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Outcomes in hospitalisations of women with Turner syndrome compared to women without Turner syndrome

Part of: Metabolic

Published online by Cambridge University Press:  19 March 2021

Isani Singh
Affiliation:
Department of Statistics, Harvard University, Cambridge, MA, USA
Lindsey M. Duca
Affiliation:
Colorado School of Public Health, Department of Epidemiology, University of Colorado | Aschutz Medical Center, Aurora, CO, USA
David Kao
Affiliation:
School of Medicine, Department of Medicine, Division of Cardiology, University of Colorado | Aschutz Medical Campus, Aurora, CO, USA
Kathryn C. Chatfield
Affiliation:
School of Medicine, Departments of Medicine and Pediatrics, Divisions of Cardiology, University of Colorado | Anschutz Medical Campus, Aurora, CO, USA
Amber D. Khanna*
Affiliation:
School of Medicine, Department of Medicine, Division of Cardiology, University of Colorado | Aschutz Medical Campus, Aurora, CO, USA
*
Author for correspondence: Amber Khanna MD, MS, Departments of Medicine and Pediatrics, 12401 E. 17th Ave, B132 Aurora, CO80045, USA. Tel: 720-848-6505; Fax: 720-848-5301. Email: Amber.Khanna@cuanschutz.edu

Abstract

Objective:

To evaluate outcomes in patients with Turner Syndrome, especially those with cardiac conditions, compared to those without Turner syndrome.

Design:

Retrospective cohort study utilising hospitalisation data from 2006 to 2012. Conditional logistic regression models are used to analyse outcomes of interest: all-cause mortality, increased length of stay, and discharge to home.

Participants:

We identified 2978 women with Turner syndrome, matched to 11,912 controls by primary diagnosis.

Results:

Patients with Turner syndrome were more likely to experience inpatient mortality (odds ratio 1.44, 95% confidence interval 1.02–2.02, p = 0.04) and increased length of stay (OR 1.31, CI 1.18–1.46, p = 0.03) than primary diagnosis matched controls, after adjusting for age, race, insurance status, and Charlson comorbidity index. Patients with Turner syndrome were 32% less likely to be discharged to home (OR 0.68, CI 0.60–0.78, p < 0.001). When restricting the sample of patients to those admitted with a cardiac diagnosis, the likelihood of mortality (OR 3.10, CI 1.27–7.57, p = 0.01) and prolonged length of stay (OR 1.42, CI 1.03–1.95, p = 0.03) further increased, while the likelihood of discharge to home further decreased (OR 0.55, CI 0.38–0.80, p = 0.001) in Turner syndrome compared to primary diagnosis matched controls. Specifically, patients with congenital heart disease were more likely to have prolonged length of stay (OR: 1.53, CI 1.18–2.00, p = 0.002), but not increased mortality or decreased discharge to home.

Conclusions:

Hospitalised women with Turner syndrome carry a higher risk of adverse outcomes even when presenting otherwise similarly as controls, an important consideration for those treating them in these settings.

Type
Original Article
Copyright
© The Author(s), 2021. Published by Cambridge University Press

