Biomaterial Database

[Hyperimmunoglobulinemia E, recurring staphylococcal infections and a defect in granulocyte chemotaxis in adults. A variant of Job's syndrome]. 1985

M E Heim, and R Herz, and P Worst, and W Queisser

Two cases in adults with recurrent staphylococcal infections associated with abnormal granulocytic chemotaxis and hyperimmunoglobulinaemia E (Job's syndrome) are described. The pathophysiological mechanisms seems to consist of an abnormal IgE reaction against staphylococcal antigens causing secondary abnormality of granulocyte function. Abnormal cellular immune function was demonstrated in vitro and in vivo. Corticosteroid administration at first proved effective in both patients. One patient developed Hodgkin's disease of the mixed type in the course of the disease.

UI	MeSH Term	Description	Entries
D007073	Immunoglobulin E	An immunoglobulin associated with MAST CELLS. Overexpression has been associated with allergic hypersensitivity (HYPERSENSITIVITY, IMMEDIATE).	IgE
D007589	Job Syndrome	Primary immunodeficiency syndrome characterized by recurrent infections and hyperimmunoglobulinemia E. Most cases are sporadic. Of the rare familial forms, the dominantly inherited subtype has additional connective tissue, dental and skeletal involvement that the recessive type does not share.	HIES, Autosomal Recessive,Hyper-IgE Syndrome, Autosomal Recessive,Hyper-Immunoglobulin E Syndrome, Autosomal Recessive,Hyperimmunoglobulin E-Recurrent Infection Syndrome,Job's Syndrome,Buckley Syndrome,HIE Syndrome,HIES, Autosomal Dominant,Hyper-IgE Recurrent Infection Syndrome, Autosomal Recessive,Hyper-IgE Syndrome,Hyper-IgE Syndrome, Autosomal Dominant,Hyper-Immunoglobulin E Syndrome, Autosomal Dominant,Hyperimmunoglobulin E, Recurrent Infection Syndrome,Hyperimmunoglobulinemia E Syndrome,Job-Buckley Syndrome,Autosomal Dominant HIES,Autosomal Dominant HIESs,Autosomal Recessive HIES,Autosomal Recessive HIESs,Buckley Syndromes,HIE Syndromes,HIESs, Autosomal Dominant,HIESs, Autosomal Recessive,Hyper IgE Recurrent Infection Syndrome, Autosomal Recessive,Hyper IgE Syndrome,Hyper IgE Syndrome, Autosomal Dominant,Hyper IgE Syndrome, Autosomal Recessive,Hyper Immunoglobulin E Syndrome, Autosomal Dominant,Hyper Immunoglobulin E Syndrome, Autosomal Recessive,Hyper-IgE Syndromes,Hyperimmunoglobulin E Recurrent Infection Syndrome,Hyperimmunoglobulinemia E Syndromes,Job Buckley Syndrome,Job Syndromes,Job-Buckley Syndromes,Jobs Syndrome,Syndrome, Job,Syndromes, Job
D008297	Male		Males
D010585	Phagocyte Bactericidal Dysfunction	Disorders in which phagocytic cells cannot kill ingested bacteria; characterized by frequent recurring infection with formulation of granulomas.	Bactericidal Dysfunction, Phagocyte,Bactericidal Dysfunctions, Phagocyte,Dysfunction, Phagocyte Bactericidal,Dysfunctions, Phagocyte Bactericidal,Phagocyte Bactericidal Dysfunctions
D002633	Chemotaxis	The movement of cells or organisms toward or away from a substance in response to its concentration gradient.	Haptotaxis
D005260	Female		Females
D006098	Granulocytes	Leukocytes with abundant granules in the cytoplasm. They are divided into three groups according to the staining properties of the granules: neutrophilic, eosinophilic, and basophilic. Mature granulocytes are the NEUTROPHILS; EOSINOPHILS; and BASOPHILS.	Granulocyte
D006689	Hodgkin Disease	A malignant disease characterized by progressive enlargement of the lymph nodes, spleen, and general lymphoid tissue. In the classical variant, giant usually multinucleate Hodgkin's and REED-STERNBERG CELLS are present; in the nodular lymphocyte predominant variant, lymphocytic and histiocytic cells are seen.	Granuloma, Hodgkin,Granuloma, Malignant,Hodgkin Lymphoma,Lymphogranuloma, Malignant,Granuloma, Hodgkin's,Granuloma, Hodgkins,Hodgkin Lymphoma, Adult,Hodgkin's Disease,Hodgkin's Lymphoma,Hodgkins Disease,Lymphocyte Depletion Hodgkin's Lymphoma,Lymphocyte-Rich Classical Hodgkin's Lymphoma,Mixed Cellularity Hodgkin's Lymphoma,Nodular Lymphocyte-Predominant Hodgkin's Lymphoma,Nodular Sclerosing Hodgkin's Lymphoma,Adult Hodgkin Lymphoma,Disease, Hodgkin,Disease, Hodgkin's,Disease, Hodgkins,Hodgkin Granuloma,Hodgkin's Granuloma,Hodgkins Granuloma,Hodgkins Lymphoma,Lymphocyte Rich Classical Hodgkin's Lymphoma,Lymphogranulomas, Malignant,Lymphoma, Hodgkin,Lymphoma, Hodgkin's,Malignant Granuloma,Malignant Granulomas,Malignant Lymphogranuloma,Malignant Lymphogranulomas,Nodular Lymphocyte Predominant Hodgkin's Lymphoma
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006942	Hypergammaglobulinemia	An excess of GAMMA-GLOBULINS in the serum due to chronic infections or PARAPROTEINEMIAS.	Hyperimmunoglobulinemia,Hypergammaglobulinemias,Hyperimmunoglobulinemias