Evaluación y tratamiento motor de una niña con diagnóstico de mielomeningocele y artritis idiopática juvenil en un hospital púbico pediátrico. Reporte de un caso / Evaluation and motor treatment of a girl diagnosed with myelomeningocele and juvenile idiopathic arthritis in a public pediatric hospital. A case report

RESUMEN Introducción: El diagnóstico de mielomeningocele asociado con otra patología es muy poco frecuente. Sólo se encontró publicado un reporte de caso de un niño con mielomeningocele, parálisis cerebral y artritis idiopática juvenil (AIJ). El objetivo de este reporte es describir la evaluación y el tratamiento de terapia física (TF) en una niña con diagnóstico de mielomeningocele y AIJ, en un hospital público pediátrico. Presentación del caso: Niña con diagnóstico prenatal de mielomeningocele, que, con un año y tres meses de edad, comenzó seguimiento en la institución en noviembre de 2015. A los cinco años, se presentó con dolor, calor, aumento de tamaño y deformidad de codos, muñecas y rodillas de un año de evolución, por lo que fue evaluada por el servicio de reumatología, solicitando estudios complementarios para diagnóstico diferencial. En julio del 2021, se confirmó el diagnóstico de AIJ, iniciando tratamiento farmacológico y de TF adaptada, con lo que alcanzó una mejoría en la actividad articular, la capacidad funcional y su calidad de vida. Conclusión: Se describió la evolución de una niña con mielomeningocele, que fue diagnosticada de AIJ a los cinco años e inició tratamiento farmacológico y de TF adaptada, presentando mejoría en la actividad articular, capacidad funcional y su calidad de vida.

ABSTRACT Introduction: The diagnosis of myelomeningocele associated with another pathology is not frequent. Only one case report of a child with myelomeningocele, cerebral palsy, and juvenile idiopathic arthritis (JIA) was found. The objective of this study is to describe the evaluation and physical therapy (PT) treatment of a girl diagnosed with myelomeningocele and JIA in a public pediatric hospital. Case presentation: A girl diagnosed prenatally with myelomeningocele started follow-up in our institution at the age of 1 year and 3 months in November 2015. At the age of 5 years, she presented with one-year history of pain, heat sensation, and increased size and deformity of elbows, wrists, and knees. She was evaluated by the rheumatology service in 2020. Imaging and laboratory studies were requested for a differential diagnosis. In July 2021, the diagnosis of JIA was confirmed. She began pharmacological treatment and adapted PT, with an improvement in her joint activity, functional capacity, and quality of life. Conclusion: This case report describes the progress of a girl with myelomeningocele diagnosed with JIA at the age of 5 years. She began pharmacological treatment and adapted PT, with an improvement in her joint activity, functional capacity, and quality of life.

Validación del Cuestionario pediátrico de calidad de vida™ (Pediatric Quality of Life Inventory™) Módulo neuromuscular, versión 3. 0 en español para Argentina / Validation of the Pediatric Quality of Life Inventory™, Neuromuscular Module, version 3. 0 in Spanish for Argentina

Objetivo: Evaluar las propiedades psicométricas del Cuestionario pediátrico de calidad de vida (PedsQLTM 3.0) Módulo Neuromuscular, versión en español para Argentina, en niños entre 2 y 18 años con enfermedades neuromusculares. Población y métodos: Estudio observacional, analítico, prospectivo, de validación, realizado en el Hospital Garrahan entre el 19 de marzo de 2019 y el 9 de marzo de 2020. A los 10-15 días se realizó el retest del cuestionario para validar en los pacientes que reportaron estabilidad. Resultados: Participaron 185 niños y sus padres. Sobre la factibilidad de la herramienta, los participantes comprendieron fácilmente su contenido. La confiabilidad resultó aceptable, con una consistencia interna de 0,82 en niños y 0,87 en padres y un coeficiente de correlación intraclase en el retest de 0,70 en niños y 0,82 en familiares. Sobre la validez del constructo se confirmaron 8 de 11 hipótesis establecidas (72,7 %). Conclusión: El cuestionario fue validado en sus propiedades psicométricas

