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1.
Orphanet J Rare Dis ; 17(1): 444, 2022 12 21.
Artículo en Inglés | MEDLINE | ID: mdl-36544157

RESUMEN

BACKGROUND/AIM: Despite optimal conventional treatment (oral phosphate supplements and active vitamin D analogs), about 40-50% of children with well-controlled X-linked hypophosphatemia (XLH) show linear growth failure, making them less likely to achieve an acceptable final height. Here, we studied the hypothesis that rhGH treatment improves final height in children with XLH and growth failure. METHODS: Two cohorts of children with XLH were included in this retrospective longitudinal analysis: (1) a cohort treated with rhGH for short stature (n = 34) and (2) a cohort not treated with rhGH (n = 29). The mean duration of rhGH treatment was 4.4 ± 2.9 years. We collected the auxological parameters at various time points during follow-up until final height. RESULTS: In rhGH-treated children, 2 years of rhGH therapy was associated with a significant increase in height from - 2.4 ± 0.9 to - 1.5 ± 0.7 SDS (p < 0.001). Their mean height at rhGH discontinuation was - 1.2 ± 0.9 SDS and at final height was - 1.3 ± 0.9 SDS corresponding to 165.5 ± 6.4 cm in boys and 155.5 ± 6.3 cm in girls. Notably, the two groups had similar final heights; i.e., the final height in children not treated with rhGH being - 1.2 ± 1.1 SDS (165.4 ± 6.8 cm in boys and 153.7 ± 7.8 cm in girls), p = 0.7. CONCLUSION: Treatment with rhGH permits to improve final height in children with XLH and growth failure, despite optimal conventional treatment. We propose therefore that rhGH therapy could be considered as an option for short stature in the context of XLH.


Asunto(s)
Enanismo , Raquitismo Hipofosfatémico Familiar , Hormona de Crecimiento Humana , Niño , Femenino , Humanos , Masculino , Estatura , Enanismo/tratamiento farmacológico , Raquitismo Hipofosfatémico Familiar/tratamiento farmacológico , Trastornos del Crecimiento/tratamiento farmacológico , Hormona del Crecimiento/uso terapéutico , Hormona de Crecimiento Humana/uso terapéutico , Estudios Retrospectivos
2.
PLoS One ; 14(10): e0223358, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31613880

RESUMEN

The majority of cognitive bias research has been conducted in Western cultures. We examined cross-cultural differences in cognitive biases, comparing Westerners' and East Asians' performance and acculturation following migration to the opposite culture. Two local (UK, Hong Kong) and four migrant (short-term and long-term migrants to each culture) samples completed culturally validated tasks measuring attentional and interpretation bias. Hong Kong residents were more positively biased than people living in the UK on several measures, consistent with the lower prevalence of psychological disorders in East Asia. Migrants to the UK had reduced positive biases on some tasks, while migrants to Hong Kong were more positive, compared to their respective home counterparts, consistent with acculturation in attention and interpretation biases. These data illustrate the importance of cultural validation of findings and, if replicated, would have implications for the mental health and well-being of migrants.


Asunto(s)
Cognición/fisiología , Cultura , Aculturación , Adulto , Atención , Sesgo , Emigración e Inmigración , Emociones , Asia Oriental , Femenino , Hong Kong , Humanos , Masculino , Test de Stroop , Análisis y Desempeño de Tareas , Reino Unido , Adulto Joven
3.
Neuroimage Clin ; 12: 940-948, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27995059

