Co-occurrence of oral pemphigus vulgaris and herpes simplex virus infection in a young patient with Crohn's disease: report of a rare case of oral lesions during anti-TFN alpha and immunomodulator therapy.

BACKGROUND: Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune disease that affects desmoglein-1 and desmoglein-3, leading to intraepithelial vesiculobullous lesions. In the oral mucosa, PV lesions can mimic other diseases such as mucous membrane pemphigoid, other forms of pemphigus, recurrent aphthous stomatitis, erythema multiforme, Stevens-Johnson syndrome, and virus-induced ulcers like herpes simplex virus (HSV), making diagnosis challenging. The co-occurrence of PV with Crohn's disease is rare and predominantly seen in younger patients. The therapeutic mainstay for both PV and Crohn's disease usually involves systemic corticosteroids combined with immunosuppressants and immunobiological drugs. Literature indicates that the use of these drugs, particularly TNF-alpha inhibitors, for managing autoimmune diseases like Crohn's can potentially induce other autoimmune diseases known as autoimmune-like syndromes, which include episodes of lupus-like syndrome and inflammatory neuropathies. There are few cases in the literature reporting the development of PV in individuals with CD undergoing infliximab therapy. CASE REPORT: A young female with severe Crohn's disease, treated with the TNF-alpha inhibitor infliximab, developed friable pseudomembranous oral ulcerations. Histopathological and immunofluorescence analyses confirmed these as PV. The treatment included clobetasol propionate and low-level photobiomodulation, which resulted in partial improvement. The patient later experienced severe intestinal bleeding, requiring intravenous hydrocortisone therapy, which improved both her systemic condition and oral lesions. Weeks later, new ulcerations caused by herpes virus and candidiasis were identified, leading to treatment with oral acyclovir, a 21-day regimen of oral nystatin rinse, and photodynamic therapy, ultimately healing the oral infections. To manage her condition, the gastroenterologists included methotrexate (25 mg) in her regimen to reduce the immunogenicity of infliximab and minimize corticosteroid use, as the patient was in remission for Crohn's disease, and the oral PV lesions were under control. CONCLUSION: Young patients with Crohn's disease should be referred to an oral medicine specialist for comorbidity investigation, as oral PV and opportunistic infections can arise during immunosuppressive therapy. The use of TNF-alpha inhibitors in patients treated for inflammatory bowel disease, such as Crohn's, should be carefully evaluated for potential side effects, including oral PV.

Presence of Merkel cell polyomavirus DNA and large-T antigen in keratinocyte carcinomas and its correlation with immunohistochemical markers p16, p53 and ki67.

BACKGROUND: Merkel cell polyomavirus (MCPyV), a human polyomavirus that is unequivocally linked to merkel cell carcinoma (MCC), has been found in association with keratinocytes carcinomas (KC), especially basal cell carcinoma (BCC) and cutaneous squamous cell carcinoma (cSCC). Nevertheless, there is scarce information about the possible involvement of MCPyV in the development of KC. OBJECTIVES: To assess the presence of MCPyV DNA and Large-T Antigen (LT-Ag) via Polymerase Chain Reaction (PCR) and Immunohistochemistry (IHC) in cases of KC, and to correlate its presence with immunohistochemical markers p16, p53, and ki67, tumor type and subtype, sun-exposed location, and epidemiological data. METHODS: The prevalence of MCPyV DNA, LT-Ag, and immunohistochemical markers p16, p53, and ki67 was assessed by PCR and Immunohistochemistry (IHC) in 127 cases of KC, these results were correlated with tumor type and subtype, sun-exposed location, and epidemiological data. RESULTS: The MCPyV DNA was detected in 42.57% (43 of 101) cases by PCR, the LT-Ag was detected in 16.4% (20 of 122) of cases, p16 in 81.5% (97 of 119), p53 in 66.4% (83 of 125), ki67 in 89% (73 of 82). No correlation between MCPyV LT-Ag and DNA confronted with tumor type, subtype, location site, and immunohistochemical markers was found. A single correlation between the MCPyV LT-Ag and cSCC tumors and peri-tumoral lymphocyte cells was noted. STUDY LIMITATIONS: Further steps need to be taken to better evaluate the MCPyV influence and its possible role in KC carcinogenesis, as the evaluation of the virus genome state, the gene sequence that encodes LT-Ag in the KC tumor cells, and in situ hybridization for viral DNA or RNA in these cells. CONCLUSIONS: Despite the frequent detection of MCPyV in KC, the data available so far does not support the hypothesis of a causal relationship between them.

