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Biomedical subjects

C Ferencz

Publications and source records attributed to C Ferencz.

At least 37 records · Page 2Linked to original sources
PubMed1993

Use of prescription and non-prescription drugs in pregnancy. The Baltimore-Washington Infant Study Group.

We analyzed use of therapeutic drugs during pregnancy by 2752 mothers of infants without major congenital malformations. During pregnancy, 68% of the women used at least one prescription or non-prescription drug. Drug use in pregnancy was significantly more common for women who were white, older, married, better educated, of higher income and occupational status, receiving private prenatal care and not living in urban areas. Number of maternal illnesses, higher socioeconomic status, white race, multiparity and use of recreational drugs explained 26% of reported drug use. The mean number of drugs reported (1.2) underestimates total drug exposure due to exclusion of some drug categories including multivitamins and illicit drugs. Since the majority of women giving birth to normal infants report use of at least one pharmacologic agent during pregnancy, attribution of adverse outcome to drug use in an individual case is rarely justified.

Abnormalities, Drug-Induced↗
PubMed1993

Congenital heart disease in adolescents and adults. Teratology, genetics, and recurrence risks.

This brief review has described historic highlights of etiologic knowledge, current concepts in the categorization of cardiovascular anomalies based upon ongoing advances in teratology, and epidemiologic evaluations of biologic and xenobiotic risk factors with emphasis on the teratogenic roles of maternal diabetes, hyperphenylalaninemia, and parental exposures to alcohol, drugs, solvents, pesticides, lead, and other toxic substances. Evidence is presented for a strong genetic basis of cardiovascular maldevelopment requiring further studies to define at-risk families. Counseling and personal and societal preventive interventions may reduce the occurrence of some forms of CHD.

Abnormalities, Drug-Induced↗
PubMed1993

Temporal trends in prevalence of cardiovascular malformations in Maryland and the District of Columbia, 1981-1988. The Baltimore-Washington Infant Study Group.

We studied the yearly occurrence of isolated cardiovascular malformations in liveborns for the period 1981-1988 in Maryland and the District of Columbia. We used Poisson regression to fit yearly prevalence at birth as a function of time for all cardiovascular malformation case types combined and for three diagnostic subgroups. We give results separately by diagnostic method and race (white/nonwhite), with categories combined when appropriate. Time trends were similar for both races except in pulmonic stenosis diagnosed by echocardiography. The yearly prevalence for all case types, all races, and all diagnostic methods combined increased from 28 to 43 per 10,000 livebirths over the 8 years, due to an increase in the rate among infants diagnosed by echocardiography. For every diagnostic-type-by-race group studied, there were increases in the prevalence for those diagnosed by echocardiography and decreases (or no change in one group) for those diagnosed by cardiac catheterization and/or surgery. Although there may have been an increase in actual occurrence of cardiovascular malformation over our study period, it is also possible that wider use of a new echocardiography technology resulted in increased detection.

Black People↗
PubMed1993

Paternal exposures and cardiovascular malformations. The Baltimore-Washington Infant Study Group.

Possible associations between paternal exposures and cardiovascular malformations were evaluated in the Baltimore-Washington Infant Study, a population based case-control investigation of congenital heart disease and environmental factors. Home interviews of case and control parents elicited information on parental home and occupational exposures. Analysis focused on twelve cardiac diagnostic groups and paternal exposures incurred during the six months preceding the pregnancy. Associations were identified between jewelry making and atrial septal defect (Odds ratio: 12.6; 95% confidence interval: 2.3-68.6) and membranous ventricular septal defect (8.1; 2.0-33.3), welding and endocardial cushion defect with Down syndrome (1.8; 1.1-3.0), lead soldering and pulmonary atresia (2.3; 1.1-4.9) and ionizing radiation and endocardial cushion defect without Down syndrome (4.7; 1.7-12.6). Ionizing radiation was found to be associated with endocardial cushion defect with Down syndrome only when father was present at interview (5.6; 1.7-17.9); a similar effect of father at interview was noted for paint stripping in relation to coarctation of the aorta (3.5; 1.5-8.0) and muscular ventricular septal defect (3.5; 1.5-8.5). Also, paint stripping was associated with hypoplastic left heart only in the presence of family history of cardiac defects (11.9; 2.4-60.0). This large study on cardiac diagnostic groups and specific preconceptional exposures provides new leads for further assessment of the role of paternal exposures on adverse pregnancy outcome.