Objective: To assess the psychometric properties of the Pediatric Quality of Life Inventory™ (PedsQL™ 3.0), Neuromuscular Module, version in Spanish for Argentina, for children aged 2-18 years with neuromuscular disease. Population and methods: Observational, analytical, prospective validation study conducted in Hospital Garrahan between March 19th, 2019 and March 9th, 2020. The retest questionnaire was administered 10-15 days later to validate it among patients who reported a stable condition. Results: A total of 185 children and their parents participated. In terms of the questionnaire's feasibility, its content was easily understood by participants. Its reliability was acceptable, with an internal consistency of 0.82 among children and 0.87 among parents and a retest intraclass correlation coefficient of 0.70 among children and 0.82 among parents. In relation to the construct validity, 8 of the 11 hypotheses established (72.7 %) were confirmed. Conclusion: The questionnaire's psychometric properties were validated.

Validation of the Pediatric Quality of Life Inventory™, Neuromuscular Module, version 3.0 in Spanish for Argentina. / Validación del Cuestionario pediátrico de calidad de vida™ (Pediatric Quality of Life Inventory™) Módulo neuromuscular, versión 3.0 en español para Argentina.

OBJECTIVE: To assess the psychometric properties of the Pediatric Quality of Life Inventory™ (PedsQL™ 3.0), Neuromuscular Module, version in Spanish for Argentina, for children aged 2-18 years with neuromuscular disease. POPULATION AND METHODS: Observational, analytical, prospective validation study conducted in Hospital Garrahan between March 19th, 2019 and March 9th, 2020. The retest questionnaire was administered 10-15 days later to validate it among patients who reported a stable condition. RESULTS: A total of 185 children and their parents participated. In terms of the questionnaire's feasibility, its content was easily understood by participants. Its reliability was acceptable, with an internal consistency of 0.82 among children and 0.87 among parents and a retest intraclass correlation coefficient of 0.70 among children and 0.82 among parents. In relation to the construct validity, 8 of the 11 hypotheses established (72.7 %) were confirmed. CONCLUSION: The questionnaire's psychometric properties were validated.

Objetivo: Evaluar las propiedades psicométricas del Cuestionario pediátrico de calidad de vida (PedsQLTM 3.0) Módulo Neuromuscular, versión en español para Argentina, en niños entre 2 y 18 años con enfermedades neuromusculares. Población y métodos: Estudio observacional, analítico, prospectivo, de validación, realizado en el Hospital Garrahan entre el 19 de marzo de 2019 y el 9 de marzo de 2020. A los 10-15 días se realizó el retest del cuestionario para validar en los pacientes que reportaron estabilidad. Resultados: Participaron 185 niños y sus padres. Sobre la factibilidad de la herramienta, los participantes comprendieron fácilmente su contenido. La confiabilidad resultó aceptable, con una consistencia interna de 0,82 en niños y 0,87 en padres y un coeficiente de correlación intraclase en el retest de 0,70 en niños y 0,82 en familiares. Sobre la validez del constructo se confirmaron 8 de 11 hipótesis establecidas (72,7 %). Conclusión: El cuestionario fue validado en sus propiedades psicométricas.

How Long does it Take to Examine Young Children with the Caries ICDAS System and how do they Respond?

A caries-epidemiological study using the ICDASepi-merged system was conducted in Colombian young children. This study aimed at associating the time needed for the clinical examination of caries and caries risk in 1 to 5-year-old children according to age and caries risk, and to assess behavior and child pain self-perception during examination according to age. After IRB approval and given parents/caregivers' informed consent, seven trained examiners assessed 1 to 5-year olds in kindergartens under local field conditions. ICDASepi-merged caries experience (depiMEmf) was assessed as follows: Initial-depi (ICDAS 1/2 without air-drying); Moderate-dM (ICDAS 3,4); Extensive-dE (ICDAS 5,6) lesions; due-to-caries fillings-f and missing-m surfaces/teeth. Caries risk was assessed with Cariogram®. Child's behavior (Frankl-Behavior-Rating-Scale) and self-perceived pain (Visual-Analogue-Scale-of-Faces) during examination were evaluated. Clinical examination time was recorded with a stopwatch. A total of 592 children participated (1-yr.: n=31; 2-yrs.: n=96; 3-yrs.: n=155; 4-yrs.: n=209, 5-yrs.: n=101). The depiMEmfs prevalence was of 79.9% and the mean 8.4±10.4. Most were high-caries-risk children (68.9%). The majority (58.9%) showed ≥ positive-behavior and ≤ light-pain self-perception (88.4%). Mean clinical examination time was around 3.5 minutes (216.9±133.9 seconds). For 5-yr. olds it corresponded to 4 minutes (240.4±145.0 seconds) vs. 2 minutes (122.8±80.1 seconds) for 1-yr. olds (Kruskal-Wallis; p=0.00). For high- and low-caries risk children it was around 4.3 minutes (255.7±118.5 seconds) and 3.3 minutes (201.3±129.4 seconds), respectively (ANOVA; p=0.01). This study demonstrates using the ICDAS system in young children is feasible, taking less than 4 minutes for the clinical examination without children behavior/pain self-perception issues.

How long does it take to examine young children with the caries icdas system and how do they respond?

Abstract A caries-epidemiological study using the ICDASepi-merged system was conducted in Colombian young children. This study aimed at associating the time needed for the clinical examination of caries and caries risk in 1 to 5-year-old children according to age and caries risk, and to assess behavior and child pain self-perception during examination according to age. After IRB approval and given parents/caregivers' informed consent, seven trained examiners assessed 1 to 5-year olds in kindergartens under local field conditions. ICDASepi-merged caries experience (depiMEmf) was assessed as follows: Initial-depi (ICDAS 1/2 without air-drying); Moderate-dM (ICDAS 3,4); Extensive-dE (ICDAS 5,6) lesions; due-to-caries fillings-f and missing-m surfaces/teeth. Caries risk was assessed with Cariogram®. Child's behavior (Frankl-Behavior-Rating-Scale) and self-perceived pain (Visual-Analogue-Scale-of-Faces) during examination were evaluated. Clinical examination time was recorded with a stopwatch. A total of 592 children participated (1-yr.: n=31; 2-yrs.: n=96; 3-yrs.: n=155; 4-yrs.: n=209, 5-yrs.: n=101). The depiMEmfs prevalence was of 79.9% and the mean 8.4±10.4. Most were high-caries-risk children (68.9%). The majority (58.9%) showed ≥ positive-behavior and ≤ light-pain self-perception (88.4%). Mean clinical examination time was around 3.5 minutes (216.9±133.9 seconds). For 5-yr. olds it corresponded to 4 minutes (240.4±145.0 seconds) vs. 2 minutes (122.8±80.1 seconds) for 1-yr. olds (Kruskal-Wallis; p=0.00). For high- and low-caries risk children it was around 4.3 minutes (255.7±118.5 seconds) and 3.3 minutes (201.3±129.4 seconds), respectively (ANOVA; p=0.01). This study demonstrates using the ICDAS system in young children is feasible, taking less than 4 minutes for the clinical examination without children behavior/pain self-perception issues.

