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Purpose: To continue the validation process of the Decoding Development Monitoring Protocol (PRADE) in software format in the validity evidence stage based on response processes., Methods: 250 individuals participated in this study, 125 individuals from private schools and 125 individuals from public schools. The assessment was carried out in person using the software that hosts the instrument's tasks, which are organized into decoding linguistically balanced words and non-words, respecting the decoding rules of Brazilian Portuguese. The software prepares an individual performance report for each participant, counting the decoding time for each stimulus, as well as the number of words decoded correctly. The data is organized considering the correct decoding time of the stimuli, decoding accuracy and percentage of correct answers. All data underwent statistical analysis using SPSS software., Results: The data indicated an important effect of the length of words and non-words on public and private school students. Furthermore, it was possible to observe the evolution of decoding, depending on the school year, in all the variables studied. In both groups, a strong influence of non-words on student performance throughout Elementary School I was observed., Conclusion: The data indicate validity in the analysis of response processes, since it was possible to adequately characterize the performance of school children public and private throughout Elementary School I, characterizing each group, as well as their differences according to the advancement of schooling.

Background: Williams-Beuren syndrome is a multisystemic disorder caused by a microdeletion of the 7q11.23 region. Although familial cases with autosomal dominant inheritance have been reported, the vast majority are sporadic., Objective: To investigate the main complaints and clinical findings of patients with Williams-Beuren syndrome., Methods: A total of 757 parents of patients registered in the Brazilian Association of Williams-Beuren Syndrome (ABSW) received a questionnaire via WhatsApp from March to July 2017., Results: In total, 229 parents answered the survey. Age of diagnosis ranged from 2 days to 34 years (median: 3 years). The main clinical findings reported by the parents were abdominal colic (83.3%), failure to thrive (71.5%), feeding difficulty in the first year (68.9%), otitis (56.6%), urinary tract infections (31.9%), precocious puberty (27.1%) and scoliosis (15.9%). Cardiac defects were present in 66% of patients, and the most frequent defect was supravalvular aortic stenosis (36%). Arterial hypertension was reported in 23%. Hypercalcemia was reported in 10.5% of patients, mainly during the first year of life. Hyperacusis and hypersociability were common complaints (both present in 89%). Other behavioral and neuropsychiatric symptoms reported by the parents included attention deficit (89%), anger crises (83%), excessive fear (66%), depression (64%), anxiety (67%) and hypersexuality (33%). The most common complaints were hypersensitivity to sounds, talkative personality, emotional dependence and learning difficulties. In 98.3%, the parents denied family history., Conclusions: Williams-Beuren syndrome requires close follow-up with different medical specialties due to their variable clinical comorbidities, including language and school learning difficulties, behavioral and psychiatric problems.

The characterization of karyotypes is an important aspect in understanding the structure and evolution of genomes. Polybia is a genus of social wasps of the family Vespidae. This genus has 58 species, but for only 8 of these chromosome number and morphology have been reported in the literature. The aim of this study was to describe and characterize the Polybia fastidiosuscula Saussure karyotype, presenting the first case of a B chromosome in Vespidae. In addition, we investigated the chromatin composition of this species through C-banding, base-specific fluorochrome staining, and physical mapping of 7 microsatellites and 18S rDNA. Four colonies of P. fastidiosuscula from Minas Gerais and Paraná states, Brazil, were analyzed. The chromosome number identified was 2n = 34, and 2 colonies presented a B chromosome. We characterized the chromatin composition of this species, analyzing the existence of different microsatellite-rich heterochromatic regions which are also enriched with AT or GC base pairs. We suggest an intraspecific origin of the B chromosome based on the homology of the heterochromatic composition with A chromosomes and also verify that the TTAGG and TCAGG sequences are not telomeric, but only microsatellites that occur in the centromeres of most chromosomes, as well as GAG and CGG., (© 2021 S. Karger AG, Basel.)