Adult↗
PubMed1992

Endocardial cushion defect: further studies of "isolated" versus "syndromic" occurrence.

The isolated occurrence of endocardial cushion defect (ECD) has been suggested to differ from its occurrence within the context of a syndrome, with regard to the nature (complete or partial) of the defect and the associated cardiovascular malformations. Analysis of data derived from the Baltimore-Washington Infant Study of congenital cardiovascular malformations supports the observation that "syndromic" ECD tends to be of the complete atrioventricular canal type and is less frequently associated with left cardiac anomalies than the isolated form. However, each syndrome has a unique impact on the overall cardiovascular "phenotype", including the ECD. This is especially true for Down and Ivemark syndromes, which are most frequently associated with ECD, but also for other syndromes as well. It is also suggested that isolated ECD is specifically associated with gastrointestinal and urinary tract anomalies. However, in Down syndrome ECD appears to be a specific cardiovascular expression of the trisomic state that is unrelated to other noncardiac malformations. Additional information on the association of ECD with other less common genetic syndromes is needed in order to further investigate the possible genetic basis of this cardiac defect.

Case-Control Studies↗
PubMed1992

Cardiomyopathy in infancy: observations in an epidemiologic study.

Cardiomyopathy (Cm), a rare form of cardiac disease in infancy, is receiving increasing attention stimulated by the availability of endocardial biopsy and new forms of therapy. Population-based information on frequency of occurrence, types, and maternal and infant characteristics of this diverse group of heart muscle disorders has been obtained in the course of an etiologic study on cardiovascular disease in infancy. The Baltimore-Washington Infant Study (BWIS) enrolled 2659 infants with heart disease and 2801 control infants between January 1, 1981 and March 31, 1987, a 6-year prevalence of 4.46/1000 live births. Fifty-six infants had cardiomyopathy, in the absence of a structural defect (prevalence 1/10,000). The cases were classified clinicopathologically as follows: dilated Cm (n = 17), hypertrophic Cm (n = 26), tumor (n = 5), endocardial fibroelastosis (n = 5), glycogen storage (n = 1), mucocutaneous lymph node syndrome (n = 1), and infarction (n = 1). Eleven syndromic associations and six metabolic disturbances indicate genetic risk factors. Some of the same syndromes occurred in other infants who had structural cardiac abnormalities. This overlap suggests that embryonic myocardial disease might sometimes be responsible for altered cardiac structures, possibly secondary to hemodynamic changes. Familial myocardial disease occurred in two infants with hypertrophic Cm. The Cm group did not differ by race and sex from controls, but the mothers were of lower educational and occupational status with less private care and with later registration for pregnancy care. The descriptive epidemiology of this population-based case group provides evidence of greater etiologic heterogeneity than has been shown in clinical reports.

Cardiomyopathies↗
PubMed1991

White-black differences in cardiovascular malformations in infancy and socioeconomic factors. The Baltimore-Washington Infant Study Group.

Cardiovascular malformations were examined for white/black variation in the Baltimore-Washington Infant Study. In this population-based case-control study, cases (n = 2,087) were live births with cardiovascular malformations ascertained through pediatric cardiology centers and 53 hospitals in Maryland, the District of Columbia, and northern Virginia between 1981 and 1987. Controls (n = 2,721) were a random sample of infants from the live-birth cohort that gave rise to the cases. The proportion of infants that were white was similar for all cases as a group and controls (0.68 and 0.67, respectively). Subgroup analysis, however, revealed an excess of white infants among cases with Ebstein's anomaly (odds ratio (OR) = 3.7, 95% confidence interval (Cl) 1.1-12.5), aortic stenosis (OR = 3.6, 95% Cl 1.7-7.6), pulmonary atresia (OR = 2.5, 95% Cl 1.0-6.1), coarctation of the aorta (OR = 2.2, 95% Cl 1.4-3.5), and D-transposition of the great arteries (OR = 1.6, 95% Cl 1.1-2.5), and a deficit of white infants among cases with pulmonary stenosis (OR = 0.6, 95% Cl 0.4-0.8) and heterotaxia (OR = 0.4, 95% Cl 0.3-0.8). These associations remained when cases were stratified by infant's age or by method of diagnosis. Controlling for socioeconomic factors attenuated the white excess for Ebstein's anomaly (OR = 3.0, 95% Cl 0.9-10.5), disclosed a white excess among cases of L-transposition of the great arteries (OR = 2.8, 95% Cl 1.0-8.0), and revealed that the white excess for aortic stenosis was limited to low and middle socioeconomic strata. These results highlight racial variations in cardiovascular malformations, suggest that socioeconomic factors account for some of this variation, and identify malformation subgroups for which further evaluation of sociocultural, environmental, and familial factors is needed.

