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- Publisher Website: 10.1016/S0303-8467(87)80045-8
- Scopus: eid_2-s2.0-0023269465
- PMID: 3665286
- WOS: WOS:A1987K375700002
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Article: Ataxia telangiectasia in Chinese children. A clinical and electrophysiological study
Title | Ataxia telangiectasia in Chinese children. A clinical and electrophysiological study |
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Authors | |
Keywords | Ataxia telangiectasia (Louis Bar Syndrome) cerebellar atrophy electrophysiological studies hepatocellular carcinoma |
Issue Date | 1987 |
Publisher | Elsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/clineuro |
Citation | Clinical Neurology And Neurosurgery, 1987, v. 89 n. 3, p. 137-144 How to Cite? |
Abstract | The clinical manifestations, immunological, chromosomal, and multimodal electrophysiological studies of five Chinese patients with ataxia telangiectasia are described. One died of hepatocellular carcinoma not associated with Hepatitis B-antigenaemia. Another died of respiratory failure. Two siblings are free of sinopulmonary infections although they are wheelchair bound. Computed tomography of the brain showed cerebellar atrophy in four cases. Nerve conduction studies showed evidence of axonal neuropathy in all cases with the earliest detection at six years. Electromyography showed mild denervation changes in two cases. Two patients had abnormal somatosensory evoked potentials and one had abnormal visual and brain stem auditory evoked potentials. The level of alpha foetal protein was elevated whereas the serum carcino-'mbryonic antigen was normal in all patients. |
Persistent Identifier | http://hdl.handle.net/10722/143505 |
ISSN | 2023 Impact Factor: 1.8 2023 SCImago Journal Rankings: 0.608 |
ISI Accession Number ID |
DC Field | Value | Language |
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dc.contributor.author | Wong, V | en_HK |
dc.contributor.author | Yu, YL | en_HK |
dc.contributor.author | ChanLui, WY | en_HK |
dc.date.accessioned | 2011-12-12T03:51:19Z | - |
dc.date.available | 2011-12-12T03:51:19Z | - |
dc.date.issued | 1987 | en_HK |
dc.identifier.citation | Clinical Neurology And Neurosurgery, 1987, v. 89 n. 3, p. 137-144 | en_HK |
dc.identifier.issn | 0303-8467 | en_HK |
dc.identifier.uri | http://hdl.handle.net/10722/143505 | - |
dc.description.abstract | The clinical manifestations, immunological, chromosomal, and multimodal electrophysiological studies of five Chinese patients with ataxia telangiectasia are described. One died of hepatocellular carcinoma not associated with Hepatitis B-antigenaemia. Another died of respiratory failure. Two siblings are free of sinopulmonary infections although they are wheelchair bound. Computed tomography of the brain showed cerebellar atrophy in four cases. Nerve conduction studies showed evidence of axonal neuropathy in all cases with the earliest detection at six years. Electromyography showed mild denervation changes in two cases. Two patients had abnormal somatosensory evoked potentials and one had abnormal visual and brain stem auditory evoked potentials. The level of alpha foetal protein was elevated whereas the serum carcino-'mbryonic antigen was normal in all patients. | en_HK |
dc.language | eng | en_US |
dc.publisher | Elsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/clineuro | en_HK |
dc.relation.ispartof | Clinical Neurology and Neurosurgery | en_HK |
dc.subject | Ataxia telangiectasia (Louis Bar Syndrome) | - |
dc.subject | cerebellar atrophy | - |
dc.subject | electrophysiological studies | - |
dc.subject | hepatocellular carcinoma | - |
dc.subject.mesh | Adolescent | en_US |
dc.subject.mesh | Ataxia Telangiectasia/*physiopathology | en_US |
dc.subject.mesh | Cerebellum/physiology | en_US |
dc.subject.mesh | Child | en_US |
dc.subject.mesh | Electromyography | en_US |
dc.subject.mesh | Electrophysiology | en_US |
dc.subject.mesh | Evoked Potentials | en_US |
dc.subject.mesh | Female | en_US |
dc.subject.mesh | Hong Kong | en_US |
dc.subject.mesh | Humans | en_US |
dc.subject.mesh | Male | en_US |
dc.subject.mesh | Neural Conduction | en_US |
dc.title | Ataxia telangiectasia in Chinese children. A clinical and electrophysiological study | en_HK |
dc.type | Article | en_HK |
dc.identifier.email | Wong, V:vcnwong@hku.hk | en_HK |
dc.identifier.authority | Wong, V=rp00334 | en_HK |
dc.description.nature | link_to_subscribed_fulltext | - |
dc.identifier.doi | 10.1016/S0303-8467(87)80045-8 | - |
dc.identifier.pmid | 3665286 | - |
dc.identifier.scopus | eid_2-s2.0-0023269465 | en_HK |
dc.identifier.volume | 89 | en_HK |
dc.identifier.issue | 3 | en_HK |
dc.identifier.spage | 137 | en_HK |
dc.identifier.epage | 144 | en_HK |
dc.identifier.isi | WOS:A1987K375700002 | - |
dc.publisher.place | Netherlands | en_HK |
dc.identifier.scopusauthorid | Wong, V=7202525632 | en_HK |
dc.identifier.scopusauthorid | Yu, YL=8094845300 | en_HK |
dc.identifier.scopusauthorid | ChanLui, WY=6507549100 | en_HK |
dc.identifier.issnl | 0303-8467 | - |