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Biomedical subjects

C Azen

Publications and source records attributed to C Azen.

At least 37 records · Page 2Linked to original sources
PubMed1996

The North American Maternal Phenylketonuria Collaborative Study, developmental assessment of the offspring: preliminary report.

Preliminary results of 2-year Bayley and 4-year McCarthy test scores are presented. To date numbers are too small to statistically correlate:- offspring from pregnancies in which diet was started prior to conception, offspring from pregnancies with phenylalanine (Phe) levels of 120-360 mumol/l versus 360-600 mumol/l, influence of home environment, influence of maternal nutrition, language development, behaviour/hyperactivity, Revised Wechsler Intelligence Score, school performance and learning disabilities. Two-year Bayley scores (mental and motor) revealed a median developmental quotient of 113 in 58 offspring from control pregnancies, 104 in 19 offspring from untreated "non-phenylketonuria (PKU) mild hyperphenylalaninaemia" (natural Phe levels < 600 mumol/l) pregnancies, 104 in 32 offspring from pregnancies whose Phe levels decreased on treatment to < 600 mumol/l by 10 weeks gestation and remained in that range for the remainder of the pregnancy, 98 in offspring from 32 pregnancies where permanent control was not achieved until 10-20 weeks and 72 in offspring from 51 pregnancies where control was not attained until after 20 weeks gestation. IQ scores determined by the McCarthy test at age 4-5 years revealed a mean of 112 in 43 offspring of control mothers, 99 in 12 offspring of "non PKU mild hyperphenylalaninaemia" women, 93 in 14 offspring whose mother's Phe levels were continuously under 600 mumol/l by 10 weeks gestation, 88 in 24 offspring from pregnancies in metabolic control by 10-20 weeks and 73 in 28 offspring of pregnancies not in metabolic control until after 20 weeks gestation. These preliminary results suggest that early and adequate dietary treatment during pregnancy in maternal PKU may provide some protection to the fetus for later intellectual development but much more data is required before definitive statements about cognition can be made.

Child Development↗
PubMed1996

Care of the adult with phenylketonuria.

Forty-three adults with classical phenylketonuria were identified by neonatal screening and treated with a phenylalanine (Phe) restricted diet. Nineteen have remained on dietary treatment with varying levels of blood Phe control and 24 have discontinued the diet at an average age of 7.8 years. Follow up at an average age of 22 years revealed that the cohort remaining on dietary treatment have achieved substantially better social and academic achievement than the 24 who discontinued dietary treatment. Another group of 19 adults who were not diagnosed until an average age of 2.5 years have also been evaluated after an average of 22 years on a Phe restricted diet. This report is based upon Wechsler Adult Intelligence Revised Test scores, attendance at college, employment and marital status.

Adolescent↗
PubMed1996

Hormone replacement therapy and cobalamin status in elderly women.

Serum cobalamin concentrations are frequently low in the elderly but the cause is often not apparent. Because oral contraceptives have been associated with low cobalamin concentrations in young women, we compared hormone use with cobalamin status in elderly women to determine whether it could account for their unexplained low cobalamin concentrations. Thirty-eight of the 111 women had abnormal cobalamin status (defined by low cobalamin, elevated methylmalonic acid, and/or elevated homocysteine concentrations) and 73 had normal status. There was no difference in hormone use between the two groups: 7 (18.4%) of the 38 cobalamin-deficient subjects used estrogens compared with 20 (27.4%) of the 73 control subjects. No differences in hormone use were apparent either when analysis was confined to abnormal serum cobalamin concentrations alone. Similarly, the 27 women taking hormones and the 84 women not taking hormones did not have significantly different serum cobalamin or serum total homocysteine concentrations. Indeed, hormone users had slightly, though not significantly, higher cobalamin concentrations and lower homocysteine concentrations than nonusers; furthermore, hormone users also had significantly lower serum methylmalonic acid concentrations. Thus, neither cobalamin concentrations nor cobalamin metabolic status were significantly worse in elderly women taking estrogen than in those not taking it (and, if anything, may have been slightly better). Hormone use does not appear to be a significant contributor to the low cobalamin concentrations or the mild metabolic evidence of cobalamin deficiency so often seen in the elderly.