Black or African American↗
PubMed1991

Birth weight and cardiovascular malformations: a population-based study. The Baltimore-Washington Infant Study.

Mean birth weights were evaluated in infants with D-transposition of the great arteries, tetralogy of Fallot, endocardial cushion defect, hypoplastic left heart syndrome, pulmonary stenosis, aortic stenosis, coarctation of the aorta, ventricular septal defect, and atrial septal defect in a population-based case-control study of congenital cardiovascular malformations in residents of Maryland, Washington, D.C., and northern Virginia (1981-1987). Study subjects were liveborn singletons without extracardiac anomalies. After adjustment for potentially confounding maternal, gestational, and infant factors, significant birth weight deficits were found for infants with tetralogy of Fallot, endocardial cushion defect, hypoplastic left heart syndrome, pulmonary stenosis, coarctation of the aorta, ventricular septal defect, and atrial septal defect. After adjustment, infants with these malformations (except coarctation of the aorta and atrial septal defect) were also significantly more likely than were controls to have low birth weight for gestational age. These findings strengthen previous evidence that certain cardiovascular malformations and low birth weight may be causally related.

Case-Control Studies↗
PubMed1991

Total anomalous pulmonary venous return: familial and environmental factors. The Baltimore-Washington Infant Study Group.

In total anomalous pulmonary venous return (TAPVR), the intrapulmonary venous plexus has failed to connect to the left atrium, so that the pulmonary veins drain into right atrial tributaries, frequently resulting in early postnatal circulatory distress. The Baltimore-Washington Infant Study (BWIS), a population-based exploratory case-control study of cardiovascular malformations (CVM), identified 41 cases of TAPVR during 1981-1987: 1.5% of all CVM (N = 2659), a regional prevalence of 6.8/100,000 live births. Of the TAPVR infants, 68% were diagnosed as neonates, 88% had surgery, and 51.2% were alive at 1 year of age. Noncardiac malformations were present in nine cases (22%); the male-female ratio was 0.78 (18.23). Compared with a control group representative of the birth cohort (N = 2,801), more TAPVR patients had low birthweight (less than 2,500 g: 16.2% vs. 6.9%, short gestational age (less than 38 weeks: 18.9% vs. 9.3%), and intrauterine growth retardation (IUGR) (26.8% vs. 5.8%). Sociodemographic findings were similar to those of controls, except that fewer TAPVR mothers received private pregnancy care (59.5% vs. 71.4%). Family history revealed no other TAPVR-affected members, but a significant linear trend of increased risk was found over the ordered malformation categories (familial noncardiac, cardiac, both). Bivariate analysis of TAPVR and exposure in life-style, hobbies, and work showed possible associations for exposure to lead (OR 2.9; 99% confidence interval [CI]: 1.2, 7.2), painting/paint stripping (OR 3.3; 99% CI: 1.3, 8.4), lead soldering (OR 13.3; 99% CI: 1.8, 99.2), and pesticides (OR 2.7; 99% CI: 1.2, 6.4). Multivariate analysis suggested an interaction between pesticide exposure and family history and, thus, a possible familial susceptibility to environmental teratogens. Although the number of TAPVR cases is small, this epidemiologic study identifies hypotheses that may be further explored in morphogenetic and epidemiology studies. Total anomalous pulmonary venous return (TAPVR) constitutes a well-defined clinical entity in which the pulmonary veins fail to enter the left atrium and instead drain into the right atrium or its systemic venous tributaries (Neill, '56; Rowe et al., '81). During intrauterine life, the malformation does not compromise the fetal circulation, since the pulmonary arterial resistance is high and the patent foramen ovale provides easy access of right atrial blood to the left side of the heart. At birth, however, the pulmonary vascular resistance begins to fall, and the presence of a severe hemodynamic disturbance becomes increasingly evident (Ferencz et al., '71).(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed1990

Maternal diabetes and cardiovascular malformations: predominance of double outlet right ventricle and truncus arteriosus.

