Psychometric Validation of a Hearing Screening Questionnaire for Preschoolers Based on Language Development Evaluation by Caregivers.

INTRODUCTION: This study aimed to validate three age-adjusted versions of a Hearing Screening Questionnaire for Preschoolers, in Brazilian Portuguese, based on parents' perception of their children's hearing and oral language. METHODS: Psychometric validation was conducted on three questionnaires, each comprising nine items with yes/no responses. Three items focused on hearing screening at birth, and six assessed hearing and oral language. The study included 152 parents and their children, who attended daycare centers in Belo Horizonte, Brazil. The children were categorized into three age bands: 12-18 months, 19-35 months, and 36-48 months. Audiological assessments, including tympanometry, transient-evoked otoacoustic emissions (TEOAE), and pure-tone audiometry (when applicable), were performed on the children. In case of abnormal findings in the previous exams, auditory brainstem response (ABR) testing was conducted. Descriptive data, false alarm, and false-negative analyses were carried out. RESULTS: Considering any type of hearing loss, whether unilateral or bilateral, the questionnaires showed a false-negative rate of 41.17% (7/17 children). However, when considering only bilateral hearing loss, the questionnaire showed a false alarm rate of 31.69% (45/142) and a false-negative rate of 30.0% (3/10). When focusing exclusively on sensorineural hearing loss, the questionnaire identified two children (1.31%), with a false-negative rate of 0% but a false-positive rate of 33.33%. CONCLUSION: Language-development-oriented questionnaires allowed quick screening of potential hearing loss in preschoolers. This study found a robust hit rate with these questionnaires. Their validation signifies a promising and cost-effective tool for conducting hearing screenings in preschool children, especially in nations lacking a comprehensive school screening policy. The validated questionnaire affords an easy-to-apply, low-cost, and effective instrument for preschool hearing screening.

Development of an immunogen containing CD4+/CD8+ T-cell epitopes for the prophylaxis of tegumentary leishmaniasis.

Tegumentary leishmaniasis (TL) is a disease of high severity and incidence in Brazil, and Leishmania braziliensis is its main etiological agent. The inefficiency of control measures, such as high toxicity and costs of current treatments and the lack of effective immunoprophylactic strategies, makes the development of vaccines indispensable and imminent. In this light, the present work developed a gene encoding multiple T-cell (CD4+/CD8+) epitope, derived from conserved proteins found in Leishmania species and associated with TL, to generate a chimeric protein (rMEP/TL) and compose a vaccine formulation. For this, six T-cell epitopes were selected by immunoinformatics approaches from proteins present in the amastigote stage and associated with host-parasite interactions. The following formulations were then tested in an L. braziliensis murine infection model: rMEP/TL in saline or associated with MPLA-PHAD®. Our data revealed that, after immunization (three doses; 14-day intervals) and subsequent challenging, rMEP/TL and rMEP/TL + MPLA-vaccinated mice showed an increased production of key immunological biomarkers of protection, such as IgG2a, IgG2a/IgG1, NO, CD4+, and CD8+ T-cells with IFN-γ and TNF-α production, associated with a reduction in CD4+IL-10+ and CD8+IL-10+ T-cells. Vaccines also induced the development of central (CD44highCD62Lhigh) and effector (CD44highCD62Llow) memory of CD4+ and CD8+ T-cells. These findings, associated with the observation of lower rates of parasite burdens in the vaccinated groups, when compared to the control groups, suggest that immunization with rMEP/TL and, preferably, associated with an adjuvant, may be considered an effective tool to prevent TL. KEY POINTS: • Rational design approaches for vaccine development. • Central and effector memory of CD4+ and CD8+ T-cells. • Vaccine comprised of rMEP/TL plus MPLA as an effective tool to prevent TL.

Development of a chimeric protein based on a proteomic approach for the serological diagnosis of human tegumentary leishmaniasis.