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References

Zhong, Q, Layman, LC. Genetic considerations in the patient with Turner syndrome—45,X with or without Mosaicism. Fertil Steril 2012; 98: 775779. doi: 10.1016/j.fertnstert.2012.08.021 CrossRefGoogle ScholarPubMed
Urbach, A, Benvenisty, N. Studying early lethality of 45,XO (Turner’s syndrome) embryos using human embryonic stem cells. PloS One 2009; 4: e4175. doi: 10.1371/journal.pone.0004175 CrossRefGoogle ScholarPubMed
Hook, EB. Chromosome abnormalities and spontaneous fetal death following amniocentesis: further data and associations with maternal age. Am J Hum Genet 1983; 35: 110116.Google ScholarPubMed
Gravholt, CH. Epidemiology of Turner syndrome. Lancet Oncol 2008; 9: 193195. doi: 10.1016/S1470-2045(08)70045-7 CrossRefGoogle ScholarPubMed
Stochholm, K, Juul, S, Juel, K, Naeraa, RW, Højbjerg Gravholt, C. Prevalence, incidence, diagnostic delay, and mortality in Turner syndrome. J Clin Endocrinol Metab 2006; 91: 38973902. doi: 10.1210/jc.2006-0558 CrossRefGoogle ScholarPubMed
Ackermann, A, Bamba, V. Current controversies in Turner syndrome: genetic testing, assisted reproduction, and cardiovascular risks. J Clin Transl Endocrinol 2014; 1: 6165. doi: 10.1016/j.jcte.2014.05.003 Google ScholarPubMed
Mortensen, KH, Andersen, NH, Gravholt, CH. Cardiovascular phenotype in Turner syndrome—integrating cardiology, genetics, and endocrinology. Endocr Rev 2012; 33: 677714. doi: 10.1210/er.2011-1059 CrossRefGoogle Scholar
Mortensen, KH, Young, L, De Backer, J, et al. Cardiovascular imaging in Turner syndrome: state-of-the-art practice across the lifespan. Heart Br Card Soc 2018; 104: 18231831. doi: 10.1136/heartjnl-2017-312658 Google ScholarPubMed
Cadoret, F, Parinaud, J, Bettiol, C, et al. Pregnancy outcome in Turner syndrome: a French multi-center study after the 2009 guidelines. Eur J Obstet Gynecol Reprod Biol 2018; 229: 2025. doi: 10.1016/j.ejogrb.2018.08.005 CrossRefGoogle ScholarPubMed
van den Hoven, AT, Duijnhouwer, AL, Eicken, A, et al. Adverse outcome of coarctation stenting in patients with Turner syndrome. Catheter Cardiovasc Interv Off J Soc Card Angiogr Interv 2017; 89: 280287. doi: 10.1002/ccd.26728 CrossRefGoogle ScholarPubMed
Son, K-A, Lee, D-Y, Yoon, B-K, Choi, D. The efficacy of long-term estrogen replacement therapy in Turner syndrome women with premature ovarian insufficiency. J Pediatr Adolesc Gynecol Published online May 27, 2019. doi: 10.1016/j.jpag.2019.05.008 CrossRefGoogle ScholarPubMed
Viuff, MH, Berglund, A, Juul, S, Andersen, NH, Stochholm, K, Gravholt, CH. Sex hormone replacement therapy in Turner syndrome: impact on morbidity and mortality. J Clin Endocrinol Metab 2020; 105. doi: 10.1210/clinem/dgz039 CrossRefGoogle ScholarPubMed
Ji, J, Zöller, B, Sundquist, J, Sundquist, K. Risk of solid tumors and hematological malignancy in persons with Turner and Klinefelter syndromes: a national cohort study. Int J Cancer 2016; 139: 754758. doi: 10.1002/ijc.30126 CrossRefGoogle ScholarPubMed
Stochholm, K, Hjerrild, B, Mortensen, KH, Juul, S, Frydenberg, M, Gravholt, CH. Socioeconomic parameters and mortality in Turner syndrome. Eur J Endocrinol 2012; 166: 10131019. doi: 10.1530/EJE-11-1066 CrossRefGoogle ScholarPubMed
Gravholt, CH, Juul, S, Naeraa, RW, Hansen, J. Morbidity in Turner syndrome. J Clin Epidemiol 1998; 51: 147158. doi: 10.1016/S0895-4356(97)00237-0 CrossRefGoogle ScholarPubMed
Schoemaker, MJ, Swerdlow, AJ, Higgins, CD, Wright, AF, Jacobs, PA, United Kingdom clinical cytogenetics group. Mortality in women with turner syndrome in Great Britain: a national cohort study. J Clin Endocrinol Metab 2008; 93: 47354742. doi: 10.1210/jc.2008-1049 CrossRefGoogle ScholarPubMed
