Successful pregnancy outcome following maternal intravenous immunoglobulin treatment in a woman with recurrent perinatal haemochromatosis.
OBJECTIVE
We report a case of successful pregnancy outcome following maternal intravenous immunoglobulin treatment in a woman with previous history of recurrent fetal hydrops secondary to perinatal haemochromatosis.


METHODS
A 32-year old woman had two successive pregnancies complicated by fetal hydrops and perinatal deaths. Pathological examination of the fetus showed severe liver destruction with siderosis of hepatocytes at extrahepatic sites, but sparing of the reticulo-endothelial elements, consistent with the diagnosis of perinatal haemochromatosis. In the subsequent pregnancy, maternal intravenous immunoglobulin was administered weekly from the 18th week of gestation until delivery by elective caesarean section at 38 weeks. The infant was treated with desferrioxamine, N-acetylcysteine, vitamins K and E.


RESULTS
The infant was born in good health, but had high serum ferritin levels, markedly elevated percent transferrin saturation, and mild transient derangement of liver and coagulation function. The infant made an excellent recovery and the treatment was stopped at 7 weeks of age. The liver and coagulation parameters and the serum ferritin levels returned to normal values.


CONCLUSIONS
Haemochromatosis should be considered in the differential diagnosis of hydrops fetalis. The recurrence risk is high, and immunomodulation with intravenous immunoglobulin treatment appears to alter the course of the disease with better infant survival.
Venkat-Raman
Narayanaswamy
N
Department of Obstetrics, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Leicester, UK. nvraman@tiscali.co.uk
Venkata-Krishnan
Radha V
RV
Howarth
Edmund S
ES
D000111:Acetylcysteine / Q000627:therapeutic use; D000328:Adult; D003676:Deferoxamine / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D005293:Ferritins / Q000097:blood; D005315:Fetal Diseases / Q000188:drug therapy*; D016166:Free Radical Scavengers / Q000627:therapeutic use; D005865:Gestational Age; D006432:Hemochromatosis / Q000151:congenital / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D015160:Hydrops Fetalis / Q000175:diagnosis; D007231:Infant, Newborn; D050498:Live Birth; D017114:Liver Failure, Acute / Q000151:congenital / Q000188:drug therapy* / Q000503:physiopathology; D011247:Pregnancy; D012008:Recurrence; D016896:Treatment Outcome; D014810:Vitamin E / Q000627:therapeutic use; D014812:Vitamin K / Q000627:therapeutic use
D016166:Free Radical Scavengers; D014812:Vitamin K; D014810:Vitamin E; D005293:Ferritins; D003676:Deferoxamine; D000111:Acetylcysteine