Leishmania braziliensis is responsible for most cases of human tegumentary leishmaniasis (HTL) and has caused a wide range of clinical manifestations, including cutaneous (CL) and mucosal leishmaniasis (ML). The diagnosis is based on criteria that consider epidemiological data, clinical findings, and laboratory tests and is hard to establish. For laboratory tests, none of the assays available can be considered gold standards for disease detection. In addition, the Montenegro skin test, essential to supporting infectologists in the clinical management of the disease, is no longer available in Brazil. Thus, the aim of this study was to develop new targets to be used in diagnostic tests for HTL. In the first step, we carried out two-dimensional gel electrophoresis, followed by mass spectrometry, combined with heat map analysis and immunoproteomics approach, and disclosed eight proteins expressed in the amastigote stage specifically recognized by serum from CL and ML patients. A chimeric protein was designed based on the combination of thirteen linear B-cell epitopes, identified by immunoinformatics analysis, from L. braziliensis proteins. Our results showed that the strategy used in this work was successful in developing an antigen to be used in immunological assays (100.0% sensitivity and specificity) in the detection of HTL cases and in comparison with results obtained from an ELISA using soluble L. braziliensis antigen (SLb-Antigen) and immunofluorescence assay (Bio-Manguinhos/FIOCRUZ). The present technology opens the door for its use in field exams by means of an immunochromatographic test, which will be even more helpful in regions without laboratory structures.Key points• Rational strategy to develop antigens.• Integration between immunoproteomic and immunoinformatics analysis.• Chimeric protein shows high performance in HTL diagnosis.

Annexin A1 Is Involved in the Resolution of Inflammatory Responses during Leishmania braziliensis Infection.

Leishmaniases are diseases caused by several Leishmania species. Leishmania (Viannia) braziliensis can cause localized cutaneous leishmaniasis (LCL), which heals spontaneously, or mucosal leishmaniasis (ML), characterized by chronic and intense inflammation and scanty parasitism. Annexin A1 (AnxA1) is a protein involved in modulation and resolution of inflammation through multiple mechanisms. In the present study, the role of AnxA1 was investigated in L. braziliensis-infected BALB/c mice. AnxA1 levels increased at the peak of tissue lesion and parasitism in infected mice. AnxA1 increased also after L. braziliensis infection of BALB/c (wild-type [WT]) bone marrow derived macrophages. Despite a lower parasite intake, parasite burden in bone marrow-derived macrophages from AnxA1-/- mice was similar to WT and associated with an early increase of TNF-α and, later, of IL-10. AnxA1-/- mice controlled tissue parasitism similarly to WT animals, but they developed significantly larger lesions at later stages of infection, with a more pronounced inflammatory infiltrate and increased specific production of IFN-γ, IL-4, and IL-10. AnxA1-/- mice also presented higher phosphorylation levels of ERK-1/2 and p65/RelA (NF-κB) and inducible NO synthase expression, suggesting that AnxA1 may be involved in modulation of inflammation in this model of experimental leishmaniasis. Finally, assessment of AnxA1 levels in sera from patients with LCL or ML revealed that ML patients had higher levels of serum AnxA1 than did LCL patients or control subjects. Collectively, these data indicate that AnxA1 is actively expressed during L. braziliensis infection. In the absence of AnxA1, mice are fully able to control parasite replication, but they present more intense inflammatory responses and delayed ability to resolve their lesion size.

Increased N200 and P300 latencies in cognitively impaired elderly carrying ApoE ε-4 allele.

OBJECTIVE: To compare the results of neuropsychological tests, evoked potentials N200 and P300 and polymorphisms of ApoE and BDNF rs6265 between patients with normal cognition and those with mild cognitive impairment (MCI) and Alzheimer's dementia (AD). METHODS: This is a cross-sectional study of elderly individuals with normal cognition and those with MCI and AD, who were submitted to evoked potential tests (N200 and P300) by means of hearing stimuli based on the auditory oddball paradigm. Genotyping was obtained by using the real-time PCR technique. RESULTS: Sixty-five patients were evaluated as follows: 14 controls, 34 with MCI and 17 with AD. N200 latency and P300 latency and amplitude were not associated with MCI and AD diagnosis. Patients with cognitive impairment (MCI or AD) showed increase in the latencies of P300 and N200. BNDF gene was not associated with cognitive impairment. CONCLUSION: Latencies of N200 and P300 increased in cognitively impaired patients with the presence of ApoE ε-4 allele.

