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Re: Fatty acidFrom: Luis Sanchez-Ramos (sanchez-ramos@worldnet.att.net)Wed Dec 30 17:26:49 1998
At Wed, 30 Dec 1998, Peter Wein wrote: >>Luis - the condition is presumably diagnosable antenatally - don't have your original post. If any other pregnancy is similarly affected, the condition being lethal, would the patient request termination of pregnancy, so making the problem irrelevant<< Perhaps it would help if I gave you additional information. Patient is 33 G20100 First pregnancy ended at 33-34 weeks' gestation due to abnormal fetal testing which required an emergency cesarean. Apgar scores 0/1/6 cord pH 6.70-metabolic acidosis. Newborn had very difficult time in the NICU but eventually was discharged and did as well as could be expected. At 8 mos of age suddenly became iull and in a matter of 5 days, died. Autopsy revealed medium and long chain fatty oxidation disorder (inborn error of metabolism). In addition, when the palcental slides were evaluated, they revealed classical "maternal floor infarction" of the placenta. This unique disorder is really a misnomer, since there is no true infarction. It consists of fibrin deposits in the decidua basalis and affects the chorinic villi and intervillous space. Frequently associated with severe FGR, oligohydramnios, and fetal death. It has a recurrence rate as high as 10%. Once again, it is not due to ischemic necrosis and there is no true infarction of the placenta. The patient is currently 25 weeks' gestation and the fetus and placenta appear normal. No evidence of oligohydramnios. However, the placenta appears to have some areas of hyperechogenic foci. I'm trying to see if the fatty oxidation in the fetus and the maternal floor infarction are somehow related. I remember a post with regard to fatty liver of pregnancy and fatty oxidation, but cannot recall when it was posted. Her child's liver had marked fatty infiltration as did many other organs.
-- Luis Sanchez-ramos, MD
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