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Multisystem burden of neurofibromatosis 1 in Denmark: registry- and population-based rates of hospitalizations over the life span

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  1. DLG4-related synaptopathy: a new rare brain disorder

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  2. NEXMIF encephalopathy: an X-linked disorder with male and female phenotypic patterns

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  3. DOORS syndrome and a recurrent truncating ATP6V1B2 variant

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  1. Psychiatric disorders in individuals with neurofibromatosis 1 in Denmark: A nationwide register-based cohort study

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  2. Risk of depression after diagnostic prostate cancer workup - A nationwide, registry-based study

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  3. Is the health literacy of informal caregivers associated with the psychological outcomes of breast cancer survivors?

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  4. Antidepressant prescriptions and associated factors in men with prostate cancer and their female partners

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  5. Risk of Depression After Radical Prostatectomy-A Nationwide Registry-based Study

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  • Line Kenborg
  • Anne Katrine Duun-Henriksen
  • Susanne O Dalton
  • Pernille E Bidstrup
  • Karoline Doser
  • Kathrine Rugbjerg
  • Camilla Pedersen
  • Anja Krøyer
  • Christoffer Johansen
  • Klaus Kaae Andersen
  • John R Østergaard
  • Hanne Hove
  • Sven Asger Sørensen
  • Vincent M Riccardi
  • John J Mulvihill
  • Jeanette F Winther
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PURPOSE: The aim was to assess lifetime risk for hospitalization in individuals with neurofibromatosis 1 (NF1).

METHODS: The 2467 individuals discharged with a diagnosis indicating NF1 or followed in a clinical center for NF1 were matched to 20,132 general population comparisons. Based on diagnoses in 12 main diagnostic groups and 146 subcategories, we calculated rate ratios (RRs), absolute excess risks (AERs), and hazard ratios for hospitalizations.

RESULTS: The RR for any first hospitalization among individuals with NF1 was 2.3 (95% confidence interval 2.2-2.5). A high AER was seen for all 12 main diagnostic groups, dominated by disorders of the nervous system (14.5% of all AERs), benign (13.6%) and malignant neoplasms (13.4%), and disorders of the digestive (10.5%) and respiratory systems (10.3%). Neoplasms, nerve and peripheral ganglia disease, pneumonia, epilepsy, bone and joint disorders, and intestinal infections were major contributors to the excess disease burden caused by NF1. Individuals with NF1 had more hospitalizations and spent more days in hospital than the comparisons. The increased risk for any hospitalization was observed for both children and adults, with or without an associated cancer.

CONCLUSION: NF1 causes an overall greater likelihood of hospitalization, with frequent and longer hospitalizations involving all organ systems throughout life.

Original languageEnglish
JournalGenetics in medicine : official journal of the American College of Medical Genetics
Volume22
Issue number6
Pages (from-to)1069-1078
Number of pages10
ISSN1098-3600
DOIs
Publication statusPublished - Jun 2020

    Research areas

  • cohort study, hospitalizations, neurofibromatosis 1, population-based

ID: 61824232