Motility, oxidative status and morphology of frozen-thawed bovine semen are not impacted by fatty acid exposure in vitro.

While sperm migrate within the reproductive tract of cows experiencing negative energy balance (NEB), they come into contact with elevated concentrations of non-esterified fatty acids (NEFA). For this reason, this study aimed to investigate the effects of three different NEFA - palmitic acid (PA), stearic acid (SA), and oleic acid (OA) - on bovine sperm motility, kinetic parameters, oxidative status, and morphology. Frozen thawed semen samples from Bos taurus bulls were incubated with varying concentrations of each fatty acid, and the sperm's characteristics were analysed at different time points. Computer-Assisted Sperm Analysis (CASA) was employed to assess sperm motility and kinetic parameters. Concurrently, the production of the reactive oxygen species (ROS) and total antioxidant capacity were measured to determine the oxidative status. Additionally, sperm morphology was evaluated. In Experiment 1, different concentrations of PA did not show significant effects on total motility, progressive motility, or any kinetic parameters analysed. Similarly, PA did not have a significant impact on the oxidative status or sperm morphology. In Experiment 2, SA at various concentrations did not lead to significant changes in total motility, progressive motility, or any kinetic parameters evaluated. Furthermore, SA did not affect oxidative status or sperm morphology. In Experiment 3, the concentrations of OA used did not result in significant changes in total motility, progressive motility, or any kinetic parameters studied. Likewise, OA did not induce any alterations in oxidative status or sperm morphology. Overall, the results from all three experiments indicate that PA, SA and OA, at the in vitro conditions and tested concentrations, do not exert detrimental effects on bovine sperm function and morphology. These results provide insights that contribute to our understanding of how fatty acids can impact the reduction of fertility rates in cows facing NEB. This, in turn, lays the foundation for additional critical investigations in this area. Further studies are necessary to validate these findings in vivo.

Challenging of treating patients with exfolliative cheilittis: Report of two cases.

Cheilitis is a term given to the inflammation that occurs in the vermillion of the lips. The exfoliative type is an uncommon form of cheilitis, which is characterized by inflammation and desquamation of the lip. It can cause aesthetic problems and compromise daily eating and phonation. The aim of this paper is to describe two cases of exfoliative cheilitis in young persons under periods of emotional stress and parafunctional habits. A 22-year-old white male and an 18-year-old black female presenting edema, intense dryness, and slight desquamation on the vermilion of the lips. In the second case, fissures with bleeding were also observed. Oral lesions were associated with intense emotional stress. The diagnosis of both was made based on the clinical presentation and the exclusion of other conditions. Although the patients have presented a significant improvement after the corticosteroid treatment, they still have a recurrence in stressful episodes. Detailed clinical examination and complementary exams are fundamental for determining associated factors and correctly diagnosing exfoliative cheilitis. Treatment can be challenging, especially in the face of relapses. Key words:Cheilitis, exfoliative cheilitis, oral lesions, stress psychological.

Evaluation of changes in antimicrobial susceptibility in bacteria infecting children and their mothers in pediatric, neonatal-intensive care unit, and gynecology/obstetrics wards of a quaternary referral hospital during the COVID-19 pandemic.

The World Health Organization released a statement warning of increased risk for the incidence of multidrug resistant microorganisms and the absence of new drugs to control such infections soon. Since the beginning of the COVID-19 pandemic, the prescription of antimicrobial agents has increased and may have accelerated the emergence of multidrug resistant (MDR) bacteria. This study aimed to evaluate maternal and pediatric infections within a hospital from January 2019 to December 2021. An observational retrospective cohort study was performed at a quaternary referral hospital in a metropolitan area of Niteroi city, Rio de Janeiro state, Brazil. A total of 196 patients' medical records were analyzed. The data from 90 (45.9%) patients were collected before the SARS-CoV-2 pandemic, 29 (14.8%) from the 2020 pandemic period, and 77 (39.3%) from the 2021 pandemic period. A total of 256 microorganisms were identified during this period. Out of those, 101 (39.5%) were isolated in 2019, 51 (19.9%) in 2020, and 104 (40.6%) in 2021. Antimicrobial susceptibility tests were performed on 196 (76.6%) clinical isolates. The exact binomial test showed that the distribution of Gram-negative bacteria was predominant. The most common microorganism was Escherichia coli (23%; n = 45), followed by Staphylococcus aureus (17.9%, n = 35), Klebsiella pneumoniae (12.8%, n = 25), Enterococcus faecalis (7.7%, n = 15), Staphylococcus epidermidis (6.6%, n = 13) and Pseudomonas aeruginosa (5.6%, n = 11). Staphylococcus aureus was the predominant species among resistant bacteria. Among the antimicrobial agents tested, the following were resistant, presented on a descending scale: penicillin (72.7%, p = 0.001, Binomial test), oxacillin (68.3%, p = 0.006, Binomial test), ampicillin (64.3%, p = 0.003, Binomial test), and ampicillin/sulbactam (54.9%, p = 0.57, Binomial test). Infections with S. aureus were 3.1 times greater in pediatrics and maternal units than in other hospital wards. Despite the global reduction in the incidence of MRSA, we observed an increase in MDR S. aureus in this study. No changes were observed in the frequency of resistance profiles of the clinical isolates after the establishment of the global SARS-CoV-2 pandemic. More comprehensive studies are needed to understand the impact of the global SARS-CoV-2 pandemic on the resistance levels of bacteria associated with neonate and pediatric patients.

