lymphocytic leukemia, and the most prevalent nonmalignant diagnosis was sickle-cell anemia. Preventive dental procedures represent most of the dental procedures undertaken in hospitalized onco-hematological pediatric patients.

Resumo Fundamento: A prevalência e os desfechos clínicos em pacientes com insuficiência cardíaca com fração de ejeção do ventrículo esquerdo preservada pós-infarto agudo do miocárdio ainda não foram bem elucidados. Objetivo: Analisar a prevalência de insuficiência cardíaca com fração de ejeção do ventrículo esquerdo preservada no infarto agudo do miocárdio e sua associação com a mortalidade. Métodos: Pacientes com infarto agudo do miocárdio (n = 1.474) foram incluídos prospectivamente. Pacientes admitidos sem insuficiência cardíaca (Killip = 1), com insuficiência cardíaca com fração de ejeção do ventrículo esquerdo preservada (Killip > 1 e fração de ejeção do ventrículo esquerdo ≥ 50%) e com insuficiência cardíaca sistólica (Killip > 1 e fração de ejeção do ventrículo esquerdo < 50%) foram comparados. A associação entre insuficiência cardíaca sistólica e com fração de ejeção do ventrículo esquerdo preservada, com a mortalidade hospitalar foi testada em modelos ajustados. Resultados: Dentre os incluídos, 1.256 (85,2%) pacientes foram admitidos sem insuficiência cardíaca (72% homens, 67 ± 15 anos), 78 (5,3%) com insuficiência cardíaca com fração de ejeção do ventrículo esquerdo preservada (59% homens, 76 ± 14 anos) e 140 (9,5%) com insuficiência cardíaca sistólica (69% homens, 76 ± 14 anos), com mortalidade, respectivamente, de 4,3; 17,9 e 27,1% (p < 0,001). A regressão logística (ajustada para sexo, idade, troponina, diabetes e índice de massa corporal) demonstrou que insuficiência cardíaca com fração de ejeção do ventrículo esquerdo preservada (odds ratio de 2,91; intervalo de confiança de 95% de 1,35-6,27; p = 0,006) e insuficiência cardíaca sistólica (odds ratio de 5,38; intervalo de confiança de 95% de 3,10-9,32; p < 0,001) se associaram à mortalidade intra-hospitalar. Conclusão: Um terço dos pacientes com infarto agudo do miocárdio admitidos com insuficiência cardíaca apresentou fração de ejeção do ventrículo esquerdo preservada. Apesar de esse subgrupo ter evolução mais favorável que os pacientes com insuficiência cardíaca sistólica, ele apresentou risco de morte três vezes maior do que o grupo sem insuficiência cardíaca. Pacientes com infarto agudo do miocárdio e insuficiência cardíaca com fração de ejeção do ventrículo esquerdo preservada apresentaram elevado risco em curto prazo e mereceram especial atenção e monitorização durante a internação hospitalar.

Abstract Background: The prevalence and clinical outcomes of heart failure with preserved left ventricular ejection fraction after acute myocardial infarction have not been well elucidated. Objective: To analyze the prevalence of heart failure with preserved left ventricular ejection fraction in acute myocardial infarction and its association with mortality. Methods: Patients with acute myocardial infarction (n = 1,474) were prospectively included. Patients without heart failure (Killip score = 1), with heart failure with preserved left ventricular ejection fraction (Killip score > 1 and left ventricle ejection fraction ≥ 50%), and with systolic dysfunction (Killip score > 1 and left ventricle ejection fraction < 50%) on admission were compared. The association between systolic dysfunction with preserved left ventricular ejection fraction and in-hospital mortality was tested in adjusted models. Results: Among the patients included, 1,256 (85.2%) were admitted without heart failure (72% men, 67 ± 15 years), 78 (5.3%) with heart failure with preserved left ventricular ejection fraction (59% men, 76 ± 14 years), and 140 (9.5%) with systolic dysfunction (69% men, 76 ± 14 years), with mortality rates of 4.3%, 17.9%, and 27.1%, respectively (p < 0.001). Logistic regression (adjusted for sex, age, troponin, diabetes, and body mass index) demonstrated that heart failure with preserved left ventricular ejection fraction (OR 2.91; 95% CI 1.35–6.27; p = 0.006) and systolic dysfunction (OR 5.38; 95% CI 3.10 to 9.32; p < 0.001) were associated with in-hospital mortality. Conclusion: One-third of patients with acute myocardial infarction admitted with heart failure had preserved left ventricular ejection fraction. Although this subgroup exhibited more favorable outcomes than those with systolic dysfunction, this condition presented a three-fold higher risk of death than the group without heart failure. Patients with acute myocardial infarction and heart failure with preserved left ventricular ejection fraction encounter elevated short-term risk and require special attention and monitoring during hospitalization.

