ABSTRACTS
767 Cystic Fibrosis Screening in the Newborn. Jeanette
GENERAL CONSIDERATIONS Congenital Malformations
in Newborn Babies. Jo~o
M.
Videirar'.4maral, M.E. Sacramento Monteiro,:.= Nuho Cordeiro Ferreira, and Wilson Lopes. Rev Port Pediatr Special number on neonatology 1977 A retrospective study of 5921 live-born and stillborn infants (5776 and 145, respectively) in a neonatal care unit in the city of Lisbon was made in order to estimate the incidence of congenital malformations (CM), both as regards the total number and the types of malformations diagnosed at birth and classified as major and minor. The incidence of CM major was 2.6%, and that of C M minor was 5.6%. The incidence of major malformations was higher in infants with low birth weights, and that of minor malformations was higher in infants with weights over 2.5 g. Malformation was one of the more important causes of neonatal mortality; it was found that 10.9% of stillborn infants and 15% of live-born babies who had died were severely malformed.--Antbnio Gentil Martins Fetal Wastage as a Result of an Alpha-Fetoprotein Screening Programme; Iv'.B. Gordon, M. J. Kitau, A. T. Letehworth, J.
G. Grundzinskas, M. M. Usherwood, and T. Chard. Lancet 1:677-678 (April), 1978 One hundred two women underwent amniocentesis for the measurement of amniotic alpha-fetoprotein following the finding of raised serum alpha-fetoprotein above the 95th percentile at 16-24 wk. The procedure was performed after ultrasonic confirmation of gestational age, placental localization, and absence of multiple pregnancy. As a result, 17.6% (18 patients) underwent termination of pregnancy. Of these 18, 15 fetuses were found to have neural tube defects, 2 had gastrointestinal abnormalities (one with exomphalos and multiple defects, the other with exomphalos alone), and 1 patient had hydrocephalus. Four patients spontaneously aborted after the procedure, one of which was abnormal with exomphalos and neural tube defect. The authors suggest that in order to reduce the number of patients submitted to amniocentesis, action should be taken only if serum alpha-fetoprotein exceeds 2.5 times the median value at that gestational age; in order to prevent the complications of amniocentesis, the procedure should be performed under direct ultrasonic observation. They suggest no way in which differentiation may be made between gastrointestinal tract abnormalities and neural tube defects on the basis of alpha-fetoprotein.--J. G. Harvey Adverse EffeCts of Routine Procedures on Preterm Infants.
B. D. Speidel. Lancet 1:864-865 (April), 1978 Using continuous monitoring of arterial oxygen tension in neonates, the author shows that a significant fall in PaO 2 occurs when the sick neonate is handled during nursing observations, blood.taking,or x~ray proced~es. Spontaneous .. r e t u r n e r the PaO 2 usually occurs; but repeated or~sequential procedures may cause a prolonged fall in PaO2 necessitating ventilatory support. The cause of the fall in PaO 2 may be related to transient apnea during handling or to right-to-left shunting of blood through the foramen ovali or ductus arteriosus during crying. The author suggests that a policy of minimal handling should be adopted in all neonatal nurseries.--J. G. Harvey
R.
Crossley, Colleen C. Berryman, and Robert B. Elliott. Lancet 2:1093-1095 (November), 1977 The authors describe a new screening test for cystic fibrosis in the neonatal period based on the pancreatic enzyme trypsin in the stool sample. A colorless substrate b e n z o y l - a r g i n i n e - p - n i t r o a n i l i d e ( B A P N A ) releases p-nitroaniline when hydrolyzed by trypsin. Stool samples from neonates who lack tryptic activity give negligible color change. The incidence of false positive results was 0.1% and 0.05% on retesting. There were no false negative results. The authors justify the new method in terms of its sensitivity and ease of interpretation and application.--J. G. Harvey Cimetidine Cholestatic Jaundice in Children. John R. Lilly,
David C. Hitch, Norman B. Javitt. J Surg Res 24:384-387 (May), 1978 Five children ages 1-14 yr with peptic ulcer disease were treated with cimetidine. Dosage was calculated at 20-30 mg/kg/day for both oral and intravenous administration. Serum bilirubin, glutamic oxaloacetic transaminase, alkaline phosphatase, and serum bile acid determinations were made prior to therapy, during therapy, and after treatment. A l l patients demonstrated prompt radiographic healing of ulceration. Elevated serum bile acids were noted in all patients during therapy, but they returned to normal after discontinuation of cimetidine. Serum bile acid is considered a more discriminating index of hepatic excretory function, and hence cholestasis, than serum bilirubin. It is speculated that cimetidine induces a ductular-type cholestasis because of its blocking effect of vagal and secretin receptors.--Richard J. .4ndrassy Hepatic Impairment in Infants Induced by Hig h Calorie Infusion.
1I. Hirai, Y. Sanada, T. Fujiwara, et al. J Jpn Soc Pediatr Surg 14:409 420 (April), 1978 Hepatic impairment was encountered in 14 of 19 newborns and 4 of 9 infants who had long-term intravenous hyperalimentation (more than 2 wk). The authors classified types of hepatic impairment induced by high calorie infusion into three groups: Transient elevation of serum transaminase without elevation of alkaline phosphatase (type I) was seen in 6 newborns and 4 infants. This type of hepatic impairment was considered to be due=to, imbalance of nutrients. Elevation of alkaline phosphatase and direct bilirubin without elevation of serum transaminase ( t y p e II) was seen in 2 newborns. The ballooning of liver cells by overloading of nutrients resulted in compression of bile canaliculi and obstructive jaundice in this group. Marked and prolonged elevation of alkaline phosphatase, direct bilirubin, and serum transaminase (type III) was seen in 6 newborns. T h e etiology of this type of impairment was considered,to be immaturity of hepatocellular enzymes.--H. Suzuki Effects of Prolonged Parenteral Nutrition In Preoperatory Treatment of Glycogenosis. J. G. Maksoud, U. Tannuri, .4.
L. Mathias, and V. .4. Carvalho Pinto. Rev Assoc Med Bras 23:211-213 (June), 1977 The authors present their experience with 2 children with glycogen storage disease treated with intravenous