| Craniofacial and brain abnormalities in Laron syndrome (primary growth hormone insensitivity). | |
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MedLine Citation:
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PMID: 11916617 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To investigate abnormalities in the craniofacial structures and in the brain in patients with Laron syndrome. DESIGN: Eleven patients with classical Laron syndrome, nine untreated adults aged 36-68 years and two children aged 4 and 9 years (the latter treated by IGF-I), were studied. METHODS: Magnetic resonance images of the brain were obtained in all the patients. One patient also underwent computed tomography. The maximal diameter of the maxillary and frontal sinuses was measured and compared with reference values, the size of the sphenoid sinus was evaluated in relation to the sella, and the mastoids were evaluated qualitatively (small or normal). The brain was evaluated for congenital anomalies and parenchymal lesions. RESULTS: In the adult untreated patients, the paranasal sinuses and mastoids were small; in six patients, the bone marrow in the base of the skull was not mature. The diploe of the calvaria was thin. On computed tomography in one adult patient, the sutures were still open. A minimal or mild degree of diffuse brain parenchymal loss was seen in ten patients. One patient demonstrated a lacunar infarct and another periventricular high signals on T2-weighted images. Two patients had cerebellar atrophy. CONCLUSIONS: The present study has demonstrated the important role IGF-I plays in the development of the brain and bony structures of the cranium. |
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Authors:
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L Kornreich; G Horev; M Schwarz; B Karmazyn; Z Laron |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: European journal of endocrinology / European Federation of Endocrine Societies Volume: 146 ISSN: 0804-4643 ISO Abbreviation: Eur. J. Endocrinol. Publication Date: 2002 Apr |
Date Detail:
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Created Date: 2002-03-27 Completed Date: 2002-06-03 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 9423848 Medline TA: Eur J Endocrinol Country: England |
Other Details:
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Languages: eng Pagination: 499-503 Citation Subset: IM |
Affiliation:
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Imaging Department, Schneider Children's Medical Center of Israel, Petah Tiqva, Israel. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Brain / abnormalities*, pathology Craniofacial Abnormalities / complications*, diagnosis Female Growth Disorders / complications*, physiopathology* Human Growth Hormone / physiology* Humans Magnetic Resonance Imaging Male Middle Aged Syndrome Tomography, X-Ray Computed |
| Chemical | |
Reg. No./Substance:
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12629-01-5/Human Growth Hormone |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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