Cancer is the third most frequent cause of death in children in Brazil. Early diagnosis and medical advances have significantly improved treatment outcomes, which has resulted in higher survival rates and the management of late side effects has become increasingly important in caring for these patients. Dental abnormalities are commonly observed as late effects of antineoplastic therapy in the oral cavity. The incidence and severity of the dental abnormalities depend on the child's age at diagnosis and the type of chemotherapeutic agent used, as well as the irradiation dose and area. The treatment duration and aggressivity should also be considered. Disturbances in dental development are characterized by changes in shape, number and root development. Enamel anomalies, such as discoloration, opacities and hypoplasia are also observed in these patients. When severe, these abnormalities can cause functional and esthetic sequelae that have an impact on the children's and adolescents' quality of life. General dentists and pediatric dentists should understand these dental abnormalities and how to identify them aiming for early diagnosis and appropriate treatment.

Type-1 (T1R) and Type-2 (T2R) leprosy reactions (LR), which affect up to 50% of leprosy patients, are aggressive inflammatory episodes of sudden onset and highly variable incidence across populations. LR are often diagnosed concurrently with leprosy, but more frequently occur several months after treatment onset. It is not uncommon for leprosy patients to develop recurring reactional episodes; however, they rarely undergo both types of LR. Today, LR are the main cause of permanent disabilities associated with leprosy and represent a major challenge in the clinical management of leprosy patients. Although progress has been made in understanding the immunopathology of LR, the factors that cause a leprosy patient to suffer from LR are largely unknown. Given the impact that ethnic background has on the risk of developing LR, host genetic factors have long been suspected of contributing to LR. Indeed, polymorphisms in seven genes [Toll-like receptors (TLR)1, TLR2, nucleotide-binding oligomerisation domain containing 2, vitamin D receptor, natural resistance-associated macrophage protein 1, C4B and interleukin-6] have been found to be associated with one or more LR outcomes. The identification of host genetic markers with predictive value for LR would have a major impact on nerve damage control in leprosy. In this review, we present the recent advances achieved through genetic studies of LR.

Acid erosion is a superficial loss of enamel caused by chemical processes that do not involve bacteria. Intrinsic and extrinsic factors, such as the presence of acid substances in the oral cavity, may cause a pH reduction, thus potentially increasing acid erosion. The aim of this study was to evaluate the microhardness of bleached and unbleached bovine enamel after immersion in a soda beverage, artificial powder juice and hydrochloric acid. The results obtained for the variables of exposure time, acid solution and substrate condition (bleached or unbleached enamel) were statistically analyzed by the ANOVA and Tukey tests. It was concluded that a decrease in microhardness renders dental structures more susceptible to erosion and mineral loss, and that teeth left unbleached show higher values of microhardness compared to bleached teeth.