Agency for Healthcare Research & Quality. Retrieved October 5, 2019, from https://www.ahrq.gov/ Google Scholar
Welcome – California Health and Human Services Open Data Portal. Retrieved October 5, 2019, from https://data.chhs.ca.gov/ Google Scholar
Data Requests | Health Statistics and Data Management | NH Department of Health and Human Services. Retrieved October 5, 2019, from https://www.dhhs.nh.gov/dphs/hsdm/requests.htm Google Scholar
Department of Health | Health Care Quality Assessment | NJ Hospital Discharge Data Collection System (NJDDCS). Retrieved October 5, 2019, from https://www.state.nj.us/health/healthcarequality/health-care-professionals/njddcs/ Google Scholar
Statewide Planning and Research Cooperative System. Retrieved October 5, 2019, from https://www.health.ny.gov/statistics/sparcs/ Google Scholar
Hospital Discharge Data Use Agreement. Retrieved October 5, 2019, from https://www.dshs.state.tx.us/THCIC/Hospitals/Download.shtm Google Scholar
Reclassification of ICD-9 Codes into Meaningful Categories for Oncology Survivorship Research : Table 2. Retrieved July 13, 2019, from https://www.hindawi.com/journals/jce/2010/569517/tab2/ CrossRefGoogle Scholar
Glidewell, J, Book, W, Raskind-Hood, C, et al. Population-based surveillance of congenital heart defects among adolescents and adults: surveillance methodology. Birth Defects Res 2018; 110: 13951403. doi: 10.1002/bdr2.1400 CrossRefGoogle ScholarPubMed
D’Hoore, W, Bouckaert, A, Tilquin, C. Practical considerations on the use of the Charlson comorbidity index with administrative data bases. J Clin Epidemiol 1996; 49: 14291433.10.1016/S0895-4356(96)00271-5CrossRefGoogle ScholarPubMed
Weiss, AJ, Elixhauser, A. Overview of hospital stays in the United States, 2012: statistical Brief #180. In: Healthcare Cost and Utilization Project (HCUP) Statistical Briefs. Agency for Healthcare Research and Quality (US), 2006. http://www.ncbi.nlm.nih.gov/books/NBK259100/ Google ScholarPubMed
Price, WH, Clayton, JF, Collyer, S, De Mey, R, Wilson, J. Mortality ratios, life expectancy, and causes of death in patients with Turner’s syndrome. J Epidemiol Community Health 1986; 40: 97102.10.1136/jech.40.2.97CrossRefGoogle ScholarPubMed
Fuchs, MM, Attenhofer Jost, CH, Said, SM, et al. Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up. World J Cardiol 2020; 12: 97106. doi: 10.4330/wjc.v12.i3.97 CrossRefGoogle ScholarPubMed
Madriago, E, Nguyen, T, McFerson, M, et al. Frequency and outcomes of cardiac operations and catheter interventions in Turner syndrome. Am J Cardiol 2012; 110: 580585. doi: 10.1016/j.amjcard.2012.04.036 CrossRefGoogle ScholarPubMed
Cunniff, C, Hassed, SJ, Hendon, AE, Rickert, VI. Health care utilization and perceptions of health among adolescents and adults with Turner syndrome. Clin Genet 1995; 48: 1722. doi: 10.1111/j.1399-0004.1995.tb04048.x CrossRefGoogle ScholarPubMed
Elsheikh, M, Dunger, DB, Conway, GS, Wass, J a. H. Turner’s syndrome in adulthood. Endocr Rev 2002; 23: 120140. doi: 10.1210/edrv.23.1.0457 Google ScholarPubMed
Aortic Dissection – Cardiovascular Disorders. Merck Manuals Professional Edition. Retrieved October 28, 2017, from https://www.merckmanuals.com/professional/cardiovascular-disorders/diseases-of-the-aorta-and-its-branches/aortic-dissection Google Scholar
Aortic dissection: When Your Aorta Tears. Mayo Clinic. Retrieved October 28, 2017, from http://www.mayoclinic.org/diseases-conditions/aortic-dissection/basics/definition/con-20032930 Google Scholar
Bondy, CA, Ceniceros, I, Lange, E, Bakalov, VK. Declining estrogen use in young women with Turner syndrome. Arch Intern Med 2006; 166: 13221322. doi: 10.1001/archinte.166.12.1322-a CrossRefGoogle ScholarPubMed