| Endocrine disorders associated with holoprosencephaly. | |
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MedLine Citation:
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PMID: 16355806 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To investigate the incidence of endocrinopathies in holoprosencephaly (HPE) and correlate the severity of the endocrinopathies with the neuroanatomic abnormalities. STUDY DESIGN: We reviewed the histories and medical records of 117 children with HPE for endocrinopathies and related treatments. Neuroimaging studies were graded for severity of HPE, hypothalamus non-separation, and pituitary abnormalities. RESULTS: Diabetes insipidus (DI) occurred in 70% of patients with classic HPE. The severity of the DI correlated with the grade of HPE and hypothalamic non-separation (p < 0.0001). Anterior pituitary dysfunctions were much less common. Hypothyroidism was identified in 11% of patients, hypocorticism in 7%, and growth hormone deficiency in 5%. Only one patient with middle interhemispheric variant of holoprosencephaly (MIH) had any of these disorders. CONCLUSIONS: Patients with HPE have a high incidence of DI that may be related to the failure of cleavage of hypothalamic nuclei. Anterior pituitary dysfunctions are much less common than DI. |
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Authors:
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Jin S Hahn; Sara M Hahn; Heather Kammann; A James Barkovich; Nancy J Clegg; Mauricio R Delgado; Eric Levey |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Journal of pediatric endocrinology & metabolism : JPEM Volume: 18 ISSN: 0334-018X ISO Abbreviation: J. Pediatr. Endocrinol. Metab. Publication Date: 2005 Oct |
Date Detail:
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Created Date: 2005-12-16 Completed Date: 2006-01-03 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9508900 Medline TA: J Pediatr Endocrinol Metab Country: England |
Other Details:
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Languages: eng Pagination: 935-41 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, Stanford University, Stanford, CA, 94305-5235, USA. jhahn@stanford.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Child Child, Preschool Diabetes Insipidus / etiology, pathology Endocrine System Diseases / etiology*, pathology Female Holoprosencephaly / complications*, pathology Humans Infant Male Pituitary Diseases / etiology, pathology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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