title: Hyperzincemia with systemic inflammation: a heritable disorder of calprotectin metabolism with rheumatic manifestations?
abstract: A boy had infantile-onset systemic inflammation, growth failure, hepatosplenomegaly, anemia, leukocytopenia, progressive muscular dystrophy, and hypercalprotectinemia, resulting in marked hyperzincemia. His mother had a history of chronic arthritis since childhood and also showed hypercalprotectinemia/hyperzincemia. We postulate an inherent defect in calprotectin metabolism.
authors.affiliation: Department of Pediatrics, Institute of Rheumatology, Tokyo Women's Medical University (TWMU), Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Saito
authors.forename: Kayoko
authors.initials: K
authors.lastname: Saito
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Hirano
authors.forename: Kiyoko
authors.initials: K
authors.lastname: Ikeya
authors.forename: Haruko
authors.initials: H
authors.lastname: Suzuki
authors.forename: Keiko
authors.initials: K
authors.lastname: Shishikura
authors.forename: Sumie
authors.initials: S
authors.lastname: Manno
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Takakuwa
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Nakagawa
authors.forename: Aiko
authors.initials: A
authors.lastname: Iwasa
authors.forename: Satoshi
authors.initials: S
authors.lastname: Fujikawa
authors.forename: Makoto
authors.initials: M
authors.lastname: Moriya
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Mizoguchi
authors.forename: Barbara E
authors.initials: BE
authors.lastname: Golden
authors.forename: Makiko
authors.initials: M
authors.lastname: Osawa
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001171:Arthritis, Juvenile / Q000150:complications / Q000235:genetics*; D002135:Calcium-Binding Proteins / Q000378:metabolism*; D003402:Creatine Kinase / Q000097:blood; D005260:Female; D006529:Hepatomegaly; D006801:Humans; D039841:Leukocyte L1 Antigen Complex; D007970:Leukopenia / Q000150:complications; D008297:Male; D008562:Membrane Glycoproteins / Q000378:metabolism*; D008661:Metabolism, Inborn Errors / Q000150:complications / Q000235:genetics*; D009136:Muscular Dystrophies / Q000150:complications; D019006:Neural Cell Adhesion Molecules / Q000378:metabolism*; D013163:Splenomegaly; D015032:Zinc / Q000097:blood*
chemical_list: D002135:Calcium-Binding Proteins; D039841:Leukocyte L1 Antigen Complex; D008562:Membrane Glycoproteins; D019006:Neural Cell Adhesion Molecules; D003402:Creatine Kinase; D015032:Zinc