Fungus-farming ants of the genus Mycetophylax exhibit intra and interspecific chromosome variability, which makes them suitable for testing hypotheses about possible chromosomal rearrangements that endure lineage diversification. We combined cytogenetic and molecular data from Mycetophylax populations from coastal environments to trace the evolutionary history of the clade in light of chromosomal changes under a historical and geographic context. Our cytogenetic analyses revealed chromosomal differences within and among species. M. morschi exhibited three distinct karyotypes and considerable variability in the localization of 45S rDNA clusters. The molecular phylogeny was congruent with our cytogenetic findings. Biogeographical and divergence time dating analyses estimated that the most recent common ancestor of Mycetophylax would have originated at about 30 Ma in an area including the Amazon and Southern Grasslands, and several dispersion and vicariance events may have occurred before the colonization of the Brazilian Atlantic coast. Diversification of the psammophilous Mycetophylax first took place in the Middle Miocene (ca. 18-10 Ma) in the South Atlantic coast, while "M. morschi" lineages diversified during the Pliocene-Pleistocene transition (ca. 3-2 Ma) through founder-event dispersal for the Northern coastal regions. Psammophilous Mycetophylax diversification fits into the major global climatic events that have had a direct impact on the changes in sea level as well as deep ecological impact throughout South America. We assume therefore that putative chromosomal rearrangements correlated with increased ecological stress during the past climatic transitions could have intensified and/or accompanied the divergence of the psammophilous Mycetophylax. We further reiterate that "M. morschi" comprises a complex of at least three well-defined lineages, and we emphasize the role of this integrative approach for the identification and delimitation of evolutionary lineages.

Background: Cri du Chat syndrome (CdCS) is a genetic syndrome caused by deletions in the short arm of chromosome 5. Although the main clinical features of CdCS are well known, the neurocognitive and behavioural characteristics of the phenotype are rarely described in detail in the literature. In this study, we analysed the main phenotypic features of CdCS from a parental perspective., Method: A questionnaire was sent to 700 Brazilian families that were registered in the Brazilian Association of CdCS. The questions involved specific domains of CdCS, such as pregnancy and birth conditions, recurrence of the disease in the family, current major health problems, and aspects of cognitive development., Results: In total, 73 questionnaires were completed: 44 females and 29 males, ranging from 9.5 months old to 40 years old (mean = 13.8 years; median = 12 years). Most of the parents noticed the typical cat-like cry at birth (94.4%). The age at diagnosis of CdCS ranged from the time of birth to 180 months (mean = 14 months; median = 6 months), while one case was diagnosed during pregnancy. In all of the cases, the diagnosis of CdCS was made by G-banding karyotype analysis. In 66.2% of the cases, the parents underwent cytogenetic investigation. A total of 52.1% of the parents answered that they did not remember what the recurrence risk of CdCS was in their family. The main health problems that were reported were as follows: swallowing problems (80.3%), feeding problems (80.3%), congenital heart disease (31.5%), spine abnormalities (28.8%), and neurological symptoms (20.5%), including seizures (11%). The behavioural problems that were reported were as follows: aggressive behaviour, stereotypies, anxiety, phobias, and genital manipulation/masturbation. Neurodevelopmental delay was reported in all of the cases. Independent walking was achieved in 72.2% of the patients. Approximately 50% of the patients never presented expressive language, and most of the patients are dependent on others for their daily activities., Conclusions: The questionnaire was a pioneer initiative in the CdCS support group, and the answers used in this study can improve the health care assistance to these patients because they focus attention on the demands from a parental perspective. In addition, nearly half of the families stated that they did not remember information regarding recurrence risk, which reinforces the importance of genetic counselling follow-up and the need for the expansion of genetic services in Brazil., (© 2018 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.)