Evaluation of a hypothetical protein for serodiagnosis and as a potential marker for post-treatment serological evaluation of tegumentary leishmaniasis patients.

The serodiagnosis for tegumentary leishmaniasis (TL) presents problems related to the sensitivity and/or specificity of the tests. In the present study, an enzyme-linked immunosorbent assay (ELISA) technique was used to evaluate the performance from a Leishmania braziliensis hypothetical protein, LbHyM, in an attempt to compare its serological reactivity with a soluble Leishmania antigenic preparation (SLA) for the serodiagnosis of cutaneous (CL) and mucosal (ML) leishmaniasis. LbHyM was predicted to be a kinesin-like protein by bioinformatics tools. Serum samples were collected from both CL and ML patients, as well as from those with Chagas disease and from healthy subjects living in endemic or non-endemic areas of TL. Also, sera were collected from patients before and after the treatments, seeking to evaluate their serological follow-up in relation to the anti-protein and anti-parasite antibody levels. When an ELISA-rLbHyM assay was performed, it proved to be significantly more sensitive than ELISA-L. braziliensis SLA in detecting both CL and ML patients. Also, when using sera from Chagas disease patients, the ELISA-rLbHyM proved to be more specific than ELISA-SLA. The anti-protein and anti-parasite antibody levels were also evaluated 6 months after the treatments, and treated patients showed significantly lower levels of specific-rLbHyM antibodies, when compared to the anti-parasite antibody levels. In conclusion, the ELISA-rLbHyM assay can be considered a confirmatory serological technique for the serodiagnosis of L. braziliensis infection and can also be used in the serological follow-up of treated patients, aiming to correlate the low anti-protein antibody levels with the improvement of the healthy state of the patients.

Immunological signature of the different clinical stages of the HTLV-1 infection: establishing serum biomarkers for HTLV-1-associated disease morbidity.

This study aimed at establishing the immunological signature and an algorithm for clinical management of the different clinical stages of the HTLV-1-infection based on serum biomarkers. A panel of serum biomarkers was evaluated by four sets of innovative/non-conventional data analysis approaches in samples from 87 HTLV-1 patients: asymptomatic carriers (AC), putative HTLV-1 associated myelopathy/tropical spastic paraparesis (pHAM/TSP) and HAM/TSP. The analysis of cumulative curves and molecular signatures pointed out that HAM/TSP presented a pro-inflammatory profile mediated by CXCL10/LTB-4/IL-6/TNF-α/IFN-γ, counterbalanced by IL-4/IL-10. The analysis of biomarker networks showed that AC presented a strongly intertwined pro-inflammatory/regulatory net with IL-4/IL-10 playing a central role, while HAM/TSP exhibited overall immune response toward a predominant pro-inflammatory profile. At last, the classification and regression trees proposed for clinical practice allowed for the construction of an algorithm to discriminate AC, pHAM and HAM/TSP patients with the elected biomarkers: IFN-γ, TNF-α, IL-10, IL-6, IL-4 and CysLT. These findings reveal a complex interaction among chemokine/leukotriene/cytokine in HTLV-1 infection and suggest the use of the selected but combined biomarkers for the follow-up/diagnosis of disease morbidity of HTLV-1-infected individuals.

Elderly people with human T-cell leukemia virus type 1-associated myelopathy present an early impairment in cognitive skills.

