Post-operative growth is different in various forms of pediatric Cushing's syndrome

    1. Constantine A Stratakis1,2
    1. 1Section on Endocrinology and GeneticsProgram on Developmental Endocrinology and Genetics (PDEGEN), Bethesda, MarylandUSA
      2Pediatric Endocrinology Inter-Institute Training ProgramEunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, 20892USA
      3Department of Pediatric EndocrinologyGeorgetown University School of Medicine, 3800 Reservoir Road, Washington, District of Columbia, 20007USA
      4Biostatistics and Clinical Epidemiology ServiceNational Institutes of Health Clinical Center, Bethesda, Maryland, 20982USA
      5Department of RadiologyNational Institutes of Health Clinical Center, Bethesda, Maryland, 20892USA
    1. Correspondence should be addressed to E Gourgari,Division of Pediatric Endocrinology Georgetown University Hospital, 4200 Wisconsin Avenue, N.W, 4th Floor, Washington DC 20016, USA; Email: evgenia.gourgari{at}

    Dear Editor,

    Cushing's syndrome (CS) is a rare disease in children it is associated with weight gain and stunting of their linear growth (McArthur et al. 1980, Magiakou et al. 1994a, Stratakis 2012). In this study, we assessed growth in pediatric patients with CS after cure, caused by either adrenocorticotropic hormone (ACTH)-dependent Cushing's disease (CD) or a form of ACTH-independent CS, and patients with micronodular adrenal hyperplasia (MAH).

    We reviewed medical records of patients who had successful transsphenoidal surgery or adrenalectomy at the NIH between the years of 2002 and 2012. A total of 18 children with CD (9 F, mean age 12.2±3.0 years) and 19 children with MAH (15 F, mean age 9.8±4.4 years) were included. All patients were evaluated under the clinical protocol 97CH0076 and 95CH0059 that were approved by the NICHD Institutional Review Board. Informed consent was signed from the patients' parents. The diagnosis of CS was established as previously reported (Batista et al. 2007). Patients that were at Tanner stage 5 at the time of surgery were excluded from the analysis of annual growth velocity and insulin-like growth factor 1 (IGF1) z scores.

    The mean follow-up after surgery was 402±27 days for the CD and 365±87 days for the MAH patients (P=0.09). No significant difference was found in terms of mean age (although CD patients were 2.4 years older), gender distribution, duration of symptoms, midnight cortisol levels, IGF1 z score, and mean urinary free cortisol at the time of surgery. The demographics are presented in Table 1. The baseline height (Ht) z scores and BMI z scores were not significantly different between the two groups (P=0.85 and P=0.66 respectively; Fig. 1A).

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    Table 1

    Clinical characteristics of CS patients at time of surgery, delta height z scores and delta BMI …

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