X-linked adrenoleukodystrophy (Q88707): Difference between revisions
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A adrenoleucodistrofia ligada ao X é uma doença peroxissomal caracterizada por desmielinização progressiva do sistema nervoso central (SNC) - cérebro e/ou medula espinhal - e insuficiência adrenal primária. As formas cerebrais juvenis (45% dos casos) afetam meninos de 5 a 12 anos de idade previamente saudáveis. A forma adulta, adrenomieloneuropatia (AMN), é caracterizada pelo aparecimento de paraparesia espástica entre os 20 e 45 anos de idade, associada a distúrbios da marcha, distúrbios urinários e disfunção sexual. | |||||||||||||||
| description / en | description / en | ||||||||||||||
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disease characterised by progressive demyelinisation of the central nervous system (CNS) (brain and/or spinal cord) and peripheral adrenal insufficiency. The cerebral forms of juvenile X-ALD (45% of the cases) affect previously healthy 5 to 12 year-old boys. The adult form, adrenomyeloneuropathy (AMN), is characterised by the onset of spastic paraparesia between 20 and 45 years of age, associated with gait disturbances, urinary disorders and sexual dysfunction. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/485676510 / rank | |||||||||||||||
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CID11:ID_485676510 | |||||||||||||||
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dki-india-ID_485676510 | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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13 August 2026
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| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
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Latest revision as of 15:05, 15 August 2026
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disease characterised by progressive demyelinisation of the central nervous system (CNS) (brain and/or spinal cord) and peripheral adrenal insufficiency. The cerebral forms of juvenile X-ALD (45% of the cases) affect previously healthy 5 to 12 year-old boys. The adult form, adrenomyeloneuropathy (AMN), is characterised by the onset of spastic paraparesia between 20 and 45 years of age, associated with gait disturbances, urinary disorders and sexual dysfunction.
| Language | Label | Description | Also known as |
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| default for all languages | ID_485676510 |
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| English | X-linked adrenoleukodystrophy |
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disease characterised by progressive demyelinisation of the central nervous system (CNS) (brain and/or spinal cord) and peripheral adrenal insufficiency. The cerebral forms of juvenile X-ALD (45% of the cases) affect previously healthy 5 to 12 year-old boys. The adult form, adrenomyeloneuropathy (AMN), is characterised by the onset of spastic paraparesia between 20 and 45 years of age, associated with gait disturbances, urinary disorders and sexual dysfunction. |
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CID11:ID_485676510
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dki-india-ID_485676510
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Concluído
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13 August 2026
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