Literature DB >> 25388128

A gender-dependent analysis of Cushing's disease in childhood: pre- and postoperative follow-up.

Laura G Libuit1, Alexander S Karageorgiadis1, Ninet Sinaii2, Nina M Nguyen May1, Margaret F Keil1, Maya B Lodish1, Constantine A Stratakis1.   

Abstract

OBJECTIVE: To analyse gender differences in the clinical presentation and recovery of paediatric patients with Cushing's disease (CD) after transsphenoidal surgery (TSS). Indeed, gender differences between paediatric patients with CD during presentation, after TSS and postoperative recovery have not been adequately studied.
DESIGN: Data were obtained and retrospectively analysed from clinical reports and biochemical tests at the time of presentation, 5-9 days after TSS and at the 6 and 12 months postoperative follow-up visits to determine hypothalamic-pituitary-adrenal axis (HPAA) recovery. PATIENTS: Data from 102 paediatric patients (48 females, 54 males, mean age 12.9 ± 3.0) with CD who underwent TSS at the National Institute of Health (NIH) Clinical Center between 1997 and 2011.
RESULTS: There was equal distribution of paediatric CD between males and females (53% vs 47%; n = 102, P = 0.484). Males were more likely than females to present with higher mean BMI Z-scores (2.2 ± 0.7 vs 1.9 ± 0.6, P = 0.0079), lower mean height Z-scores (-1.2 ± 1.3 vs -0.7 ± 1.1, P = 0.0467) and higher median plasma ACTH (12.2 vs 8.5 pmol/l; P = 0.0495). Females did not present more frequently with any single sign or symptom. No significant differences were found between males and females for CD cure rates 5-9 days after TSS (87.0% males vs 87.5% females, P = 1.0), long-term cure rates (86.5% vs 93.7%; n = 69; P = 0.4374) and HPAA recovery time (11.2 ± 2.5 vs 11.7 ± 2.5 months; n = 47; P = 0.1992).
CONCLUSIONS: Paediatric CD is found to have equal distribution between males and females, but male patients present with elevated BMI and potentially shorter height and higher plasma ACTH. There is no significant difference in the cure rate or HPAA recovery time after TSS between males and females. Published 2014. This article is a U.S. Government work and is in the public domain in the USA.

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Year:  2015        PMID: 25388128      PMCID: PMC6342464          DOI: 10.1111/cen.12644

Source DB:  PubMed          Journal:  Clin Endocrinol (Oxf)        ISSN: 0300-0664            Impact factor:   3.478


  12 in total

Review 1.  Neonatal Cushing Syndrome: A Rare but Potentially Devastating Disease.

Authors:  Christina Tatsi; Constantine A Stratakis
Journal:  Clin Perinatol       Date:  2017-12-12       Impact factor: 3.430

2.  The Gene of the Ubiquitin-Specific Protease 8 Is Frequently Mutated in Adenomas Causing Cushing's Disease.

Authors:  Luis G Perez-Rivas; Marily Theodoropoulou; Francesco Ferraù; Clara Nusser; Kohei Kawaguchi; Constantine A Stratakis; Fabio Rueda Faucz; Luiz E Wildemberg; Guillaume Assié; Rudi Beschorner; Christina Dimopoulou; Michael Buchfelder; Vera Popovic; Christina M Berr; Miklós Tóth; Arif Ibrahim Ardisasmita; Jürgen Honegger; Jerôme Bertherat; Monica R Gadelha; Felix Beuschlein; Günter Stalla; Masayuki Komada; Márta Korbonits; Martin Reincke
Journal:  J Clin Endocrinol Metab       Date:  2015-05-05       Impact factor: 5.958

Review 3.  Diagnosis and Clinical Genetics of Cushing Syndrome in Pediatrics.

Authors:  Constantine A Stratakis
Journal:  Endocrinol Metab Clin North Am       Date:  2016-06       Impact factor: 4.741

4.  Coagulation Profile Dynamics in Pediatric Patients with Cushing Syndrome: A Prospective, Observational Comparative Study.

Authors:  Leah Birdwell; Maya Lodish; Amit Tirosh; Prashant Chittiboina; Meg Keil; Charlampos Lyssikatos; Elena Belyavskaya; Richard A Feelders; Constantine A Stratakis
Journal:  J Pediatr       Date:  2016-08-02       Impact factor: 4.406

Review 5.  Genetics of Cushing's disease: an update.

Authors:  L G Perez-Rivas; M Reincke
Journal:  J Endocrinol Invest       Date:  2015-07-25       Impact factor: 4.256

6.  Kidney Stones as an Underrecognized Clinical Sign in Pediatric Cushing Disease.

Authors:  Sara H Rahman; Georgios Z Papadakis; Margaret F Keil; Fabio R Faucz; Maya B Lodish; Constantine A Stratakis
Journal:  J Pediatr       Date:  2015-12-15       Impact factor: 4.406

7.  Aortic pulse wave velocity in children with Cushing syndrome: A window into a marker of early cardiovascular disease.

Authors:  Hailey Blain; Ninet Sinaii; Deena Zeltser; Charalampos Lyssikatos; Elena Belyavskaya; Margaret Keil; David A Bluemke; Constantine Stratakis; Wiphada Patricia Bandettini; Maya Lodish
Journal:  Endocrinol Diabetes Metab       Date:  2019-01-15

8.  Lipoprotein particles in patients with pediatric Cushing disease and possible cardiovascular risks.

Authors:  Angeliki Makri; Anita Cheung; Ninet Sinaii; Alan T Remaley; Maureen Sampson; Meg Keil; Elena Belyavskaya; Charalampos Lyssikatos; Maria De La Luz Sierra; Constantine A Stratakis; Maya Lodish
Journal:  Pediatr Res       Date:  2019-05-21       Impact factor: 3.756

9.  Decreased lymphocytes and increased risk for infection are common in endogenous pediatric Cushing syndrome.

Authors:  Christina Tatsi; Rebecca Boden; Ninet Sinaii; Meg Keil; Charalampos Lyssikatos; Elena Belyavskaya; Sergio D Rosenzweig; Constantine A Stratakis; Maya B Lodish
Journal:  Pediatr Res       Date:  2017-12-06       Impact factor: 3.756

10.  Sexual Dimorphism in Cellular and Molecular Features in Human ACTH-Secreting Pituitary Adenomas.

Authors:  Francesca Pecori Giraldi; Maria Francesca Cassarino; Antonella Sesta; Mariarosa Terreni; Giovanni Lasio; Marco Losa
Journal:  Cancers (Basel)       Date:  2020-03-13       Impact factor: 6.639

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