| 1. |
- Bonnard, Åsa, et al.
(författare)
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The risk of cholesteatoma in individuals with first-degree relatives surgically treated for the disease
- 2023
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Ingår i: JAMA Otolaryngology - Head and Neck Surgery. - : American Medical Association (AMA). - 2168-6181 .- 2168-619X. ; 149:5
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Tidskriftsartikel (refereegranskat)abstract
- IMPORTANCE: Cholesteatoma in the middle ear is not regarded as a hereditary disease, but case reports of familial clustering exist in the literature, as well as observed familial cases in the clinical work. However, the knowledge regarding cholesteatoma as a hereditary disease is lacking in the literature. OBJECTIVE To assess the risk of cholesteatoma in individuals with a first-degree relative surgically treated for the same disease.DESIGN, SETTING, AND PARTICIPANTS: In this nested case-control study in the Swedish population between 1987 and 2018 of first-time cholesteatoma surgery identified from the Swedish National Patient Register, 2 controls per case were randomly selected from the population register through incidence density sampling, and all first-degree relatives for cases and controls were identified. Data were received in April 2022, and analyses were conducted between April and September 2022.EXPOSURE: Cholesteatoma surgery in a first-degree relative.MAIN OUTCOMES AND MEASURES: The main outcome was first-time cholesteatoma surgery. The association between having a first-degree relative with cholesteatoma and the risk of cholesteatoma surgery in the index persons was estimated by odds ratios (ORs) and 95% CIs through conditional logistic regression analysis.RESULTS: Between 1987 and 2018, 10 618 individuals with a first-time cholesteatoma surgery (mean [SD] age at surgery, 35.6 [21.5] years; 6302 [59.4%] men) were identified in the Swedish National Patient Register. The risk of having a cholesteatoma surgery was almost 4 times higher in individuals having a first-degree relative surgically treated for the disease (OR, 3.9; 95% CI, 3.1-4.8), but few cases were exposed overall. Among the 10 105 cases with at least 1 control included in the main analysis, 227 (2.2%) had at least 1 first-degree relative treated for cholesteatoma, while the corresponding numbers for controls were 118 of 19 553 control patients (0.6%). The association was stronger for individuals under the age of 20 years at first surgery (OR, 5.2; 95% CI, 3.6-7.6) and for a surgery involving the atticus and/or mastoid region (OR, 4.8; 95% CI, 3.4-6.2). There was no difference in the prevalence of having a partner with cholesteatoma between cases and controls (10 cases [0.3%] and 16 controls [0.3%]; OR, 0.92; 95% CI, 0.41-2.05), which implies that increased awareness does not explain the association.CONCLUSIONS AND RELEVANCE: In this Swedish case-control study using nationwide register data with high coverage and completeness, the findings suggest that the risk of cholesteatoma in the middle ear is strongly associated with a family history of the condition. Family history was nevertheless quite rare and can therefore only explain a limited number of all cases; these families could be an important source for information regarding the genetic background for cholesteatoma disease.
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| 2. |
- Elmstedt, Sixten, et al.
(författare)
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Cancer patients hospitalised in the last week of life risk insufficient care quality : a population-based study from the Swedish Register of Palliative Care
- 2019
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Ingår i: Acta Oncologica. - : Taylor & Francis Group. - 0284-186X .- 1651-226X. ; 58:4, s. 432-438
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Tidskriftsartikel (refereegranskat)abstract
- Background: One-quarter of all cancer deaths in Sweden occur in hospitals. If the place of death affects the quality of end-of-life (EOL) is largely unknown.Methods: This population-based, retrospective study included all adults cancer deaths reported to the Swedish Register of Palliative Care in 2011-2013 (N = 41,729). Hospital deaths were compared to deaths occurring in general or specialised palliative care, or in nursing homes with respect to care quality indicators in the last week of life. Odds ratios (OR) with 95% confidence intervals (CI) were calculated with specialised palliative home care as reference.Results: Preferred place of death was unknown for 63% of hospitalised patients and consistent with the actual place of death in 25% compared to 97% in palliative home care. Hospitalised patients were less likely to be informed when death was imminent (OR: 0.3; CI: 0.28-0.33) as were their families (OR: 0.51; CI: 0.46-0.57). Validated screening tools were less often used in hospitals for assessment of pain (OR: 0.32; CI: 0.30-0.34) or other symptoms (OR: 0.31; CI: 0.28-0.34) despite similar levels of EOL symptoms. Prescriptions of as needed drugs against anxiety (OR: 0.27; CI: 0.24-0.30), nausea (OR: 0.19; CI: 0.17-0.21), or pulmonary secretions (OR: 0.29; CI: 0.26-0.32) were less prevalent in hospitals. Bereavement support was offered after 57% of hospital deaths compared to 87-97% in palliative care units and 72% in nursing homes.Conclusions: Dying in hospital was associated with inferior end-of-life care quality among cancer patients in Sweden.
