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Article: Immunodeficiency in methylmalonic acidaemia

TitleImmunodeficiency in methylmalonic acidaemia
Authors
Keywordschildren, Chinese
immunodeficiency
methylmalonic acidaemia
Issue Date1992
Citation
Journal Of Paediatrics And Child Health, 1992, v. 28 n. 2, p. 180-183 How to Cite?
AbstractThree Chinese infants with methylmalonic acidaemia were described. They presented in the neonatal period with recurrent episodes of poor feeding, lethargy, apnoea and severe acidosis. The diagnosis was established by increased methylmalonic acid concentration in the plasma and/or urine. Pancytopenia was a prominent feature in all three patients. Only patient three had assessment of lymphocyte subsets and it showed diminished population of B-lymphocytes and a reversed CD4/CD8 ratio. All three patients were unresponsive to vitamin B12. They experienced severe infections including Gram-negative septicaemia, candidiasis and Pneumocystis carinii pneumonia which caused their deaths. Patients with this disease should be regarded as having severe immunodeficiency, and in addition to optimal metabolic control, they should be treated aggressively for any suspected infective episodes.
Persistent Identifierhttp://hdl.handle.net/10722/147919
ISSN
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorWong, SNen_HK
dc.contributor.authorLow, LCKen_HK
dc.contributor.authorLau, YLen_HK
dc.contributor.authorNicholls, Jen_HK
dc.contributor.authorChan, MYPen_HK
dc.date.accessioned2012-05-29T06:09:54Z-
dc.date.available2012-05-29T06:09:54Z-
dc.date.issued1992en_HK
dc.identifier.citationJournal Of Paediatrics And Child Health, 1992, v. 28 n. 2, p. 180-183en_HK
dc.identifier.issn0004-993Xen_HK
dc.identifier.urihttp://hdl.handle.net/10722/147919-
dc.description.abstractThree Chinese infants with methylmalonic acidaemia were described. They presented in the neonatal period with recurrent episodes of poor feeding, lethargy, apnoea and severe acidosis. The diagnosis was established by increased methylmalonic acid concentration in the plasma and/or urine. Pancytopenia was a prominent feature in all three patients. Only patient three had assessment of lymphocyte subsets and it showed diminished population of B-lymphocytes and a reversed CD4/CD8 ratio. All three patients were unresponsive to vitamin B12. They experienced severe infections including Gram-negative septicaemia, candidiasis and Pneumocystis carinii pneumonia which caused their deaths. Patients with this disease should be regarded as having severe immunodeficiency, and in addition to optimal metabolic control, they should be treated aggressively for any suspected infective episodes.en_HK
dc.languageengen_US
dc.relation.ispartofJournal of Paediatrics and Child Healthen_HK
dc.subjectchildren, Chineseen_HK
dc.subjectimmunodeficiencyen_HK
dc.subjectmethylmalonic acidaemiaen_HK
dc.subject.meshAmino Acid Metabolism, Inborn Errors - Blood - Immunologyen_US
dc.subject.meshAmino Acids - Analysisen_US
dc.subject.meshChromatography, Gasen_US
dc.subject.meshChromatography, High Pressure Liquiden_US
dc.subject.meshFemaleen_US
dc.subject.meshHumansen_US
dc.subject.meshImmunohistochemistryen_US
dc.subject.meshImmunologic Deficiency Syndromes - Complications - Immunologyen_US
dc.subject.meshInfant, Newbornen_US
dc.subject.meshLymphocyte Subsets - Immunologyen_US
dc.subject.meshMaleen_US
dc.subject.meshMethylmalonic Acid - Blooden_US
dc.subject.meshSepsis - Complicationsen_US
dc.titleImmunodeficiency in methylmalonic acidaemiaen_HK
dc.typeArticleen_HK
dc.identifier.emailLow, LCK: lcklow@hkucc.hku.hken_HK
dc.identifier.emailLau, YL: lauylung@hku.hken_HK
dc.identifier.emailNicholls, J: jmnichol@hkucc.hku.hken_HK
dc.identifier.authorityLow, LCK=rp00337en_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.identifier.authorityNicholls, J=rp00364en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid1562372-
dc.identifier.scopuseid_2-s2.0-0026606726en_HK
dc.identifier.volume28en_HK
dc.identifier.issue2en_HK
dc.identifier.spage180en_HK
dc.identifier.epage183en_HK
dc.identifier.isiWOS:A1992HL83300013-
dc.identifier.scopusauthoridWong, SN=7404590305en_HK
dc.identifier.scopusauthoridLow, LCK=7007049461en_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK
dc.identifier.scopusauthoridNicholls, J=7201463077en_HK
dc.identifier.scopusauthoridChan, MYP=36941297300en_HK

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