Jump to ContentJump to Main Navigation
Show Summary Details
More options …

Journal of Pediatric Endocrinology and Metabolism

Editor-in-Chief: Kiess, Wieland

Ed. by Bereket, Abdullah / Darendeliler, Feyza / Dattani, Mehul / Gustafsson, Jan / Luo, Fei Hong / Mericq, Veronica / Toppari, Jorma

IMPACT FACTOR 2018: 1.239

CiteScore 2018: 1.22

SCImago Journal Rank (SJR) 2018: 0.507
Source Normalized Impact per Paper (SNIP) 2018: 0.562

See all formats and pricing
More options …
Volume 32, Issue 7


Diagnosis of cyclic Cushing’s disease manifests as early morning hyperglycemia in a patient with previously well-controlled type 1 diabetes

Carol Singer-Granick / James K. Liu
  • Professor of Neurosurgery, Rutgers New Jersey Medical School, Department of Neurosurgery, Newark, NJ, USA
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ David BleichORCID iD: https://orcid.org/0000-0001-7253-4018 / Lissette Cespedes
  • Corresponding author
  • Assistant Professor of Medicine, Division of Endocrinology, Diabetes and Metabolism, Rutgers New Jersey Medical School, 185 South Orange Avenue, MSB I-588, Newark, NJ 07103, USA
  • Email
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2019-06-28 | DOI: https://doi.org/10.1515/jpem-2018-0506



Cyclic Cushing’s disease (CCD) is reported to occur in approximately 15% of patients with Cushing’s disease (CD). CCD is a rare phenomenon in children.

Case presentation

A Portuguese female with well-controlled type 1 diabetes (T1DM) on an insulin pump developed transient uncontrolled blood sugar every morning. Increased basal and bolus insulin dosing was ineffective in lowering blood sugar and she began to miss school because of nausea, vomiting, fatigue, but no ketoacidosis. Therefore, other causes of sporadic hyperglycemia were explored. Multiple 6-h urinary free cortisol (UFC) samples revealed a spike in cortisol coincident with severe hyperglycemia. Pituitary magnetic resonance imaging (MRI) revealed a 3.5 mm microadenoma and inferior petrosal sinus sampling of adrenocorticotropic hormone (ACTH) after corticotropin releasing hormone (CRH) stimulation confirmed ACTH-dependent CD. Endoscopic endonasal tumor resection led to resolution of early morning hyperglycemia and symptoms.


Our case illustrates an atypical presentation of CCD. There are no previous case reports of a pediatric patient with T1DM and CCD. Unexplained hyperglycemia in a patient with previous well-controlled T1DM should prompt assessment of other causes. CCD can be easily be missed if timed 6-h UFC measurements are not obtained.

Keywords: Cushing’s disease; cyclic; type 1 diabetes


  • 1.

    Lacroix A, Feelders RA, Stratakis CA, Nieman LK. Cushing’s syndrome. Lancet 2015;386:913–27.PubMedCrossrefWeb of ScienceGoogle Scholar

  • 2.

    Sharma ST, Nieman LK, Feelders RA. Cushing’s syndrome: epidemiology and developments in disease management. Clin Epidemiol 2015;7:281–93.Web of SciencePubMedGoogle Scholar

  • 3.

    Stratakis CA. Diagnosis and clinical genetics of Cushing syndrome in pediatrics. Endocrinol Metab Clin North Am 2016;45:311–28.CrossrefPubMedWeb of ScienceGoogle Scholar

  • 4.

    Alexandraki KF, Kaltsas GA, Isidori AM, Akker SA, Drake WM, et al. The prevalence and characteristic features of cyclicity and variability in Cushing’s disease. Eur J Endocrinol 2009;160:1011–8.PubMedWeb of ScienceCrossrefGoogle Scholar

  • 5.

    Meinardi JR, Wolffenbuttel BR, Dullaart RF. Cyclic Cushing’s syndrome: a clinical challenge. Eur J Endocrinol 2007;157:245–54.Web of ScienceCrossrefPubMedGoogle Scholar

  • 6.

    Noctor E, Gupta S, Brown T, Farrell M, Javadpour M, et al. Paediatric cyclical Cushing’s disease due to corticotroph cell hyperplasia. BMC Endocr Disord 2015;15:27.CrossrefPubMedWeb of ScienceGoogle Scholar

  • 7.

    Mantero F, Scaroni CM, Albiger NE. Cyclic Cushing’s syndrome: an overview. Pituitary 2004;7:203–7.PubMedCrossrefGoogle Scholar

  • 8.

    Atkinson B, Mullan KR. What is the best approach to suspect cyclical Cushing syndrome? Strategies for managing Cushing’s syndrome with variable laboratory data. Clin Endocrinol 2011;75:27–30.CrossrefGoogle Scholar

  • 9.

    Batista D, Gennari M, Riar J, Chang R, Keil MF, et al. An assessment of petrosal sinus sampling for localization of pituitary microadenomas in children with Cushing’s disease. J Clin Endocrinol Metab 2006;91:221–4.CrossrefGoogle Scholar

  • 10.

    Lonser RR, Wind JJ, Nieman LK, Weil RJ, DeVroom HL, et al.Outcome of surgical treatment of 200 children with Cushing’s disease. J Clin Endocrinol Metab 2013;98: 892–901.Web of ScienceCrossrefPubMedGoogle Scholar

  • 11.

    Magiakou MA, Mastorakos G, Oldfield EH, Gomez MT, Doppman JL, et al. Cushing’s syndrome in children and cdolescents – presentation, diagnosis, and therapy. N Engl J Med 1994; 331:629–36.CrossrefPubMedGoogle Scholar

About the article

Corresponding author: Lissette Cespedes, MD, Assistant Professor of Medicine, Division of Endocrinology, Diabetes and Metabolism, Rutgers New Jersey Medical School, 185 South Orange Avenue, MSB I-588, Newark, NJ 07103, USA, Phone: +973-972-6170, Fax: +973-972-5185, E-mail: bleichda@njms.rutgers.edu

Received: 2018-11-20

Accepted: 2019-03-24

Published Online: 2019-06-28

Published in Print: 2019-07-26

Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

Research funding: None declared.

Employment or leadership: None declared.

Honorarium: None declared.

Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.

Disclosures: The authors have no disclosures.

Citation Information: Journal of Pediatric Endocrinology and Metabolism, Volume 32, Issue 7, Pages 785–789, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: https://doi.org/10.1515/jpem-2018-0506.

Export Citation

© 2019 Walter de Gruyter GmbH, Berlin/Boston.Get Permission

Comments (0)

Please log in or register to comment.
Log in