Annabel Teberg

A new disorder of purine metabolism with behavioral manifestations

Hyperuricemia has been observed in a 3-year-old boy with mental retardation, dysplastic teeth, failure to cry with tears, absence of speech, and unusual, autistic behavior. Increased synthesis of purines de novo was documented by a rate of conversion of glycine to uric acid that was seven times that of control. The activity of the enzyme hypoxanthine guanine phosphoribosyl transferase was normal, while that of adenine phosphoribosyl transferase was increased. These observations are interpreted to reflect a distinct disease productive of hyperuricemia very early in life.

Effect of phototherapy in preterm infants on growth in the neonatal period

A total of 120 preterm infants were randomly divided at 24 hr of age into three groups: Group I, controls; Group II, continuous phototherapy for 5 days; and Group III, intermittent phototherapy (12 hr on and 12 hr off) for 5 days. At the end of week 1 80% of the control group regained and surpassed their birth weight as opposed to 44 and 57.6% in the continuous and intermittent phototherapy groups, respectively. In weeks 2 and 3 both phototherapy groups had greater weight gain than the control group. Similar but less marked differences were observed in body lenth and head circumference in the three groups. Data suggest decreased growth during phototherapy with subsequent catch-up in growth during weeks 2 and 3. Differences were less marked between infants on intermittent (rather than continuous) phototherapy and controls. Increased metabolic demands and decreased intestinal absorption during phototherapy may be two of the factors responsible for the observed differences in growth in the three groups.

Factors affecting nursery survival of very low birth weight infants

398 infants with birthweight (BW) 500-1500 g born from January 2 1982 to December 1983 were studied to determine incidence and survival rate by BW and gestational age (GA) categories and to determine causes of death and factors influencing mortality. 58% of the group survived. Factors other than those in the perinatal and postnatal period did not significantly influence survival. Infants with BW below 1000 g delivered by elective C-section had better survival than those delivered vaginally. Survival increased progressively with increasing BW and GA categories with GA more than BW being the limiting factor. Eleven (6.6%) of the deaths in the very low birth weight infants occurred during the nursery period after 28 days of age. These deaths would not have been addressed in the neonatal mortality.

Effect of phototherapy on growth of low-birth-weight infants--two-year follow-up.

The effect of phototherapy on subsequent growth of low-birth-weight infants has been evaluated at two-year follow-up of infants from two separate controlled neonatal studies. Growth of weight, length, and head circumference in the treated infants of Group I was slower than that of control subjects. Growth was comparable in treated infants and control subjects in Group II, and the two control groups, although not concurrent were also similar. Infants whose heads were growing at a normal rate, even though below 2 SD from the norms, were developing normally. Only infants who had deceleration in rate of head growth were abnormal. It is unlikely that phototherapy was responsible for the slow growth patterns seen in Group I, since these patterns were not reproducible in Group II.

Streptococcal disease in Southern California.

Summary 1. Evidence has been presented toindicate that streptococcosis occurs with similar frequency in Southern California as in other areas throughout the United States. 2. A small percentage (2 to 6 percent) of the normal childhood population harbor streptococci. 3. A small percentage (3 to 18 per cent) of respiratory disease in children is due to streptococci, the actual percentage depending upon the time of the year. 4. We found it difficult to predictstreptococcal infection on the basis of symptoms and physical findings alone and therefore strongly recommend a throat culture as a necessary laboratory aid. 5. The acute phase reactants are valuable laboratory aids in differentiating uncomplicated from complicated streptococcosis.

PNPs in Follow-Up Care Of High-Risk Infants

Among infants born with a weight of less than 1,500 gm. during the 1950s, 50 percent or more subsequently were found to have significant physical, neurological, or developmental handicaps(1-3). Improved survival rates in this highrisk group create a need for reliable, long-term data to evaluate the prevalence of significant problems. Through development of a model project with a pediatric nurse practitioner giving primary care and as patient advocate, we hoped to accomplish two goals: 1. To develop a follow-up program with continuity of care that would improve patient-professional relationships and patient satisfaction, compliance, and follow-up. 2. To evaluate the neurological, physical, and developmental status of high-risk infants of low birth weight and the relationship of this status to changes in perinatal

EFFECT OF INTRAUTERINE GROWTH RETARDATION ON PREMATURE INFANTS OF SIMILAR GESTATIONAL AGE

Previous evaluation of 35 SGA and AGA very low birth weight infants (VLBW) of similar birth weight (BW) showed differences in developmental quotient but none in neurologic outcome at one year of age (Ped. Res. 20:165A, 1985). To determine the effect of gestational age (GA) on the outcome of the VLBW infant, 20 SGA infants were compared to 20 AGA infants matched for GA, year of birth, race, gender and socio-economic status. Anomalies and intrauterine infections were excluded. SGA infants had a mean GA of 30.8 wks and a mean BW of 1006g. AGA infants had a mean GA of 30.6 wks and a mean BW of 1294g. All infants were evaluated at term, at 20 and 40 wks corrected for prematurity. Results showed that at birth the SGA infants were lighter, shorter with smaller head circumference (p<.005). In the nursery the SGA infants required respiratory assistance for longer periods (p<.005) and remained in the hospital longer (p<.001). More SGA infants were classified as neurologically suspect and abnormal at 20 wks. (p<.01) and at 40 wks. (p<.005). The Gesell developmental quotient was lower in the SGA infants reaching significance at 40 wks (p<.001). At one year of age 30% of the SGA and 5% of the AGA demonstrated neuromotor handicaps (p<.05). In conclusion, comparing the neurodevelopraental outcome of the VLBW infants by similar GA rather than by BW demonstrated the increased risk of intrauterine growth retardation for the immature brain.