Cytopathological diagnosis of herpes simplex virus infection causing oral aphthous ulcers in a patient with acute lymphocytic leukemia: case report / Diagnóstico citopatológico de infecção pelo vírus herpes simples causando úlceras aftosas orais em paciente com leucemia linfocítica aguda: relato de caso

Aim:This paper aims to report an atypical oral HSV infection diagnosed by cytopathological examination in a patient with acute lymphocytic leukemia. Case report: A nine-year-old white female was admitted with acute lymphocytic leukemia, presenting ulcers covered with pseudomembrane and spontaneous bleeding on the left soft palate, measuring approximately 2 cm, as well as other ulcers measuring 1 cm on the left lateral border of the tongue. Exfoliative cytopathology revealed neutrophils and cytopathic effects of HSV in the keratinocytes in a fibrin background. Based on the exfoliative examination, the diagnosis of HSV infection was obtained. Conclusion: Oral HSV infection can be atypical in immunocompromised patients and can cause high morbidity and mortality. Healthcare professionals, especially those working in a hospital environment, should be aware of the possibility of HSV infection in atypical lesions in these patients and evaluate the need to include antiviral prophylactic therapy.

Objetivo:Este trabalho tem como objetivo relatar uma infecção oral atípica por HSV diagnosticada por exame citopatológico em um paciente com leucemia linfocítica aguda. Relato de caso:Paciente do sexo feminino, nove anos, branca, portadora de leucemia linfocítica aguda, apresentando úlceras recobertas por pseudomembrana e sangramento espontâneo em palato mole esquerdo medindo aproximadamente 2 cm, além de outras úlceras medindo 1 cm na borda lateral esquerda da língua. A citopatologia esfoliativa revelou neutrófilos e efeitos citopáticos do HSV nos ceratinócitos em um fundo de fibrina. Com base no exame esfoliativo, foi obtido o diagnóstico de infecção por HSV. Conclusão: A infecção oral por HSV pode ser atípica em pacientes imunocomprometidos e pode causar alta morbidade e mortalidade. Os profissionais de saúde, principalmente os que atuam em ambiente hospitalar, devem estar atentos à possibilidade de infecção pelo HSV em lesões atípicas nesses pacientes e avaliar a necessidade de inclusão de terapia antiviral profilática.

Oral lichenoid lesion in association with chemotherapy treatment for non-Hodgkin lymphoma or lichen planus? Review of the literature and report of two challenging cases.

BACKGROUND: The diagnosis of oral lichenoid lesions (OLL) remains a challenge for clinicians and pathologists. Although, in many cases, OLL cannot be clinically and histopathologically distinguishable from oral lichen planus (OLP), one important difference between these lesions is that OLL has an identifiable etiological factor, e.g. medication, restorative material, and food allergy. The list of drugs that can cause OLL is extensive and includes anti-inflammatory drugs, anticonvulsants, antihypertensives, antivirals, antibiotics, chemotherapeutics, among others. This work aimed to perform a literature review of OLL related to chemotherapy drugs and to report two cases of possible OLL in patients with B-cell and T-cell non-Hodgkin lymphomas in use of chemotherapy and adjuvant medications. We also discuss the challenge to clinically and histopathologically differentiate OLL and OLP. CASE PRESENTATION: In both cases, oral lesions presented reticular, atrophic, erosive/ulcerated, and plaque patterns. The diagnosis of OLL was initially established in both cases by the association of histopathology and history of onset of lesions after the use of medications. Although the patients have presented a significant improvement in the oral clinical picture for more than 2 years of follow-up, they still have some lesions. CONCLUSION: A well-detailed anamnesis associated with the drug history, temporal relationship of the appearance of the lesions, and follow-up of patients are fundamental for the diagnosis of OLL related to drugs. Nevertheless, its differentiation from OLP is still a challenge.

