COVID-19 and Sickle Cell Disease in the Province of Quebec, Canada: Outcomes after Two Years of the Pandemic.

BACKGROUND: Patients with sickle cell disease (SCD) are considered at higher risk of severe COVID-19 infection. However, morbidity and mortality rates are variable among countries. To date, there are no published reports that document outcomes of SCD patients with COVID-19 in Canada. METHODS: A web-based registry was implemented in June 2020 capturing outcomes of SCD patients with COVID-19 from March 2020 to April 2022 and comparing them to the general population of Quebec, Canada. RESULTS: After 24 months of the pandemic, 185 SCD patients with confirmed SARS-CoV-2 infection were included in the registry. Overall, the population was young (median age 12 years old) and had few comorbidities. No deaths were reported. Risk of hospitalization and admission to intensive care unit (ICU) because of COVID-19 was higher in patients with SCD than in the general population (relative risks (RR) 5.15 (95% confidence interval (95% CI) 3.84-6.91), p ˂ 0.001 and 4.56 (95% CI 2.09-9.93) p ˂ 0.001). A history of arterial hypertension or acute chest syndrome in the past 12 months was associated with a higher risk of severe disease (RR = 3.06 (95% CI 1.85-5.06) p = 0.008 and 2.27 (95% CI 1.35-3.83) p = 0.01). Hospitalized patients had lower hemoglobin F than non-hospitalized patients (12% vs. 17%, p = 0.02). For those who had access to vaccination at the time of infection, 25 out of 26 patients were adequately vaccinated and had mild disease. CONCLUSIONS: The SCD population is at higher risk of severe disease than the general population. However, we report favorable outcomes as no deaths occurred. Registries will continue to be critical to document the impact of novel COVID-19 specific therapy and vaccines for the SCD population.

Verbal and Nonverbal Mismatch Negativity in Children with Typical Development: Variables Analysis.

Introduction Mismatch negativity (MMN) is a promising instrument for the investigation of different auditory disorders, as it does not need behavioral responses. Objective To analyze the influence of the ear, gender and age variables in the MMN in children with typical development; and to compare the different measures of this potential, using verbal and nonverbal stimuli in the sample studied, providing reference values. Methods Observational, descriptive, cross-sectional, quantitative study, with 23 children, aged from 5 to 11 years and 11 months old, divided by age group. Mismatch negativity was performed using verbal and nonverbal stimuli, and the data was analyzed by means of the statistical Student t -test. Results No significant differences were noted for the ear, gender and age variables in the MMN with both stimuli. There were significant differences for the latency, duration and area variables when the stimuli were compared. The reference values established for nonverbal stimuli were: latency 249.8 milliseconds, amplitude 2.28 µv, duration 82.97 milliseconds, and area 137.3 microvolt x microseconds (µVx µs); as for the verbal stimuli, they were: latency 265.3 milliseconds, amplitude - 2.82 µv, duration 110.5 milliseconds, and area 225.5 microvolt x microseconds (µVx µs). Conclusion The variables studied did not influence the recordings of the MMN. Latency, duration and area of the MMN with verbal stimuli were higher. It was possible to furnish reference values for children with typical development in the age group studied.

Molecular-based and antibody-based targeted pharmacological approaches in childhood acute lymphoblastic leukemia.

Introduction: Despite the significant survival improvement in childhood acutelymphoblastic leukemia (ALL), 15-20% of patients continue to relapse; outcomes following relapse remain suboptimal and have room for further improvement. Advances in genomics have shed new insights on the biology of ALL, led to the discovery of novel genomically defined ALL subtypes, refined prognostic significance and revealed new therapeutic vulnerabilities.Areas covered: In this review, the authors provide an overview of the genomic landscape of childhood ALL and highlight recent advances in molecular-based and antibody-based pharmacological approaches in the treatment of childhood ALL, from emerging preclinical evidence to published results of completed clinical trials.Expert opinion: Molecularly targeted therapies and immunotherapies have expanded the horizons of ALL therapy and represent promising therapeutic avenues for high-risk and relapsed/refractory ALL. These novel therapies are now moving into frontline ALL therapy and may define new treatment paradigms that aim to further improve survival and reduce chemotherapy-related toxicities in the management of pediatric ALL.

Mismatch Negativity in children with Phonological Disorders.

