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RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study
BACKGROUND: Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childhood and adolescence is influenced by endocrine and hypothalamic long-term sequelae. A legal status of the degree of disability (GdB), according...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9164938/ http://dx.doi.org/10.1093/neuonc/noac079.030 |
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author | Boekhoff, Svenja Beckhaus, Julia Eveslage, Maria Friedrich, Carsten Müller, Hermann L |
author_facet | Boekhoff, Svenja Beckhaus, Julia Eveslage, Maria Friedrich, Carsten Müller, Hermann L |
author_sort | Boekhoff, Svenja |
collection | PubMed |
description | BACKGROUND: Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childhood and adolescence is influenced by endocrine and hypothalamic long-term sequelae. A legal status of the degree of disability (GdB), according to the German Social Code Book V that is worthy of support, provides financial means for psychosocial rehabilitation and participation of craniopharyngioma survivors. The aim of this study was to determine the association of clinical/psychosocial characteristics and quality of life (QoL) indicators with the resulting GdB. PATIENTS AND METHODS: HIT-Endo is a German registry study on craniopharyngioma patients aged ≤ 18 years at diagnosis and included before the year 2000. In a sample of 108 patients, the degree of disability and the association with endocrine, ophthalmological, neuropsychological and psychosocial parameters was analyzed after a mean follow-up period of 16 years (95% CI: 9.8-36.4). RESULTS: 44 patients (41%) did not receive a GdB, three patients (3%) received a GdB of 30-40, 43 patients (40%) a GdB of 50-90 and 18 patients (17%) the maximum GdB of 100. Higher GdB were associated with lower education, higher body mass index standard deviation and a higher degree of visual impairment and hypothalamic involvement of the craniopharyngeoma. Patients with a GdB of 100 reported loss in physical and cognitive function, as well as fatigue, dyspnea, and pain conditions, and limitations in social and occupational contexts. They further had a lower functional capacity (German daily life ability scale (FMH)) compared to those with a smaller GdB. CONCLUSION: The GdB is associated with psychosocial and physical impairments and reflects the long-term consequences of craniopharyngioma during childhood and adolescence. A low functional capacity (assessed by FMH) may indicate the eligibility for a high GdB in later life of craniopharyngioma survivors. |
format | Online Article Text |
id | pubmed-9164938 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-91649382022-06-05 RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study Boekhoff, Svenja Beckhaus, Julia Eveslage, Maria Friedrich, Carsten Müller, Hermann L Neuro Oncol Craniopharyngioma and Rare Tumors BACKGROUND: Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childhood and adolescence is influenced by endocrine and hypothalamic long-term sequelae. A legal status of the degree of disability (GdB), according to the German Social Code Book V that is worthy of support, provides financial means for psychosocial rehabilitation and participation of craniopharyngioma survivors. The aim of this study was to determine the association of clinical/psychosocial characteristics and quality of life (QoL) indicators with the resulting GdB. PATIENTS AND METHODS: HIT-Endo is a German registry study on craniopharyngioma patients aged ≤ 18 years at diagnosis and included before the year 2000. In a sample of 108 patients, the degree of disability and the association with endocrine, ophthalmological, neuropsychological and psychosocial parameters was analyzed after a mean follow-up period of 16 years (95% CI: 9.8-36.4). RESULTS: 44 patients (41%) did not receive a GdB, three patients (3%) received a GdB of 30-40, 43 patients (40%) a GdB of 50-90 and 18 patients (17%) the maximum GdB of 100. Higher GdB were associated with lower education, higher body mass index standard deviation and a higher degree of visual impairment and hypothalamic involvement of the craniopharyngeoma. Patients with a GdB of 100 reported loss in physical and cognitive function, as well as fatigue, dyspnea, and pain conditions, and limitations in social and occupational contexts. They further had a lower functional capacity (German daily life ability scale (FMH)) compared to those with a smaller GdB. CONCLUSION: The GdB is associated with psychosocial and physical impairments and reflects the long-term consequences of craniopharyngioma during childhood and adolescence. A low functional capacity (assessed by FMH) may indicate the eligibility for a high GdB in later life of craniopharyngioma survivors. Oxford University Press 2022-06-03 /pmc/articles/PMC9164938/ http://dx.doi.org/10.1093/neuonc/noac079.030 Text en © The Author(s) 2022. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Craniopharyngioma and Rare Tumors Boekhoff, Svenja Beckhaus, Julia Eveslage, Maria Friedrich, Carsten Müller, Hermann L RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study |
title | RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study |
title_full | RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study |
title_fullStr | RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study |
title_full_unstemmed | RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study |
title_short | RARE-05. Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the HIT-ENDO study |
title_sort | rare-05. legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up – results of the hit-endo study |
topic | Craniopharyngioma and Rare Tumors |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9164938/ http://dx.doi.org/10.1093/neuonc/noac079.030 |
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