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Re: Fatty acidFrom: Peter Wein (p.wein@obsgyn-mercy.unimelb.EDU.AU)Wed Dec 30 18:05:36 1998
> >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 > Oh - I know this condition as massive intervillous fibrin deposition - have had couple of patients with it, and together with our pathologist have never managed to find any particular association - very little about it other than some pathology references. One of the patients had it twice, and we successfully treated her in her next pregnancy with heparin/aspirin - normal baby - although I don't know of long-term follow up. The post about AFLP and fatty acid oxidation was from Terence Jones in 1996 I think: From: Terrence.Jones@ncal.kaiperm.org Mon, 28 Oct 1996 14:28 -0800 (PST) "Garry, somewhere out there, where the zebra's and unicorns roam, is the consideration of long-chain fatty-acid deficiency (LCAHD) and its assoc with SIDS. The obstetrical significance would be that of AFLP (acute fatty liver of pregnancy), or stigmata thereof, in the mother. This topic came up around this time last year, and Dr. Copel (now on sabbatical from Yale, and Ob-Gyn-l) offered the help of Dr. Rinaldi (also from Yale), for eval of the newborn should the Mom have features c/w AFLP. It would be quite unlikely in the case you've presented in view of the antecedent hx, but always worth mentioning for those managing AFLP. For more on this topic, see below. For a general review on recurrent SIDS see Oyen, Am J Ped 8/96, vol 144: 300-5. This study, from Norway, followed 352,475 Mother/Child pairs from 1967- 88. The recurrence rate (5.8 overall) was stratified for a number of variables, including prematurity, malformations, infection, etc. There have been a number of studies of cigarette smoking, summarized by Taylor, J Ped 6/95, vol 126:887-91; with a suggestion that smoking-cessation may contribute to a possible 30% reduction in occurrence'. Leaving the unicorns behind, and heading somewhere over the rainbow, there's also the consideration for MELAS variants (if not interested, press PF3 now!). As mentioned in a previous post, regarding carnitine supplementation, these mitochondrial disorders may affect resp. chain complex function. A report by Yoon (Ped Res 5/93, vol 33:433-40), describes a defect in complex III-IV in liver/kidney/muscle (with muscle predominating). The newborns (2 from same mother) both died within a few days after term birth. The mother, and her twin sib, had a hx of recurrent pregnancy loss. Southern blot analysis of the affected children's mitochondrial DNA failed to demonstrate any deletions. When studied after denaturing gradient gel analysis (detects single base changes), there was revealed an extra band not seen in the mother. This was due to an A-to-G mutation at 15923 that causes t-RNA transcription dysfunction. The mother's twin sib, at 16 wks pregnant, underwent amnio which failed to demonstrate the defect in cultured amniocytes. This was confirmed on cord blood analysis of the newborn, which remains alive and healthy. Other aspects of mitochondrial dysfunction may be assoc with cardiomyopathy, and dysrhytmic phenomena (Sato Am Heart J (10/94) vol 128:733- 41). Tho these have also been assoc with sudden death in the newborn period, they are phenotypically demonstrable at birth. For the few remaining who have any interest in AFLP and SIDS, as related to lond-chain 3-hydroxyacyl-CoA dehydrogenase def, the mutation has been traced to a G-to-C at 1528 (G1528C) of the alpha-subunit of the tri-functional protein on both alleles (Sims, Proc Nat'l Acad Sci USA 1/95, vol 92:841-5). " Peter Wein
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