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Pia Burman


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Prevalence of Nelson’s syndrome after bilateral adrenalectomy in patients with cushing’s disease : a systematic review and meta-analysis


  • Eleni Papakokkinou
  • Marta Piasecka
  • Hanne Krage Carlsen
  • Dimitrios Chantzichristos
  • Daniel S. Olsson
  • Per Dahlqvist
  • Maria Petersson
  • Katarina Berinder
  • Sophie Bensing
  • Charlotte Höybye
  • Britt Edén Engström
  • Pia Burman
  • Cecilia Follin
  • David Petranek
  • Eva Marie Erfurth
  • Jeanette Wahlberg
  • Bertil Ekman
  • Anna Karin Åkerman
  • Erik Schwarcz
  • Gudmundur Johannsson
  • Henrik Falhammar
  • Oskar Ragnarsson

Summary, in English

Purpose: Bilateral adrenalectomy (BA) still plays an important role in the management of Cushing's disease (CD). Nelson’s syndrome (NS) is a severe complication of BA, but conflicting data on its prevalence and predicting factors have been reported. The aim of this study was to determine the prevalence of NS, and identify factors associated with its development. Data sources: Systematic literature search in four databases. Study Selection: Observational studies reporting the prevalence of NS after BA in adult patients with CD. Data extraction: Data extraction and risk of bias assessment were performed by three independent investigators. Data synthesis: Thirty-six studies, with a total of 1316 CD patients treated with BA, were included for the primary outcome. Pooled prevalence of NS was 26% (95% CI 22–31%), with moderate to high heterogeneity (I2 67%, P < 0.01). The time from BA to NS varied from 2 months to 39 years. The prevalence of NS in the most recently published studies, where magnet resonance imaging was used, was 38% (95% CI 27–50%). The prevalence of treatment for NS was 21% (95% CI 18–26%). Relative risk for NS was not significantly affected by prior pituitary radiotherapy [0.9 (95% CI 0.5–1.6)] or pituitary surgery [0.6 (95% CI 0.4–1.0)]. Conclusions: Every fourth patient with CD treated with BA develops NS, and every fifth patient requires pituitary-specific treatment. The risk of NS may persist for up to four decades after BA. Life-long follow-up is essential for early detection and adequate treatment of NS.


  • Genomics, Diabetes and Endocrinology
  • EXODIAB: Excellence in Diabetes Research in Sweden

Publishing year






Document type

Journal article




  • Gastroenterology and Hepatology


  • Bilateral adrenalectomy
  • Corticotroph adenoma
  • Cushing’s disease
  • Nelson’s syndrome



Research group

  • Genomics, Diabetes and Endocrinology


  • ISSN: 1386-341X