| 11. |
- Lovén, Jakob, et al.
(författare)
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MYCN-regulated microRNAs repress estrogen receptor-alpha (ESR1) expression and neuronal differentiation in human neuroblastoma.
- 2010
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Ingår i: Proceedings of the National Academy of Sciences of the United States of America. - : Proceedings of the National Academy of Sciences. - 0027-8424 .- 1091-6490. ; 107:4, s. 1553-8
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Tidskriftsartikel (refereegranskat)abstract
- MYCN, a proto-oncogene normally expressed in the migrating neural crest, is in its amplified state a key factor in the genesis of human neuroblastoma (NB). However, the mechanisms underlying MYCN-mediated NB progression are poorly understood. Here, we present a MYCN-induced miRNA signature in human NB involving the activation and transrepression of several miRNA genes from paralogous clusters. Several family members derived from the miR-17 approximately 92 cluster, including miR-18a and miR-19a, were among the up-regulated miRNAs. Expression analysis of these miRNAs in NB tumors confirmed increased levels in MYCN-amplified samples. Specifically, we show that miR-18a and miR-19a target and repress the expression of estrogen receptor-alpha (ESR1), a ligand-inducible transcription factor implicated in neuronal differentiation. Immunohistochemical staining demonstrated ESR1 expression in human fetal sympathetic ganglia, suggesting a role for ESR1 during sympathetic nervous system development. Concordantly, lentiviral restoration of ESR1 in NB cells resulted in growth arrest and neuronal differentiation. Moreover, lentiviral-mediated inhibition of miR-18a in NB cells led to severe growth retardation, outgrowth of varicosity-containing neurites, and induction of neuronal sympathetic differentiation markers. Bioinformatic analyses of microarray data from NB tumors revealed that high ESR1 expression correlates with increased event-free survival in NB patients and favorable disease outcome. Thus, MYCN amplification may disrupt estrogen signaling sensitivity in primitive sympathetic cells through deregulation of ESR1, thereby preventing the normal induction of neuroblast differentiation. Collectively, our findings demonstrate the molecular consequences of abnormal miRNA transcription in a MYCN-driven tumor and offer unique insights into the pathology underlying MYCN-amplified NB.
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| 12. |
- Nisell, Margret, et al.
(författare)
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The Imperforate Anus Psychosocial Questionnaire (IAPSQ) : its construction and psychometric properties.
- 2009
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Ingår i: Child and Adolescent Psychiatry and Mental Health. - London : Springer Science and Business Media LLC. - 1753-2000. ; 3:1, s. article number: 15-
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Tidskriftsartikel (refereegranskat)abstract
- The origin of the present study was to develop the liaison work between the disciplines of child and adolescent psychiatry and paediatric surgery and nursing, so as to improve the quality of treatment and care of a group of children with imperforate anus (IA) and their families. Imperforate anus is a congenital disease involving a deformity of the anorectum. The early surgery and invasive follow-up treatment associated with IA may affect the child psychosocially, including the child-parent relationship. By developing and testing a questionnaire for children born with anorectal anomalies, a tool for measuring psychosocial functioning can be realized. METHODS: First, a literature review on "Imperforate Anus" was performed. Second, an exploratory interview study was conducted with patients/adolescents with IA and their parents. The findings from these interviews were the foundation for construction of the questionnaire. The Imperforate Anus Psychosocial Questionnaire (IAPSQ) was tested and revised three times before its completion. It contains 45 items on Likert scales. A total of 87 children completed the IAPSQ: 25 children with IA and two comparison groups. Face and content validity were considered. The Rasch approach, an item response theory model, was used to evaluate the psychometric properties of the IAPSQ, where item difficulty and person ability are concurrently approximated. RESULTS: The findings of the Rasch analysis revealed that the psychological dimension was reasonable, and that person reliability (0.83) was moderate and item reliability (0.95) was sufficient. The social dimension showed satisfactory item reliability (0.87). The person reliability (0.52) of the social dimension was weak. Content validity seemed to be established and construct validity was recognized on the psychological dimension. CONCLUSION: The IAPSQ provides a reasonably valid and reliable measure of psychosocial functioning for clinical use among children with IA, although some revisions are suggested for the next version of the IAPSQ. By using the Rasch model, we discovered that specific items should be discarded and other items should be reformulated to make the questionnaire more "on target". The social dimension has to be expanded with further items to reasonably capture a social dimension.
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| 13. |
- Olsson, Gunilla Maria, et al.