BACKGROUND: Cerebral changes occur in individuals with human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy (HAM) and seem to predominate in subcortical areas. Little is known about the cognitive decline in the elderly living with HTLV-1. OBJECTIVE: To evaluate the cognitive aging of individuals infected with HTLV-1 aged ≥ 50 years. METHODS: This is a cross-sectional study of former blood donors infected with HTLV-1 who have been followed in the cohort of the Interdisciplinary Research Group on HTLV-1 since 1997. The groups of study consisted of 79 HTLV-1 infected individuals aged ≥ 50 years, with 41 of them presenting symptomatic HAM and 38 being asymptomatic carriers, and 59 seronegative individuals (controls) aged ≥ 60 years. All were submitted to the P300 electrophysiological test and neuropsychological tests. RESULTS: Individuals with HAM presented delayed P300 latency in relation to the other groups, and this latency delay increased progressively with aging. The performance of this group in the neuropsychological tests was also the worst. The HTLV-1- asymptomatic group performance was similar to that of the control group. CONCLUSIONS: Individuals with HAM presented cognitive decline that progressed with aging and, although HTLV-1-asymptomatic carriers appear to present cognitive aging similar to that of healthy elderly people, concern about a subclinical cognitive impairment is warranted in this population.

ANTECEDENTES: Alterações cerebrais ocorrem em indivíduos com mielopatia associada ao vírus da leucemia de células T humanas tipo 1 (HTLV-1) (HAM) e parecem predominar em áreas subcorticais. Pouco se sabe sobre o declínio cognitivo em idosos vivendo com HTLV-1. OBJETIVO: Avaliar o envelhecimento cognitivo de indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos. MéTODOS: Trata-se de um estudo transversal com ex-doadores de sangue infectados pelo HTLV-1 acompanhados na coorte do Grupo Interdisciplinar de Pesquisa em HTLV-1 há 20 anos. Os grupos de estudo foram compostos por 79 indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos, sendo que 41 apresentavam HAM e 38 eram portadores assintomáticos, e 59 indivíduos soronegativos (controles) com idade ≥ 60 anos. Todos foram submetidos ao teste eletrofisiológico P300 e testes neuropsicológicos. RESULTADOS: Indivíduos com HAM apresentaram atraso na latência do P300 em relação aos demais grupos, e esse atraso de latência aumentou progressivamente com o envelhecimento. O desempenho desse grupo nos testes neuropsicológicos também foi o pior. O desempenho do grupo HTLV-1- assintomático foi semelhante ao do grupo controle. CONCLUSãO: Indivíduos com HAM apresentaram declínio cognitivo que progrediu com o envelhecimento e, embora os portadores assintomáticos do HTLV-1 pareçam apresentar envelhecimento cognitivo semelhante ao dos idosos saudáveis, justifica-se a preocupação com um comprometimento cognitivo subclínico nessa população.

Epidemiology, treatment, and prevention of human T-cell leukemia virus type 1-associated diseases.

Human T-cell leukemia virus type 1 (HTLV-1), the first human retrovirus to be discovered, is present in diverse regions of the world, where its infection is usually neglected in health care settings and by public health authorities. Since it is usually asymptomatic in the beginning of the infection and disease typically manifests later in life, silent transmission occurs, which is associated with sexual relations, breastfeeding, and blood transfusions. There are no prospects of vaccines, and screening of blood banks and in prenatal care settings is not universal. Therefore, its transmission is active in many areas such as parts of Africa, South and Central America, the Caribbean region, Asia, and Melanesia. It causes serious diseases in humans, including adult T-cell leukemia/lymphoma (ATL) and an incapacitating neurological disease (HTLV-associated myelopathy/tropical spastic paraparesis [HAM/TSP]) besides other afflictions such as uveitis, rheumatic syndromes, and predisposition to helminthic and bacterial infections, among others. These diseases are not curable as yet, and current treatments as well as new perspectives are discussed in the present review.

Galvanic vestibular stimulation and its applications: a systematic review.