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| 3. |
- Frederiksen, Line Elmerdahl, et al.
(författare)
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Psychiatric disorders in childhood cancer survivors in Denmark, Finland, and Sweden : a register-based cohort study from the SALiCCS research programme
- 2022
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Ingår i: The Lancet Psychiatry. - 2215-0366 .- 2215-0374. ; 69
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Tidskriftsartikel (refereegranskat)abstract
- Background: A childhood cancer diagnosis and treatment-induced somatic late effects can affect the long-term mental health of survivors. We aimed to explore whether childhood cancer survivors are at higher risk of psychiatric disorders later in life than their siblings and the general population. Methods: In this register-based cohort study (part of the Socioeconomic Consequences in Adult Life after Childhood Cancer [SALiCCS] research programme), we included 5-year survivors of childhood cancer diagnosed before 20 years of age between Jan 1, 1974 and Dec 31, 2011, in Denmark, Finland, and Sweden. In Denmark and Sweden, 94·7% of individuals were born in a Nordic country (ie, Denmark, Finland, Iceland, Norway, or Sweden); similar information was not available in Finland. Data on ethnicity were not collected. Survivors were compared with their siblings and randomly selected individuals from the general population who were matched to the survivors by year of birth, sex, and geographical region. We followed up our study population from 5 years after the childhood cancer diagnosis or corresponding calendar date for matched individuals (the index date) until Aug 11, 2017, and assessed information on hospital contacts for any and specific psychiatric disorders. For siblings, the index date was defined as 5 years from the date on which they were of the same age as their sibling survivor when diagnosed with cancer. Findings: The study population included 18 621 childhood cancer survivors (9934 [53·3%] males and 8687 [46·7%] females), 24 775 siblings (12 594 [50·8%] males and 12 181 [49·2%] females), and 88 630 matched individuals (47 300 [53·4%] males and 41 330 [46·6%] females). The cumulative incidence proportion of having had a psychiatric hospital contact by 30 years of age between Jan 1, 1979, and Aug 11, 2017, was 15·9% (95% CI 15·3–16·5) for childhood cancer survivors, 14·0% (13·5–14·5) for siblings, and 12·7% (12·4–12·9) for matched individuals. Despite a small absolute difference, survivors were at higher relative risk of any psychiatric hospital contact than their siblings (1·39, 1·31–1·48) and matched individuals (hazard ratio 1·34, 95% CI 1·28–1·39). The higher risk persisted at the age of 50 years. Survivors had a higher burden of recurrent psychiatric hospital contacts and had more hospital contacts for different psychiatric disorders than their siblings and the matched individuals. Interpretation: Childhood cancer survivors are at higher long-term risk of psychiatric disorders than their siblings and matched individuals from the general population. To improve mental health and the overall quality of life after childhood cancer, survivorship care should include a focus on early signs of mental health problems, especially among high-risk groups of survivors. Funding: NordForsk, Aarhus University, Swedish Childhood Cancer Foundation, Danish Health Foundation, and Swiss National Science Foundation.
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| 4. |
- Modée Borgström, Agnes, et al.
