title: Immunoglobulin abnormalities and effects of enzyme replacement therapy in children with Gaucher disease.
abstract: Hyperimmunoglobulinemia is documented in patients with Gaucher disease of all ages. We investigated the frequency of hyperimmunoglobulinemia in 12 pediatric patients with type I and III Gaucher disease and the effects of enzyme replacement therapy on these abnormalities. The incidence of hyperimmunoglobulinemia was 77%, 66%, and 60% at the diagnosis, before and after ERT, respectively. Immunoglobulin G abnormalities were the most commonly seen isotype abnormality. After enzyme replacement therapy normalization of IgA and IgM levels were recorded but decline in IgG levels was less likely to occur. This study indicated the higher frequency of hyperimmunoglobulinemia in pediatric Gaucher patients.
authors.affiliation: Faculty of Medicine, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, Hacettepe University, Ankara Turkey. ztarikan@hotmail.com
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Arıkan-Ayyıldız
authors.forename: Aysel
authors.initials: A
authors.lastname: Yuce
authors.forename: Nuray
authors.initials: N
authors.lastname: Uslu-Kızılkan
authors.forename: Hülya
authors.initials: H
authors.lastname: Demir
authors.forename: Figen
authors.initials: F
authors.lastname: Gurakan
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D056947:Enzyme Replacement Therapy; D005260:Female; D005776:Gaucher Disease / Q000188:drug therapy / Q000276:immunology*; D005962:Glucosylceramidase / Q000627:therapeutic use*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000276:immunology; D007070:Immunoglobulin A / Q000097:blood; D007074:Immunoglobulin G / Q000097:blood; D007075:Immunoglobulin M / Q000097:blood; D007223:Infant; D008297:Male; D011994:Recombinant Proteins; D055815:Young Adult
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D011994:Recombinant Proteins; D005962:Glucosylceramidase; C090568:imiglucerase