Resumo Um estudo epidemiológico de cárie usando o sistema ICDAS foi realizado em crianças pequenas colombianas. O objetivo deste estudo foi associar o tempo necessário para o exame clínico da cárie e o risco de cárie em crianças de 1 a 5 anos de acordo com a idade e o risco de cárie e avaliar a autopercepção do comportamento e da dor na criança durante o exame, de acordo com a idade. Após a aprovação do comitê de ética e do consentimento informado dos pais/responsáveis, sete examinadores treinados avaliaram crianças de 1 a 5 anos em creches em condições locais de campo. A experiência de cárie do ICDAS (depiMEmf) foi avaliada da seguinte forma: Epi-depi inicial (ICDAS 1/2 sem secagem ao ar); Moderado-dM (ICDAS 3,4); lesões extensas de dE (ICDAS 5,6); restaurações devido a cárie -f e superfícies/dentes ausentes-m. O risco de cárie foi avaliado com Cariogram®. O comportamento de crianças (Frankl-Behavior-Rating-Scale) e a autopercepção de dor (Escala Visual-Analógica-de-Rostos) durante o exame foram avaliados. O tempo de exame clínico foi registrado com um cronômetro. 592 crianças participaram (1 ano: n = 31; 2 anos: n = 96; 3 anos: n = 155; 4 anos: n = 209, 5 anos: n = 101 ). A prevalência do depiMEmfs foi de 79,9% e a média de 8,4 ± 10,4. A maioria era de crianças com alto risco de cárie (68,9%). A maioria (58,9%) apresentou ≥ comportamento positivo e ≤ autopercepção de dor leve (88,4%). O tempo médio de exame clínico foi em torno de 3,5 min (216,9 ± 133,9 s). Para crianças de 5 anos, correspondeu a 4 min (240,4 ± 145,0 s) vs. 2 min (122,8 ± 80,1 s) para crianças de 1 ano de idade (Kruskal-Wallis; p = 0,00). Para crianças com alto e baixo risco de cárie, foi em torno de 4,3 min (255,7 ± 118,5 s) e 3,3 min (201,3 ± 129,4 s), respectivamente (ANOVA; p = 0,01). Este estudo demonstra que a utilização do sistema ICDAS em crianças pequenas é viável, levando menos de 4 min para o exame clínico sem problemas de autopercepção de comportamento/ dor em crianças.

Differential reactivity of salivary igA and igG against streptococcus mutans proteins in humans with different caries experience.

Dental caries is an infectious disease which still constitutes a public health concern. It begins at an early age and is caused mainly Streptococcus mutans (S. mutans). The aim of this study was to characterize the salivary humor immune response to S. mutans proteins in patients with caries, with history of caries and without caries, in order to determine which S. mutans proteins participate in the immunological response in subjects with different caries experience. Saliva was collected by spontaneous salivation for 5 minutes from 60 subjects aged 18 to 30 years, classified according to their caries experience as: without caries (Group I), with active caries (Group II) and with history of caries (Group III). The antigens derived from S. mutans by sonication were recognized by salivary IgA and IgG by Western Blot. The results showed that all the individuals studied recognized S. mutans proteins with molecular weights in the range of 8 to 191 kDa, with similar recognition profiles for salivary IgA and IgG. Subjects without caries recognized the 29 kDa protein, also known as S. mutans Antigen A, via salivary IgA, differing from patients with caries and history of caries, who recognized it via IgG. The protective response against S. mutans is mediated by IgA. To conclude, a differential response to the 29 kDa protein between study individuals may be indicative of resistance to dental caries and may have a protective role in the induction of IgA antibodies against dental caries, as found in the group without caries, in contrast to subjects with active caries and history of caries.

Differential reactivity of salivary igA and igG against streptococcus mutans proteins in humans with different caries experience

Dental caries is an infectious disease which still constitutes a public health concern. It begins at an early age and is caused mainly Streptococcus mutans (S. mutans). The aim of this study was to characterize the salivary humor immune response to S. mutans proteins in patients with caries, with history of caries and without caries, in order to determine which S. mutans proteins participate in the immunological response in subjects with different caries experience. Saliva was collected by spontaneous salivation for 5 minutes from 60 subjects aged 18 to 30 years, classified according to their caries experience as: without caries (Group I), with active caries (Group II) and with history of caries (Group III). The antigens derived from S. mutans by sonication were recognized by salivary IgA and IgG by Western Blot. The results showed that all the individuals studied recognized S. mutans proteins with molecular weights in the range of 8 to 191 kDa, with similar recognition profiles for salivary IgA and IgG. Subjects without caries recognized the 29 kDa protein, also known as S. mutans Antigen A, via salivary IgA, differing from patients with caries and history of caries, who recognized it via IgG. The protective response against S. mutans is mediated by IgA. To conclude, a differential response to the 29 kDa protein between study individuals may be indicative of resistance to dental caries and may have a protective role in the induction of IgA antibodies against dental caries, as found in the group without caries, in contrast to subjects with active caries and history of caries.