Head circumference and anthropometric changes and their relation to plexiform and skin neurofibromas in sporadic and familial neurofibromatosis 1 Brazilian adults: a cross-sectional study.

BACKGROUND: Neurofibromatosis 1 (NF1) is a common autosomal dominant syndrome with complete penetrance and highly variable expressivity. The cutaneous neurofibroma (Cnf) and plexiform neurofibroma (Pnf), café-au-lait spots, and freckle-like lesions are common in NF1, but many other manifestations can occur. We aimed to evaluate head circumference, height, weight, body mass index (BMI), head circumference-to-height ratio (HCHR) and waist-hip ratio (WHR) in adult NF1 Brazilian individuals versus a paired control group and investigate their correlation with the presence of clinically visible Pnfs, and number of "skin neurofibromas" (Snf), which include both cutaneous and subcutaneous neurofibromas. METHODS: A case-control study was conducted with 168 individuals, 84 with NF1 and 84 without NF1, paired by sex and age. Head circumference and anthropometric measurements, Snf quantification, evaluation of clinically visible Pnf and familial inheritance were accessed. RESULTS: Prevalence of macrocephaly was significantly higher in NF1 women. Height and weight were significantly lower in both males and females with NF1. HCHR was higher in the NF1 group than in the control group for both sexes. BMI was significantly lower in men with NF1. Waist and hip circumferences were significantly reduced in NF compared with the controls, but the mean WHR was significantly lower only in NF1 women. No correlation was found between the Snf and head circumference and anthropometric measurements, sex or family history. The presence and larger size of clinically visible plexiform neurofibromas were associated with normal stature (p = 0.037 and p = 0.003, respectively). CONCLUSIONS: NF1 individuals have increased prevalence of macrocephaly, short stature, low BMI, and reduced abdominal fat. There is no relation between head circumference and anthropometric data with family history, or neurofibromas.

Congenital sebaceous choristoma of the tongue: A rare case report.

The most common oral choristomas are consisted of thyroid tissue and bone. The presence of sebaceous glands in the oral mucosa, especially in the buccal mucosa and labial mucosa, is often considered a normal anatomical variation since they are observed in about 80% of the population and are called ectopic sebaceous glands or Fordyce's granules. However, the presence of these glands on the tongue is rare, with only 11 cases in the dorsum of the tongue reported in the English literature, and it is considered a choristoma. This paper aims to report the third case in the literature of a congenital sebaceous choristoma on the tongue. An 11-year-old white male patient presented a firm sessile papule, without color alteration, measuring 0.4 cm x 0.3 cm in diameter, in the middle third of the dorsum of the tongue with a slight increased size in the last months. Histopathological examination showed an invagination of the epithelium into the connective tissue, forming a ductal structure covered by stratified squamous epithelium. The deeper areas had normal well-differentiated sebaceous glands, with ducts connected to the central duct. Considering clinical and histopathological findings the diagnosis was sebaceous choristoma. Despite being rare, sebaceous choristomas should also be considered in the differential diagnosis of tongue abnormalities or lesions. Although the pathogenesis is not well understood, the present report, as a congenital choristoma case in the midline, reinforces the hypothesis of a disorder with embryological origin and a possible relationship with thyroglossal duct remnants. Key words:Choristoma, Oral Mucosa, Tongue.

Antibiofilm activity of the biosurfactant and organic acids against foodborne pathogens at different temperatures, times of contact, and concentrations.

Biofilm formation has been suggested to play a significant role in the survival of pathogens in food production. Interest in evaluating alternative products of natural origin for disinfectant use has increased. However, there is a lack of information regarding the effects of biosurfactants and organic acids on Salmonella enterica serotype Enteritidis, Escherichia coli, and Campylobacter jejuni biofilms, mainly considering temperatures found in environments of poultry processing, as well as simulating the contact times used for disinfection. The aim of this study was to evaluate the antibiofilm activity of rhamnolipid, malic acid, and citric acid on the adhesion of S. Enteritidis, E. coli, and C. jejuni on polystyrene surfaces at different temperatures (4, 12, and 25 °C), compound concentrations, and times of contact (5 and 10 min), and to analyze the potential use of these compounds to disrupt formed biofilms. All three compounds exhibited antibiofilm activity under all analyzed conditions, both in the prevention and removal of formed biofilms. Contact time was less important than temperature and concentration. The antibiofilm activity of the compounds also varied according to the pathogens involved. In the food industry, compound selection must consider the temperature found in each stage of product processing and the target pathogens to be controlled.

