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- W4378674826 abstract "Neurofibromatosis 1 (NF1) is a multisystem disease which can affect nearly every organ system. The aim of our study was to describe the in-hospital population with NF1 in France. We conducted a nationwide retrospective cohort study using the French hospital administrative database. A total of 11,425 patients with NF1 (53.4% female, 19,080 person years) were identified from January 2013, through December 2019. Twenty-three percent had at least one diagnosis of a comorbidity or NF1-associated complication or disease; and it was highest in the 10-15years age group. A total of 2,601 (22.8%) had a diagnosis of cancer. There were 366 (3.2%) in-hospital deaths; and we observed a standardized mortality ratio (SMR) of 4.14 [95% Confidence Interval (CI) 3.71; 4.56] with a higher SMR in women and in the 10-15years age group. The standardized incident ratio (SIR) of cancer was 10.3 [95%CI 9.6-11.1]. We observed high SIR values of cancer in childhood, with a decrease toward that of the general population by age 70 years. We observed high SIRs for NF1-associated cancers: central nervous system SIR 195.4 [95%CI 172.2-220.9] and small intestine SIR 102.9 [95%CI 71.7-143.2]. The study provides a better understanding of the prognosis in people living with NF1." @default.
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- W4378674826 date "2023-05-01" @default.
- W4378674826 modified "2023-10-14" @default.
- W4378674826 title "In-Hospital Clinical Features, Morbidity, and Mortality of Patients with Neurofibromatosis 1 in France: A Nationwide, Population-Based Retrospective Cohort Study" @default.
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- W4378674826 doi "https://doi.org/10.1016/j.jid.2023.04.034" @default.
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