OBJECTIVE: Galvanic vestibular stimulation has been evaluated in the context of vestibular rehabilitation. The objective was to identify evidence in the scientific literature about the clinical applications of galvanic vestibular stimulation. METHODS: In this systematic review, the articles describing the applications of galvanic vestibular stimulation were extracted from PubMed, Web of Science, MEDLINE, Scopus, LILACS and SciELO databases. The survey was limited to articles published in English, Portuguese and Spanish. All the articles about the clinical applications of galvanic vestibular stimulation were compiled. Repeated articles in the databases, literature review articles, case reports, letters and editorials were excluded. The descriptors included: galvanic vestibular stimulation, postural balance, central nervous system diseases, vestibular diseases, spinal cord diseases and cognition. RESULTS: The search strategy resulted in the initial selection of 994 articles; the reading of titles and abstracts was accomplished in 470 articles and the complete reading in 23 articles. Clinical applications of galvanic vestibular stimulation included Ménière's disease, vestibular neuritis, bilateral vestibular disorders, vestibular schwannoma, Parkinson's disease, ischemic central lesions, motor myelopathies, anxiety disorders, cognition and memory. CONCLUSION: Galvanic vestibular stimulation has been considered a potentially useful strategy for balance rehabilitation, since it has the effect of stimulating the central connections related to the postural balance, favoring new neuronal synapses that allow the partial or total recovery of postural imbalance.

Impact assessment of different DNA extraction methods for non-invasive molecular diagnosis of tegumentary leishmaniasis.

The aim of this study was to evaluate two methods of nucleic acid extraction (spin-column-based method - commercial kit and direct boil - DB) from swab sampling compared to biopsy sampling for the diagnosis of tegumentary leishmaniasis (TL), (cutaneous - CL and mucocutaneous - MCL forms). The impact of these nucleic acid extraction protocols on different types of PCR and LAMP techniques were compared regarding nucleic acid quality, molecular assays accuracy, indirect quantitation, and costs. The evaluated patients were 57 TL cases (36 CL and 21 MCL) and 34 non-cases. Swab samples extracted by the DB method showed a higher DNA degradation rate and worse DNA quality in comparison to the commercial kit. Molecular tests performed on biopsy samples showed identical or higher performance in all analysis, as compared to their own performance on swab samples for TL (CL and MCL). However, only the SSU rRNA TaqMan™ RT-PCR test showed a significant difference between the performance of biopsy and swab samples extracted by commercial kit. The kDNA-cPCR coupled with swab extracted by commercial kit showed the highest accuracy (95.6%) for TL diagnosis. The sensitivity of the LAMP-RT 18S method in swab samples extracted with a commercial kit (82.5%) was close to that found in biopsy samples (86%) for TL diagnosis. The DB extraction method presented the lowest cost. The use of swab as a minimally-invasive sampling method, associated with an efficient nucleic acid extraction protocol, may represent a low-cost alternative for the diagnosis of CL and MCL.

Mapping linear B-cell epitopes of the Tryparedoxin Peroxidase and its implications in the serological diagnosis of tegumentary leishmaniasis.

Diagnosis of tegumentary leishmaniasis (TL) is essential to avoid permanent damage and severe functional sequelae and there is an urgent need to discover new antigens. The present study aimed to comprehensively evaluate the potential use of the Tryparedoxin Peroxidase (TryP) as an antigen for serological tests. The proposal integrates data from immunoproteomics with immunoinformatics, in addition to a precise analysis of protein levels in the evolutionary stages of the parasite by flow cytometry. To evaluate the performance in the diagnosis of TL, Enzyme-Linked Immunosorbent Assay (ELISA) assays were performed using the recombinant protein and the respective B-cell epitope, followed by an analysis of the contribution of this peptide in the recognition of the protein by patients, evaluated by serum depletion assays. We showed that the TryP has a linear B-cell epitope with high divergence compared to orthologs from Trypanosoma cruzi and Homo sapiens. The results also show high expression and positive cells for TryP (TryP+) in the infective metacyclic promastigotes (MET) and intracellular (24 and 48 hours) stages. From the depletion assays, it was possible to confirm the contribution of the peptide in the specific recognition of the TryP protein by patients with TL (13.7-15.9%). ELISA using the peptide showed high performance in the diagnosis compared to the recombinant TryP (rTryP), Soluble Leishmania braziliensis Antigen (sLba) and Immunofluorescence Assay (IFA) with accuracy of 94.29, 89.29, 65.00 and 37.14%, respectively). We can conclude that the MNEPAPP peptide is a potential antigen for the diagnosis of TL.