Dentinogenic ghost cell tumor with focal atypical features suggesting ghost cell odontogenic carcinoma: Report of a challenging diagnosis.

Dentinogenic ghost cell tumor (DGCT) represents a rare benign odontogenic neoplasm that can appear in a central or peripheral form and may rarely undergo malignant transformation to ghost cell odontogenic carcinoma (GCOC). We aim to report a case of a central DGCT with focal cytological malignant aspects. A 24-year-old woman exhibited a painful enlargement and dental mobility in the left posterior maxilla for about one year, which appeared as an expansive well-defined hypodense maxillary image with hyperdense foci invading ipsilateral maxillary sinus. Incisional biopsy showed a predominantly solid hyperchromatic basaloid epithelium presenting cellular pleomorphism and mitotic activity, admixed with abundant ghost cell aggregates and dentinoid material. The lesion was immunopositive for p53 and had 21% of Ki-67 proliferation index (PI). These microscopic features suggested initially a GCOC diagnosis. Partial left maxillectomy was performed without complications. The surgical specimen presented an exuberant variation of the epithelial parenchyma, including ameloblastomatous, fusiform, and cribriform areas, with numerous ghost cells and dentinoid material, lacking any signs of malignancy. The final diagnosis was DGCT. The patient is in a strict regular follow-up for over two years, and there are no signs of recurrence.

Comparative Analysis Between Dentinogenic Ghost Cell Tumor and Ghost Cell Odontogenic Carcinoma: A Systematic Review.

Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The aim of this study was to perform a comparative systematic review of the clinicopathological, genetic, therapeutic, and prognostic features of DGCT and GCOC. The electronic search was performed until December 2020 on seven electronic databases. Case reports, series, and research studies with enough histopathological criteria for diagnosis and all genomic studies were included. Both DGCT and GCOC showed a male prevalence (p = 0.043), with mandibular and maxillary predilections, respectively (p = 0.008). Peripheral DGCT (DGCTp) affected most elderly people (p < 0.001), and central DGCT (DGCTc) and GCOC occurred mainly in younger individuals. Unilateral enlargement of maxilla or mandible was the most common clinical sign associated with a radiolucent or mixed image. Ameloblastomatous epithelium was often present in both neoplasms. Basaloid and large cells with vesicular nuclei were also frequently seen in GCOC. ß-catenin expression and mutations (CTNNB1 gene) were found in DGCT and GCOC. Conservative surgery was mostly used for DGCTp, while radical resection was chosen for DGCTc and GCOC. High recurrence rates were found in DGCTc and GCOC. Metastasis occurred in 16.7% of GCOC cases and the 5-year survival rate was 72.6%. DGCT and GCOC share numerous clinicopathological features and demand a careful histopathological evaluation, considering the overlap features with other odontogenic tumors and the possibility of malignant transformation of DGCT. A strict regular post-operative follow-up is mandatory due to high recurrence rates and metastatic capacity in GCOC.

Neurofibromin expression by normal salivary glands.

INTRODUCTION: Neurofibromin, a protein encoded by the NF1 gene, is mutated in neurofibromatosis 1, one of the most common genetic diseases. Oral manifestations are common and a high prevalence of hyposalivation was recently described in individuals with neurofibromatosis 1. Although neurofibromin is ubiquitously expressed, its expression levels vary depending on the tissue type and developmental stage of the organism. The role of neurofibromin in the development, morphology, and physiology of salivary glands is unknown and a detailed expression of neurofibromin in human normal salivary glands has never been investigated. AIM: To investigate the expression levels and distribution of neurofibromin in acinar and ductal cells of major and minor salivary glands of adult individuals without NF1. MATERIAL AND METHOD: Ten samples of morphologically normal major and minor salivary glands (three samples of each gland: parotid, submandibular and minor salivary; and one sample of sublingual gland) from individuals without neurofibromatosis 1 were selected to assess neurofibromin expression through immunohistochemistry. Immunoquantification was performed by a digital method. RESULTS: Neurofibromin was expressed in the cytoplasm of both serous and mucous acinar cells, as well as in ducts from all the samples of salivary glands. Staining intensity varied from mild to strong depending on the type of salivary gland and region (acini or ducts). Ducts had higher neurofibromin expression than acinar cells (p = 0.003). There was no statistical association between the expression of neurofibromin and the type of the salivary gland, considering acini (p = 0.09) or ducts (p = 0.50) of the four salivary glands (parotid, submandibular, minor salivary, and sublingual gland). Similar results were obtained comparing the acini (p = 0.35) and ducts (p = 0.50) of minor and major salivary glands. Besides, there was no correlation between the expression of neurofibromin and age (p = 0.08), and sex (p = 0.79) of the individuals, considering simultaneously the neurofibromin levels of acini and duct (n = 34). CONCLUSION: Neurofibromin is expressed in the cytoplasm of serous and mucous acinar cells, and ductal cells of salivary glands, suggesting that this protein is important for salivary gland function.