The assessment of language skills in early childhood can provide important information about the future of literacy and academic performances. Children with reading difficulties should be identified early in their education, before they suffer from shortcomings and experience failures and feel discouraged at school. Considering the importance of early identification of language disorders and the shortage of standardized instruments for the Brazilian scenario, the overall objective of this study was to translate and adapt the Dyslexia Early Screening Test - Second Edition (DEST-2) to, subsequently, verify its applicability and efficacy in preschoolers who had Brazilian Portuguese as their native language. The study was composed of 20 children of both genders, regularly enrolled in a public school in the metropolitan region of São Paulo, none had any complaints related to learning and no indicators of sensory, neurological, cognitive, or behavioral disorders. It was observed that there was no need for significant changes to the original structure of the DEST-2 or in their administration instructions format. The performance of the children in the translated and in the national exams that were used as a benchmark was compatible, suggesting that the adjustments made met the equivalences needed to utilize this instrument with Brazilian children. A randomized study that will complement the preliminarily data obtained is in progress. Taking into consideration the linguistic and cultural diversity of Brazil, it is imperative that the translated version of the DEST-2 can be applied on a large scale and in several states of the country, in order to allow the use of this instrument as a language assessment tool in Brazil.

Purpose: To compare the occurrence of speech disfluencies during narrative production in children with specific language impairment (SLI) and their age-matched peers., Methods: The study included 60 children aged between 7 and 10 years, 40 with typical language development and 20 with SLI. For data collection, a series of 15 stories was used, each one represented by pictures composed of four scenes. Narratives were transcripted and the speech disfluencies presented on them were classified as stuttering-like disfluencies (part-word repetition, single-syllable word repetition, and dysrhythmic phonation - prolongations, blocks and broken words) or other disfluencies (interjection, revision/abandoned utterances, and multisyllable/phrase repetition). The disfluency categories were compared in each group and its occurrence was also compared between groups., Results: The occurrence of stuttering-like and other disfluencies did not differ among children with typical language development, whereas children with SLI produced other disfluencies. Between-group comparison showed that children with SLI produced more disfluencies of both types than their age-matched peers., Conclusion: Children with SLI showed more speech disfluencies during narrative production than their age-matched peers, and the most common disfluencies used by them were not typical of people who stutter (interjection, revision/abandoned utterances, and multisyllable/phrase repetition).

We determined the sensitivity, specificity and efficiency of speech-evoked Auditory Brainstem Response (ABR) as a diagnostic support for Auditory Processing Disorder (APD) and specific language impairment (SLI). Speech-evoked ABRs were elicited using the five-formant syllable/da/. The waveforms V, A, C, D, E, F, and O of all groups were analyzed. The sensitivity and specificity were calculated, and receiver operating characteristic analyses were performed to determine the optimum cut-off. Seventy-five children who were native speakers of Brazilian-Portuguese participated. The participants included 25 children with APD, 25 children with SLI and 25 with typical development. Statistical analysis demonstrated a cut-off for latency values of 6.48, 7.51, 17.82, 22.33, 30.79, 39.54 and 48.00 for V, A, C, D, E, F, and O waves, respectively. The A wave exhibited superior balance for the APD group. For the SLI group, the A, D and O waves exhibited the best balance. Furthermore, when analyzing the APD and SLI groups separately, better sensitivity values were observed for the SLI group than the APD group. Speech-evoked ABR is a useful test to identify auditory processing disorders and language impairment. Furthermore, this study represented an important step forward in establishing the clinical utility of speech-evoked ABR in Brazilian Portuguese-speaking children., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Purposes: To translate and adapt the Clinical Evaluation of Language Functions--4th Edition (CELF-4) to Brazilian Portuguese., Method: One hundred and sixty normal language development school children between the ages of seven and ten, half from public schools and the other half from private schools, both located on the east side of São Paulo., Results: CELF-4's translation and adjustment to Brazilian Portuguese language showed equivalence between the original and translated versions, which demonstrates that there were no significant changes in the test's form and content. Cronbach's α test was used in order to verify CELF-4's subtests internal consistency, in other words, if every subtest measures consistently the evaluated constructors. In this analysis, we observed that by excluding right or wrong items, and problematic items from the pool (those different from the rest of the group), all analyzed subtest presented satisfactory internal consistency, except for the Word Association Task for eight years old., Conclusion: Most subtests, as well as the Pragmatic Profile and the Observational Evaluation Scale, were simply translated, dismissing significant adaptations. The alterations performed were due to morphosyntactic and phonological differences between both languages. CELF-4's translated and adapted version to Brazilian Portuguese was able to characterize the language performance in the studied population.

