[Therapy of the Steinbrinck-Chediak-Higashi-Syndrom (author's transl)].
The Steinbrinck-Chediak-Higashi Syndrome is characterized by morphological and functional alterations of polymorphonuclear leukocytes. The impaired function in the polymorphonuclear leukocytes may be related to abnormal microtubular assembly. The ingestion of 200 mg/d of ascorbic acid potentiated chemotaxis and bactericidal activity of polymorphonuclear leukocytes from a patient with this disease. Furthermore, the treatment with ascorbic acid reduced the spontaneous cap formation by these leukocytes, demonstrating the improvement of microtubular assembly. The life long treatment with ascorbic acid may affect the clinical course of these patients favourably.
D001205:Ascorbic Acid / Q000008:administration & dosage / Q000627:therapeutic use; D002462:Cell Membrane / Q000032:analysis; D002609:Chediak-Higashi Syndrome / Q000188:drug therapy* / Q000276:immunology; D006098:Granulocytes / Q000648:ultrastructure; D006801:Humans; D007223:Infant; D037102:Lectins; D008297:Male; D008870:Microtubules / Q000648:ultrastructure; D010587:Phagocytosis; D011975:Receptors, Mitogen / Q000032:analysis
D037102:Lectins; D011975:Receptors, Mitogen; D001205:Ascorbic Acid
Therapie des Steinbrinck-Chediak-Higashi-Syndroms.