A Clinicopathologic Study on the Role of Estrogen, Progesterone, and Their Classical and Nonclassical Receptors in Cutaneous Neurofibromas of Individuals With Neurofibromatosis 1.

OBJECTIVES: To evaluate the expression of progesterone receptor (PR), estrogen receptor (ER), and G protein-coupled estrogen receptor 1 (GPER-1) in cutaneous neurofibromas (cNFs) and their correlation with demographic, clinical, and laboratory data of individuals with neurofibromatosis 1 (NF1). The association of PROGINS polymorphism and PR expression in cNFs, as well as the serum steroidal hormones and the number of cNFs, was investigated. METHODS: The sample comprised 80 large and 80 small cNFs from 80 individuals with NF1. PR, ER, GPER-1, and Ki-67 expression were investigated by immunohistochemistry in tissue micro- and macroarrays and quantified using a digital computer-assisted method. The number of cNFs, the levels of serum 17ß estradiol and progesterone, and the PROGINS polymorphism were identified. RESULTS: Twelve (8.5%) small cNFs were weakly positive for ER, 131 (92.3%) cNFs expressed PR, and all (100%) cNFs expressed GPER-1. Large cNFs showed a higher expression of PR (P < .0001) and GPER-1 (P = .019) and had a higher intensity of staining for these receptors (P < .0001). The cell proliferation index was positively correlated with PR (P = .001). Persons with more cNFs had higher serum levels of progesterone (P = .001). CONCLUSIONS: These findings emphasize the role of estrogen and progesterone in cNF development and suggest that these hormones may act on cNF cells via a noncanonical pathway through GPER-1.

Fenilcetonúria associada à alergia à proteína do leite de vaca / Phenylketonuria Associated with Cow Milk Protein Allergy

Introdução: a fenilcetonúria (PKU) é uma doença do metabolismo da fanilalanina cujo tratamento se baseia na introdução precoce de uma fórmula com restrição de fenilalanina. Relato do caso: uma menina, com diagnóstico de PKU a partir da triagem neonatal, com 82 dias de vida, recebeu tratamento dietético com fórmula com restrição de fenilalanina associada à fórmula láctea e desenvolveu alergia à proteína do leite de vaca (APLV) com sintomas cutâneos e gastrointestinais. Conclusão: o manejo dietético da PKU pode precipitar a ocorrência da APLV.

Introduction: Phenylketonuria (PKU) is a disease of the metabolism of phanylalanine whose treatment is based on the early introduction of a phenylalanine-restricted formula. Case report: A girl with 82 days of life with PKU diagnosis from neonatal screening received dietary treatment with a phenylalanine-restricted formula associated with the milk formula. She developed allergy to cow's milk protein (APLV) with cutaneous symptoms and gastrointestinal disorders. Conclusion: Dietary management of PKU may precipitate the occurrence of APLV.

Behaviour and Prevention of 5'Fluorouracil and Doxorubicin-induced Oral Mucositis in Immunocompetent Patients with Solid Tumors: A Randomised Trial.

PURPOSE: This study evaluated the efficacy of four methods to prevent chemotherapy-induced oral mucositis (OM) in patients with solid tumors (ST). In addition, the behaviour of OM was investigated in these oncological patients. MATERIALS AND METHODS: Forty-eight patients, aged 27-84, were randomly allocated to different groups from the first day of chemotherapy (CT), in the following sequence: group 1: intensive oral care programme (IOCP); group 2: 0.12% chlorhexidine mouthrinse; group 3: 0.03% triclosan mouthrinse; group 4: low-level laser therapy (LLLT). Oral mucositis was evaluated on the 7th and 14th days by means of the Oral Mucositis Assessment Scale (OMAS). RESULTS: Thirty-one (64.5%) patients developed OM in the first cycle of CT and the pain was significantly associated with OM severity (p < 0.0001). The statistically significantly worst OMAS score was found for the lips and buccal mucosa (p < 0.0001). Despite a lack of statistical significance, IOCP and LLLT notably demonstrated potential effects to prevent OM in patients who presented with only oral erythema (75%) and lower peak of severity during the follow up, respectively. CONCLUSIONS: Improved oral care awareness is needed in patients undergoing 5'fluorouracil and doxorubicin, mainly to avoid pain caused by oral mucositis. Oral mucositis was more prevalent and aggressive in oral sites exposed to chronic trauma. The IOCP and LLLT approaches showed positive results to prevent oral mucositis and should be further investigated in similar and larger samples.

