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(Spon. by John Kattwinkel) . University of V i r g i n i a Medical Center, Department of P e d i a t r i c s , C h a r l o t t e s v i l l e . D e t e r i o r a t i o n of Doppler-derived a n t e r i o r c e r e b r a l a r t e r y flow v e l o c i t i e s has been described i n newborns with evidence of b r a i n death (Ann. Neurol. 14:302-307, 1983). Since these changes suggest a progressive decrease i n c e r e b r a l perfusion, we c o r r e l a t e d s e q u e n t i a l Doppler flow v e l o c i t i e s with technetium c e r e b r a l angiograms i n 2 asphyxiated neonates.Case 1 was a 3800 gram term baby who developed l e t h a r g y and s e i z u r e s within 24 h r s . of a d i f f i c u l t d e l i v e r y . CT scan revealed c e r e b r a l edema and m a l l subdural hemorrhage. Doppler flow s t u d i e s (Medasonics D-10, 5 MHz probe) performed on day 3 showed r e t r o g r a d e d i a s t o l i c flow. Peak s y s t o l i c flow diminished on day 4. A technetium angiogram on day 4 showed markedly diminished c e r e b r a l perfusion and repeat study on day 9 a f t e r l o s s of a l l brainstem f u n c t i o n s showed absence of i n t r a c r a n i a l circul a t i o n . Case 2 was a 3400 gram baby with severe b i r t h asphyxia and s e i z u r e s . CT scan revealed small v e n t r i c l e s and subarachnoid hemorrhage. Doppler flows were normal on day 1. Over the next 4 days, d i a s t o l i c flow reversed and peak s y s t o l i c flow diminished. Technetium study on day 6 showed absence of i n t r ac r a n i a l flow. Both p a t i e n t s expired.These p a t i e n t s confirm the e a r l i e r a s s o c i a t i o n between a d e t e r i o r a t i o n of Doppler flow v e l o c i t i e s and c l i n i c a l evidence of b r a i n death. Lack of c e r e b r a l perfusion was documented with technetium angiograms. The Brindled mouse mutant i s a model f o r t h e Kinky Hair syndrome. Male Brindled hemizygous s u c k l i n g mice demonstrate poor growth and neurologic d e t e r i o r a t i o n beginning during the f i r s t week of l i f e and ending w i t h d e a t h by t h e end of t h e second week. Newborn Brindled c a r r i e r s a r e asymptomatic with normal b r a i n copper c o n c e n t r a t i o n ; l i v e r copper c o n c e n t r a t i o n is low and t h e r e n a l copper i s high. Newborn c a r r i e r pups suckled by Brindled dams p u t on low copper d i e t s on t h e day of p a r t u r i t i o n , however, show d e f i c i e n t weight g a i n by t h e age of 6 days and by 12 days weigh approximately one-half t h e i r normal l i t t e r -m a t e s . Neurologic symptoms developed i n t h e copper-deficient young c a r r i e r s by age 12-14 days w h i l e t h e i r normal l i t t e r -m a t e s remained asymptomatic. Tissue copper s t u d i e s demonstrate t h a t t h e l i v e r and b r a i n copper c o n c e n t r a t i o n i n t h e c a r r i e r is no d i f f e r e n t from t h a t of t h e normals, although the r e n a l copper is g r e a t e r than twice a s high. On copper-deficie...

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Neonatal survival of very low birth weight (VLBW) infants(600-999 grams) and long-term outcome of (LBW) infants (600-1499 grams.) are correlated w1th del1very resuscitation management were reviewed and rated. 11 0ptimal delivery care" was defined as appropriate intrapartum care and a controlled, uncomplicated delivery. 11 0ptimal resuscitation 11 required that a team be present at delivery and encounter no technical problems. Optimal delivery room (DR) management occurred when both obstetric and pediatric care were appropriate. Adequate information was available to rate 80% of cases. Survival of VLBW infants was significantly improved with optimal DR care (p < 0.05). DR care had the greatest impact on 700-999 grams infarrts. In this group, 68% of survivors had "optimal care" while only 31% of the neonatal deaths (ND) had "optimal care" (p < O.OlJ. Neonatal deaths also occurred later with optimal DR care (p < 0.02). Unavoidable obstetrical complications occurred in 14 deliveries of VLBW infants (11 ND and 3 survivors) (p < 0.05). All of the survivors had optimal resuscitation. Only 1 of the 3 survivors was normal at follow-up. Vaginal breech delivery of LBW infants had a significant neonatal mortality compared to vertex vaginal delivery (p < 0.01) or breech C-section delivery (\' < 0.02). Optimal DR management was also associated with norma long-term outcome for LBW infants. Fifty \'ercent with optimal DR care and 30% without were normal at fo low-up (p < 0.05). With improvement in Newborn Intensive Care, the effects of intrapartum and delivery room management can now be measured in both neonatal survival and long-term outcome. These findings have SEecial significance for level I who transport LBW infants after delivery and