Aged↗
PubMed1995

Tumor marker trends in asymptomatic women at risk for ovarian cancer: relevance for ovarian cancer screening.

The purpose of our study was to examine single and serial determinations of five biomarkers in asymptomatic women at risk for epithelial ovarian cancer to define median values and trends in a cancer-free cohort. Women were enrolled in the Gilda Radner Ovarian Cancer Detection program at Cedars-Sinai Medical Center, Los Angeles, California from July 1, 1991, to July 1, 1993. Biomarkers studied include CA-125, LASA, DM-70K, UGP, and HER-2. Biomarker values that were analyzed included those obtained on the first patient visit and in a subset of values evaluated at 6-month follow-ups. A total of 590 women were included in the study; 425 were premenopausal and 165 were postmenopausal. No one in this study group has developed ovarian cancer within a 12-month follow-up period. Postmenopausal women had significantly lower values for CA-125 (12 vs 19 U/ml, P = 0.0001) and higher values of LASA (16.6 vs 15.4 mg/dl, P = 0.0001), DM/70K (11 vs 0 U/ml, P < 0.001), UGP (3.3 vs 0.3 U/ml, P < 0.001), and HER-2 (12 vs 11 U/ml, P = 0.009) than premenopausal women. Less than 5% of the women had elevated tumor marker values on both screens except for CA-125, where 15% of women studied had two consecutive visits with elevated values (> 35 U/ml). Two women demonstrated an exponential rise in CA-125 between visits. Premenopausal women differ in median biomarker values compared to postmenopausal women. Elevations in single tumor markers, combinations of markers, and exponential increases of markers have all produced false-positive results for ovarian cancer within the 12-month follow-up period. Exponential increases produced the smallest false positive rate (n = 2), but further study and follow-up are needed to determine the efficacy of these tests in ovarian cancer screening.

Adult↗
PubMed1995

Lupus in the 1980s: III. Influence of clinical variables, biopsy, and treatment on the outcome in 150 patients with lupus nephritis seen at a single center.

Of 500 patients with systemic lupus erythematosus observed at our center, 150 fulfilled criteria for lupus nephritis. Of these 150 patients, 91% were female, and 67% were white. The mean age of onset was 26.2 years, and the mean follow-up duration was 11.7 years. Biopsies (n = 142) performed on 107 patients showed the following World Health Organization (WHO) class distribution: class I, n = 1; class II, n = 13; class III, n = 19; class IV, n = 69; class V, n = 17; class VI, n = 8; and class not determinable, n = 15. Ninety-five patients were nephrotic. Therapeutic intervention courses given to all patients (n = 356) included parenteral (IV) cyclophosphamide (n = 58), high-dose oral steroids (n = 126), pulse steroids (n = 49), apheresis (n = 39), azathioprine (n = 43), oral cyclophosphamide (n = 5), nitrogen mustard (n = 27), and chlorambucil (n = 6). In addition to examining the course of disease for various subsets, various predictors for fatality and end-stage renal disease (ESRD) were analyzed. Descriptive data for the short-term response to five therapies are provided for the complete patient sample, proliferative disease, and nephrotic syndrome. Twenty patients died, primarily from cardiovascular complications and sepsis, with 97% and 92% 5- and 10-year survival rates, respectively. Twenty-nine were dialyzed, and 11 were transplanted. Risk of ESRD by WHO class at 5 years was as follows: class III, 0%; IV, 9%; V, 16% (P = .04 for class V v other patterns).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

Abnormal somatosensory evoked potentials in patients with classic galactosemia: correlation with neurologic outcome.