Prevalence and clinicopathological characteristics of lipomatous neurofibromas in neurofibromatosis 1: An investigation of 229 cutaneous neurofibromas and a systematic review of the literature.

BACKGROUND: Lipomatous neurofibroma (Lnf) is a histopathological variant with adipocytes noted among cells of cutaneous neurofibromas. We aimed to investigate the prevalence and clinicopathological features of Lnfs of neurofibromatosis 1 (NF1)-associated cutaneous neurofibromas and to review the literature systematically. We also evaluated the expression of leptin (a hormone involved in lipid metabolism) in neurofibromas to better understand the pathogenesis of Lnfs. METHODS: A prospective histologic study was conducted on 229 cutaneous neurofibromas from 85 NF1 individuals. Leptin expression was immunohistochemically evaluated in 111 cutaneous neurofibromas. To systematically review the literature, two authors independently performed literature searches without year restriction. RESULTS: Forty (17.5%) neurofibromas were lipomatous. Lnfs were significantly larger lesions and associated with females. Eighteen (7.9%) of all neurofibromas had multinucleated floret-like giant cells, and these were associated with Lnfs. All neurofibromas expressed leptin. We systematically reviewed 13 articles. Three large studies investigated Lnfs mainly in sporadic neurofibromas and suggested that 0.3% to 8.0% of tumors (NF1 and sporadic) are Lnfs. CONCLUSION: In NF1, Lnfs are common, mainly in larger tumors and women. All cutaneous NF1-neurofibromas express leptin. It is unknown if the expression of leptin accounts for the lipomatous variant, but it may have a role in the pathogenesis of cutaneous neurofibroma.

Biofilm formation by Salmonella Enteritidis and Salmonella Typhimurium isolated from avian sources is partially related with their in vivo pathogenicity.

Salmonella Enteritidis and Salmonella Typhimurium are among the most prevalent serotypes isolated from salmonellosis outbreaks and poultry. Salmonella spp. have the capacity to form biofilms on several surfaces, which can favour survival in hostile environments, such as slaughterhouses. Salmonella strains present differences in pathogenicity. However, there is little information regarding the pathogenicity of S. Enteritidis and S. Typhimurium isolated from avian sources and their relationship to biofilm production. The aim of this study was to use a novel pathogenicity index and a biofilm production assay to evaluate their relationships within these serotypes. In addition, we detected the presence of the spiA and agfA genes in these strains. Biofilm formation was investigated at two temperatures (37 °C and 28 °C) using microtiter plate assay, and the results were compared with the individual pathogenicity index of each strain. PCR was used to detect spiA and agfA, virulence genes associated with biofilm production. S. Enteritidis and S. Typhimurium strains were capable of producing biofilm at 37 °C and 28 °C. Sixty-two percent and 59.5% of S. Enteritidis and 73.8% and 46.2% of S. Typhimurium produced biofilm at 37 °C and 28 °C, respectively. Biofilm production at 37 °C was significantly higher in both serotypes. Only S. Enteritidis was capable of adhering strongly at both temperatures. Biofilm production was related to pathogenicity index only at 28 °C for S. Enteritidis. spiA and agfA were found in almost all strains and were not statistically associated with biofilm production.

Biofilm formation capacity of Salmonella serotypes at different temperature conditions / Capacidade de produção de biofilme por cepas de diferentes sorovares de Salmonella em quatro temperaturas de incubação

