       Document 0329
 DOCN  M9630329
 TI    [X-chromosome recessive lymphoproliferative disease (XLP): molecular
       genetic studies]
 DT    9603
 AU    Schuster V; Grimm T; Kress W; Seidenspinner S; Belohradsky BH; Muller P;
       Kreth HW; Kinderklinik, Universitat Wurzburg.
 SO    Klin Padiatr. 1995 Sep-Oct;207(5):271-6. Unique Identifier : AIDSLINE
       MED/96058829
 AB    X-linked lymphoproliferative disease (XLP) is a rare worldwide occurring
       inherited immunodeficiency which is triggered by Epstein-Barr virus
       infection. Clinical phenotypes in 21 affected males from 5 German
       families with XLP ranged from severe and fatal infectious mononucleosis
       (57%) to acquired hypogammaglobulinaemia (28%), malignant lymphoma
       (28%), aplastic anaemia (19%) and hypergammaglobulinaemia M (19%).
       Molecular genetic studies with various polymorphic X-chromosomal DNA
       markers in 14 XLP families mapped the XLP gene locus to Xq25-q26.
       Haplotype analysis enables detection of XLP-positive and XLP-negative
       males already before EBV-infection as well as diagnosis of healthy
       female carriers within XLP families.
 DE    Child  Child, Preschool  Chromosome Mapping  DNA Probes/DIAGNOSTIC USE
       English Abstract  Female  Genes, Recessive/*GENETICS  Genetic
       Markers/GENETICS  Haplotypes  Herpesvirus 4, Human/GENETICS
       Heterozygote Detection  Human  Infectious Mononucleosis/GENETICS
       Linkage (Genetics)/*GENETICS  Lymphoproliferative Disorders/*GENETICS
       Male  Pedigree  Phenotype  Sex Chromosome Abnormalities/*GENETICS
       Support, Non-U.S. Gov't  *X Chromosome  JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

