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. (J Pediatr 2002;140:267-9)