(författare)
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Occurrence of mucosa-affecting diseases of the upper airways in middle ear cholesteatoma patients : a nationwide case-control study
- 2024
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Ingår i: European Archives of Oto-Rhino-Laryngology. - : Springer. - 0937-4477 .- 1434-4726.
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Tidskriftsartikel (refereegranskat)abstract
- PURPOSE: Exploring a possible link between upper airway inflammation and the development of cholesteatoma by studying the association between mucosa-affecting diseases of the upper airways and cholesteatoma surgery.METHODS: This is a nationwide case-control study of 10,618 patients who underwent surgery for cholesteatoma in Sweden between 1987 and 2018. The cases were identified in the National Patient Register and 21,235 controls matched by age, sex and place of residency were included from national population registers. Odds ratios (OR) and corresponding 95% confidence intervals were used to assess the association between six types of mucosa-affecting diseases of the upper airways and cholesteatoma surgery.RESULTS: Chronic rhinitis, chronic sinusitis and nasal polyposis were more common in cholesteatoma patients than in controls (OR 1.5 to 2.5) as were both adenoid and tonsil surgery (OR > 4) where the strongest association was seen for adenoid surgery. No association was seen between allergic rhinitis and cholesteatoma.CONCLUSION: This study supports an association between mucosa-affecting diseases of the upper airways and cholesteatoma. Future studies should aim to investigate the mechanisms connecting mucosa-affecting diseases of the upper airways and cholesteatoma formation regarding genetic, anatomical, inflammatory and mucosa properties.
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| 5. |
- Mogensen, Hanna, et al.
(författare)
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Educational attainment in survivors of childhood cancer in Denmark, Finland, and Sweden
- 2024
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Ingår i: British Journal of Cancer. - 0007-0920 .- 1532-1827. ; 130:2, s. 260-268
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Tidskriftsartikel (refereegranskat)abstract
- Background: Survivors of childhood cancer may face difficulties at school. We investigated whether childhood cancer affects attainment of upper secondary education, in a register-based cohort study from Denmark, Finland, and Sweden, where we limit bias from selection and participation.Methods: From the national cancer registers, we identified all long-term survivors of childhood cancer diagnosed aged 0–14 years in 1971–2005 (n = 7629), compared them to matched population comparisons (n = 35,411) and siblings (n = 6114), using odds ratios (OR) and 95% confidence intervals (CI).Results: Overall, 6127 survivors (80%) had attained upper secondary education by age 25, compared to 84% among comparison groups. Elevated OR for not attaining this level were mainly confined to survivors of central nervous system (CNS) tumours (ORSurv_PopComp2.05, 95%CI: 1.83–2.29). Other risk groups were survivors who had spent more time in hospital around cancer diagnosis and those who had hospital contacts in early adulthood, particularly psychiatric. Survivors of all cancer types were less likely to have attained upper secondary education without delay.Conclusions: Although survivors of childhood cancer experienced delays in their education, many had caught up by age 25. Except for survivors of CNS tumours, survivors attained upper secondary education to almost the same extent as their peers.
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| 6. |
- Mogensen, Hanna, et al.
(författare)
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Number of siblings and survival from childhood leukaemia : a national register-based cohort study from Sweden
- 2021
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Ingår i: British Journal of Cancer. - Stockholm : Karolinska Institutet, Institute of Environmental Medicine. - 0007-0920 .- 1532-1827.
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Tidskriftsartikel (refereegranskat)abstract
- Background: Previous studies suggest worse leukaemia survival for children with siblings, but the evidence is sparse, inconsistent and does not consider clinical factors. We explored the associations between number of siblings in the household, birth order, and survival from childhood acute lymphoid leukaemia (ALL) and acute myeloid leukaemia (AML). Methods: In this nationwide register-based study we included all children aged 1-14, diagnosed with ALL and AML between 1991-mid 2015 in Sweden (n=1692). Using Cox regression models, we estimated hazard ratios (HRs) and 95% confidence intervals (CIs) according to number of siblings and birth order, adjusting for known prognostic and sociodemographic factors. Results: A tendency towards better ALL survival among children with one, or ≥2, siblings was observed, adjHRs (95% CI): 0.73 (0.49-1.10) and 0.63 (0.40-1.00), respectively. However, this was mainly limited to children with low risk profiles. An indication of better AML survival among children with siblings was seen, adjHRs (95% CI) 0.68 (0.36-1.29) and 0.71 (0.34-1.48) but diminished after adjusting for birth order. Conclusion: Our results do not support previous findings that a larger number of siblings is associated with poorer survival. Inconsistencies might be explained by underlying mechanisms that differ between settings, but chance cannot be ruled out.
