SIDS and subsequent pregnancy

From: Terrence.Jones@ncal.kaiperm.org
Mon Oct 28 15:36:52 1996


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).

By now, my guess is only Dr. Bui is still reading! (with the possible exception of Dr Bergstrom - who will be checking proper paragraph and sentence struckcha) :) Dr. Bui, along the lines of Terry Dubose's description (per Dr. Fineberg) {see 9/96 ob-gyn-l}, have you noticed an increase in the incidence of VCI (velamentous cord insertion) in the donor twin in TOPS (Twin Olio-Poly Syndrome)? Also, as you mentioned in your Mo-Mo (monochorionic/ monamnionic) description of morbidity {see Ob-Gyn-l 7/95}, quite a number of problems arise from cord accidents. I'm not sure if it was Dr. Moise, or Dr. Copel, who frowned upon fenestration in TOPS as this might convert the pregnancy to monoamniotic. My guess is these fenestrations are too small to allow passage of fetal small parts or umbilical cord -- can you comment on this? (Not that it would change management, as this appears to be the only salvaging method avail. in this setting, and our perinatologists will freq. offer this technique.) Terry Jones Kaiser San Fran Dept Ob/Gyn





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