OBJECTIVE: to characterize the latency, amplitude and area variables of the Mismatch Negativity (MMN) elicited with verbal stimuli in children with PD, in addition to assessing whether this potential can be a useful tool in capturing auditory perception and discrimination deficits related to this disorder. METHODS: MMN was recorded using a combination of speech contrast consisting of acoustic syllables [da vs ta], as the standard and deviant stimuli, in 34 children aged between 5 and 8 years. 14 children of the sample were already diagnosed with Phonological Disorder (PD) while 19 were characterized with typical development. RESULTS: No statistically significant differences were observed for the MMN responses recorded between children with PD and their typically developed peers. CONCLUSION: The results suggest that the MMN may not be the most suitable procedure to assess auditory perception and discrimination deficits that could potentially be related to PD.

An unusual abdominal wall mass in a child.

Abdominal tumors are one of the most common types of pediatric cancer. Therefore, they should always be included in the differential diagnosis of abdominal masses. Here, we present the case of a child whose initial hypothesis of diagnosis contemplated this possibility. Later, it was demonstrated that the abdominal mass found was secondary to a common parasitosis. A 2-year old, moderately malnourished and pale white boy was referred with a history of a rapidly growing, well-limited, middle abdominal mass. The mass was 10 by 3 cm, hard and poorly movable, apparently involving both abdominal rectus muscles. A complete resection was performed, revealing an abdominal wall abscess, with intense eosinophilic proliferation, secondary to a local and intense reaction to innumerous Ascaris lumbricoides eggs. Extra luminal infestations with Ascaris, that usually form peritoneal granulomas have been previously described. However, neither external trauma nor fistula, that could explain the superficial presence of the eggs, was found. This description reinforces the relevance of infectious diseases within the differential diagnosis of abdominal masses, particularly in areas with high prevalence of parasitic infestations.

Potencial evocado auditivo de longa latência em crianças com desenvolvimento típico / Long-latency auditory potential in children with typical development / Potencial evocado auditivo de larga latencia en niños con desarrollo típico

Introdução: O Potencial Evocado Auditivo de Longa Latência é utilizado para avaliar o processamento cortical da informação acústica. Objetivo: Descrever e comparar os valores de latência e amplitude do Potencial Evocado Auditivo de Longa Latência em crianças. Além disso, fornecer valores de referência. Material e Método: Estudo transversal e quantitativo, no qual foram avaliados 20 indivíduos com idade entre 5 e 9 anos e 11 meses, com desenvolvimento típico. Realizou-se tal potencial auditivo, com o equipamento Smart EP (Intelligent Hearing Systems), com estímulos verbais utilizando fones de inserção. Teste de Wilcoxon e teste de Mann-Whitney foram utilizados. Resultados: Não houve diferença estatisticamente significante em relação aos valores de latência e amplitude dos componentes deste potencial, considerando as variáveis orelha e gênero. Forneceram-se os valores de referência para latência e amplitude dos componentes deste potencial. Conclusão: A partir deste estudo foi possível propor valores de referência para os componentes deste potencial para a amostra estudada, considerando que não houve diferença estatisticamente significante para as variáveis estudadas.

Introduction: The Long Latency auditory evoked potential is used to evaluate the cortical processing of the acoustic information. Objective: To describe and compare the values of latency and amplitude of the Long Latency Evoked Potential in children. Also, to provide reference values. Material and Method: Quantitative and cross-sectional study. 20 individuals were evaluated with ages between 5:00 -9:11 with typical development. Evoked Potential was performed with the equipment Smart Ep (Intelligent Hearing Systems) with verbal stimuli using insert headphones. Wilcoxon test and Mann-Whitney test were used. Results: There were no statistical significant differences between latency values and components amplitude of this potential, considering variables of ear and gender. The following reference values for components latency and amplitude potential were obtained. Conclusion: By this study, it was possible to propose values of reference for the components of the potential into the sample, considering that no statistically significance difference was found in the variables studied.

