Parenting Styles and Coping Strategies in PKU Early Detected Children

Abstract Phenylketonuria (PKU) requires tight control to prevent neurocognitive impairment but reports show that patients may present mild cognitive defects related to higher impulsivity. We hypothesize that chronic intervention may influence the parents and child bonding and the child´s resources to face problems. To describe the PKU parenting styles perceived by the children (PS) and their coping strategies (CS) assessing their relationship with impulsivity, 30 early diagnosed and adequately treated PKU children and 30 non PKU aged-paired controls (CG) were compared. The Argentine Children´s Coping Questionnaire, Argentine Scale Perception of the Relationship with Parents, WISC IV Comprehension Subtest, and CPT II test were administered. PKU PS were based on control: strict to pathologic in the mother and acceptable in the father (both p<0.05 vs. CG). Children significantly sought greater support and showed less emotional control when facing conflicts. These characteristics positively correlated with maternal control r:.383 and r:.398 (both p<0.05). Impulsivity was higher in PKU (p<0.05) but didn´t associate with PS or CS. Maternal strict control wasn´t linked to the higher impulsivity found (possibly neurobiologically based). Nevertheless, if both factors are present, patients may develop a psychological and/or behavioral trait of greater dependency and impulsivity that must be considered in their follow-up.

Estilos parentales y estrategias de afrontamiento en pacientes con hipotiroidismo congénito detectado y tratado en forma temprana / Parenting styles and coping strategies among patients with early detected and treated congenital hypothyroidism

El hipotiroidismo congénito (HC) como enfermedad crónica impacta en la vinculación padres-hijo y en recursos del niño para afrontar situaciones conflictivas. Objetivos. Describir estilos parentales desde la percepción del hijo con HC y sus estrategias de afrontamiento. Población y métodos. Niños de entre 9 y 10 años con HC detectado por pesquisa neonatal y adecuadamente tratado y un grupo sin HC (grupo control). Se utilizó el cuestionario argentino de afrontamiento y la escala argentina de percepción de la relación con los padres y el subtest comprensión de la Wechsler Intelligence Scale for Children III (WISC III). Se compararon los resultados con el análisis multivariante de la varianza (multivariate analysis of variance; MANOVA, por sus siglas en inglés). Resultados. Se incluyeron 60 niños con HC; percibían a su madre con una modalidad de control estricto y a su padre con más aceptación. Buscaban mayor apoyo y se paralizaban más ante situaciones conflictivas que los 60 niños sin patología. Conclusión. Estos hallazgos podrían asociarse a mayor dependencia. Deben considerarse en la atención del HC.

Congenital hypothyroidism (CH), as any chronic disease, has an impact on the parent-child relationship and on the child's resources to cope with conflicting situations. Objectives. To describe parenting styles according to the perception of children with CH and their coping strategies. Population and methods. Children aged 9-10 years who had CH detected by newborn screening and had received adequate treatment and a group without CH (control group). The Argentine Coping Questionnaire, the Argentine Scale for the Perception of Parent Relations, and the comprehension subtest of the Wechsler Intelligence Scale for Children III (WISC III) were used. Results were compared using a multivariate analysis of variance (MANOVA). Results. Sixty children with CH were included; they perceived that their mothers exercised a strict control and that their fathers showed more acceptance. They sought more support and became paralyzed more often in conflicting situations than the 60 children without CH. Conclusion. These findings may be associated with a higher level of dependence. They should be taken into consideration in CH care.

Parenting styles and coping strategies among patients with early detected and treated congenital hypothyroidism. / Estilos parentales y estrategias de afrontamiento en pacientes con hipotiroidismo congénito detectado y tratado en forma temprana.

INTRODUCTION: Congenital hypothyroidism (CH), as any chronic disease, has an impact on the parent-child relationship and on the child's resources to cope with conflicting situations. OBJECTIVES: To describe parenting styles according to the perception of children with CH and their coping strategies. POPULATION AND METHODS: Children aged 9-10 years who had CH detected by newborn screening and had received adequate treatment and a group without CH (control group). The Argentine Coping Questionnaire, the Argentine Scale for the Perception of Parent Relations, and the comprehension subtest of the Wechsler Intelligence Scale for Children III (WISC III) were used. Results were compared using a multivariate analysis of variance (MANOVA). RESULTS: Sixty children with CH were included; they perceived that their mothers exercised a strict control and that their fathers showed more acceptance. They sought more support and became paralyzed more often in conflicting situations than the 60 children without CH. CONCLUSION: These findings may be associated with a higher level of dependence. They should be taken into consideration in CH care.