This cross-cultural study investigates the impact of background experience on four verbal and visuo-spatial working memory (WM) tasks. A total of 84 children from low-income families were recruited from the following groups: (1) Portuguese immigrant children from Luxembourg impoverished in terms of language experience; (2) Brazilian children deprived in terms of scholastic background; (3) Portuguese children from Portugal with no disadvantage in either scholastic or language background. Children were matched on age, gender, fluid intelligence, and socioeconomic status and completed four simple and complex span tasks of WM and a vocabulary measure. Results indicate that, despite large differences in their backgrounds and language abilities, the groups exhibited comparable performance on the visuo-spatial tasks dot matrix and odd-one-out and on the verbal simple span task digit recall. Group differences emerged on the verbal complex span task counting recall with children from Luxembourg and Portugal outperforming children from disadvantaged schools in Brazil. The study suggests that whereas contributions of prior knowledge to digit span, dot matrix, and odd-one-out are likely to be minimal, background experience can affect performance on counting recall. Implications for testing WM capacity in children growing up in poverty are discussed.

Purpose: The purpose of this study was to establish a profile of the spelling patterns studied in students from public and private schools and to describe a word spelling task for clinical and educational settings., Methods: Eighty-two fourth grade students belonging to the elementary school of public and private schools in São Paulo, ranging in age from nine to ten years, took part in this study. The spelling task consisted of a list of ten high frequency words (HFW), ten low frequency words (LFW), and ten pseudowords (PW), in which the typology and number of spelling errors were described. To compare the average number of mistakes on the HFWs, LFWs, and PWs, we used an analysis of variance and Tukey's multiple comparisons (p<0.05). Using a cluster analysis, homogeneous groups were formed based on their performance., Results: Results indicated that the average number of mistakes in the LFWs was higher than in the HFWs (p=0.000) and PWs (p=0.000), and the number of mistakes in the HFWs was lower than in the PWs (p=0.009). The highest number of mistakes was found in the following categories: "others", "rule generalization", "omission", "voiced-voiceless", and "addition". There were no mistakes of the type "ão-am" and "blend-separation" in the HFWs., Conclusion: Spelling errors are a part of the process of learning to write, and students can show some variance in spelling performance. Furthermore, students need to be stimulated to analyze words and their aspects of phonology, morphology, and semantics. An analysis from the types of errors is not enough to plan intervention programs, but instead is necessary to understand the strategies that the child uses to write.

Purpose: To investigate the conflict resolution abilities of children with Specific Language Impairment, and to verify whether the time of speech-language therapy correlates to the performance on the conflict resolution task., Methods: Participants included 20 children with Specific Language Impairment (Research Group) and 40 children with normal language development (Control Group), with ages ranging from 7 years to 8 years and 11 months. To assess the conflict resolution abilities, five hypothetical contexts of conflict were presented. The strategies used by the children were classified and scored by the following levels: level 0 (solutions that do not match the other levels), level 1 (physical solutions), level 2 (unilateral solutions), level 3 (cooperative solutions), and level 4 (mutual solutions)., Results: Statistical analysis showed group effect for the variable total score. There was a difference between the groups for modal development level, with higher level of modal development observed in the Control Group. There was no correlation between the period of speech-language therapy attendance and the total score., Conclusion: Children with Specific Language Impairment present difficulties in solving problems, in view of the fact that they mainly use physical and unilateral strategies. There was no correlation between the time of speech-language therapy and performance in the task.