Stresses such as cold and drought can impair plant yield and induce a highly complex array of responses. Sugarcane (Saccharum spp.) is cultivated in tropical and subtropical areas and is considered a cold-sensitive plant. We previously showed that cold stress induces the expression of several genes in in vitro sugarcane plantlets. Here we characterize one of those genes, SsNAC23, a member of the NAC family of plant-specific transcription factors, which are induced by low temperature and other stresses in several plant species. The expression of SsNAC23 was induced in sugarcane plants exposed to low temperatures (4ºC). With the aim of further understanding the regulatory network in response to stress, we used the yeast two-hybrid system to identify sugarcane proteins that interact with SsNAC23. Using SsNAC23 as bait, we screened a cDNA expression library of sugarcane plants submitted to 4ºC for 48 h. Several interacting partners were identified, including stress-related proteins, increasing our knowledge on how sugarcane plants respond to cold stress. One of these interacting partners, a thioredoxin h1, offers insights into the regulation of SsNAC23 activity.

INTRODUÇÃO: Durante a era pré-microbiológica, era comum a visão de que doenças, hoje sabidamente infecciosas, eram hereditárias. Com a descoberta dos microorganismos e seu papel na patogênese de diversas patologias, chegou-se a propor que a exposição ao patógeno era condição suficiente para explicar infecção. Hoje, está claro que infecção é o resultado de uma complexa interação entre patógeno e hospedeiro, dependendo portanto, em última análise, do make-up genético de ambos os organismos. A dermatologia oferece diversos exemplos de doenças infecciosas em diferentes graus de entendimento de suas bases moleculares. Nesta revisão, resumimos os principais avanços na direção da dissecção do componente genético controlando suscetibilidade do ser humano a doenças infecciosas de importância na dermatologia. Doenças amplamente estudadas, como a hanseníase e a leishmaniose, são discutidas sob o ponto de vista da genética tanto do hospedeiro quanto do patógeno. Outras, como micobacterioses raras, micoses e sífilis, são apresentadas como boas oportunidades para pesquisa na área de genética de infecção.

BACKGROUND: In the pre-microbiological era, it was widely accepted that diseases, today known to be infectious, were hereditary. With the discovery of microorganisms and their role in the pathogenesis of several diseases, it was suggested that exposure to the pathogen was enough to explain infection. Nowadays, it is clear that infection is the result of a complex interplay between pathogen and host, therefore dependant on the genetic make-up of the two organisms. Dermatology offers several examples of infectious diseases in different stages of understanding of their molecular basis. In this review, we summarize the main advances towards dissecting the genetic component controlling human susceptibility to infectious diseases of interest in dermatology. Widely investigated diseases such as leprosy and leishmaniasis are discussed from the genetic perspective of both host and pathogen. Others, such as rare mycobacterioses, fungal infections and syphilis, are presented as good opportunities for research in the field of genetics of infection.

OBJECTIVES: The objective of this paper was to characterize the population seen at the dentistry unit of the hematology-oncology service of the Oncology-Hematology Service, Instituto da Criança at the Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo. Oral problems resulting from cancer therapy increase the risk of infection, length of hospital stay, treatment cost and negative impact on the course and prognosis of the disease. METHOD: Of the 367 medical records of cancer patients seen from November 2007 until December 2008: 186 with a cancer diagnosis and complete clinical data were selected, while 20 with a cancer diagnosis and incomplete records were excluded; 161 medical records with only hematological diagnosis were also excluded. The following characteristics were assessed: ethnicity, gender, age, diagnosis and characteristics of the neoplasm, cancer therapy status and performed dental procedures. RESULTS: Review of 1,236 visits indicated that 54% (n=100) of the patients had blood cancers, 46% (n=86) had solid tumors and 63% were undergoing anticancer therapy. The proportion of males (52.7%) in the study population was slightly greater. The most common cancer was acute lymphocytic leukemia (32.2%). Cancer occurred more often among those patients aged 5 to 9 years. The most common dental procedures were restorative treatment, preventive treatment and removal of infectious foci. CONCLUSION: The characteristics of the studied population were similar to those of the general Brazilian and global populations, especially regarding gender and diagnosis distributions. The aim of implementation of the dentistry unit was to maintain good oral health and patients' quality of life, which is critical to provide oral care and prevent future oral problems.