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| 7. |
- Mogensen, Hanna
(författare)
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Socioeconomic factors and childhood cancer : survival and long-term consequences
- 2022
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Childhood cancer is rare and survival rates have increased substantially over the past 60 years as a result of better diagnostics and treatments. Despite this, childhood cancer is the most common disease-related cause of death among children aged 1-14 years in Sweden. Moreover, there are indications that the progress in survival has not been gained by all groups equally and socioeconomic differences in childhood cancer survival have been observed across the world. However, if such differences are seen within Sweden, a country with universal healthcare that is free of charge for children, has not previously been investigated. The increased survival rates have also resulted in a growing population of adult childhood cancer survivors. Survivors may face various late health effects but less is known about the socioeconomic consequences of having had cancer as a child. To address these issues, the overall aims of this thesis were to determine if and how survival from childhood cancer varies by socioeconomic factors and to investigate long-term socioeconomic consequences, in particular educational attainment, in young adult survivors of childhood cancer. The research questions were addressed in four studies that utilized information in the Nordic population and health data registers. Study I-III investigated socioeconomic and familial factors in relation to survival from childhood cancer. Study I was a cohort study including children diagnosed with cancer in Sweden from 1991 to 2010. In this study we observed socioeconomic differences in overall survival – children of parents with a lower level of education had worse survival from childhood cancer, while no differences were observed for household income. The differences by parental education were seen already in the first year after diagnosis. These findings, together with the publication of a seminal study from the US, led us to investigate potential socioeconomic differences in early mortality from childhood cancer. In Study III, we included children diagnosed with cancer in Sweden and Denmark during 1991-2014 and assessed the association between parental socioeconomic factors and early mortality (defined as deaths occurring within 90 days after cancer diagnosis). We observed that children from disadvantaged backgrounds were at increased risk of early mortality, with parental education and maternal income showing the most pronounced associations. Associations with later mortality (defined as deaths occurring 1 to 5 years after cancer diagnosis), were attenuated or close to unity in this study. In Study II we investigated another aspect of social family circumstances, number of siblings and birth order, in relation to survival from acute lymphoid leukaemia (ALL) and acute myeloid leukaemia (AML) in children diagnosed 1991-2015 in Sweden. In this study we found no evidence supporting a previously suggested hypothesis of lower survival after leukaemia among children with siblings, but we rather observed the opposite. We also included clinical information for children diagnosed with ALL and saw that the superior survival among children with siblings was seen mainly within children with low-risk profiles. In Study IV, we compared attainment of upper secondary education in young adult survivors of childhood cancer, matched population comparisons, and the survivors’ siblings in Sweden, Denmark and Finland. We observed that delays in attaining this education level were more common among survivors of all cancer types compared to their peers. However, by the age of 25 years, many survivors had caught up with regard to upper secondary education. Particular risk groups were survivors diagnosed with central nervous system (CNS) tumours, and survivors diagnosed with ALL in the early time period. We also saw that survivors who had spent more time in hospital around the time of diagnosis or had hospital contacts, in particular for psychiatric diseases, in the age range 20-24 years were at increased risk of not having attained upper secondary education by age 25 years. In conclusion, we have observed social inequalities in survival from childhood cancer in the Nordic countries with universal healthcare access. The differences were seen already early in the disease course, and these findings need further attention. We have also shown that although survivors of childhood cancer may experience delays in their education, many catch up. However, there are vulnerable risk groups that need further support. The results in this thesis are based on information from nationwide, population and health data registers, which minimized the risk of bias from non-participation, loss to follow-up, and self-reports. Moreover, the included studies highlight the potential and importance of Nordic collaborations in research of rare diseases.