INTRODUCCIÓN: El hipotiroidismo congénito (HC) como enfermedad crónica impacta en la vinculación padres-hijo y en recursos del niño para afrontar situaciones conflictivas. OBJETIVOS: Describir estilos parentales desde la percepción del hijo con HC y sus estrategias de afrontamiento. POBLACIÓN Y MÉTODOS: Niños de entre 9 y 10 años con HC detectado por pesquisa neonatal y adecuadamente tratado y un grupo sin HC (grupo control). Se utilizó el cuestionario argentino de afrontamiento y la escala argentina de percepción de la relación con los padres y el subtest comprensión de la Wechsler Intelligence Scale for Children III (WISC III). Se compararon los resultados con el análisis multivariante de la varianza (multivariate analysis of variance; MANOVA, por sus siglas en inglés). RESULTADOS: Se incluyeron 60 niños con HC; percibían a su madre con una modalidad de control estricto y a su padre con más aceptación. Buscaban mayor apoyo y se paralizaban más ante situaciones conflictivas que los 60 niños sin patología. CONCLUSIÓN: Estos hallazgos podrían asociarse a mayor dependencia. Deben considerarse en la atención del HC.

Perfiles cognitivos en pacientes con hipotiroidismo congénito detectado y tratado en forma temprana / Cognitive profiles of patients with early detected and treated congenital hypothyroidism

Introducción. Los niños con hipotiroidismo congénito (HC) detectados por pesquisa neonatal y tratados adecuadamente presentarían defectos cognitivos leves. Objetivos. Evaluar el coeficiente intelectual de niños con HC e identificar la presencia de déficits cognitivos específicos. Población y métodos. Se seleccionó un grupo de 60 niños con HC, de entre 9 y 10 años, detectados por pesquisa neonatal y tratados adecuadamente desde el primer mes de vida, y se comparó con un grupo control de 60 niños sin HC de la misma edad. Fueron criterios de inclusión la ausencia de patología intercurrente, concurrencia a jornada escolar simple y padres con nivel escolar mínimo de secundaria completa. En entrevistas individuales, se administraron la escala de inteligencia Wechsler para niños, tercera edición, figura compleja de Rey, test Woodcock-Muñoz revisado, Conners Continuous Performance Test II, test Illinois de aptitudes psicolingüísticas, test de fluidez verbal, test de cubos de Knox, Trail Making Test, test de caras y test de los 5 dígitos. Se realizó el análisis estadístico con pruebas t de Student (muestras independientes) ajustado por Bonferroni (p < 0,002). Resultados. Aun dentro del rango normal promedio, hubo diferencias significativas entre grupos en el coeficiente intelectual total y de ejecución (tamaño del efecto pequeño y moderado, respectivamente). Los niños hipotiroideos presentaron, en el área de ejecución, significativamente menor desempeño en velocidad de procesamiento, tiempos de reacción, atención, flexibilidad cognitiva, visoconstrucción y memoria a largo plazo. No hubo diferencia significativa entre grupos en el área verbal. Conclusiones. Los niños hipotiroideos congénitos, sin discapacidad mental, presentaron defectos cognitivos leves, que deben ser tenidos en cuenta para su atención integral.

Introduction. Children with congenital hypothyroidism (CH) detected by newborn screening and adequately treated may have mild cognitive deficits. Objectives. To assess the intelligence quotient of children with CH and identify the presence of specific cognitive deficits. Population and methods. A group of 60 children with CH detected by newborn screening, who were aged 9-10 years old and received adequate treatment since their first month of life was selected and compared to a control group of 60 children without CH in the same age range. Inclusion criteria: children without concurrent diseases, who were attending school in a single shift, and whose parents had at least completed secondary education. The following tests were administered during individual interviews: the Wechsler Intelligence Scale for Children (third edition), the Rey complex figure test, the Woodcock-Muñoz revised test, the Conners Continuous Performance Test II, the Illinois Test of Psycholinguistic Abilities, the verbal fluency test, the Knox Cube Test, the Trail Making Test, the faces test, and the 5 digit test. The statistical analysis was done using Student's t tests (for independent samples) with Bonferroni's correction (p < 0.002). Results. Even within the normal average range, significant differences were observed between both groups in terms of total intelligence quotient and performance intelligence quotient (small and moderate effect sizes, respectively). In terms of performance, children with hypothyroidism had a significantly poorer performance in processing speed, reaction times, attention, cognitive flexibility, visuoconstruction, and long-term memory. No significant differences were found between both groups in the verbal area. Conclusions. Children with congenital hypothyroidism and without mental disability had mild cognitive deficits, which should be taken into account for a comprehensive patient care.