O objetivo deste estudo foi avaliar in vitro três sistemas adesivos: um monocomponente com condicionamento ácido total (G1 Prime & Bond 2.1), um "primer" autocondicionante (G2 Clearfil SE Bond) e um adesivo autocondicionante (G3 One Up Bond F), através de resistência ao cisalhamento ao esmalte de dentes humanos, avaliando o tipo de fratura por estereomicroscopia, seguindo as normas ISO para testes adesivos. Trinta pré-molares hígidos foram seccionados ao meio em sentido mésio-distal, incluídos em resina acrílica, polidos até lixa d'água de granulação 600 e aleatoriamente divididos em três grupos (n = 20). Cilindros de resina composta foram adicionados às superfícies de teste. Os espécimes foram armazenados em água destilada (37°C/24 h), termociclados por 500 ciclos (5°C-55°C) e submetidos ao teste de cisalhamento com velocidade de 0,5 mm/min, sendo o tipo de fratura analisado sob estereomicroscopia e os dados submetidos à análise estatística Anova, Tukey e Qui-quadrado (5%). As médias de resistência adesiva foram: G1: 18,13 ± 6,49 MPa, (55% de fraturas coesivas em resina); G2: 17,12 ± 5,80 MPa (90% de fraturas adesivas) e G3 10,47 ± 3,14 MPa (85% de fraturas adesivas). Em termos de resistência adesiva, não houve diferenças significantes entre G1 e G2, tendo G3 apresentado diferença significante em relação aos demais grupos. G1 apresentou tipo de fratura diferente de G2 e G3. Em conclusão, apesar de os sistemas adesivos com condicionamento ácido total e "primer" autocondicionante terem apresentado valores de resistência adesiva similares, o tipo de fratura apresentado por eles foi diferente, o que pode ter implicações clínicas.

The purpose of this study was to evaluate in vitro three adhesive systems: a total etching single-component system (G1 Prime & Bond 2.1), a self-etching primer (G2 Clearfil SE Bond), and a self-etching adhesive (G3 One Up Bond F), through shear bond strength to enamel of human teeth, evaluating the type of fracture through stereomicroscopy, following the ISO guidance on adhesive testing. Thirty sound premolars were bisected mesiodistally and the buccal and lingual surfaces were embedded in acrylic resin, polished up to 600-grit sandpapers, and randomly assigned to three experimental groups (n = 20). Composite resin cylinders were added to the tested surfaces. The specimens were kept in distilled water (37°C/24 h), thermocycled for 500 cycles (5°C-55°C) and submitted to shear testing at a crosshead speed of 0.5 mm/min. The type of fracture was analyzed under stereomicroscopy and the data were submitted to Anova, Tukey and Chi-squared (5%) statistical analyses. The mean adhesive strengths were G1: 18.13 ± 6.49 MPa, (55% of resin cohesive fractures); G2: 17.12 ± 5.80 MPa (90% of adhesive fractures); and G3: 10.47 ± 3.14 MPa (85% of adhesive fractures). In terms of bond strength, there were no significant differences between G1 and G2, and G3 was significantly different from the other groups. G1 presented a different type of fracture from that of G2 and G3. In conclusion, although the total etching and self-etching systems presented similar shear bond strength values, the types of fracture presented by them were different, which can have clinical implications.

Os paragangliomas do corpo carotídeo são lesões incomuns, embora representem a maioria dos paragangliomas da cabeça e pescoço. A presença de lesão bilateral é rara. Os autores apresentam um caso de paraganglioma bilateral, dando ênfase aos aspectos clínicos, diagnósticos e tratamento.

Carotid body paragangliomas are uncommon lesions although in the head and neck region, these lesions are the most frequent ones. Bilateral lesions are rare. The authors present a case of bilateral paraganglioma, its clinical aspects, diagnosis and treatment.