Most studies on the relationship of maternal diabetes to cardiovascular malformations (CVM) have been prospective investigations of pregnancy outcome and therefore could not identify associations with rare cardiac lesions. The results of a retrospective study shed new light on the risks of specific cardiac defects in diabetic pregnancies. The Baltimore-Washington Infant Study, a population-based case-control investigation of CVM, provides information on maternal diabetes reported in personal interviews. Among 2259 mothers of cases, 35 (1.5%) reported diabetes present before pregnancy (called "overt") and 95 (4.2%) reported diabetes only during pregnancy (called "gestational"). Among 2,801 mothers of controls, 14 (0.5%) had overt diabetes and 83 (3.0%) had gestational diabetes. Malformation-specific risks were expressed as odds ratios (OR) with 99.5% confidence intervals (CI). The strongest associations with overt maternal diabetes were found with double outlet right ventricle (OR 21.33; 99.5% CI 3.34, 136.26), and truncus arteriosus (OR 12.81; 99.5% CI 1.43, 114.64). No significant diagnosis-specific associations were found with gestational diabetes. Non-cardiac malformations were present in 23% of infants with CVM whose mothers had overt diabetes and in 26% of infants with CVM whose mother had gestational diabetes, in 32% of infants with CVM whose mothers did not have diabetes, and in 4% of controls. Double outlet right ventricle and truncus arteriosus are malformations dependent upon neural-crest-cell-derived ectomesenchymal tissues; these are precisely the conotruncal abnormalities that result from experimental ablation of the neural crest in chick embryos. The association with diabetes suggests a further etiologic link between these two lesions.

Case-Control Studies↗
PubMed1989

Congenital cardiovascular malformations in twins and triplets from a population-based study.

Data from the Baltimore-Washington Infant Study of congenital cardiovascular malformations permitted detailed analysis of congenital cardiovascular malformations in 62 twins and 3 triplets and 2303 singleton cases. A probability sample of controls (n = 2793) included 43 twins. The case prevalence of multiple births was 28 of 1000, compared with a 15 of 1000 prevalence among controls (chi 2 = 5.7). There were more girls among case twins than among case singletons and controls (chi 2 = 9.0). Monozygosity was no more frequent in case twins than in controls. Looping defects occurred in 4 monozygotic twin pairs compared with only 1 dizygotic twin pair. The twinning process itself may be implicated in the development of congenital cardiovascular malformations in some of these infants, especially those with looping defects, but concordance of types of defects in 4 of 65 pairs implicates genetic factors in the determination of some forms of congenital cardiovascular malformations.

Diseases in Twins↗
PubMed1989

Congenital cardiovascular malformations: questions on inheritance. Baltimore-Washington Infant Study Group.

The Baltimore-Washington Infant Study, an epidemiologic investigation of congenital heart disease, searches for genetic and environmental risk factors. Among 2,102 infants with heart disease, 17.5% had a noncardiac abnormality of chromosomal or genetic origin, whereas among 2,328 control infants, only 0.7% had a genetic abnormality. Familial cardiovascular malformations encountered can be grouped into five distinct etiologic mechanisms. Single gene effects may be responsible for the specific histologic and biochemical changes in familial atrial septal defect with conduction disturbance and also in idiopathic ventricular hypertrophy. Left heart lesions showed familial concordance by the presumed morphogenetic mechanism of abnormal embryonic blood flow with phenotypes of varying severity. Pulmonary stenosis appeared with familial heritable disorders, as well as a partially concordant lesion with tetralogy of Fallot. Ventricular septal defect with transposition of the great arteries (one sibling pair) and with truncus arteriosus (two sibling pairs) indicate forme fruste expression of conotruncal defects. Endocardial cushion defect occurred with and without Down's syndrome in members of three families, suggesting inheritance of a defect affecting cellular migration. Heritable blood coagulopathies occurred in case families and not in control families. The associated of hemophilia and transposition, observed also by others, is extremely unlikely by chance and suggests genetic errors of endothelial cell function. The description of specific families from a population-based study emphasizes biologic questions on the nature of the inheritance of cardiovascular malformations.