In classic galactosemia, long-term neurologic sequelae can include low cognitive functioning and a curious neurologic syndrome with tremors, dysmetria, and ataxia. An abnormal white-matter signal on cerebral magnetic resonance imaging (MRI) is present in almost all patients; some have mild cerebral or cerebellar atrophy and focal white-matter lesions. The present study was undertaken to assess the integrity of myelinated pathways by recording somatosensory evoked potentials. Results were correlated with age at diagnosis, severity of illness, age at evoked potentials, neurologic examination, MRI studies and cognitive outcome as measured by the Woodcock-Johnson Revised Standard Cognitive Battery. Evoked potentials were abnormal in 17 (28%) of 60 patients who had median nerve, and 26 (77%) of 34 patients who had posterior tibial nerve studies. Abnormalities of the central rather than the peripheral nervous system were most common. Evoked potentials correlated with severity of presenting symptoms (P = .011), age at evoked potential testing (P = .029), and presence of focal white-matter lesions on MRI (P = .049). Results of neurophysiologic testing showed no correlation with the Woodcock-Johnson Battery. Patients with classic galactosemia may have abnormal conduction along myelinated pathways that is associated with other central deficits. Myelin, which contains galactose, may be adversely affected in this inborn error of metabolism.

Adolescent↗
PubMed1994

The international collaborative study of maternal phenylketonuria: status report 1994.

Neonatal screening for phenylketonuria (PKU) has created a problem as females with PKU are reaching child-bearing age. Surveys have revealed that maternal phenylalanine blood concentrations greater than 1200 mumol/l are associated with fetal microcephaly, congenital heart defects and intrauterine growth retardation. It is estimated that as many as 3000 hyperphenylalaninemic females may be at risk of producing these fetal abnormalities. To examine this problem, the international maternal PKU collaborative study was developed to evaluate the efficacy of a phenylalanine-restricted diet in reducing fetal morbidity. Preliminary findings have indicated that phenylalanine restriction should begin before conception for females with PKU planning a pregnancy. Dietary control should maintain maternal blood phenylalanine levels between 120 and 360 mumol/l and should provide adequate energy, protein, vitamin and mineral intake. Pregnant hyperphenylalaninemic females who achieved metabolic control after conception or by the 10th week of pregnancy had a better offspring outcome than anticipated. The results of 402 pregnancies are reviewed.

Adult↗
PubMed1994

A reanalysis of the Cu-7 intrauterine contraceptive device clinical trial and the incidence of pelvic inflammatory disease: a paradigm for assessing intrauterine contraceptive device safety.

OBJECTIVE: We calculated and compared the incidence of pelvic inflammatory disease in a 10% random sample of the Cu-7 intrauterine contraceptive device (G.D. Searle & Co., Skokie, Ill.) clinical trial with the rates reported to the Food and Drug Administration and those in subsequent trials published in the world literature. STUDY DESIGN: A 10% random sample of the Cu-7 clinical trial was examined because calculations had demonstrated this random sample to be sufficient in size (n = 1614) to detect a difference in rates of pelvic inflammatory disease from those reported to the Food and Drug Administration. An audit of a subset of the patient files, compared with the original files in Skokie, Illinois, confirmed that the files available for analysis were complete. Standard definitions were used to identify cases of pelvic inflammatory disease and to calculate rates of pelvic inflammatory disease. The world literature on Cu-7 clinical trials was reviewed. RESULTS: The calculated crude and Pearl index rates of pelvic inflammatory disease were consistent with those rates previously reported to the Food and Drug Administration and published in the medical literature. Life-table pelvic inflammatory disease rates were not different between nulliparous and parous women and pelvic inflammatory disease did not differ from basal annual rates in fecund women. CONCLUSION: On the basis of the analysis of this 10% sample, the pelvic inflammatory disease patient rates reported to the Food and Drug Administration for the entire Cu-7 clinical trial are accurate and are similar to those published in the world literature.

Adult↗
PubMed1993

The North American Collaborative Study of Maternal Phenylketonuria. Status report 1993.

