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Biomedical subjects

C Azen

Publications and source records attributed to C Azen.

9 recordsLinked to original sources
PubMed1992

Maternal phenylketonuria collaborative study, obstetric aspects and outcome: the first 6 years.

OBJECTIVE: The purpose of this study was to evaluate the efficacy of a phenylalanine-restricted diet in reducing fetal morbidity associated with maternal hyperphenylalaninemia in women of childbearing age with blood phenylalanine levels greater than 240 mumol/L (greater than 4 mg/dl) on an unrestricted diet. STUDY DESIGN: Two hundred thirteen pregnant women with hyperphenylalaninemia that resulted in 134 live births have been enrolled in the study. Outcome measures were subject to the chi 2 test, Fisher exact test, analysis of variance, t test, or Wilcoxon nonparametric test for analysis. RESULTS: Optimal fetal outcome appeared to occur when blood phenylalanine levels less than 600 mumol/L (less than 10 mg/dl) were achieved by 8 to 10 weeks' gestation and maintained throughout pregnancy (trimester averages of less than or equal to 360 mumol/L (less than or equal to 6 mg/dl). Initiation of dietary therapy during the third trimester of pregnancy appears to have little beneficial effect on the fetus. CONCLUSIONS: Preconceptual counseling and early entrance into a prenatal care program is essential in achieving optimal fetal outcome in women with hyperphenylalaninemia.

Congenital Abnormalities↗
PubMed1990

A preliminary report of the collaborative study of maternal phenylketonuria in the United States and Canada.

The Maternal Phenylketonuria Collaborative Study (MPKUCS), encompassing all the United States and provinces of Canada, is a prospective, longitudinal investigation designed to ascertain the efficacy of phenylalanine-restricted therapy in protecting the fetus from high maternal phenylalanine concentrations in women with hyperphenylalaninaemia. Preliminary findings are reported for 147 pregnancies for whom the recommended therapeutic range of blood phenylalanine was 120-360 mumols/L. Sixty-three pregnancies had complete data for analysis. Dietary control was attempted prior to conception in 10 out of 63 women. Significant negative correlations were noted in length, weight and head circumference and blood phenylalanine concentrations during pregnancy. Average reported phenylalanine levels by trimester for 63 hyperphenylalaninaemic pregnancies resulting in live births revealed that no group requiring treatment achieved levels below 360 mumols/L until the third trimester. Median birth measurement percentiles revealed that all groups studied generally had smaller head size compared with birth length and weight. Those started on diet after the first trimester achieved a head circumference below the 10th percentile. The implication of small head circumference for subsequent intellectual development is unclear at this time. Furthermore, the study must evaluate more offspring of women having optimal preconception and pregnancy restriction of phenylalanine.

Canada↗
PubMed1989

Comparative efficacy and safety of cefmetazole or cefoxitin in the prevention of postoperative infection following vaginal and abdominal hysterectomy.

We evaluated three 1-g doses of cefmetazole in comparison with the standard three 2-g doses of cefoxitin for prophylaxis in vaginal or abdominal hysterectomy to determine efficacy and safety. The antibiotics were administered intravenously 30-90 min before the incision and were followed with additional intravenous doses 8 and 16 h or 6 and 12 h later, respectively. The patients received povidone-iodine vaginal preparations before surgery; vaginal packs, when used, contained no antibiotic agents. Vaginal cultures were obtained before the vaginal preparation, at the time of discharge from hospital and when there was a suggestion of operative site infection. The activity of both antibiotics against these organisms was tested. Patient demographic characteristics and surgical procedures were similar in each treatment group. The difference between the primary failure rates with the two antibiotics (2 of 35 (5.7%) with cefmetazole and 2 of 16 (12.5%) with cefoxitin) did not reach statistical significance, and results were similar for the two routes of hysterectomy. Cefmetazole was more active than cefoxitin against the majority of the aerobic and anaerobic organisms recovered, although approximately 20-30% of the isolates showed resistance, or intermediate sensitivity, generally to both antibiotics.

Adult↗
PubMed1988

Blood phenylalanine levels and intelligence of 10-year-old children with PKU in the National Collaborative Study.

Dietary intakes and blood phenylalanine levels of 125 10-year-old children with PKU are reported. Of those patients, 59 discontinued the special diet at 6 years, 16 discontinued at 8 years, 5 discontinued at 6 years but returned to the diet at 8 1/2 years, and 45 continued the diet to 10 years. Phenylalanine intake was determined by 2-day diet records. Blood phenylalanine concentrations revealed a lack of clear distinction between the diet groups; therefore, regression analysis was performed on the group as a whole. The analyses revealed significant relationships between blood phenylalanine levels from ages 6 to 10 years and each of the cognitive outcome variables, after the influences of parental IQ and the age at which treatment was initiated were controlled for. Intelligence test scores were also related to phenylalanine levels between ages 3 1/2 and 5 1/2 years. The findings suggest that dietary restriction of phenylalanine should continue in patients with PKU through at least 10 years of age.

Child↗
PubMed1977

Intellectual assessment of 111 four-year-old children with phenylketonuria.

Of the 216 children with phenylketonuria (PKU) who were initially enrolled in the Collaborative Study of Children Treated for Phenylketonuria, 203 were placed on dietary therapy between 3 and 92 days of age. Of these, 111 are now at least 4 years of age and constituted the sample for the present analysis. Their mean IQ on the Stanford Binet Intelligence Scale was 93 (1972 norms). The children assigned to two treatment groups based on "moderate" and "low" serum phenylalanine levels were comparable on their IQs at age 4, although many of the children could not be maintained in the specified categories. Females scored a significantly higher mean IQ than males (97 vs 90). Those children for whom dietary treatment was initiated during the first month of life scored a mean IQ of 95, compared with 85 for those initially treated from 31 to 65 days. However, the interpretation of dietary inception data may have been contaminated by familial and psychosocial factors. The PKU Collaborative Study is still in progress in 15 clinics located in 11 states.

Child, Preschool↗
PubMed1976

A computerized procedure for estimating nutrient intake.

A procedure was devised for computing intake in terms of calories, total protein, phenylalanine, carbohydrate, and fat. The procedure used a magnetic tape containing 3,122 numbered food items. The nutrient composition of each food was reported for 100 g of the edible portion of the food. In addition, diet diaries were prepared in which the foods eaten during the preceding 24-hr period, the code for each food corresponding to the number for the same item on the magnetic tape, and the number of units of each food eaten were recorded. A computer program then was written that calculated the amounts of intake per day for each nutrient. Application of the procedure for 42 consecutive days on the daily diet records of 43 adult carriers of the phenylalanine hydroxylase enzyme formed the data base used to determine if aspartame significantly increased levels of phenylalanine in the blood. Adaptations of the procedure permit calculations of intake for periods from 1 to 30 days and analyses of additional nutrients including calcium, phosphorous, iron, vitamin A, thiamine, riboflavin, niacin, and ascorbic acid.

Computers↗