Effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia.
BACKGROUND
Hyperzincaemia and hypercalprotectinaemia with systemic inflammation, recurrent infections, hepatosplenomegaly, arthritis, anemia, cutaneous inflammation, and failure to thrive is an extremely rare disease and no therapy is reported.


OBJECTIVE
To evaluated the effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia in terms of serum cytokine level changes before and after treatment.


METHODS
A 10-year-old girl was admitted suffering from pyoderma gangrenosum, hepatosplenomegaly, anemia that was unresponsive to iron supplementation, persistent inflammation, arthritis, and increased serum zinc. The level of serum calprotectin was extremely high; therefore, we diagnosed hyperzincaemia and hypercalprotectinaemia and started cyclosporine A treatment. Twelve cytokines in serum were measured before and one year after treatment.


RESULTS
Cyclosporine A was very effective. Her skin lesion and joint pain were alleviated and quality of life was markedly improved. C-reactive protein had decreased and anemia had improved. While zinc levels had fallen, calprotectin remained at an extremely high level. Of the cytokines examined, interleukin -6 serum levels had fallen and interleukin -8 showed a marked reduction after treatment.


CONCLUSIONS
Cyclosporine A is effective for hyperzincaemia and hypercalprotectinaemia. Serum interleukin -8 may be useful in assessing the therapeutic effects of cyclosporine A in hyperzincaemia and hypercalprotectinaemia.
Sugiura
Tokio
T
Department of Pediatrics, Neonatology and Congenital Disorders, Nagoya City University, Graduate School of Medical Sciences, Nagoya, Japan.
Goto
Kenji
K
Ito
Kouichi
K
Ban
Kyoko
K
Okada
Shoji
S
Moriyama
Akihiko
A
Togari
Hajime
H
D002648:Child; D016572:Cyclosporine / Q000627:therapeutic use*; D016207:Cytokines / Q000097:blood; D005260:Female; D006529:Hepatomegaly / Q000097:blood; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation / Q000188:drug therapy; D039841:Leukocyte L1 Antigen Complex / Q000097:blood*; D008661:Metabolism, Inborn Errors / Q000097:blood*; D008664:Metal Metabolism, Inborn Errors / Q000097:blood*; D011711:Pyoderma / Q000097:blood; D013163:Splenomegaly / Q000097:blood; D015032:Zinc / Q000097:blood*
D016207:Cytokines; D007166:Immunosuppressive Agents; D039841:Leukocyte L1 Antigen Complex; D016572:Cyclosporine; D015032:Zinc