Epidemiologic Methods↗
PubMed1989

Congenital cardiovascular malformations associated with chromosome abnormalities: an epidemiologic study.

The Baltimore-Washington Infant Study is a population-based case-control study that seeks to identify risk factors for cardiovascular malformations. Between 1981 and 1986, a total of 2102 infants with cardiovascular malformations were ascertained, among whom 271 (12.9%) also had a chromosome abnormality. Among 2328 random control subjects, only two had a chromosome abnormality. Down syndrome with cardiovascular malformations had a maternal age-adjusted regional prevalence of 4.33/10,000 for the white population and 3.70/10,000 for the nonwhite population. Endocardial cushion defect, the predominant cardiac abnormality in Down syndrome (60.1%), rarely occurred as an isolated cardiac lesion (2.8%). The absence of transpositions and the rarity of heterotaxias and of right- and left-sided obstructive lesions in trisomies indicate that there may be a genetic influence on specific embryologic mechanisms. Alimentary tract lesions were more common in Down syndrome than among euploid patients with heart disease and more severe than in control subjects. Urinary tract lesions also occurred in excess of the rate in control subjects. The coexistence of these major malformations with heart disease raises the possibility of incomplete expression of the VA(C)TER (vertebral, anal, cardiac, tracheal, esophageal renal) association. The selective association of chromosome abnormalities with certain cardiovascular defects is now beginning to be explained by reported embryologic studies on cellular characteristics. An explanation of the negative association with transposition and obstructive lesions requires further multidisciplinary studies on genetic and epigenetic factors.

Chromosome Aberrations↗
PubMed1989

Increased prevalence of ventricular septal defect: epidemic or improved diagnosis.

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P less than .025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P less than .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P less than .001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P less than .001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."

Cardiac Catheterization↗
PubMed1988

Assessing familial aggregation of congenital cardiovascular malformations in case-control studies.

Recent data indicate that the familial aggregation of congenital cardiovascular malformations (CCVM) varies with the type of defect in the index case. Using a logistic regression model that allows for dependence among family members, we calculated the risk of any CCVM to case relatives compared with relatives of controls. Data from 3,908 first-degree relatives of 570 matched cases and controls identified from 1981 through 1985 by the Baltimore-Washington Infant Study were used in the analyses. Overall risk for any CCVM in case relatives was increased four-fold over that of control relatives. While relatives of cases with flow lesions (including right and left heart defects, as well as perimembranous ventricular septal defect [VSD]) had a five-fold increase in risk, the risk to relatives of nonflow lesion cases did not differ significantly from the risk to relatives of controls. Sex, maternal age, miscarriage history in the mother, and birth order had no apparent effect on risk among siblings. However, there was an indication of increased risk in relatives of nonwhite cases with VSD compared to relatives of matched controls. However, with these data it was not possible to distinguish between environmental and genetic sources of this familial aggregation.

Black People↗
PubMed1988

Twenty-four-hour ambulatory blood pressure in normotensive adolescent children of hypertensive and normotensive parents.

Twenty-four-hour ambulatory blood pressure measurements were obtained during usual daily activities from two groups of normotensive adolescents: Cases had at least one hypertensive parent, and controls had both parents normotensive. Automatic measurements were programmed at 7.5-minute intervals. For each subject, within each of three time periods, the data were summarized by descriptors of the frequency distribution and the frequency spectrum. The time periods were sleep, school hours, and other waking hours. Regression analyses examined the relation between the blood pressure monitoring descriptors and case-control status adjusted for non-blood-pressure variables found to relate to case-control status. In stepwise logistic regression analysis with case-control status as the dependent variable, it was found that the mean level of diastolic pressure during school hours was significant, whereas the standard resting measurement was not. In linear regression analyses with blood pressure monitoring descriptors and standard resting measurements as dependent variables, several monitoring descriptors were found to be significantly related to the interaction between case-control status and certain variables that can affect blood pressure, such as weight and the presumed stress of school; no such relations were found for the standard resting measurements. These findings demonstrate the superiority of the ambulatory monitoring variables over the standard resting measurements in studying case-control differences.

Adolescent↗