Biomaterial Database

[Hyper-IgE syndrome--two case reports of unusual complications]. 1994

P Sehrt, and S Zielen, and V Gerein, and G Herrmann, and D Hofmann

Zentrum der Kinderheilkunde, Universität Frankfurt/Main.

The hyper-IgE syndrome (HIS) is a complex immunologic disease, caused by an unknown basic defect. We report on two cases showing complications, which have not been described so far. Case 1: A 15-year-old boy suffering from HIS developed a liver tumour with severe eosinophilic infiltration and degranulation. The transformation process of the liver histologically resembled focal nodular hyperplasia. Therapy with cyclosporine A did not lead to clinical benefit. Now, a therapeutic attempt with interferon gamma is made. Case 2: In a 17-year-old female HIS patient, multiple papillomas and ulcers of the mucous membrane, caused by infection with human papilloma virus, emerged in the ENT region. Under treatment with interferon alpha, papillomatosis could be restrained. As therapy of the hyper-IgE syndrome only symptomatic treatment has been recommended so far. Future therapies should strive for systemic immunomodulation by application of cytokines or soluble cytokine receptors like interferons or sIL-4R.

UI	MeSH Term	Description	Entries
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
D008113	Liver Neoplasms	Tumors or cancer of the LIVER.	Cancer of Liver,Hepatic Cancer,Liver Cancer,Cancer of the Liver,Cancer, Hepatocellular,Hepatic Neoplasms,Hepatocellular Cancer,Neoplasms, Hepatic,Neoplasms, Liver,Cancer, Hepatic,Cancer, Liver,Cancers, Hepatic,Cancers, Hepatocellular,Cancers, Liver,Hepatic Cancers,Hepatic Neoplasm,Hepatocellular Cancers,Liver Cancers,Liver Neoplasm,Neoplasm, Hepatic,Neoplasm, Liver
D008297	Male		Males
D010212	Papilloma	A circumscribed benign epithelial tumor projecting from the surrounding surface; more precisely, a benign epithelial neoplasm consisting of villous or arborescent outgrowths of fibrovascular stroma covered by neoplastic cells. (Stedman, 25th ed)	Papilloma, Squamous Cell,Papillomatosis,Papillomas,Papillomas, Squamous Cell,Papillomatoses,Squamous Cell Papilloma,Squamous Cell Papillomas
D010610	Pharyngeal Neoplasms	Tumors or cancer of the PHARYNX.	Cancer of Pharynx,Pharyngeal Cancer,Cancer of the Pharynx,Neoplasms, Pharyngeal,Neoplasms, Pharynx,Pharnyx Cancer,Pharynx Neoplasms,Cancer, Pharnyx,Cancer, Pharyngeal,Cancers, Pharnyx,Cancers, Pharyngeal,Neoplasm, Pharyngeal,Neoplasm, Pharynx,Pharnyx Cancers,Pharyngeal Cancers,Pharyngeal Neoplasm,Pharynx Cancer,Pharynx Cancers,Pharynx Neoplasm
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths