TY - JOUR
T1 - Defective humoral and cellular immune functions associated with veno-occlusive disease of the liver
AU - Etzioni, A.
AU - Benderly, A.
AU - Rosenthal, E.
AU - Shehadah, V.
AU - Austander, L.
AU - Lathat, N.
AU - Pollack, S.
PY - 1987/4
Y1 - 1987/4
N2 - We describe veno-occlusive disease of the liver associated with humoral and cellular immune defects in two siblings. Another child, with aplastic anemia, died before the age of 1 year. No consangulinity was found in the family. Both infants had lymphopenia and hypogammaglobulinemia; the surviving infant has defective in vitro immunoglobulin production after stimulation of lymphocytes with pokeweed mifogen, increased proportions of OKT8 positive cells, defective proliferative responses to phytomitogens, and decreased help for immunoglobulin production. A therapeutic trial with cimetidine, an H2 receptor antagonist, has not changed the immunologic status of the surviving child.
AB - We describe veno-occlusive disease of the liver associated with humoral and cellular immune defects in two siblings. Another child, with aplastic anemia, died before the age of 1 year. No consangulinity was found in the family. Both infants had lymphopenia and hypogammaglobulinemia; the surviving infant has defective in vitro immunoglobulin production after stimulation of lymphocytes with pokeweed mifogen, increased proportions of OKT8 positive cells, defective proliferative responses to phytomitogens, and decreased help for immunoglobulin production. A therapeutic trial with cimetidine, an H2 receptor antagonist, has not changed the immunologic status of the surviving child.
UR - http://www.scopus.com/inward/record.url?scp=0023117520&partnerID=8YFLogxK
U2 - 10.1016/s0022-3476(87)80546-2
DO - 10.1016/s0022-3476(87)80546-2
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C2 - 3559802
AN - SCOPUS:0023117520
SN - 0022-3476
VL - 110
SP - 549
EP - 554
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 4
ER -