Lian gong as a Therapeutic Treatment Option in Primary Care for Patients with Dizziness: A Randomized Controlled Trial.

Introduction Dizziness is one of the most common reasons for seeking primary health care. Vestibular rehabilitation (VR) is a conventional treatment method for peripheral balance disorders that effectively decreases symptoms. Lian gong [LG] is believed to benefit patients with dizziness and to reduce the impact of the condition on quality of life by stimulating visual fixation, attention, body balance, and neuroplasticity. Objective The present study aimed to evaluate the effects of LG on the impact of dizziness on quality of life and fear of falling in primary health care patients. Methods This was a two-arm, parallel randomized clinical trial that included 36 patients with dizziness not caused by central changes. After specific medical evaluations and indications for treatment, the participants were randomly assigned to 3 groups: the LG group ( n = 11), the VR group ( n = 11), and the control group ( n = 14). The interventions were conducted collectively over a period of 12 weeks. Results Lian gong reduced the influence of dizziness on quality of life in physical (1.8 points, 95% confidence interval [CI]: 0.2-3.4), functional (4.0 points, 95% CI: 2.1-5.9), and emotional domains of quality of life (4.4 points, 95% CI: 1.7-7.2), with no differences compared with VR. There were similar concerns among the groups about the risk of falling. Conclusion Lian gong was shown to be an effective balance rehabilitation strategy to reduce the impact of dizziness on quality of life, with similar results to those of VR.

Alterations in early auditory evoked potentials in multiple sclerosis patients.

UNLABELLED: Alterations in early auditory evoked potentials (EAEP) in individuals with demyelinating disease are suggestive of lesions in the brainstem. AIM: this study aims to evaluate the prevalence of hearing disorders and altered EAEP in multiple sclerosis (MS) patients. MATERIALS AND METHOD: sixteen female and nine male patients with a defined diagnosis of multiple sclerosis took part in this study. All individuals underwent hearing and EAEP tests. The wave forms were categorized according to Jerger (1986). RESULTS: fifty EAEP tests were carried out; 70% were classified as type I (normal response) according to Jerger's criteria. Altered EAEP results in at least one ear were classified into types II, III, IV or V according to Jerger. Females accounted for 31.25% of alterations, and males 44.44%, adding up to 36% of all cases. CONCLUSIONS: these findings stress the importance of looking at EAEP in cases where there is suspicion of demyelinating disease and in patients with a defined diagnosis for MS.

[Otoneurological manifestations associated with antiretroviral therapy]. / Manifestações otoneurológicas associadas à terapia anti-retroviral.

Ototoxicity and antiretroviral therapy seem to be associated. The aim of this study was to evaluate this possible correlation. Evaluations were carried out on 779 medical records from HIV-infected patients who were being regularly followed up, of whom 162 were being treated with antiretroviral therapy and 122 were untreated (controls). The patients undergoing treatment were older (mean: 42 years), had had serological confirmation for longer times (80 months) and had smaller viral loads (P = 0.00). CD4+ was similar between the groups (P = 0.60). In the treated group, three cases (1.8%) of idiopathic hearing loss and two (1.3%) of otosclerosis-related hearing loss were observed, which both started after antiretroviral therapy. No statistical difference relating to idiopathic hearing loss was found between the groups. While descriptive studies consider possible ototoxicity associated with antiretroviral therapy, this possible adverse effect was not related to the antiretroviral therapy in this study. Conversely, otosclerosis might have been correlated with antiretroviral therapy. This issue deserves to be studied.

Interpretation and use of caloric testing.