La caries dental es una enfermedad infecciosa que continua siendo un problema de salud publica, inicia a temprana edad y es causada principalmente por Streptococcus mutans (S. mutans). El objetivo de este estudio fue caracterizar la respuesta inmune humoral salival, ante las proteinas de S. mutans, en pacientes con caries, historia de caries e individuos libres de caries, para asi establecer que proteinas de S. mutans participan en la respuesta inmunologica en los diferentes estadios de caries. La saliva de 60 individuos entre 18 y 30 anos de edad, clasificados de acuerdo al estado de caries: libres de caries (grupo I), caries activa (grupo II) e historia de caries (grupo III), se colecto por salivacion espontanea durante 5 minutos. Los antigenos derivados de S. mutans por sonicacion, fueron reconocidos por IgA e IgG salivales por Western Blot. Los resultados mostraron que todos los individuos estudiados reconocen las proteinas de S. mutans en el rango de 8 a 191 kDa de peso molecular con perfiles de reconocimiento similares para IgA e IgG salival. Se encontro que los sujetos libres de caries reconocen por IgA salival la proteina de 29 kDa, tambien llamada Antigeno A de S. mutans, de manera diferente que los pacientes con caries e historia de caries quienes reconocieron la proteina via IgG. La respuesta protectora frente a S. mutans es mediada por IgA. En conclusion, una respuesta diferencial a la proteina de 29 kDa entre los individuos estudiados, puede ser indicativo de resistencia a la caries dental y tener un papel protector en la induccion de anticuerpos IgA frente a la caries dental, como se encontro en el grupo libre de caries, a diferencia de los sujetos con historia de caries y caries activa.

Cytokines produced by CD4+ T cells against a synthetic GTF-I(1301-1322) peptide of Streptococcus mutans in naturally sensitized humans.

Streptococcus mutans (S. mutans) is the main etiological agent in dental caries. Its virulence factors are the proteins PAc and glucosyltransferase (GTF), which are related to its physiopathogenia and have been used in research for a dental caries vaccine. It was reported that using experimental animal models, GTF-I(1301-1322) synthetic peptide from the GLU region of the GTFs has Tepitopes, induces production of serum antibodies in saliva and reduces the presence of caries, but little is known about the cellular response in naturally sensitized humans. The aim of this study was to observe whether GTF-I(1301-1322) peptide is capable of activating CD4+ T cells in PBMC from naturally sensitized humans, to classify the response and to establish the relationship with dental caries. The study was conducted on 30 individuals classified into the following 3 groups: active caries (AC), History of Caries (HC), and free of caries (H). A blood sample was drawn from each individual. Specific antigen stimulation and flow cytometry analyses were used to determine cells producing the cytokines IFN-gamma (type 1 cytokine) and IL-13 (type 2 cytokine). Cell memory response to GTF-I(1301-1322) peptide was found in naturally sensitized humans. Three different responses were detected: TH0, TH1, and NR. The percentage of CD4+ T cells producing the cytokines IFN-gamma (type 1 cytokine) was greater than the percentage producing IL-13 (p=0.006). No statistically significant differences were found among the three groups for the other variables studied (p < or = 0.05). In conclusion, specific cellular immune responses against the GTF-I(1301-1322) peptide of S. mutans does not differ between individuals who are naturally sensitized, caries- resistant or with caries.