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Pituitary-dependent Cushing disease and primary adrenocortical nodular dysplasia in childhood

Presentation of 4 cases

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Abstract

Four patients with Cushing syndrome were followed for 1.5–12 years. The main clinical symptoms were marked growth retardation and truncal obesity. Two patients had pituitary-dependent Cushing disease, while the other two had primary adrenocortical nodular dysplasia.

The treatment of choice of pituitary-dependent Cushing disease is transsphenoidal resection of the microadenoma while in primary adrenocortical nodular dysplasia bilateral adrenalectomy with subsequent steroid replacement is recommended. In our pre-or early pubertal patients catch-up growth occurred following surgical therapy and in all cases pubertal development proceeded normally.

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Authors and Affiliations

  1. Department of Pediatric Surgery, University Hospital/Sophia Children's Hospital, Rotterdam, The Netherlands

    F. W. J. Hazebroek

  2. Department of Pathology, Erasmus University, Rotterdam, The Netherlands

    F. W. J. ten Kate

  3. Division of Endocrinology, Department of Pediatrics, Sophia Children's Hospital, Erasmus University, Gordelweg 160, 3038 GE, Rotterdam, The Netherlands

    R. H. J. Houwen & S. L. S. Drop

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  1. R. H. J. Houwen
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  2. S. L. S. Drop
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  3. F. W. J. Hazebroek
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  4. F. W. J. ten Kate
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Houwen, R.H.J., Drop, S.L.S., Hazebroek, F.W.J. et al. Pituitary-dependent Cushing disease and primary adrenocortical nodular dysplasia in childhood. Eur J Pediatr 141, 101–108 (1983). https://doi.org/10.1007/BF00496799

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  • DOI: https://doi.org/10.1007/BF00496799

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