Successful pregnancy outcome following maternal intravenous
immunoglobulin treatment in a woman with recurrent perinatal
haemochromatosis.
Venkat-Raman N, Venkata-Krishnan RV, Howarth ES
Prenat Diagn. 2006 Nov 13;
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. Copyright (c) 2006 John Wiley &
Sons, Ltd.
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