SOVREMENNAYA PEDIATRIYA.2019.3(99):50-55; doi 10.15574/SP.2019.99.50

Kazytska N. M., Stepanenko T. І., Drobych S. O.
SЕ «Dnipropetrovsk Medical Academy Health Minisrty of Ukraine», Dnipro, Ukraine
Dnipropetrovsk Specialized Clinical Medical Center for the Health of Mother and Child prof. MF Rudneva «DOR», Dnipro, Ukraine

Neonatal hemochromatosis (NG) is a rare disease that is a consequence of fetal liver damage associated with abnormal iron accumulation in the newborn organs. The article presents modern views on the development of NG, the role of regulatory molecules of feedback between hepcidin and ferroportin in controlling the placental iron flow and its accumulation in the liver, and further formation of multiorgan lesions. A clinical case describes a full-term baby who was born in a satisfactory condition, but with a gradual development of a clinical picture of liver damage, and subsequently multiorgan disorders of the central nervous system, adrenal glands, kidneys, gastrointestinal tract, and progression of anemia. Considering the clinical manifestations and the appearance of an unusual blood color («rotten cherry»), the child was suspected to have NG, which was confirmed by high serum ferritin levels (the maximum level was 3076 ng/ml), MRI signs of brain damage, liver, adrenal glands, alloantibody in the mother's serum. A replacement blood transfusion of one-group blood was performed and intravenous immunoglobulin was administered. The child's condition stabilized until the 110th day of life with the recovery of liver function. Currently (age 1 year and 5 months) the child has no manifestations of liver failure, but a pronounced delay in physical and neuropsychic development.

Key words: neonatal hemochromatosis, ferritin, MRI of internal organs.

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Article received: Dec 22, 2018. Accepted for publication: Mar 13, 2019.