Anterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients

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dc.contributor.authorNour, Munier A.
dc.contributor.authorLuca, Paola
dc.contributor.authorStephure, David
dc.contributor.authorWei, Xing-Chang
dc.contributor.authorKhan, Aneal
dc.date.accessioned2018-09-27T11:21:41Z
dc.date.available2018-09-27T11:21:41Z
dc.date.issued2016-11-28
dc.date.updated2018-09-27T11:21:41Z
dc.description.abstractHypopituitarism is a clinically important diagnosis and has not previously been reported in Hunter syndrome. We contrast two cases with anatomic pituitary anomalies: one with anterior panhypopituitarism and the other with intact pituitary function. Patient 1, a 10-year-old boy with Hunter syndrome, was evaluated for poor growth and an ectopic posterior pituitary gland. Endocrine testing revealed growth hormone (GH) deficiency, secondary adrenal insufficiency, and tertiary hypothyroidism. An improvement in growth velocity with hormone replacement (GH, thyroxine, and corticosteroid) was seen; however, final adult height remained compromised. Patient 2, a 13-year-old male with Hunter syndrome, was evaluated for growth failure. He had a large empty sella turcica with posteriorly displaced pituitary. Functional endocrine testing was normal and a trial of GH-treatment yielded no significant effect. Panhypopituitarism associated with pituitary anomalies has not been previously reported in Hunter syndrome and was an incidental finding of significant clinical importance. In the setting of documented anterior hypopituitarism, while hormone replacement improved growth velocity, final height remained impaired. In patient 2 with equivocal GH-testing results, treatment had no effect on linear growth. These cases highlight the importance of careful clinical assessment in Hunter syndrome and that judicious hormone replacement may be indicated in individual cases.
dc.description.versionPeer Reviewed
dc.identifier.citationMunier A. Nour, Paola Luca, David Stephure, Xing-Chang Wei, and Aneal Khan, “Anterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients,” Case Reports in Pediatrics, vol. 2016, Article ID 4328492, 5 pages, 2016. doi:10.1155/2016/4328492
dc.identifier.doihttps://doi.org/10.1155/2016/4328492
dc.identifier.urihttp://hdl.handle.net/1880/108116
dc.identifier.urihttps://doi.org/10.11575/PRISM/45775
dc.language.rfc3066en
dc.rights.holderCopyright © 2016 Munier A. Nour et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
dc.titleAnterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients
dc.typeJournal Article
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