SAETHRE-CHOTZEN SYNDROME ASSOCIATED WITH DEFECTIVE NEUTROPHIL CHEMOTAXIS

被引:5
作者
ETZIONI, A
OBEDEANU, N
BENDERLY, A
GERSHONIBARUCH, R
机构
[1] TECHNION ISRAEL INST TECHNOL,RAMBAM MED CTR,DEPT MICROBIOL,HAIFA,ISRAEL
[2] TECHNION ISRAEL INST TECHNOL,RAMBAM MED CTR,DEPT CLIN IMMUNOL & GENET,HAIFA,ISRAEL
[3] TECHNION ISRAEL INST TECHNOL,FAC MED,HAIFA,ISRAEL
来源
ACTA PAEDIATRICA SCANDINAVICA | 1990年 / 79卷 / 03期
关键词
Chemotaxis; Neutrophil; Saethre-Chotzen syndrome;
D O I
10.1111/j.1651-2227.1990.tb11477.x
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
An 11-month-old male infant with Saethre-Chotzen syndrome and recurrent respiratory infections is described. Persistent extremely high leukocytosis warranted evaluation of neutrophil functions. It was found that the opsonophagocytic activity was normal, but neutrophil chemotaxis was markedly decreased. Further studies pointed to an intracellular neutrophil defect causing this motility dysfunction
引用
收藏
页码:375 / 379
页数:5
相关论文
共 17 条
  • [1] AESE J, 1970, J PEDIATR, V76, P928
  • [2] ACROCEPHALOSYNDACTYLY TYPE-III - CHOTZENS SYNDROME
    BARTSOCAS, CS
    WEBER, AL
    CRAWFORD, JD
    [J]. JOURNAL OF PEDIATRICS, 1970, 77 (02) : 267 - +
  • [3] A FAMILY WITH THE SAETHRE-CHOTZEN SYNDROME
    BIANCHI, E
    ARICO, M
    PODESTA, AF
    GRANA, M
    FIORI, P
    BELUFFI, G
    [J]. AMERICAN JOURNAL OF MEDICAL GENETICS, 1985, 22 (04): : 649 - 658
  • [4] NEUTROPHIL ACTIN DYSFUNCTION AND ABNORMAL NEUTROPHIL BEHAVIOR
    BOXER, LA
    HEDLEYWH.T
    STOSSEL, TP
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1974, 291 (21) : 1093 - 1099
  • [5] DEFECTIVE NEUTROPHIL CHEMOTAXIS IN HYPOGAMMAGLOBULINEMIA AND SELECTIVE IGA DEFICIENCY
    DAMELIO, R
    ROSSI, P
    LEMOLI, S
    AIUTI, F
    [J]. CLINICAL IMMUNOLOGY AND IMMUNOPATHOLOGY, 1980, 16 (03): : 287 - 296
  • [6] EASMON CSF, 1980, IMMUNOLOGY, V41, P903
  • [7] DEFECTIVE HUMORAL AND CELLULAR IMMUNE FUNCTIONS ASSOCIATED WITH VENOOCCLUSIVE DISEASE OF THE LIVER
    ETZIONI, A
    BENDERLY, A
    ROSENTHAL, E
    SHEHADAH, V
    AUSLANDER, L
    LAHAT, N
    POLLACK, S
    [J]. JOURNAL OF PEDIATRICS, 1987, 110 (04) : 549 - 554
  • [8] DEFICIENCY OF THE ADHESIVE PROTEIN COMPLEX LYMPHOCYTE FUNCTION ANTIGEN-1, COMPLEMENT RECEPTOR TYPE-3, GLYCOPROTEIN-P150,95 IN A GIRL WITH RECURRENT BACTERIAL-INFECTIONS - EFFECTS ON PHAGOCYTIC-CELLS AND LYMPHOCYTE FUNCTIONS
    FISCHER, A
    SEGER, R
    DURANDY, A
    GROSPIERRE, B
    VIRELIZIER, JL
    LEDEIST, F
    GRISCELLI, C
    FISCHER, E
    KAZATCHKINE, M
    BOHLER, MC
    DESCAMPSLATSCHA, B
    TRUNG, PH
    SPRINGER, TA
    OLIVE, D
    MAWAS, C
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1985, 76 (06) : 2385 - 2392
  • [9] ENHANCEMENT OF RANDOM MIGRATION AND CHEMOTACTIC RESPONSE OF HUMAN LEUKOCYTES BY ASCORBIC-ACID
    GOETZL, EJ
    WASSERMAN, SI
    GIGLI, I
    AUSTEN, KF
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1974, 53 (03) : 813 - 818
  • [10] HILL HR, 1975, J LAB CLIN MED, V86, P703