Cognitive profiles of patients with early detected and treated congenital hypothyroidism. / Perfiles cognitivos en pacientes con hipotiroidismo congénito detectado y tratado en forma temprana.

INTRODUCTION: Children with congenital hypothyroidism (CH) detected by newborn screening and adequately treated may have mild cognitive deficits. OBJECTIVES">To assess the intelligence quotient of children with CH and identify the presence of specific cognitive deficits. POPULATION AND METHODS: A group of 60 children with CH detected by newborn screening, who were aged 9-10 years old and received adequate treatment since their first month of life was selected and compared to a control group of 60 children without CH in the same age range. Inclusion criteria: children without concurrent diseases, who were attending school in a single shift, and whose parents had at least completed secondary education. The following tests were administered during individual interviews: the Wechsler Intelligence Scale for Children (third edition), the Rey complex figure test, the Woodcock-Muñoz revised test, the Conners Continuous Performance Test II, the Illinois Test of Psycholinguistic Abilities, the verbal fluency test, the Knox Cube Test, the Trail Making Test, the faces test, and the 5 digit test. The statistical analysis was done using Student's t tests (for independent samples) with Bonferroni's correction (p < 0.002). RESULTS: Even within the normal average range, significant differences were observed between both groups in terms of total intelligence quotient and performance intelligence quotient (small and moderate effect sizes, respectively). In terms of performance, children with hypothyroidism had a significantly poorer performance in processing speed, reaction times, attention, cognitive flexibility, visuoconstruction, and long-term memory. No significant differences were found between both groups in the verbal area. CONCLUSIONS: Children with congenital hypothyroidism and without mental disability had mild cognitive deficits, which should be taken into account for a comprehensive patient care.

Introducción. Los niños con hipotiroidismo congénito (HC) detectados por pesquisa neonatal y tratados adecuadamente presentarían defectos cognitivos leves. Objetivos. Evaluar el coeficiente intelectual de niños con HC e identificar la presencia de déficits cognitivos específicos. Población y métodos. Se seleccionó un grupo de 60 niños con HC, de entre 9 y 10 años, detectados por pesquisa neonatal y tratados adecuadamente desde el primer mes de vida, y se comparó con un grupo control de 60 niños sin HC de la misma edad. Fueron criterios de inclusión la ausencia de patología intercurrente, concurrencia a jornada escolar simple y padres con nivel escolar mínimo de secundaria completa. En entrevistas individuales, se administraron la escala de inteligencia Wechsler para niños, tercera edición, figura compleja de Rey, test WoodcockMuñoz revisado, Conners Continuous Performance Test II, test Illinois de aptitudes psicolingüísticas, test de fluidez verbal, test de cubos de Knox, Trail Making Test, test de caras y test de los 5 dígitos. Se realizó el análisis estadístico con pruebas t de Student (muestras independientes) ajustado por Bonferroni (p < 0,002). Resultados. Aun dentro del rango normal promedio, hubo diferencias significativas entre grupos en el coeficiente intelectual total y de ejecución (tamaño del efecto pequeño y moderado, respectivamente). Los niños hipotiroideos presentaron, en el área de ejecución, significativamente menor desempeño en velocidad de procesamiento, tiempos de reacción, atención, flexibilidad cognitiva, visoconstrucción y memoria a largo plazo. No hubo diferencia significativa entre grupos en el área verbal. Conclusiones. Los niños hipotiroideos congénitos, sin discapacidad mental, presentaron defectos cognitivos leves, que deben ser tenidos en cuenta para su atención integral.

STINGRAY: system for integrated genomic resources and analysis.

BACKGROUND: The STINGRAY system has been conceived to ease the tasks of integrating, analyzing, annotating and presenting genomic and expression data from Sanger and Next Generation Sequencing (NGS) platforms. FINDINGS: STINGRAY includes: (a) a complete and integrated workflow (more than 20 bioinformatics tools) ranging from functional annotation to phylogeny; (b) a MySQL database schema, suitable for data integration and user access control; and (c) a user-friendly graphical web-based interface that makes the system intuitive, facilitating the tasks of data analysis and annotation. CONCLUSION: STINGRAY showed to be an easy to use and complete system for analyzing sequencing data. While both Sanger and NGS platforms are supported, the system could be faster using Sanger data, since the large NGS datasets could potentially slow down the MySQL database usage. STINGRAY is available at http://stingray.biowebdb.org and the open source code at http://sourceforge.net/projects/stingray-biowebdb/.