(författare)
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The Adolescent Adjustment Profile (AAP) in comparisons of patients with obesity, phenylketonuria or neurobehavioural disorders
- 2008
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Ingår i: Nordic Journal of Psychiatry. - : Informa UK Limited. - 0803-9488 .- 1502-4725. ; 62:1, s. 66-76
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Tidskriftsartikel (refereegranskat)abstract
- Psychosocial development in children with chronic disease is a key issue in paediatrics. This study investigated whether psychosocial adjustment could be reliably assessed with the 42-item Adolescent Adjustment Profile (AAP) instrument. The study mainly focused on adjustment-to-obesity measurement, although it compared three patient groups with chronic conditions. All phenylketonuria (PKU) patients in Sweden between ages 9 and 18 and their parents and teachers were invited to participate. Patients with neurobehavioural syndromes and obesity were age- and gender-matched with PKU patients. Healthy children constituted a reference group. Psychosocial adjustment was measured using the AAP, which is a multi-informant questionnaire that contains four domains. Information concerning parents' socio-economic and civil status was requested separately. Respondents to the three questionnaires judged the PKU patients to be normal in all four domains. Patients with neurobehavioural syndromes demonstrated less competence and the most problems compared with the other three groups. According to the self-rating, the parent rating and the teacher rating questionnaires, obese patients had internalizing problems. The parent rating and the teacher rating questionnaire scored obese patients as having a lower work capacity than the reference group. Compared with the reference group, not only families with obese children but also families with children with neurobehavioural syndromes had significantly higher divorce rates. Obese patients were also investigated with the Strength and Difficulties Questionnaire (SDQ), another instrument that enables comparison between two measures of adjustment. The AAP had good psychometric properties; it was judged a useful instrument in research on adolescents with chronic diseases.
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| 14. |
- Strondl, Annika, et al.
(författare)
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Characterisation and control of powder properties for additive manufacturing
- 2014
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Konferensbidrag (övrigt vetenskapligt/konstnärligt)abstract
- Powder characterization and handling in PM are important issues and required powder properties will vary between different component manufacturing processes. By understanding and controlling these, the final material properties for different applications can be improved and become more reliable. In this study, metal powders used in additive manufacturing have been investigated regarding particle size and shape using dynamic image analysis. In parallel, powder flow characteristics have been evaluated with a powder rheometer. Correlations within the results have been found between particle shape and powder flow characteristic that could explain certain effects of the powder processing in the additive manufacturing processes.
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| 15. |
- Strondl, Annika, et al.
(författare)
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Characterization and Control of Powder Properties for Additive Manufacturing
- 2015
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Ingår i: JOM. - : Springer Science and Business Media LLC. - 1047-4838 .- 1543-1851. ; 67:3, s. 549-554
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Tidskriftsartikel (refereegranskat)abstract
- Powder characterization and handling in powder metallurgy are important issues and the required powder properties will vary between different component manufacturing processes. By understanding and controlling these, the final material properties for different applications can be improved and become more reliable. In this study, the metal powders used in additive manufacturing (AM) in terms of electron beam melting and selective laser melting have been investigated regarding particle size and shape using dynamic image analysis. In parallel, powder flow characteristics have been evaluated with a powder rheometer. Correlations within the results have been found between particle shape and powder flow characteristics that could explain certain effects of the powder processing in the AM processes. The impact, however, in the processing performance as well as in ultimate material properties was found to be limited.
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| 16. |
- Wigander, Helena, et al.
(författare)
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Quality of life and functional outcome in Swedish children with low anorectal malformations : a follow-up study
- 2019
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Ingår i: Pediatric surgery international (Print). - : Springer. - 0179-0358 .- 1437-9813. ; 35:5, s. 583-590
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: The aim was to investigate the quality of life and bowel function in children with low anorectal malformations (ARM).ADDITIONAL AIM: To evaluate the Swedish version the Hirschsprung's Disease/Anorectal Malformation Quality of life Questionnaire (HAQL).METHODS: Forty-four children and their parents were invited to complete the HAQL and the Bowel Function Score (BFS). Healthy children participated as controls and completed the HAQL.RESULTS: Seventeen children and 18 mothers completed the HAQL. The children reported impaired function in the physical symptom (PH) fecal continence (FC) and laxative diet (LD) domains compared to controls. Compared with their mothers, they reported impaired physical function and more symptoms in the emotional functioning (EMF) and PH domains. 27 families completed the BFS; 63% reported normal bowel function, 33% moderate outcome and one patient, comprising 4%, poor outcome. Evaluation of the HAQL, FC, EMF and PH domains showed no obvious conflicts.CONCLUSIONS: The children did not differ much regarding their QoL, even though they appeared to have impaired bowel function and worse emotional functioning compared to controls. The mothers underestimated their children's physical symptoms and overestimated their emotional functioning. Evaluated domains in the HAQL appear to work as intended, but the questionnaire needs further development.
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| 17. |
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