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Free Content Neuroimaging Review of Pediatric Endocrinopathies

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The hypothalamic-pituitary axis regulates many important functions in a child, including growth, puberty, and maintenance of physiologic homeostasis. Dysfunction of the hypothalamic-pituitary axis can produce hormone disturbances that result in various pediatric neuroendocrinopathies, including an isolated growth hormone deficiency, multiple pituitary hormone deficiencies, precocious or delayed puberty, central diabetes insipidus, and elevated pituitary hormone conditions (eg, hyperprolactinemia). Although the hormonal abnormalities in a child with endocrinopathy are typically well characterized with clinical and laboratory evaluations by an endocrinologist, neuroimaging with brain MR imaging can be critical for identifying an underlying etiology to guide prognosis and treatment. Neuroendocrinopathies can be congenital, such as from genetic mutations involved in hypothalamic-pituitary axis development, or acquired, such as with pediatric suprasellar masses, trauma, or inflammatory conditions, or after cranial irradiation. This article reviews the clinical presentations, pathophysiologies, etiologies, and MR imaging findings in children who present with specific types of neuroendocrinopathies.

Learning Objective: To review the pathophysiologies, etiologies, and associated neuroimaging findings in the most commonly imaged pediatric neuroendocrinopathies.
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Keywords: ADH = antidiuretic hormone; DI = diabetes insipidus; FSH = follicle-stimulating hormone; GHRH = growth hormone‐releasing hormone; GH = growth hormone; GnRH = gonadotropin-releasing hormone; HPA = hypothalamic-pituitary axis; LCH = Langerhans cell histiocytosis; LH = luteinizing hormone; MAS = McCune-Albright syndrome; MEN1 = multiple endocrine neoplasia type 1; NF1 = neurofibromatosis type 1; OPG = optic pathway glioma; PPBS = posterior pituitary bright spot; RCC = Rathke cleft cyst; SD = standard deviation; SOD = septooptic dysplasia; WHO = World Health Organization

Document Type: Research Article

Publication date: February 1, 2020

More about this publication?
  • Neurographics is the peer-reviewed, bimonthly educational journal of the American Society of Neuroradiology. The journal comprises articles selected from material presented at the ASNR Annual Meeting. Neurographics also publishes other high-quality submissions that are primarily educational and have a high emphasis on a pictorial approach. Neurographics offers CME credit for reading review articles and completing quiz-based self-assessment activities.

    Visit to view all available CME courses.

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