RESUMEN

Working memory ability matures through puberty and early adulthood. Deficits in working memory are linked to the risk of onset of neurodevelopmental disorders such as schizophrenia, and there is a significant temporal overlap between the peak of first episode psychosis risk and working memory maturation. In order to characterize the normal working memory functional maturation process through this critical phase of cognitive development we conducted a systematic review and coordinate based meta-analyses of all the available primary functional magnetic resonance imaging studies (n = 382) that mapped WM function in healthy adolescents (10-17 years) and young adults (18-30 years). Activation Likelihood Estimation analyses across all WM tasks revealed increased activation with increasing subject age in the middle frontal gyrus (BA6) bilaterally, the left middle frontal gyrus (BA10), the left precuneus and left inferior parietal gyri (BA7; 40). Decreased activation with increasing age was found in the right superior frontal (BA8), left junction of postcentral and inferior parietal (BA3/40), and left limbic cingulate gyrus (BA31). These results suggest that brain activation during adolescence increased with age principally in higher order cortices, part of the core working memory network, while reductions were detected in more diffuse and potentially more immature neural networks. Understanding the process by which the brain and its cognitive functions mature through healthy adulthood may provide us with new clues to understanding the vulnerability to neurodevelopmental disorders.


Asunto(s)
Envejecimiento , Encéfalo/fisiología , Memoria a Corto Plazo/fisiología , Adolescente , Desarrollo del Adolescente , Adulto , Encéfalo/crecimiento & desarrollo , Mapeo Encefálico , Niño , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Vías Nerviosas/crecimiento & desarrollo , Vías Nerviosas/fisiología , Adulto Joven
4.
J Pers Disord ; 29(1): 71-8, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24932872

RESUMEN

Borderline personality disorder (BPD) often goes unrecognized, and therefore a short but accurate screening tool is desired. The present study investigated the psychometric properties of the 10-item McLean Screening Instrument for BPD (MSI-BPD) in 159 well-diagnosed female participants. The MSI-BPD showed excellent internal consistency (α = .90). When compared to BPD diagnoses based on a structured clinical interview (SCID-II), the MSI-BPD showed substantial congruent validity (receiver operating characteristic area under the curve = 0.96). The cutoff point proposed by the developers of the MSI-BPD (7 or more) showed high specificity (.96) and good sensitivity (.71). The optimal cutoff point in the present study (5 or more) showed somewhat lower specificity (.86), but importantly better sensitivity (.94). Taken together, the Dutch version of the MSI-BPD demonstrated good psychometric properties for a screening tool.


Asunto(s)
Trastorno de Personalidad Limítrofe/diagnóstico , Determinación de la Personalidad/estadística & datos numéricos , Psicometría/estadística & datos numéricos , Encuestas y Cuestionarios , Adolescente , Adulto , Trastorno de Personalidad Limítrofe/psicología , Femenino , Humanos , Persona de Mediana Edad , Países Bajos , Escalas de Valoración Psiquiátrica , Pruebas Psicológicas , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Factores Socioeconómicos , Adulto Joven
5.
Psychiatry Res ; 219(3): 513-7, 2014 Nov 30.
Artículo en Inglés | MEDLINE | ID: mdl-25060832

RESUMEN

In depression, approach deficits often impair everyday social functioning, but empirical findings on performance-based measurements of approach-avoidance behavior remain conflicting. To investigate action tendencies in patients with depression, the current study used an explicit version of the Approach-Avoidance Task (AAT). In this task, participants responded to emotional faces by either pulling a joystick toward (approach) or pushing it away from themselves (avoid). Reaction times to happy and angry expressions with direct and averted gaze were assessed in 30 patients with major depressive disorder and 20 matched healthy controls. In contrast to healthy individuals, depressed patients did not show approach-avoidance tendencies, i.e., there was no dominant behavioral tendency and they reacted to happy and angry expressions likewise. These results indicate that behavioral adjustments to different emotional expressions, gaze directions or motivational demands were lacking in depression. Crucially, this distinguishes depressed patients not only from healthy individuals, but also from other clinical populations that demonstrate aberrant approach-avoidance tendencies, e.g., patients with social anxiety or psychopathy. As responding flexibly to different social signals is integral to social interactions, the absence of any social motivational tendencies seems maladaptive, but may also provide opportunities for modifying action tendencies in a therapeutic context.


Asunto(s)
Trastorno Depresivo Mayor/psicología , Emociones , Expresión Facial , Conducta Social , Adulto , Ira , Conducta , Depresión , Femenino , Felicidad , Humanos , Relaciones Interpersonales , Masculino , Persona de Mediana Edad , Motivación , Tiempo de Reacción , Adulto Joven
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