| Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. | |
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MedLine Citation:
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PMID: 9141516 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We report nine consecutive children and adolescents [five females and four males; aged 2 yr 8 months (m) to 18 yr 1 m] studied over the last 5 yr with idiopathic central diabetes insipidus. In addition to vasopressin deficiency, anterior pituitary hormone deficiencies were detected, either on evaluation at presentation or during follow-up studies over the following 3 yr. Four patients had an increased concentration of plasma PRL. One patient had multiple pituitary hormone deficiencies at diagnosis, and two others developed the same by 21 m of follow-up. Brain magnestic resonance imaging scans, performed at presentation, were originally interpreted as normal in four of nine patients, except for absence of the bright posterior pituitary signal; after retrospective review, two of nine were considered normal. All of the brain magnetic resonance imaging (MRI) scans showed positive findings by 14 m of follow-up. The first abnormal finding in all patients was isolated pituitary stalk thickening. Evaluation of cerebrospinal fluid (CSF) for hCG was positive in three of eight evaluated patients; the three positive CSF values were found at presentation and 3 and 9 m after presentation. All eight patients assessed were negative for CSF alpha-fetoprotein and cytology, and no patient had serum tumor markers. Transsphenoidal biopsy of the lesion in seven of nine patients showed a germinoma in six patients and inflammatory cells in one. The six patients with documented germinoma comprise 31% of the intracranial germinomas diagnosed in this age group at the University of California-San Francisco during the last 5 yr. The patient with mononuclear inflammatory cells on biopsy along with one other patient have had spontaneous resolution of their stalk thickening. So-called "idiopathic" central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. Normal brain MRI scans or scans that show isolated pituitary stalk thickening merit follow-up with serial contrast enhanced brain MRI for the early detection of an evolving occult hypothalamic-stalk lesion. CSF evaluation is recommended at presentation because elevated CSF hCG may precede MRI abnormalities. |
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Authors:
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S L Mootha; A J Barkovich; M M Grumbach; M S Edwards; S E Gitelman; S L Kaplan; F A Conte |
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Publication Detail:
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Type: Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: The Journal of clinical endocrinology and metabolism Volume: 82 ISSN: 0021-972X ISO Abbreviation: J. Clin. Endocrinol. Metab. Publication Date: 1997 May |
Date Detail:
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Created Date: 1997-05-23 Completed Date: 1997-05-23 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0375362 Medline TA: J Clin Endocrinol Metab Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1362-7 Citation Subset: AIM; IM |
Affiliation:
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Department of Pediatrics, University of California, San Francisco 94143, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Biopsy Brain Neoplasms / complications*, pathology Child Child, Preschool Chorionic Gonadotropin / blood, cerebrospinal fluid Diabetes Insipidus / etiology*, pathology Female Germinoma / complications*, pathology Humans Hypothalamus / pathology* Magnetic Resonance Imaging Male Pituitary Gland / pathology* Pituitary Hormones, Anterior / deficiency Vasopressins / deficiency alpha-Fetoproteins / analysis |
| Grant Support | |
ID/Acronym/Agency:
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5T32-DK-07161/DK/NIDDK NIH HHS; M01-RR-01271/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Chorionic Gonadotropin; 0/Pituitary Hormones, Anterior; 0/alpha-Fetoproteins; 11000-17-2/Vasopressins |
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