neonatal hemochromatosis ( nh ) is defined by liver disease associated with siderosis of the extrahepatic tissues 1 . the most frequent cause of nh is a gestational alloimmune disorder called gestational alloimmune liver disease , which results from the transplacental transfer of maternal immunoglobulin g ( igg ) antibodies 2 . although about 80% of nh patients can survive without liver transplantation because of exchange transfusions and intravenous ig therapy , some of the patients with more serious disease require transplantation 3 . nh with rtd has been reported in only five cases , all of which were stillborn or resulted in early neonatal death 58 . we describe a case of surviving patient who had nh with rtd and underwent peritoneal dialysis and liver transplantation from her father . a 29-year - old woman was referred to our hospital at 35  weeks gestation in her second pregnancy with atypical genital bleeding . she had been diagnosed with poor fetal growth and oligohydramnios from 30  weeks of gestation . a female infant was born by spontaneous vaginal delivery at 35  weeks with a birth weight of 1544  g ( 2.8 standard deviations , sd ) , a birth length of 42  cm ( 1.6 sd ) , and a head circumference of 29  cm ( 1.8 sd ) . apgar scores were 1 ( 1  min ) and 6 ( 5  min ) . physical examination revealed calvarial bone hypoplasia with enlarged fontanels and hepatosplenomegaly , but with no other abnormalities . renal ultrasound showed hyperechogenicity of both kidneys , but the size and morphology were normal . anuria , skull ossification defects , and renal hyperechogenicity suggested renal tubular dysgenesis . because the anuria persisted longer than 48  h and serum creatinine had increased to 3.0  mg / dl , peritoneal dialysis was performed on day 3 ( fig.1 ) . spontaneous urination was first observed on day 6 , and it increased gradually ; therefore , peritoneal dialysis was discontinued on day 10 . finally , serum creatinine reached normal levels ( 0.5  mg / dl ) on day 30 . ivig , intravenous immunoglobulin ; d - bil , direct bilirubin ; pt - inr , prothrombin time / international normalized ratio . hepatic failure was suspected on day 9 because of hepatosplenomegaly , a poor coagulation profile ( pt - inr : 2.07 ) , jaundice ( direct - bilirubin : 9.1  mg / dl ) , thrombocytopenia ( platelets : 7.2    10/mm ) , and hypoglycemia . congenital infections and metabolic disorders were ruled out by blood examinations . on the basis of the clinical course of acute liver failure and the complication of rtd , nh was suspected . however , she had a normal iron level of 138  g / dl and a ferritin level of 280  ng / ml . moreover , magnetic resonance imaging of the abdomen did not reveal hepatic and extrahepatic siderosis on day 15 . to obtain a definitive diagnosis , a liver biopsy was performed on day 30 , concomitant with the surgical ligation of the ductus arteriosus . the liver biopsy revealed an increase in stainable iron within the hepatocytes , confirming our diagnosis of nh ( fig.2 ) . moreover , a marked loss of hepatocytes and cholestasis was detected and the remaining hepatocytes were multinucleated . ( b ) parenchymal fibrosis was extensive in most areas ( azan staining , original magnification 100 ) . iron staining was predominantly both inside and outside the hepatocytes ( berlin blue staining , original magnification 200 ) . vitamin e was administered starting at day 16 ( 25  iu / kg / day ) , whereas vitamin k was intravenously administered periodically after birth . she received continuous infusions of ffp and repeated transfusions of platelets because of her overwhelming liver dysfunction . her liver function did not improve ; however , she gained weight , reaching 2500  g , which indicated that she was able to accept a liver transplantation from an adult ( fig.1 ) . a living donor liver transplantation with segment 2 monosubsegment graft ( 130  g ) for her was performed on day 59 . the liver extracted from patient was replaced by small nodules and overall fibrosis was progressing . microscopically , the liver showed almost total distortion of the lobular architecture , loss of the hepatocytes , and a diffuse increase in fibrous tissue . a high degree of iron deposition in hepatocytes was observed , which was definitely findings in neonatal hemochromatosis . first , the infusion of ffp and peritoneal dialysis were effective in treating persistent hypotension and renal failure in rtd with nh . to the best of our knowledge , second , repeated exchange transfusions improved the jaundice and coagulopathy , facilitating the patient s growth for subsequent liver transplantation regardless of her status as a preterm infant . nh with rtd has only been reported in five cases , all of which were fatal or resulted in early neonatal death because of coagulopathy and liver failure 57 . in the case reported here , ffp infusion and peritoneal dialysis were effective in maintaining systemic blood pressure and organ perfusion . this is supported by a recent report on a neonatal case of inherited rtd with an angiotensinogen gene mutation ; this patient survived for more than 18  months with a treatment regimen that comprised ffp infusion and peritoneal dialysis 9,10 . because the diagnosis of rtd was made on the basis of the clinical symptoms and the hyperechogenicity of both kidneys in the present case , a kidney biopsy was required to confirm the diagnosis . however , the kidney biopsy was difficult to perform because of the presence of severe coagulopathy . liver failure did not improve regardless of a plurality of times of exchange transfusion and other medications . moreover , her liver biopsy showed the remaining hepatocytes were multinucleated . on basis of clinical course and biopsy observation , we determined surgical liver transplantation was essential in this case . most liver transplantations for children in japan are performed using the liver from one of the patient s parents . our patient was very young , because of which we had to wait until her body weight reached at least 2500  g to accept an adult liver , and we thus treated her jaundice and coagulopathy with exchange transfusions . fortunately , she developed no serious hemorrhage but instead showed weight gain , indicating growth . our case report indicates that even a premature infant of nh with rtd can survive with peritoneal dialysis and exchange transfusions .