Caloric testing is an otoneurologic evaluation of the status of the vestibular-ocular reflex; it allows an evaluation of each labyrinth separately. The main aspects on the use and interpretation of caloric testing are reviewed. METHOD: A systematic review of papers published in the past one hundred years on caloric testing was undertaken. The inclusion criteria were: cross-sectional, longitudinal, original articles, reviews and meta-analyses. Reviews of patient charts, case reports and editorials were excluded. The key-words were: caloric testing, nystagmus, vestibular system, directional preponderance, labyrinth predominance, monothermal caloric test, ice water caloric testing, Bell s phenomenon. The databases were: COCHRAINE, MEDLINE, LILACS, CAPES. RESULTS: Ninety-three of 818 abstracts fulfilled the inclusion criteria. After reading these articles, 55 were selected for this study. These papers discussed the basics of caloric testing, the types of stimulation, the interpretation of results, variables, artifacts, and the monothermal and ice water caloric test. FINAL COMMENTS: Caloric testing reference values may vary according to each unit; the the cutoff point is defined based on local studies. Attention to the technique is essential to maximize test sensitivity.

Otorhinolaryngological and esophageal manifestations of epidermolysis bullosa.

UNLABELLED: Epidermolysis bullosa (EB) is a group of skin diseases with different clinical manifestations and varied inheritance patterns. Blisters may appear spontaneously or following minimal trauma to the skin or mucosa. AIM: this paper aims to describe the otorhinolaryngological manifestations and esophageal complications related to EB, and the experience in treating patients with esophageal stenosis secondary to this disease. MATERIALS AND METHOD: this descriptive study enrolled 60 patients with EB seen from June 1999 to December 2006 at the Head and Neck Surgery Service of X Hospital, a reference center for EB. RESULTS: the patients' mean age was 14.5 years. Twenty-eight (46.6%) were females and 32 (53.4%) were males. Eight (13.4%) were diagnosed with epidermolysis bullosa simplex, while 51 (85%) had epidermolysis bullosa dystrophica; one (1.6%) patient had one acquired EB. Lips, mouth, tongue and ears were the most frequently involved sites (32 patients - 53.3%). Dysphagia was found in 28 patients (46.6%). After esophageal dilatation the symptoms subsided. CONCLUSION: EB is a rare disease and patients must be sent for treatment at reference centers. Physicians treating patients for EB must be aware of the measures required to improve the quality of the treatment provided without putting the patients in harm's way.

Flexible nasolaryngoscopy accuracy in laryngomalacia diagnosis.

UNLABELLED: Laryngomalacia is the most common cause of stridor in infancy. Diagnosis is established by associating the clinical manifestations with laryngoscopic findings in a dynamic form. AIM: To analyze diagnostic accuracy of laryngomalacia through flexible nasolaryngoscopy performed by four different examiners. Form of studying: Comparison of diagnostic tests (clinical study). MATERIAL AND METHOD: A protocol of videolaryngoscopic evaluation for patients with laryngomalacia was created encompassing the following items: anterior displacement of the arytenoids; omega-shaped epiglottis; short aryepiglottic folds; posterior displacement of epiglottis; vocal folds being visible or not; edema of the posterior larynx. Four different examiners analyzed the videolaryngoscopic examinations of 18 patients with established diagnosis of laryngomalacia previously established by clinical data, epidemiology and anatomical traits. The four observers knew previously that the patients had laryngomalacia and which criteria should be analyzed in the evaluation protocol. Observers were unaware of the results each one found. RESULTS: diagnostic agreement average considering all parameters evaluated was of 88.2%. DISCUSSION/CONCLUSION: Dynamic flexible nasolaryngoscopy is a proven diagnostic method, regardless of physician experience.

[Presbyvertigo as a cause of dizziness in elderly]. / Presbivertigem como causa de tontura no idoso.