Introducción: El Potencial Evocado Auditivo de Larga Latencia es usado para evaluar el procesamiento cortical de la información acústica. Objetivo: Describir y comparar los valores de latencia y amplitud del Potencial Evocado Auditivo de Larga Latencia en niños. Además, proporcionar valores de referencia. Material y método: Estudio transversal y cuantitativo, en el cual fueron evaluados 20 individuos con edad entre 5 a 9 años y 11 meses, con desarrollo típico. El potencial auditivo se evaluó con el equipo Smart EP(Intelligent Hearing Systems), con estímulos verbales utilizando auriculares de inserción. La prueba de Wilcoxon y la prueba de Mann-Whitneyfueron utilizadas. Resultados: No hubo diferencia estadísticamente significativa en relación a los valores de latencia y amplitud de los componentes de este potencial, considerando las variables oreja y género. Se han proporcionado los valores de referencia para la latencia y la amplitud de los componentes de este potencial. Conclusión: A partir de este estudio fue posible proponer valores de referencia para los componentes de este potencial para la muestra estudiada, considerando que no hubo diferencia estadísticamente significativa para las variables estudiadas.

An unusual abdominal wall mass in a child

ABSTRACT Abdominal tumors are one of the most common types of pediatric cancer. Therefore, they should always be included in the differential diagnosis of abdominal masses. Here, we present the case of a child whose initial hypothesis of diagnosis contemplated this possibility. Later, it was demonstrated that the abdominal mass found was secondary to a common parasitosis. A 2-year old, moderately malnourished and pale white boy was referred with a history of a rapidly growing, well-limited, middle abdominal mass. The mass was 10 by 3 cm, hard and poorly movable, apparently involving both abdominal rectus muscles. A complete resection was performed, revealing an abdominal wall abscess, with intense eosinophilic proliferation, secondary to a local and intense reaction to innumerous Ascaris lumbricoides eggs. Extra luminal infestations with Ascaris, that usually form peritoneal granulomas have been previously described. However, neither external trauma nor fistula, that could explain the superficial presence of the eggs, was found. This description reinforces the relevance of infectious diseases within the differential diagnosis of abdominal masses, particularly in areas with high prevalence of parasitic infestations.

Audiometria de reforço visual em lactentes nascidos a termo e pré-termo: nível mínimo de resposta / Visual reinforcement audiometry in infants who born term and pre-term: minimum response level / Audiometría de refuerzo visual en infantes nacidos a término y pre ­ término: nivel mínimo de respuesta

Objetivo: analisar os Níveis Mínimos de Respostas (nmrs) em lactentes, nascidos a termo e pré-termo e investigar a relação entre os nmrs e os diferentes Indicadores de Risco para Deficiência Auditiva (irdas). Método: a amostra foi composta por 114 lactentes que apresentaram resultado "passa" na Triagem Auditiva Neonatal (TAN), de seis a oito meses de idade, distribuídos em dois grupos conforme a idade gestacional. Grupo Termo: 75 lactentes. Grupo Pré-termo: 39 lactentes. Realizou-se a Audiometria de Reforço Visual (ARV) com o audiômetro pediátrico, buscando os nmrs. Resultados: não houve diferença estatisticamente significante entre NMR em relação ao lado de apresentação. Os nmrs em 2 khz e 4 khz no Grupo Termo foram menores nos bebês do gênero feminino. Ao comparar nmrs dos dois grupos, não houve diferença estatisticamente significante. Os maiores nmrs apareceram entre os lactentes que apresentaram os seguintes IRDA: permanência na Unidade de Terapia Intensiva (UTI), uso de medicação ototóxica, ventilação mecânica e uso de fumo pela mãe durante o período gestacional. Conclusão: não houve diferença estatisticamente significante nos nmrs em relação ao lado de apresentação dos estímulos e entre os grupos. Observou-se que os lactentes do gênero feminino, do Grupo Termo, apresentaram menores nmrs nas frequências de 2KHz e 4KHz. Alguns irdas influenciaram no desempenho dos lactentes na ARV.

Objective: To analyze the Minimum Response Level (MRL) in infants, born term and preterm, considering some variables. In addition, to investigate the relationship between MRL and different Risk Indicators for Hearing Loss (RIHL). Methods: The sample consisted of 114 infants who had result "pass" in Newborn Hearing screening, from six to eight months old, distributed into two groups according to gestational age. Term group: 75 infants and Pre-term group: 39 infants. The Visual Reinforcement Audiometry (VRA) was made with a pediatric audiometer, seeking the MRL. Results: There was no statistically significant difference in the MRL regarding the side of the presentation. The MRL's in 2 khz and 4 khz in the Term Group were lower in female babies. Comparing the MRL between the two groups there was not statistically significant difference. The higher MRL's are among the infants with the following RIHL: Intensive care unit (ICU) hospitalization, use of ototoxic drugs, mechanical ventilation and tobacco use by the mother during pregnancy. Conclusion: There was no statistically significant difference in the MRL regarding the side of the presentation of the stimuli and between groups. It was observed that the female infants of the term group had lower mrls in the frequencies of 2KHz and 4KHz. Some RIHL influenced the performance of infants in the VRA.

