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Pediatric Diseases: Blood Disease (2), mother-child immunity comes to an issue- Neonatal Hemochromatosis (GALD)

Develops disorder of liver function at early postnatal period.

Neonatal Hemochromatosis is the rare disease where abnormality of hepatic function appears at early postnatal period, and deposition of iron is recognized at several histological organs other than reticuloendothelial, like liver, pancreas or heart. Not applying only to neonatal hepatic cirrhosis, it has a characteristic of having many cases where fetal agenesis in the womb has already been recognized. This is not a inherited disease.

Antibody in maternal blood disables neonatal liver.

IgG antibody produced in maternal blood against the natal liver placentally transferred to natal liver, then antibody activate the complement (CSb-9 complex) and affecting hepatic damage. Because of this, neonatal blood hepcidin is decreased so that fetal becomes iron excess state. (Fig.1) In other words, it is described that the gestational alloimmune liver disease (GALD) is the mechanism of pathogenesis for neonatal hemochromatosis.

For the case fall into failure of the liver, it leads to a poor prognosis.

There is systemic failure immediately after birth (respiration/cardiovascular failure), fetal growth failure, fetal edema or hepatic failure symptom, and low signal indicating iron deposition to several organs including liver by MRI T2 enhanced image. They say that the repetition of sibling pathogenesis is high. Transferrin, which is a carrier of glycoprotein, saturation rate is getting high.

There is a case which mis birth, immature birth, intrauterine growth disorder, or amniotic fluid reduction is recognized already in fetal period. There are many cases that abnormality of hepatic function or hepatic failure symptom has appeared at early postnatal period, and when a case falls into failure of liver, prognosis becomes worse.

The report says that administration of immune globulin is effective for prevention.

Diagnosis are made by image findings with MRI exam, high value of blood glucose ferritin and transferrin saturation rate, in addition, iron deposition in salivary gland tissue has histopathologically proved by small-mouth salivary gland biopsy.

For medicine treatment, the report has listed exchange transfusion, cocktail treatment (antioxidant agent, iron chelating agent), immune globulin (IVG) treatment. Refractory case is subject to liver transplantation. And for expectant mother, the report state that the continuous administration of IVIG after 14 weeks of pregnancy has prevented the occurrence of the symptom.

Fig. 1 Mechanism of pathogenesis of GALD