Academic Journal
Adrenoleukodystrophy in a Chinese boy
| العنوان: | Adrenoleukodystrophy in a Chinese boy |
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| المؤلفون: | Wong, V |
| بيانات النشر: | //www.elsevier.com/locate/braindev Netherlands |
| سنة النشر: | 1992 |
| المجموعة: | University of Hong Kong: HKU Scholars Hub |
| مصطلحات موضوعية: | Oleic acid, Erucic acid, Adrenoleukodystrophy, Very long chain fatty acid, Gammaglobulin, Oleic Acids/therapeutic use, Adrenoleukodystrophy/diet therapy/drug therapy/*pathology, Brain/pathology, Child, Preschool, Erucic Acids/therapeutic use, Fludrocortisone/therapeutic use, Humans, Hydrocortisone/therapeutic use, Magnetic Resonance Imaging, Male, gamma-Globulins/therapeutic use |
| الوصف: | We report the first Chinese boy with adrenoleukodystrophy (ALD) who presented with hyperpigmentation, behavioral change and demyelination shown in magnetic resonance imaging of the brain. ALD was confirmed by the elevation of very long chain fatty acid in the serum and biochemical evidence of adrenal insufficiency. A trial of special diet with restriction of very long chain fatty acid and addition of glyceryl trierucate/glycerol trioleate oil (GTEO or Lorenzo's oil) failed to prevent clinical deterioration. The child had progressive visual loss and spastic tetraparesis despite dietary manipulation, adrenal steroid replacement and intravenous gammaglobulin treatment. ; link_to_subscribed_fulltext |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 0387-7604 |
| Relation: | Brain and Development; Brain And Development, 1992, v. 14 n. 4, p. 276-277; 277; WOS:A1992JL67300018; 1279999; eid_2-s2.0-0026709327; 276; http://hdl.handle.net/10722/143491; 14 |
| الاتاحة: | http://hdl.handle.net/10722/143491 |
| رقم الانضمام: | edsbas.34734E3 |
| قاعدة البيانات: | BASE |
| تدمد: | 03877604 |
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