title: A child with X-linked agammaglobulinemia and Kawasaki disease: an unusual association.
abstract: An association of X-linked agammaglobulinemia (XLA) with Kawasaki disease (KD) is very uncommon. Only two case reports are available so far in pediatric literature. Patients with XLA have recurrent infections and physical examination have absent lymph nodes and tonsils. Laboratory investigations reveal hypogammaglobulinemia and reduced or absent B cells on flow cytometry. KD is a medium vessel vasculitis. Here, we report a 12 year old boy with X-linked agammaglobulinemia on regular replacement intravenous immunoglobulin who developed KD on follow-up. This is an uncommon occurrence.
authors.affiliation: Allergy-Immunology Unit, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Dhrubajyoti
authors.initials: D
authors.lastname: Sharma
authors.affiliation: Allergy-Immunology Unit, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Sandesh
authors.initials: S
authors.lastname: Guleria
authors.affiliation: Allergy-Immunology Unit, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Deepti
authors.initials: D
authors.lastname: Suri
authors.affiliation: Allergy-Immunology Unit, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Amit
authors.initials: A
authors.lastname: Rawat
authors.affiliation: Allergy-Immunology Unit, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
authors.forename: Ravinder
authors.initials: R
authors.lastname: Garg
authors.affiliation: Allergy-Immunology Unit, Advanced Pediatrics Center, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India. surjitsinghpgi@rediffmail.com.
authors.forename: Surjit
authors.initials: S
authors.lastname: Singh
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D002648:Child; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D007155:Immunologic Factors / Q000008:administration & dosage; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D009154:Mutation
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
keywords: Intravenous immunoglobulin; Primary immunodeficiency; Vasculitis