Line Kenborg1, Anne Katrine Duun-Henriksen2, Susanne O Dalton3, Pernille E Bidstrup3, Karoline Doser3, Kathrine Rugbjerg4, Camilla Pedersen4, Anja Krøyer4, Christoffer Johansen3,5, Klaus Kaae Andersen2, John R Østergaard6, Hanne Hove7,8, Sven Asger Sørensen9, Vincent M Riccardi10, John J Mulvihill11, Jeanette F Winther4,12. 1. Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark. kenborg@cancer.dk. 2. Statistics and Pharmacoepidemiology, Danish Cancer Society Research Center, Copenhagen, Denmark. 3. Survivorship Unit, Danish Cancer Society Research Center, Copenhagen, Denmark. 4. Childhood Cancer Research Group, Danish Cancer Society Research Center, Copenhagen, Denmark. 5. Department of Oncology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark. 6. Centre for Rare Diseases, Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark. 7. Department of Clinical Genetics, Copenhagen University Hospital, Copenhagen, Denmark. 8. The RAREDIS Database, Section of Rare Diseases, Department of Clinical Genetics, Copenhagen University Hospital, Copenhagen, Denmark. 9. Department of Neurogenetics, Institute of Cellular and Molecular Medicine, The Panum Institute, University of Copenhagen, Copenhagen, Denmark. 10. The Neurofibromatosis Institute, La Crescenta, CA, USA. 11. Department of Pediatrics, University of Oklahoma, Oklahoma City, OK, USA. 12. Department of Clinical Medicine, Faculty of Health, Aarhus University, Aarhus, Denmark.
Abstract
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.
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.
Authors: Roope A Kallionpää; Sirkku Peltonen; Jussi Leppävirta; Minna Pöyhönen; Kari Auranen; Hannu Järveläinen; Juha Peltonen Journal: J Med Genet Date: 2020-06-22 Impact factor: 6.318
Authors: Karoline Doser; Hanne Hove; John R Østergaard; Pernille E Bidstrup; Susanne O Dalton; Mette Møller Handrup; Cecilie Ejerskov; Anja Krøyer; Mia Aagaard Doherty; Jens Richardt Møllegaard Jepsen; John J Mulvihill; Jeanette F Winther; Line Kenborg Journal: BMJ Open Date: 2022-09-20 Impact factor: 3.006