Neonatal screening for phenylketonuria (PKU) has created an unexpected problem as females with PKU are reaching childbearing age. Surveys have revealed that maternal phenylalanine blood concentrations above 1200 mumol/L are associated with microcephaly, mental retardation, congenital heart defects, and intrauterine growth retardation among their offspring. It is estimated that as many as 3000 hyperphenylalaninemic females may be at risk for producing these fetal abnormalities. To examine this problem, the North American Maternal PKU Collaborative Study has been developed to evaluate the efficacy of a phenylalanine-restricted diet in reducing fetal morbidity. Preliminary findings indicate that phenylalanine restriction should begin before conception for females with PKU planning a pregnancy. Dietary control should maintain maternal blood phenylalanine levels between 120 and 360 mumol/L and should provide adequate energy, protein, vitamin, and mineral intake. Pregnant hyperphenylalaninemic females who achieved metabolic control after conception or by the 10th week of pregnancy had a better offspring outcome than anticipated.

Adolescent↗
PubMed1993

Effect of hypogonadism and deficient calcium intake on bone density in patients with galactosemia.

Forty children and adults with classic galactosemia had vertebral bone density determined by standard quantitative computed tomography at 3.4 to 44.2 years of age. Compared with age- and sex-matched control subjects, patients with galactosemia had diminished bone density (p = < 0.001). Prepubertal patients of both sexes had bone density determinations below those of the control group (p = 0.008); similar findings were seen in postpubertal patients as well (women, p = 0.001; men, p = 0.008). Women receiving replacement estrogen-progestin therapy for premature ovarian failure had abnormal bone density (136.3 +/- 17.3 mg/cm3 vs 166.0 +/- 17.5 mg/cm3 for control subjects; p = 0.002); patients with evidence of ovarian insufficiency not receiving replacement sex steroids had even lower bone density (92.4 +/- 14.3 mg/cm3 vs 160.2 +/- 20.2 mg/cm3 for control subjects; p < 0.001). Calcium intake for the entire galactosemia group was 540 +/- 344 mg/day. Calcium intake correlated positively with bone density in women given exogenous estrogen (r = 0.87; p = 0.002) and in men (r = 0.74; p = 0.009). Thus the diminished mineralization of bones appears to be another abnormality associated with galactosemia. The results of our study suggest that this is likely secondary to abnormal levels of sex steroids in female patients, low calcium intake, and perhaps an intrinsic defect in the normal galactosylation of the collagen matrix of bone caused by the enzyme defect. Strategies to improve bone formation should be considered to diminish morbidity in patients with this inborn error of metabolism.

Adolescent↗
PubMed1993

Maternal tobacco use and substance abuse: reported prevalence rates and associations with the delivery of small for gestational age neonates.

OBJECTIVE: To determine the changes in reported prevalence rates of tobacco use and substance abuse in a population of pregnant women, as well as to evaluate the associations between such use and sociodemographic characteristics and the delivery of small for gestational age (SGA) neonates. METHODS: We studied 7741 women who delivered at Cedars-Sinai Medical Center from 1986-1990. Antenatal and delivery information was entered prospectively into the computerized perinatal data base. Subjects were classified according to tobacco use and substance abuse status. We defined SGA as a birth weight less than the tenth percentile for gestational age at delivery. Univariate and multivariate analyses were used to determine the associations between SGA and tobacco use, substance abuse, and sociodemographic characteristics. RESULTS: We found that the reported prevalence rates of tobacco use and substance abuse declined between 1986-1990 (10 versus 6% and 7 versus 2%, respectively; P < .001). Tobacco use and substance abuse were reported as being highest in black women (11 and 6%) and lowest in Asian and Hispanic women (4 and 3%), a significant difference (P < .001). Tobacco use and race-ethnicity were found to have the strongest independent associations with SGA. The incidence of SGA was highest in black women identified as tobacco users and substance abusers. CONCLUSIONS: The reported rates of tobacco use and substance abuse varied by year, race-ethnicity, and insurance status. There were significant associations between maternal tobacco use, substance abuse, and race-ethnicity and the incidence of SGA neonates.

Adult↗
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↗