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

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