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Article: Immunologic profile of patients with protein-losing enteropathy complicating congenital heart disease

TitleImmunologic profile of patients with protein-losing enteropathy complicating congenital heart disease
Authors
KeywordsCongenital Heart Disease
Immunologic Profile
Protein-Losing Enteropathy
Issue Date2002
PublisherSpringer New York LLC. The Journal's web site is located at http://link.springer.de/link/service/journals/00246
Citation
Pediatric Cardiology, 2002, v. 23 n. 6, p. 587-593 How to Cite?
AbstractThe immunologic profile of patients with congenital heart disease complicated by protein-losing enteropathy (PLE) is undefined. The aim of this study was to assess the lymphocyte subpopulation and immunglobulin (Ig) pattern in patients with PLE complicating congenital heart disease. The immunologic profile of six patients with congenital heart disease complicated by PLE was compared to that of controls without PLE matched for age and cardiac interventions. Enteric protein loss was documented by Tc99m-labeled albumin scintigraphy. The lymphocyte subpopulations were enumerated using flow cytometry, whereas serum IgG, IgA, and IgM concentrations were measured by the turbidimetric technique. The cardiac diagnoses included complex cyanotic heart disease post-Fontan procedure (n = 3), and one each of tetralogy of Fallot, restrictive cardiomyopathy, and valvar pulmonary stenosis. In patients with PLE, the T lymphocyte (CD3 +) count was significantly lower (300 ± 186 vs 2070 ± 1171/μl, p = 0.017); both the helper/inducer lymphocytes (CD4 +) (127 ± 158 vs 927 ± 377/μl, p = 0.006) and suppressor/cytotoxic lymphocytes (CD8 +) (129 ± 49 vs 850 ± 695/μl, p = 0.057) were reduced with reversal of CD4 +/CD8 + ratio (0.81 ± 0.68 vs 1.64 ± 0.89, p = 0.027). Furthermore, IgG level was significantly reduced (5.12 ± 2.84 vs 12.5 ± 1.58 g/L, p = 0.005) and IgA level tended to be lower (1.36 ± 1.37 vs 2.50 ± 0.80 g/L, p = 0.095). In contrast, the B lymphocyte (CD19 +) count (340 ± 151 vs 618 ± 427/μl, p = 0.25), natural killer cell count (CD16 +56 +CD3 -) (252 ± 212 vs 276 ± 251/μl, p = 0.85), and IgM level (0.98 ± 0.59 vs 1.12 ± 0.25 g/L, p = 0.67) were similar for both groups. None of the patients developed opportunistic or severe viral infections. Abnormal immunologic profile of both the cellular and humoral arms of the immune system occurs in patients with congenital heart disease complicated by PLE. Nonetheless, these abnormalities perhaps appear quantitative rather than qualitative in nature, although further functional studies of antibody production and lymphocyte proliferation assays are required to support this proposition.
Persistent Identifierhttp://hdl.handle.net/10722/170321
ISSN
2015 Impact Factor: 1.452
2015 SCImago Journal Rankings: 0.732
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorCheung, YFen_US
dc.contributor.authorTsang, HYHen_US
dc.contributor.authorKwok, JSYen_US
dc.date.accessioned2012-10-30T06:07:29Z-
dc.date.available2012-10-30T06:07:29Z-
dc.date.issued2002en_US
dc.identifier.citationPediatric Cardiology, 2002, v. 23 n. 6, p. 587-593en_US
dc.identifier.issn0172-0643en_US
dc.identifier.urihttp://hdl.handle.net/10722/170321-
dc.description.abstractThe immunologic profile of patients with congenital heart disease complicated by protein-losing enteropathy (PLE) is undefined. The aim of this study was to assess the lymphocyte subpopulation and immunglobulin (Ig) pattern in patients with PLE complicating congenital heart disease. The immunologic profile of six patients with congenital heart disease complicated by PLE was compared to that of controls without PLE matched for age and cardiac interventions. Enteric protein loss was documented by Tc99m-labeled albumin scintigraphy. The lymphocyte subpopulations were enumerated using flow cytometry, whereas serum IgG, IgA, and IgM concentrations were measured by the turbidimetric technique. The cardiac diagnoses included complex cyanotic heart disease post-Fontan procedure (n = 3), and one each of tetralogy of Fallot, restrictive cardiomyopathy, and valvar pulmonary stenosis. In patients with PLE, the T lymphocyte (CD3 +) count was significantly lower (300 ± 186 vs 2070 ± 1171/μl, p = 0.017); both the helper/inducer lymphocytes (CD4 +) (127 ± 158 vs 927 ± 377/μl, p = 0.006) and suppressor/cytotoxic lymphocytes (CD8 +) (129 ± 49 vs 850 ± 695/μl, p = 0.057) were reduced with reversal of CD4 +/CD8 + ratio (0.81 ± 0.68 vs 1.64 ± 0.89, p = 0.027). Furthermore, IgG level was significantly reduced (5.12 ± 2.84 vs 12.5 ± 1.58 g/L, p = 0.005) and IgA level tended to be lower (1.36 ± 1.37 vs 2.50 ± 0.80 g/L, p = 0.095). In contrast, the B lymphocyte (CD19 +) count (340 ± 151 vs 618 ± 427/μl, p = 0.25), natural killer cell count (CD16 +56 +CD3 -) (252 ± 212 vs 276 ± 251/μl, p = 0.85), and IgM level (0.98 ± 0.59 vs 1.12 ± 0.25 g/L, p = 0.67) were similar for both groups. None of the patients developed opportunistic or severe viral infections. Abnormal immunologic profile of both the cellular and humoral arms of the immune system occurs in patients with congenital heart disease complicated by PLE. Nonetheless, these abnormalities perhaps appear quantitative rather than qualitative in nature, although further functional studies of antibody production and lymphocyte proliferation assays are required to support this proposition.en_US
dc.languageengen_US
dc.publisherSpringer New York LLC. The Journal's web site is located at http://link.springer.de/link/service/journals/00246en_US
dc.relation.ispartofPediatric Cardiologyen_US
dc.subjectCongenital Heart Diseaseen_US
dc.subjectImmunologic Profileen_US
dc.subjectProtein-Losing Enteropathyen_US
dc.titleImmunologic profile of patients with protein-losing enteropathy complicating congenital heart diseaseen_US
dc.typeArticleen_US
dc.identifier.emailCheung, YF:xfcheung@hku.hken_US
dc.identifier.authorityCheung, YF=rp00382en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1007/s00246-001-0078-zen_US
dc.identifier.pmid12530489-
dc.identifier.scopuseid_2-s2.0-0036879060en_US
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0036879060&selection=ref&src=s&origin=recordpageen_US
dc.identifier.volume23en_US
dc.identifier.issue6en_US
dc.identifier.spage587en_US
dc.identifier.epage593en_US
dc.identifier.isiWOS:000179055100003-
dc.publisher.placeUnited Statesen_US
dc.identifier.scopusauthoridCheung, YF=7202111067en_US
dc.identifier.scopusauthoridTsang, HYH=7006768516en_US
dc.identifier.scopusauthoridKwok, JSY=7006208874en_US

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