Tissue zinc levels in a child with hypercalprotectinaemia and hyperzincaemia: A case report and a review of the literature

Abstract

Her grossly elevated serum calprotectin concentration is probably able to raise circulating total Zn concentrations without raising ionized concentrations, but this Zn remains confined to the circulating blood as well as to excreted body fluids, particularly faeces.

Hair Zinc Levels and the Efficacy of Oral Zinc Supplementation in Patients with Atopic Dermatitis

“…Human hair is a convenient source, being easy to obtain and robust, and its storage and preparation for analysis are straightforward since no special preservation techniques are required. The nutrient source for hair growth is the blood supply, which contains traces of anything ingested by the individual (9,34). Moreover, the concentrations of trace elements in hair are at least 10 times higher than in blood, serum and urine (10).…”

Calcium Ion Gradients Modulate the Zinc Affinity and Antibacterial Activity of Human Calprotectin

“…30,31 Lastly, hypercalprotectinanemia is a rare disease of erroneous Zn(II) metabolism characterized by anemia, inflammation, recurrent infections, and hyperzincanemia. 33,34 Patients present >1000-fold elevated levels of plasma CP and high plasma zinc concentrations, indicating excessive zinc-bound CP in circulation and Zn(II) depletion from tissues. Taking these clinical observations together, molecular-level understanding of how CP and Zn(II) contribute to the pathophysiology of various human diseases is necessary and may provide new insights into therapeutic development.…”

“…Links between CP and zinc have been identified for human pathologies that include inflammation and cancer, 30,31 amyloid formation in the ageing prostate, 32 and erroneous zinc metabolism. 33,34 Molecular-level understanding of how CP contributes to bacterial and mammalian physiology in broad terms requires elucidation of both its metal-binding properties and the biological significance of the metal-bound forms. Motivated by the multiple links between CP and Zn(II) in the host/pathogen interaction and human disease, we focus on the zinc-binding properties of CP in this work.…”

Whole genome microarray expression analysis in blood identifies pathways linked to signs and symptoms of a patient with hypercalprotectinaemia and hyperzincaemia

“…We hypothesized that by the use of a high‐quality whole‐genome mRNA expression array we would be able to identify plausible mechanisms behind the signs and symptoms that our patient displayed. The hypercalprotectinaemia is explained plausibly by an up‐regulation of the calprotectin complex genes ( S100A8, S100A9, but also S100A12 ), and the serum hyperzincaemia is most probably simply a reflection of the increase in this zinc‐transporting protein, as we found previously that tissue zinc levels (hair, nails) are not increased in this patient . As there was no mutation in the calprotectin‐raising gene PSTPIP1 , the mechanism for the Hc/Hz‐like biomarker changes must lie elsewhere. The thrombocytosis reflects a general up‐regulation of the JAK/STAT pathway; for instance, by oncostatin ( OSM ), IL18R1 and SOCS3 .…”

“…The first case in Sweden, described by us , was atypical in that gastrointestinal bleeding and diarrhoea were presenting symptoms and continued to be major clinical problems, in addition to pronounced growth retardation and persistent pronounced thrombocytosis. Despite the hyperzincaemia in serum there were no signs of zinc overload in tissues .…”