Document Detail

B and T cell immunity in patients with lysinuric protein intolerance.
MedLine Citation:
PMID:  10361230     Owner:  NLM     Status:  MEDLINE    
Lysinuric protein intolerance (LPI) is characterized by defective cellular transport of the dibasic amino acids, secondary dysfunction of the urea cycle, aversion to dietary protein, failure to thrive, hepatosplenomegaly and osteoporosis. Because several patients have suffered from recurrent respiratory infections and/or severe generalized varicella, and a few have developed systemic lupus, vasculitis or other autoimmune diseases, we have now evaluated the function of patients' immune systems. Serum concentrations of one to three IgG subclasses were decreased in 10 of the 12 patients studied. Antibody titres against diphtheria, tetanus and Haemophilus influenzae (Hib) were below the detection limit of the assay in four, three and eight of the 11 patients examined, respectively. (Re)vaccination of these 11 patients led to satisfactory responses against tetanus, but two patients still failed to develop measurable antibodies against diphtheria, two against Hib and six against one or more of the three serotypes of 23-valent pneumococcus vaccine. The proportions of T cells of all lymphocytes and the proliferative responses of the peripheral blood mononuclear cells were normal. In conclusion, humoral immune responses in some patients with LPI are defective and these patients may benefit from intravenous immunoglobulin therapy.
M Lukkarinen; K Parto; O Ruuskanen; O Vainio; H Käyhty; R M Olander; O Simell
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Clinical and experimental immunology     Volume:  116     ISSN:  0009-9104     ISO Abbreviation:  Clin. Exp. Immunol.     Publication Date:  1999 Jun 
Date Detail:
Created Date:  1999-06-23     Completed Date:  1999-06-23     Revised Date:  2013-06-11    
Medline Journal Info:
Nlm Unique ID:  0057202     Medline TA:  Clin Exp Immunol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  430-4     Citation Subset:  IM    
Department of Paediatrics, University of Turku, Finland.
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MeSH Terms
Amino Acid Metabolism, Inborn Errors / immunology*,  therapy
Ammonia / blood
B-Lymphocytes / immunology*
Child, Preschool
Immune Tolerance
Immunoglobulin G / blood
Immunoglobulins, Intravenous / therapeutic use
Lysine / urine*
Middle Aged
T-Lymphocytes / immunology*
Reg. No./Substance:
0/Immunoglobulin G; 0/Immunoglobulins, Intravenous; 56-87-1/Lysine; 7664-41-7/Ammonia

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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