Evaluar los Nivel Mínimo de Respuesta(NMRs) en infantes, nacidos a término y pretérmino, teniendo en cuenta algunas variables. Además de investigar la relación entre el NMR y diferentes indicadores de riesgo para la pérdida auditiva(IRPAs). Metodos: La muestra fue constituida por 114 infantes que aprobaran el la Screeening Auditivo para Recién (SARN), com seis a ocho meses, divididos en dos grupos según la edad gestacional. Grupo Término: 75 infantes y Grupo pre-término: 39 infantes. La Audiometría de Refuerzo Visual (ARV) se realizó con el audiómetro pediátrico, buscando el NMR. Resultados: No hubo diferencia estadísticamente significativa entre NMR en relación al lado de la presentación. El NMR en 2 KHz y 4 KHz en el Grupo Término fueron inferiores en bebés de sexo femenino. Al comparar el NMR entre los dos grupos no hubo diferencia estadísticamente significativa. Los infantes que presentaron los IRPA: estancia en la unidad de terapia intensiva (UTI), el uso de medicamentos ototóxicos, la ventilación mecánica y el uso de tabaco por la madre durante el embarazo presentaron mayor NMRs. Conclusión: No hubo diferencia estadísticamente significativa en los NMRs en relación al lado de la presentación de los estímulos y entre los grupos. Se observó que los infantes del sexo femenino del Grupo Término tenían NMRs más bajos en la frecuencia de 2 KHz y 4 KHz. Algunos IRPAs influyen en el rendimiento de los infantes en la ARV.

Resolução temporal em idosos / Temporal resolution in elderly

RESUMO Objetivo: detectar a sensibilidade, para avaliar a habilidade de resolução temporal, comparando os testes Randon Gap Detection Test (RGDT) e Gaps- In-Noise (GIN) além de sugerir valores de referência nestes testes para idosos. Métodos: participaram 38 idosos, 24 mulheres e 14 homens, com idades entre 60 e 82 anos, com audição normal ou perda auditiva neurossensorial até moderada; com simetria entre as orelhas; timpanograma tipo A, reflexos acústicos presentes, com queixa de processamento auditivo. Todos Foram submetidos a avaliação básica para caracterizar a audição periférica, RGDT e GIN. Resultados: a sensibilidade encontrada para o teste RGDT na identificação da habilidade de resolução temporal foi de 88,64% e no GIN de 67,65%. Os valores médios para o limiar de detecção de gap no teste GIN encontraram-se em torno de 8ms e para o teste RGDT em 23,13ms. Conclusão: o teste RGDT demonstrou maior sensibilidade para detecção da alteração da habilidade de resolução temporal. Os valores dos limiares de resolução temporal, sugeridos como valores de referência para idosos, são de 8ms para o GIN e 23,13 ms para o RGDT.

ABSTRACT Purpose: detecting the sensitivity to evaluate the temporal resolution ability, by comparing Randon Gap Detection Test (RGDT) and Gaps- In-Noise (GIN) tests, in addition to suggesting reference values in these tests for elderly people. Methods: 38 elderly people, 24 women and 14 men, aged between 60 and 82 years, with normal hearing or sensorineural to moderate hearing loss; with symmetry between the ears; Type A tympanogram, acoustic reflex, with auditory processing complaints. All patients underwent basic evaluations to characterize the peripheral hearing - RGDT and GIN. Results: the sensitivity found for RGDT regarding the identification of the temporal resolution ability was 88.64% and 67.65% in GIN. The mean values for the gap detection thresholds in GIN test were around 8ms and for the RGDT test in 23,13ms. Conclusion: RGDT test presented greater sensitivity for detecting the change in temporal resolution ability. The values of temporal resolution thresholds suggested as reference values for the elderly people, are 8ms for GIN and 23.13 ms for RGDT.