Oral signs of intravenous chemotherapy with 5- Fluorouracil and Leucovorin calcium in colon cancer treatment

Several studies have shown how cytostatics may cause hypofunction of salivary glands but failed to elucidate any potentially related side effects. Keeping in mind the sialochemical assistance and the role of saliva on the homeostasis of the stomatognathic system, the aim of this study was to establish potential gland disorders in patients submitted to 5- Fluorouracil (5-Fu) and Leucovorin calcium(LV) as well as their correlation with certain oral health disorders that diminish the quality of life. Materials and methods: the focus of this research was observational and longitudinal. Twenty-five patients diagnosed with colon cancer at an initial, intermediate and late phase submitted to specifically devised therapy were assessed. Clinical history, oral health indexes and basal or stimulated saliva samples were recorded. Results: Basal and stimulated flow dropped in the intermediate stage. Stimulated saliva pH decreased during treatment. On basal saliva, urea, sodium and potassium rose during the intermediate phase. Löe and Silness rates as well as simplified bleeding increased during therapy but reverted by the end of the treatment. Depth index of the vestibular gingival sulcus rose during the intermediate phase but did not return. Conclusion: This treatment caused functional salivary gland disorders as evidenced by basal and stimulated hyposialia, and acidification of stimulated saliva pH during the intermediate phase. Increase in basal urea may be due to proteic catabolism arising from plasma or glands. Variation in Na+ and K+ of basal saliva concentrates might be assumed as a possible duct disorder. Recovery of bleeding and Löe and Silness rates may point to a transient inflammatory effect associated to a decrease in salivary flow. Increase in the depth rates of the periodontal vestibular sulcus could be correlated with a higher risk of periodontal disease (AU)

Oral signs of intravenous chemotherapy with 5-Fluorouracil and Leucovorin calcium in colon cancer treatment.

UNLABELLED: Several studies have shown how cytostatics may cause hypofunction of salivary glands but failed to elucidate any potentially related side effects. Keeping in mind the sialochemical assistance and the role of saliva on the homeostasis of the stomatognathic system, the aim of this study was to establish potential gland disorders in patients submitted to 5- Fluorouracil (5-Fu) and Leucovorin calcium (LV) as well as their correlation with certain oral health disorders that diminish the quality of life. MATERIALS AND METHODS: the focus of this research was observational and longitudinal. Twenty-five patients diagnosed with colon cancer at an initial, intermediate and late phase submitted to specifically devised therapy were assessed. Clinical history, oral health indexes and basal or stimulated saliva samples were recorded. RESULTS: Basal and stimulated flow dropped in the intermediate stage. Stimulated saliva pH decreased during treatment. On basal saliva, urea, sodium and potassium rose during the intermediate phase. Löe and Silness rates as well as simplified bleeding increased during therapy but reverted by the end of the treatment. Depth index of the vestibular gingival sulcus rose during the intermediate phase but did not return. CONCLUSION: This treatment caused functional salivary gland disorders as evidenced by basal and stimulated hyposialia, and acidification of stimulated saliva pH during the intermediate phase. Increase in basal urea may be due to proteic catabolism arising from plasma or glands. Variation in Na+ and K+ of basal saliva concentrates might be assumed as a possible duct disorder. Recovery of bleeding and Löe and Silness rates may point to a transient inflammatory effect associated to a decrease in salivary flow. Increase in the depth rates of the periodontal vestibular sulcus could be correlated with a higher risk of periodontal disease.

ProtozoaDB: dynamic visualization and exploration of protozoan genomes.

ProtozoaDB (http://www.biowebdb.org/protozoadb) is being developed to initially host both genomics and post-genomics data from Plasmodium falciparum, Entamoeba histolytica, Trypanosoma brucei, T. cruzi and Leishmania major, but will hopefully host other protozoan species as more genomes are sequenced. It is based on the Genomics Unified Schema and offers a modern Web-based interface for user-friendly data visualization and exploration. This database is not intended to duplicate other similar efforts such as GeneDB, PlasmoDB, TcruziDB or even TDRtargets, but to be complementary by providing further analyses with emphasis on distant similarities (HMM-based) and phylogeny-based annotations including orthology analysis. ProtozoaDB will be progressively linked to the above-mentioned databases, focusing in performing a multi-source dynamic combination of information through advanced interoperable Web tools such as Web services. Also, to provide Web services will allow third-party software to retrieve and use data from ProtozoaDB in automated pipelines (workflows) or other interoperable Web technologies, promoting better information reuse and integration. We also expect ProtozoaDB to catalyze the development of local and regional bioinformatics capabilities (research and training), and therefore promote/enhance scientific advancement in developing countries.