Purpose: In this study, the authors examined the comprehension of sentences with predicates and reflexives that are linked to a nonadjacent noun as a test of the hierarchical ordering deficit (HOD) hypothesis. That hypothesis and more modern versions posit that children with specific language impairment (SLI) have difficulty in establishing nonadjacent (hierarchical) relations among elements of a sentence. The authors also tested whether additional working memory demands in constructions containing reflexives affected the extent to which children with SLI incorrectly structure sentences as indicated by their picture-pointing comprehension responses., Method: Sixteen Brazilian Portuguese-speaking children (8;4-10;6 [years;months]) with SLI and 16 children with typical language development (TLD) matched for age (± 3 months), gender, and socioeconomic status participated in 2 experiments (predicate and reflexive interpretation). In the reflexive experiment, the authors also manipulated working memory demands. Each experiment involved a 4-choice picture selection sentence comprehension task., Results: Children with SLI were significantly less accurate on all conditions. Both groups made more hierarchical syntactic construction errors in the long working memory condition than in the short working memory condition., Conclusion: The HOD hypothesis was not confirmed. For both groups, syntactic factors (structural assignment) were more vulnerable than lexical factors (prepositions) to working memory effects in sentence miscomprehension.

Objective: This study aimed to examine drug packaging and labeling, identifying similarities among them that may lead to medication errors, which may occur by unintentional substitution, in different sectors of the pharmacy of a university hospital in northeastern Brazil., Methods: Cross-sectional observational study, which included 300 pharmaceutical presentations (150 pairs) that were photographed from May to December 2010. Concordance analysis of data related to the pictures of possibly similar packaging and labels was validated using the Kappa index., Results: Of all drugs evaluated (n = 150), about 43% of "possibly similar drugs" were in the central pharmacy (n = 65) and were related to small-volume parenteral solutions. The strength of interobserver agreement in the category "very similar to each other" was considered "satisfactory" (Kappa = 0584) in 90.66% of the drugs evaluated (n = 136). The overall Kappa analysis of the study was 0.488. Variables with statistical significance were: "same color label or packaging", with the respective percentages for both primary and secondary packaging (52%-44%), p = 0.028; the variable "same color of drug presentation" obtained similar values and statistical significance to the previous variable; for the variable "same arrangement of words", the values found for both packages were close to 50%, p = 0.001; and for the variable "same color of the words", the percentages were: (50.7% - 44%) (p = 0.008)., Conclusion: Our results indicate similarities related to the labeling of drugs with potential for errors, especially in dispensing, storage, and administration if preventive measures are not adopted.

Objectives: To analyze the association between resting heart rate and blood pressure in male children and adolescents and to identify if this association is mediated by important confounders., Study Design: Cross-sectional study carried out with 356 male children and adolescents from 8 to 18 years old. Resting heart rate was measured by a portable heart rate monitor according to recommendations and stratified into quartiles. Blood pressure was measured with an electronic device previously validated for pediatric populations. Body fatness was estimated by a dual-energy x-ray absorptiometry., Results: Obese subjects had values of resting heart rate 7.8% higher than nonobese (P = .001). Hypertensive children and adolescents also had elevated values of resting heart rate (P = .001). When the sample was stratified in nonobese and obese, the higher quartile of resting heart rate was associated with hypertension in both groups of children and adolescents., Conclusions: This study confirms the existence of a relationship between elevated resting heart rate and increased blood pressure in a pediatric population, independent of adiposity, ethnicity and age., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Background: The narrative abilities provide valuable information about the linguistic, cognitive and social development of school-age children with typical language development (TLD)., Aim: To examine the temporal ordering of figures and the narrative abilities of school-age children with TLD in relation to the type of discourse presented., Method: Participants of this study included 60 children with TLD aged between seven and ten years of age. Fifteen stories were used in the study. Each story was illustrated by four figures. The sequences of figures were created and classified as mechanical, behavioral and intentional according to the relationships presented among the characters. Data were transcribed and analyzed regarding the type of discourse (descriptive, causal and intentional) and the type of organization of the figures., Results: No differences between age groups were observed for temporal ordering. For all age groups, the most frequently presented discourse type was the causal one. Statistically significant differences were observed among age groups for the causal and intentional discourse type. Inasmuch as the age increased, school-age children with TLD reduced the use of the descriptive discourse and increased the use of the intentional one., Conclusion: The ability of temporal ordering is already developed in children with TLD at seven-years of age. The type of discourse was influenced by age and by the type of story presented.