CAUSE OF DEATH IN VERY LOW BIRTH WEIGHT PREDOMINANTLY HISPANIC POPULATION: RELATIONSHIP TO BIRTH WEIGHT AND GESTATIONAL AGE

Although mortality rate has been reported to be directly related to birthweight (BW) and gestational age (GA), there is little data regarding the impact of these factors related to cause of death in the very low BW infants of Hispanic heritage. During the 18 month period from January 1982 to July 1983 there were 22,903 deliveries at the LAC/USC Medical Center, a tertiary perinatal center with a predominantly (87%) Hispanic population. There were 289 live born infants with BW between 500-1500 g. One hundred twenty five or 43% of these infants died. Among the non-survivors, 60% were male, Apgar score of ≤ 5 were found in 88% and 60% at 1 and 5 minutes respectively, 80% required assisted ventilation and 60% had respiratory distress syndrome (RDS). In infants with BW ≤ 1000g the mortality was 73% and the major causes of death were immaturity (48%), birth asphyxia (19%) and RDS (15%). In infants with BW 1001-1500g the mortality was 20% and the major causes of death were congenital anomalies (25%), intraventricular hemorrhage (25%) and RDS (22%). The mortality of the infants ≤ 28 weeks gestation was 76% compared to 27% in infants > 28 weeks. When infants weighed ≤ 1000g, survival was independent upon GA rather than BW. However, when infants weighed > 1000g, GA was not a factor since the outcome was similar in all categories. We conclude that the reduction of nursery mortality in our center is quality dependent upon the prevention of extreme prematurity (GA < 28 wks.) rather than upon BW≤1000g.

DECLINE IN MENTAL DEVELOPMENT AND INFANT-MOTHER ATTACHMENT SCORES IN INFANTS OF SUBSTANCE ABUSE MOTHERS

A cohort of 24 physically and neurologically normal infants followed in a special pediatric clinic designed for care of off-spring of substance abusing mothers† was evaluated at ages 1 and 2 years with the Bayley Scales of Infant Development and with a modification of the Ainsworth-Bell Infant-Mother Attachment Scale. The 11 male and 13 female infants were from a low income Hispanic background. Mean Bayley Mental Scores (MDI) declined from 95.5 at 1 year to 87.3 at 2 years. At 1 year of age 80% of children had normal MDI scores (mean=95.5) and 20% had suspect scores (mean=83.0). When re-tested at age 2, 67% were normal (mean=87.3), 29% were suspect (mean=80.8) and 1 female infant was abnormal (67.0). Of the 7 infants with suspect scores, 5 were male and 2 were female. When infant-mother attachment behavior was evaluated at 1 year, 83% were found to be normal, 17% suspect or abnormal. Normal second year attachment behavior was found in 71% of the sample with 29% suspect or abnormal. All 7 children with suspect/abnormal attachment behavior at 2 years showed a decline in mental score from the first year. Four of these had fallen from normal to suspect category. In summary, infants of substance abuse mothers showed a decline in Bayley mental scores and a decline in appropriate attachment behavior over the first 2 years. Test results suggest that male infants may be more vulnerable to this decline. Health providers must recognize that these families need long term support. †Herion, PCP, polydrugs.

RELATIONSHIP BETWEEN LARGE FOR GESTATIONAL AGE AND APPROPRIATE FOR GESTATIONAL AGE INFANTS OF CLASS B DIABETIC MOTHERS

To determine the relationship between intrauterine growth and neonatal morbidity in the infant of class B diabetic mother, we compared 145 appropriate for gestational age (AGA) infants and 41 large for gestational age (LGA) infants born from July 1985 to July 1986. California Intrauterine Growth Curves were used to categorize the infants. The mean birth weight of the AGA infants was 3260g compared with 4098g (p<0.001) of the LGA infants. Forty-five % of the AGA infants were born by C Section compared with 64% of the LGA infants (p<0.05). No differences were found in mean gestational age (AGA 37.8 weeks vs LGA 37.9 weeks), Apgar scores, initial calcium level, initial or peak bilirubin. Differences were found in glucose value done 30 minutes after birth (dextrostix), 39.7mg% in AGA vs 35.0mg% in LGA (p<0.001), in lowest glucose value (dextrostix), 33.3 mg% in AGA vs 31.4 mg% in LGA (p<0.005), and in highest hematocrit, 59.7% in AGA vs 61.2% in LGA (p<.01). There were no differences in need for intravenous glucose supplementation, in volume or in time of initiation of oral feeds. The great majority of infants did very well and did not require special observation after 12 hours. Only 12.5% of AGA and 9.8% of LGA infants remained hospitalized after five days. We conclude that the major management differences between the groups related to method of delivery and that minor differences did not affect the nursery course and management between the AGA and LGA infants.