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Titolo:
Enhanced lymphocyte proliferation in patients with adrenoleukodystrophy treated with erucic acid (22 : 1)-rich triglycerides
Autore:
Barmaki Pour, R; Stockler-Ipsiroglu, S; Hunneman, DH; Gahr, M; Korenke, GC; Pabst, W; Hanefeld, F; Peters, A;
Indirizzi:
Univ Gottingen, Childrens Hosp, Dept Neuropediat, D-3400 Gottingen, Germany Univ Gottingen Gottingen Germany D-3400 ediat, D-3400 Gottingen, Germany Univ Vienna, Dept Pediat, A-1010 Vienna, Austria Univ Vienna Vienna Austria A-1010 a, Dept Pediat, A-1010 Vienna, Austria Univ Hosp Giessen, Dept Med Informat, Giessen, Germany Univ Hosp Giessen Giessen Germany , Dept Med Informat, Giessen, Germany Univ Dresden, Dept Pediat, Dresden, Germany Univ Dresden Dresden Germany niv Dresden, Dept Pediat, Dresden, Germany Univ Freiburg, Dept Pediat, D-7800 Freiburg, Germany Univ Freiburg Freiburg Germany D-7800 t Pediat, D-7800 Freiburg, Germany
Titolo Testata:
JOURNAL OF INHERITED METABOLIC DISEASE
fascicolo: 2, volume: 23, anno: 2000,
pagine: 113 - 119
SICI:
0141-8955(200003)23:2<113:ELPIPW>2.0.ZU;2-T
Fonte:
ISI
Lingua:
ENG
Soggetto:
X-LINKED ADRENOLEUKODYSTROPHY; LORENZO OIL; THERAPY; ADRENOMYELONEUROPATHY; THROMBOCYTOPENIA;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
13
Recensione:
Indirizzi per estratti:
Indirizzo: Stockler-Ipsiroglu, S Univ Childrens Hosp, Wahringer Gurtel 18-20, A-1090 Vienna, Austria Univ Childrens Hosp Wahringer Gurtel 18-20 Vienna Austria A-1090
Citazione:
R. Barmaki Pour et al., "Enhanced lymphocyte proliferation in patients with adrenoleukodystrophy treated with erucic acid (22 : 1)-rich triglycerides", J INH MET D, 23(2), 2000, pp. 113-119

Abstract

Lymphocytopenia and depression of natural killer cells have been observed in patients with adrenoleukodystrophy (ALD) treated with glycerol trioleateand glycerol trierucate ('Lorenzo's oil'). To investigate possible alterations of cellular immunoreactivity, we measured lymphocyte proliferation in response to mitogens (PHA, Con A, PWM, OKT3) in 27 patients on treatment and in 14 patients without treatment. In patients on treatment, lymphocyte proliferation in response to the mitogens PHA and Con A was significantly higher than in patients without treatment. Lymphocyte proliferation in patients without treatment was comparable to that of normal control lymphocytes. Additionally, we found increased concentrations of erucic acid, C-22:1, in lymphocytes from patients with treatment. The enhanced proliferation of lymphocytes in response to mitogens is an indication of increased reactivity ofcellular immunity to unspecific immunological stimuli. Long-term side-effects on cellular immunoreactivity have to be considered in ALD patients treated with Lorenzo's oil.

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Documento generato il 26/10/20 alle ore 05:21:10