Background: verb acquisition., Aim: to verify quantitative and qualitative verb diversity in the spontaneous speech of Brazilian Portuguese-speaking preschoolers and verb acquisition from 2 to 4 years of age., Method: participants were sixty preschoolers equally matched on gender and age, divided into three groups: GI (2-year-old children), GII (3-year-old children) and GIII (4-year-old children). Spontaneous speech samples obtained through playing interactions, in educational contexts, were collected, and verb productions were listed., Results: children used 167 different verbs. The quantitative analysis indicated significant statistical differences between all groups (p< .001), with gradual increase of verb usage from 2 to 4 years of age. However, no statistical difference (p= .956) was observed between genders., Conclusion: preschoolers improved their use of verbs during the initial stages of language acquisition, irrespective of their gender.

Background: Phonological working memory and typical language development in children., Aim: To describe and discuss findings of researches concerning the assessment of phonological working memory in normally developing children undertaken since the 1980's., Aim: To present a systematic literature review regarding the phonological working memory and its relationship with normal language development., Method: A systematic review of the literature on phonological working memory and its relationship with language development skills of normal children was made. The used material involved books, monographs, thesis, dissertations and articles stored in Lilacs, Pubmed, Scielo and Medline databases. An analysis was made considering the applied tests and the results regarding the effects of extension and age and the relationship of these variables with phonological working memory for speakers of English and the Brazilian Portuguese language., Conclusion: According to the consulted literature, studies indicate a relationship between the phonological and lexical knowledge and the phonological working memory in children with normal language development.

Background: translation and cross-cultural adaptation of instruments to the Brazilian Portuguese language., Aim: in Brazil, in the area of Speech-Language Pathology, there is a significant lack of commercially available formal and objective instruments that have as a purpose the evaluation and diagnosis of different communication disorders. Some researchers have tried to ease this problem by translating instruments available in other languages. Such procedure also contributes to the development of cross-cultural studies, which can bring knowledge about the communication disorders and their specificities in different languages. The aim of the present study is to discuss the procedures that should be adopted in this process., Conclusion: it is fundamental to adopt appropriate methodological procedures when translating and adapting foreign assessment instruments.

Background: application of an American early language test in Brazilian Portuguese-speaking subjects., Aim: to compare the performance of normal Brazilian Portuguese and American English-speaking subjects on the Test of Early Language Development - Third Edition (TELD-3)., Method: participants of this study were 120 subjects of both genders, ranging in age from 2:00 to 7:11 years (20 subjects in each age range). Subjects were selected and tested individually by the researcher. A comparative analysis of the subjects' performances with that observed in English-speaking children was made., Results: the results indicated that Brazilian and American subjects presented similar performances until the age of 4:11 years. Portuguese-speaking children of 5, 6 and 7 years presented higher scores on the receptive subtest of Form A when compared to their American pairs. Portuguese-speaking subjects of 5 and 6 years presented lower scores on the expressive subtest of Form B when compared to their American pairs., Conclusion: the translation of diagnostic instruments that are already available in other languages can lessen the lack of such resources in countries where these instruments are not yet available. Furthermore, this practice can allow cross-cultural studies which are relevant for researches on child language and language disabilities.

8 patients with bone marrow failure after a caesium-137 radiation accident were treated with recombinant human granulocyte-macrophage colony stimulating factor (rHuGM-CSF). The 7 who were evaluable had prompt increases in granulocytes and bone marrow cellularity. 2 patients died of radiation toxicity and haemorrhage and 2 of bacterial sepsis acquired before the start of rHuGM-CSF treatment. 4 patients survive, including 2 who were treated early and never became infected. This therapeutic approach to radiation-induced granulocytopenia may therefore be useful after radiation and nuclear accidents.