BACKGROUND: dizziness is a frequent complaint in the geriatric population and has a negative impact in the life quality of these individuals. AIM: to correlate the types of dizziness and alterations in the caloric test and to verify the frequency of presbyvertigo in an elderly population with dizziness. METHOD: The records of 132 patients aged over 60 and with balance disturbance were reviewed. These patients were evaluated in the Otoneurology Service of the Clinical Hospital of the Federal University of Minas Gerais between the years of 1998 and 2007. The variables considered for analysis were: epidemiologic data, clinical history, associated diseases and the result of the caloric test. Patients with positional and central vertigo were excluded from the analysis. RESULTS: The research sample consisted of 120 patients, with an average age was of 70 years, being 71% (n=87) of sample women. Vertigo with the duration of a few minutes and of a daily frequency was the most frequent type of dizziness. In relation to the caloric test, normal results were observed in 73% of the sample. Altered results included unilateral weakness (14%), bilateral weakness (10%) and hyperactive caloric response (3%). Correlating the caloric test with the type of dizziness, bilateral weakness was associated with postural instability (p=0.006; IC=2 - 419). CONCLUSION: dizziness in elderly has many causes. Bilateral weakness of the vestibular function can be related to presbyvertigo and must be considered in aged individuals who present unbalance. Metabolic, psychiatric, disautonomic, orthopedic, visual and proprioceptive disturbances may be the cause of dizziness in aged individuals who present normal vestibular evaluation.

Elderly people with human T-cell leukemia virus type 1-associated myelopathy present an early impairment in cognitive skills / Idosos com mielopatia associada ao vírus da leucemia de células T humanas tipo 1apresentam comprometimento precoce em habilidades cognitivas

Abstract Background Cerebral changes occur in individuals with human T-cell leukemia virus type 1 (HTLV-1 )-associated myelopathy (HAM) and seem to predominate in subcortical areas. Little is known about the cognitive decline in the elderly living with HTLV-1. Objective To evaluate the cognitive aging of individuals infected with HTLV-1 aged ≥ 50 years. Methods This is a cross-sectional study of former blood donors infected with HTLV-1 who have been followed in the cohort of the Interdisciplinary Research Group on HTLV-1 since 1997. The groups of study consisted of 79 HTLV-1 infected individuals aged ≥ 50 years, with 41 of them presenting symptomatic HAM and 38 being asymptomatic carriers, and 59 seronegative individuals (controls) aged ≥ 60 years. All were submitted to the P300 electrophysiological test and neuropsychological tests. Results Individuals with HAM presented delayed P300 latency in relation to the other groups, and this latency delay increased progressively with aging. The performance of this group in the neuropsychological tests was also the worst. The HTLV-1- asymptomatic group performance was similar to that of the control group. Conclusions Individuals with HAM presented cognitive decline that progressed with aging and, although HTLV-1-asymptomatic carriers appear to present cognitive aging similar to that of healthy elderly people, concern about a subclinical cognitive impairment is warranted in this population.

Resumo Antecedentes Alterações cerebrais ocorrem em indivíduos com mielopatia associada ao vírus da leucemia de células T humanas tipo 1 (HTLV-1) (HAM) e parecem predominar em áreas subcorticais. Pouco se sabe sobre o declínio cognitivo em idosos vivendo com HTLV-1. Objetivo Avaliar o envelhecimento cognitivo de indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos. Métodos Trata-se de um estudo transversal com ex-doadores de sangue infectados pelo HTLV-1 acompanhados na coorte do Grupo Interdisciplinar de Pesquisa em HTLV-1 há 20 anos. Os grupos de estudo foram compostos por 79 indivíduos infectados pelo HTLV-1 com idade ≥ 50 anos, sendo que 41 apresentavam HAM e 38 eram portadores assintomáticos, e 59 indivíduos soronegativos (controles) com idade ≥ 60 anos. Todos foram submetidos ao teste eletrofisiológico P300 e testes neuropsicológicos. Resultados Indivíduos com HAM apresentaram atraso na latência do P300 em relação aos demais grupos, e esse atraso de latência aumentou progressivamente com o envelhecimento. O desempenho desse grupo nos testes neuropsicológicos também foi o pior. O desempenho do grupo HTLV-1- assintomático foi semelhante ao do grupo controle. Conclusão Indivíduos com HAM apresentaram declínio cognitivo que progrediu com o envelhecimento e, embora os portadores assintomáticos do HTLV-1 pareçam apresentar envelhecimento cognitivo semelhante ao dos idosos saudáveis, justificase a preocupação com um comprometimento cognitivo subclínico nessa população.