AACP Newsletter, Volume 12,
Number 4, Autumn 1998
Research into Fetal Alcohol Syndrome
with Ann P. Streissguth, Ph.D and Sterling K. Clarren, MD as authors
Clarren SK. Alvord EC Jr, Sumi SM. Streissguth AP, Smith DW
Brain malformations related to prenatal exposure to ethanol
Journal of Pediatrics. 92(1):64-7, 1978 Jan.
Microcephaly and mental retardation have been principal features of the fetal alcohol syndrome. This article describes the neuropathologic findings in four human neonates who were exposed to large quantities of ethanol at frequent intervals during gestation. The findings suggest that intrauterine exposure to ethanol can result in structural abnormalities of the brain. All four brains displayed similar malformations stemming from errors in migration of neuronal and glial elements. Hydrocephalus was one consequence of the malformations in two of the infants. Furthermore, the brain alterations may be the only distinct abnormality produced by in utero ethanol exposure. Only two of the four subjects were diagnosed as having the fetal alcohol syndrome from external criteria.
Streissguth AP, Clarren SK, Jones KL
Natural history of the fetal alcohol syndrome: a 10-year follow-up of eleven patients
Lancet. 2(8446):85-91, 1985 Jul 13.
Of the eleven children who were the first to be diagnosed as having the fetal alcohol syndrome ten years ago, two are now dead, one is lost to follow-up, and the remaining eight continue to be growth deficient and dysmorphic. With menarche, which occurred with normal timing, the female patients developed increased body fat. The mothers were all severe chronic alcoholics. Four of the eight known survivors are of borderline intelligence and have needed some remedial teaching. The other four are severely handicapped intellectually and need complete supervision outside the home. The degree of growth deficiency and intellectual handicap was directly related to the extent of craniofacial abnormalities. New features of the syndrome include dental malalignments, malocclusions, and eustachian tube dysfunction, which may relate embryologically to midface hypoplasia.
Little RE, Streissguth AP, Guzinski GM, Uhl CN, Paulozzi L, Mann SL, Young A. Clarren SK, Grathwohl HL
An evaluation of the pregnancy and health program
Alcohol Health & Research World 10(1):44-53, 71, 75, 1985 Fall.
No Abstract.
Streissguth AP, Sampson PD, Barr HM, Clarren SK, Martin DC.
Studying alcohol teratogenesis from the perspective of the fetal alcohol syndrome: methodological and statistical issues
Annals of the New York Academy of Sciences. 477:63-86, 1986.
Alcohol is a teratogenic drug and the effects appear to be grossly dose related. The severest effects are observable clinically as the fetal alcohol syndrome and are associated with heavy prenatal alcohol exposure and a history of chronic maternal abuse of alcohol. Hypotheses for subtler behavioral effects associated with lower levels of exposure can be generated from observation of the behavioral effects in FAS. Behavioral effects associated with various levels of prenatal alcohol exposure in humans include poor sucking and poor habituation in the newborn, poorer mental and motor development in infancy, and attentional and reaction time effects at four and seven years of age. Human behavioral teratology studies are necessarily complex due to the large number of covariates that affect behavior, modify the effects of teratogens, and influence interpretation. Challenging problems exist in assessing exposure, outcomes, and covariates. Common to assessment of all these classes of variables are the multiplicity of measurement and the indirect nature of the measurement. Answering specific questions about timing and dose effects demands careful statistical modeling procedures and large, complex databases. Large databases and indirect measurement problems suggest factor analytic extensions of current regression methodology, which we have proposed in this paper.
Clarren SK, Sampson PD, Larsen J, Donnell DJ, Barr HM, Bookstein FL, Martin DC, Streissguth AP
Facial effects of fetal alcohol exposure: assessment by photographs and morphometric analysis
American Journal of Medical Genetics. 26(3):651-66, 1987 Mar.
This study was designed to assess the limits of alcohol-related facial dysmorphogenesis. Standard full face and lateral facial photographs were obtained on 21 7-year-old children who had been exposed gestationally to known, heavy quantities of ethanol. Only two of these children had been previously considered to have definite fetal alcohol syndrome (FAS). Similar photographs of 21 other 7-year-old children with negligible gestational ethanol exposure were obtained for control purposes. Copies of the 42 photographs were given to each of seven expert clinicians who were asked to select any child with an FAS-related facial appearance. Six of seven judges were accurate in identifying children with high levels of alcohol exposure as having a fetal alcohol-affected face. A set of homologous points on the photographs were then digitized and analyzed by newly developed morphometric methods to determine the facial shape characteristics that distinguish the selected photographs of highly exposed children. The analysis confirmed that the judges specifically identified children with facial changes consistent with those previously published as defining the face of the FAS: short palpebral fissures, a relatively long and flat midface, and a retrusive mandible. This methodology may be useful in more accurately delineating the facial phenotype in other conditions diagnosed primarily on the basis of subjective clinical criteria.
Streissguth AP, Aase JM, Clarren SK, Randels SP, LaDue RA, Smith DF
Fetal alcohol syndrome in adolescents and adults
JAMA 265(15):1961-7, 1991 Apr 17
Department of Psychiatry, Child Development/Mental Retardation Center, Seattle, WA.
Comment in: JAMA, 1991 Aug 28;266(8):1077
Fetal alcohol syndrome is a specific recognizable pattern of malformation. Manifestations in 61 adolescents and adults suffering from alcohol teratogenesis are presented. After puberty, the faces of patients with fetal alcohol syndrome or fetal alcohol effects were not as distinctive. Patients tended to remain short and microcephalic, although their weight was somewhat closer to the mean. The average IQ was 68, but the range of IQ scores widely varied. Average academic functioning was at the second- to fourth-grade levels, with arithmetic deficits most characteristic. Maladaptive behaviors such as poor judgment, distractibility, and difficulty perceiving social cues were common. Family environments were remarkably unstable. Fetal alcohol syndrome is not just a childhood disorder; there is a predictable long-term progression of the disorder into adulthood, in which maladaptive behaviors present the greatest challenge to management.
Sampson PD, Streissguth AP, Bookstein FL, Little RE, Clarren SK, Dehaene P, Hanson JW,
Graham JM Jr.
Incidence of fetal alcohol syndrome and prevalence of alcohol-related neurodevelopmental disorder [Review] [59 refs]
Teratology. 56(5):317-26, 1997 Nov.
Department of Statistics, University of Washington, Seattle 98195, USA.
Comment in: Teratology 1999 Aug;60(2):51-2
We critique published incidences for fetal alcohol syndrome (FAS) and present new estimates of the incidence of FAS and the prevalence of alcohol-related neurodevelopmental disorder (ARND). We first review criteria necessary for valid estimation of FAS incidence. Estimates for three population-based studies that best meet these criteria are reported with adjustment for underascertainment of highly exposed cases. As a result, in 1975 in Seattle, the incidence of FAS can be estimated as at least 2.8/1000 live births, and for 1979-81 in Cleveland, approximately 4.6/1,000. In Roubaix, France (for data covering periods from 1977-1990), the rate is between 1.3 and 4.8/1,000, depending on the severity of effects used as diagnostic criteria. Utilizing the longitudinal neurobehavioral database of the Seattle study, we propose an operationalization of the Institute of Medicine's recent definition of ARND and estimate its prevalence in Seattle for the period 1975-1981. The combined rate of FAS and ARND is thus estimated to be at least 9.1/1,000. This conservative rate--nearly one in every 100 live births--confirms the perception of many health professionals that fetal alcohol exposure is a serious problem. [References: 59]
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