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| 8. |
- Stachurski, Mikolaj, et al.
(författare)
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The impact of waiting time on hearing outcome and patients' satisfaction after cholesteatoma surgery
- 2023
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Ingår i: Acta Oto-Laryngologica. - : Taylor & Francis. - 0001-6489 .- 1651-2251. ; 143:8, s. 662-668
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Tidskriftsartikel (refereegranskat)abstract
- Background: Cholesteatoma is a formation of epithelium mass in the middle ear. Surgery aims to prevent complications while maintain or improve hearing.Aims/Objectives: To determine if waiting time until cholesteatoma surgery affects hearing outcome and patients’ satisfaction.Material and Methods: A retrospective cohort study performed at the only Ear Nose Throat clinic in one county in Sweden. Sixty concomitant surgeries, both first time and revisions, were included.Results: Of the 60 surgeries, 33 (55%) were performed within a 3-month period. The mean waiting time was 1.4 months. In the remaining 27 cases, the mean waiting time was 8.6 months. Both groups had preoperatively similar air conduction pure tone average (AC PTA4), 47.3 dB and 47.0 dB respectively. The mean AC PTA4 gain was greater in the group with waiting time ≤3 months (8.6 dB) compared to the >3 months group (1.2 dB, p = 0.040). The patients’ satisfaction was lower in the latter group, but the difference was not statistically significant.Conclusions: This study indicates that longer waiting time to cholesteatoma surgery has a negative impact on postoperative hearing results but not on patients’ satisfaction.Significance: The outcome of this study suggests that waiting time to surgery can be a factor determining postoperative hearing results.
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| 9. |
- Sørensen, Gitte V., et al.
(författare)
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Late mortality among survivors of childhood acute lymphoblastic leukemia diagnosed during 1971–2008 in Denmark, Finland, and Sweden : A population-based cohort study
- 2022
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Ingår i: Pediatric Blood and Cancer. - : Wiley. - 1545-5009 .- 1545-5017. ; 69:1
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Investigate all-cause and cause-specific late mortality after childhood acute lymphoblastic leukemia (ALL) in a population-based Nordic cohort. Methods: From the cancer registries of Denmark, Finland, and Sweden, we identified 3765 five-year survivors of ALL, diagnosed before age 20 during 1971–2008. For each survivor, up to five matched comparison subjects were randomly selected from the general population (n = 18,323). Causes of death were classified as relapse related, health related, and external. Late mortality was evaluated by cumulative incidences of death from 5-year survival date. Mortality hazard ratios (HR) were evaluated with Cox proportional models. Results: Among the survivors, 315 deaths occurred during a median follow-up of 16 years from 5-year survival date (range 0–42). The majority were attributable to relapse (n = 224), followed by second neoplasm (n = 45). Cumulative incidence of all-cause late mortality at 15 years from diagnosis decreased gradually over treatment decades, from 14.4% (95% confidence interval [CI]: 11.6–17.2) for survivors diagnosed during 1971–1981, to 2.5% (95% CI: 1.3–3.7) for those diagnosed during 2002–2008. This was mainly attributable to a reduction in relapse-related deaths decreasing from 13.4% (95% CI: 10.7–16.1) for survivors diagnosed during 1971–1981 to 1.9% (95% CI: 0.9–2.8) for those diagnosed during 2002–2008. Health-related late mortality was low and did not change substantially across treatment decades. Compared to comparison subjects, all-cause mortality HR was 40 (95% CI: 26–61) 5–9 years from diagnosis, and 4.4 (95% CI: 3.4–5.6) ≥10 years from diagnosis. Conclusions: Survivors of ALL have higher late mortality than population comparison subjects. Among the survivors, there was a temporal reduction in risk of death from relapse, without increments in health-related death.
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