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M9480562.TXT
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1994-08-20
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Document 0562
DOCN M9480562
TI A family of selective immunodeficiency with normal immunoglobulins:
possible autosomal dominant inheritance.
DT 9410
AU Higuchi S; Awata H; Nunoi H; Tsuchiya H; Naoe H; Igarashi H; Matsuda I;
Department of Paediatrics, Kumamoto University Medical School,; Japan.
SO Eur J Pediatr. 1994 May;153(5):328-32. Unique Identifier : AIDSLINE
MED/94307290
AB We report here our findings in two Japanese siblings who experienced
recurrent bacterial and viral infections since early infancy. Recent
symptoms included diarrhoea, conjunctivitis, rashes, headache, sore
throat, joint pain, vomiting and vertigo, all similar to those seen in
toxic shock syndrome, except for shock. These symptoms improved
following gammaglobulin treatment. Staphylococcus aureus with coagulase
type IV was continuously isolated from nasal smears producing toxic
shock syndrome toxin-1 (TSST-1). Serum antibodies did not or only poorly
responded to TSST-1, diphtheria toxoid, varicella virus and rubella
virus, whereas total and subclass levels of serum immunoglobulin and in
vitro DNA synthesis of lymphocytes stimulated by TSST-1, Staph. aureus,
varicella vaccine and mitogens were normal. In the family, ten other
members in three generations (five males: five females) including the
mother had similar clinical symptoms. Thus, the disease may be inherited
in an autosomal dominant fashion.
DE Antibodies, Bacterial/BIOSYNTHESIS Antibodies, Viral/BIOSYNTHESIS Case
Report Child Child, Preschool Diphtheria Toxoid/IMMUNOLOGY
Enterotoxins/IMMUNOLOGY Family Health Female *Genes, Dominant
Herpesvirus 3, Human/IMMUNOLOGY Human Immunoglobulins/*BIOSYNTHESIS
Immunologic Deficiency Syndromes/*GENETICS/IMMUNOLOGY Male Pedigree
Rubella Virus/IMMUNOLOGY JOURNAL ARTICLE
SOURCE: National Library of Medicine. NOTICE: This material may be
protected by Copyright Law (Title 17, U.S.Code).