Salmonella spp. are one of the most important agents of foodborne disease in several countries, including Brazil. Poultry-derived products are the most common food products, including meat and eggs, involved in outbreaks of human salmonellosis. Salmonella has the capacity to form biofilms on both biotic and abiotic surfaces. The biofilm formation process depends on an interaction among bacterial cells, the attachment surface and environmental conditions. These structures favor bacterial survival in hostile environments, such as slaughterhouses and food processing plants. Biofilms are also a major problem for public health because breakage of these structures can cause the release of pathogenic microorganisms and, consequently, product contamination. The aim of this study was to determine the biofilm production capacity of Salmonella serotypes at four different temperatures of incubation. Salmonella strains belonging to 11 different serotypes, isolated from poultry or from food involved in salmonellosis outbreaks, were selected for this study. Biofilm formation was investigated under different temperature conditions (37°, 28°, 12° and 3°C) using a microtiter plate assay. The tested temperatures are important for the Salmonella life cycle and to the poultry-products process. A total of 92.2% of the analyzed strains were able to produce biofilm on at least one of the tested temperatures. In the testing, 71.6% of the strains produced biofilm at 37°C, 63% at 28°C, 52.3% at 12°C and 39.5% at 3°C, regardless of the serotype. The results indicate that there is a strong influence of temperature on biofilm production, especially for some serotypes, such as S. Enteritidis, S. Hadar and S. Heidelberg. The production of these structures is partially associated with serotype. There were also significant differences within strains of the same serotype, indicating that biofilm production capacity may be strain-dependent.(AU)

Salmonella spp. são um dos mais importantes agentes causadores de doenças transmitidas por alimentos em vários países, inclusive no Brasil. Produtos avícolas e ovos são os principais alimentos envolvidos na transmissão dos sorovares de Salmonella que são responsáveis por surtos de salmonelose em humanos. Salmonella possui a capacidade de formar biofilmes em diversas superfícies. O processo de formação de biofilme depende da interação entre as células bacterianas, a superfície de adesão e as condições do ambiente onde a bactéria se encontra. Estas estruturas favorecem a sobrevivência bacteriana em ambientes hostis, como em matadouros-frigoríficos e em indústrias processadoras de alimentos. Biofilmes são um grande problema em saúde pública, pois a ruptura destas estruturas pode provocar a liberação de microrganismos patogênicos e, consequentemente, a contaminação dos produtos. O objetivo deste estudo foi avaliar a capacidade de produção de biofilme por diferentes sorovares de Salmonella submetidos a quatro temperaturas de incubação. Cepas de Salmonella de 11 sorovares foram selecionadas. A produção de biofilme foi avaliada através do método de incubação em microplacas de poliestireno incubadas a 37°, 28°, 12° e 3°C. Estas temperaturas são importantes durante o ciclo de vida de Salmonella e para o processamento de produtos avícolas. Do total de cepas avaliadas, 92,2% foram capazes de produzir biofilme em pelo menos uma das quatro temperaturas testadas. Neste estudo, 71,6% das cepas produziram biofilme a 37°C, 63% a 28°C, 52,3% a 12°C e 39,5% a 3°C, independentemente do sorovar. Os resultados indicam uma forte influência da temperatura na produção de biofilme, especialmente para os sorovares S. Enteritidis, S. Hadar e S. Heidelberg. A produção de biofilme está parcialmente associada com o sorovar da cepa. Também foi observado que existe variação quanto à produção destas estruturas dentro de um mesmo sorovar, indicando que possivelmente a produção de biofilme é cepa-dependente.(AU)

Hydrocephalus associated to congenital Zika syndrome: does shunting improve clinical features?

PURPOSE: Congenital Zika syndrome (CZS) is a new entity with little information about its course and natural history. It is known that prenatal infection by Zika virus is associated to disrupted nervous system development, leading to typical neurological disabilities and deformities. Some children present progressive ventriculomegaly and hydrocephalus associated to aggravation of seizures and neurological impairment. The aim of this study is to evaluate the development of hydrocephalus and the impact of ventriculoperitoneal shunt insertion in the clinical condition of these children. METHODS: Data was obtained from chart review, direct interviews with patients' parents, direct neurological examination, and analysis of pre- and postoperative neuroimages. RESULTS: A group of 115 patients had CZS diagnosis from November 2015 to July 2017. Among them, 21 (18.3%) patients had ventricular enlargement noted on follow-up CT scans. Six children (28.6%) underwent a ventriculoperitoneal shunt and all had some improvement after surgery concerning either waking time during the day and better interaction. Overall improvement was also noted in seizures. Spasticity decrease and more cervical control were also achieved. In two out of six cases, a slight increase in parenchymal length could be noted on the CT scans. CONCLUSION: This series points out the possibility of hypertensive hydrocephalus development in CZS patients. Affected children may benefit from VP shunt insertion. These findings suggest a dual pathology association: fetal brain disruption and primary cortical malformation by the virus itself and hypertensive hydrocephalus. This is already seen in some cases of congenital rubella, toxoplasmosis, or cytomegalovirus-associated hydrocephalus.