title: [Dubowitz syndrome with immunodeficiency and solid malignant tumor in two siblings (author's transl)].
abstract: The main features of Dubowiz syndrome are bird headed dwarfism and a typical facial configuration. Including two own patients 13 cases among 8 siblings are known in the literature. Two own cases of Dubowitz syndrome in two sisters are described, one of them with hypogammaglobulinemia and neuroblastoma, the other one with complete Ig A deficiency and malignant lymphoma. A propable relationship between immundeficiency and malignancies is discussed. Until 1973 151 malignant tumors with primary immundeficiency had been registrated. This paper is the first description of two cases of Dubowitz syndrome with immunodeficiency and malignant neoplasms.
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications; D002648:Child; D002675:Child, Preschool; D004392:Dwarfism / Q000151:congenital* / Q000235:genetics; D004406:Dysgammaglobulinemia / Q000150:complications; D005147:Facial Bones / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000235:genetics; D008223:Lymphoma / Q000150:complications* / Q000235:genetics; D009447:Neuroblastoma / Q000150:complications* / Q000235:genetics; D010375:Pedigree; D013577:Syndrome
chemical_list: D007070:Immunoglobulin A
vernacular_title: Dubowitz-Syndrom mit Immundefizienz und malignem Neoplasma bei zwei Geschwistern.