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Query: UMLS:C0917816 (mental retardation)
 15,867 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This review of the organic aspects of mental retardation considers this handicap among the other chronic neuropediatric disorders and is one of the numerous possible manifestations of a cerebral damage occurring in a developing nervous system. The relative frequency of the different etiologies is discussed in relation to the severity of the mental handicap. The prenatal causes of mental deficiency which are the most frequent are reviewed and a distinction is made between malformations and pseudomalformations. Particular mention of the fetal alcohol syndrome is made. The role of perinatal medicine in the epidemiology of mental retardation is discussed and the diagnostic approach of a particular child with mental retardation is presented with the personal experience of the author in the pediatric department, CHUV, Lausanne. The possible measures to prevent mental retardation are finally discussed.
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PMID:[Mental deficiency: organic aspects]. 48 89

Two unrelated children with the fetal alcohol syndrome are described. Features of the syndrome include mental deficiency, growth delay, and craniofacial, limb and cardiac anomalies. The finding of this spectrum of abnormalities raises the possibility of alcohol ingestion during pregnancy. The deleterious effects of alcohol on the developing fetus are emphasized. In view of the high prevalence of alcoholism in this country the fetal syndrome must be regarded as a serious and preventable cause of morbidity.
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PMID:The fetal alcohol syndrome. 56 11

Four cases of the fetal alcohol syndrome are described, and the clinical features are reviewed. The most consistently found features are pre- and postnatal growth retardation, small palpebral fissures and mental deficiency. In view of this, chronic alcoholic women shold avoid pregnancy until they have weaned off alcohol, but should it occur then the question of termination must be considered.
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PMID:The fetal alcohol syndrome. 56 12

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. Futhermore, 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.
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PMID:Brain malformations related to prenatal exposure to ethanol. 61 80

In a sample of 20 patients with the fetal alcohol syndrome, ages 9 months to 21 years, the average IQ was 65, with a range of 16 to 105; 60% of the patients had IQ's more than two standard deviations below the mean. The sample included patients who ranged in severity of dysmorphogenesis from mild to severe. The severity of the dysmorphic features was related to degree of mental deficiency; children with the most severe manifestations of FAS had an average IQ of 55, whereas children with lesser manifestations had an average IQ of 82. All children were growth deficient for height and/or head circumference, but in this study only height was significantly correlated with IQ. Environmental and behavioral characteristics of the sample are discussed, and two adults with the fetal alcohol syndrome are presented.
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PMID:Intelligence, behavior, and dysmorphogenesis in the fetal alcohol syndrome: a report on 20 patients. 63 74

Fetal alcohol syndrome is the leading known cause of mental retardation. The syndrome, defined as growth retardation, midface hypoplasia, and neurologic dysfunction, represents only part of the spectrum of fetal alcohol effects. The biochemical mechanism of teratogenesis is unknown. In adults, metabolites of ethanol, FAEE, are known to accumulate in major organs. The formation of FAEE is catalyzed by a family of enzymes, FAEE synthases. Our hypothesis is that accumulation of FAEE in the embryo results in fetal alcohol syndrome. We have developed assays for FAEE and FAEE synthase activity using mg of tissue. Using these assays, we have shown the following: Human placenta, mouse placenta, heart, and liver are active in catalyzing the formation of FAEE. One h after maternal ethanol administration on gestational d 14, mouse placenta and fetuses accumulated significant quantities of FAEE. The fatty acid incorporated into FAEE was tissue dependent. Tissues from pregnant animals given ethanol on gestational d 7 showed persistence of FAEE on gestational d 14. We conclude that: 1) human and mouse placentas have significant FAEE synthase activity, 2) mouse heart, liver, placenta, and fetal tissues accumulate significant amounts of FAEE after maternal ethanol exposure, 3) there is tissue specificity for the fatty acid incorporated into FAEE, and 4) FAEE may persist for 7 d in placentas. These results provide a basis for further research into the role of FAEE in the development of fetal alcohol syndrome.
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PMID:Fetal alcohol syndrome and fatty acid ethyl esters. 160 26

Thirty years after recognition of the fetal alcohol syndrome, a study of 105 affected individuals who reached adulthood provides insight into the long-term outcome of this condition. The typical facial dysmorphism undergoes odd changes, often with a long face and a bulky nose and chin, in contrast to the appearance of affected infants. The growth failure becomes slightly less marked. The malformations, which occur in 3/4 of severe forms, increase the disability although function is sometimes better than expected. In contrast, the microcephaly becomes more pronounced. This explains the psychic disorders which are the main prognostic factor: mental retardation in the most severely affected patients or severe learning disabilities and, in every case, behavior disorders and marked instability. Similar disorders are often found in siblings with no apparent dysmorphism underlining the extent of the problem and the urgent need for prophylaxis. Some of the observations made in adults, confronted to embryologic data, lead to useful inferences with respect to prophylaxis.
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PMID:[Outcome of children of alcoholic mothers (study of 105 cases followed to adult age) and various prophylactic findings]. 161 36

A syndrome involving facial abnormalities (telecanthus, epicanthus, broad flattened nose, large inverted W-shaped mouth and malformed ears), malformed extremities (camptodactyly, clinodactyly, interdigital webbing and joint hyperlaxity) and mental retardation is described in a girl at birth and at 11 years old. A comparison with Pashayan-Pruzansky syndrome, fetal alcohol syndrome, VATER association, Marden-Walker syndrome and Tel-Hashomer syndrome is discussed. We suggest this patient represents a new malformation syndrome or an extreme phenotypic variant of one of the above-mentioned syndromes.
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PMID:Mental retardation with blepharo-naso-facial abnormalities and hand malformations: a new syndrome? 163 41

Among children referred to our genetic clinics for mental or growth retardation we identified 8 of their mothers with the fetal alcohol syndrome. This was complete in 5 and partial in 3. All of their alcoholic mothers had died. Most of the patients were unwed mothers with mental retardation and no elementary education. One of them was also alcoholic and her third offspring had the syndrome. The etiology of this syndrome and possible preventive measures are discussed.
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PMID:[Progeny in women with fetal alcohol syndrome]. 172 14

A revised estimate of the national economic impact of fetal alcohol syndrome (FAS)-related abnormalities was conducted. The present evaluation included "corrections" for background rates of low birth weight, and costs normally incurred for housing and food regardless of whether an individual requires institutionalization or not. Additional anomalies were included along with hospital costs provided for in diagnostic-related groups. The current estimate for annual costs related to FAS is $249.7 million, which is about $80 million less than our previous estimate. This estimate is lower due to a slightly lower number of cases with low birth weight, a correction for costs that would otherwise have occurred, and exclusion of costs (about $75 million) for annual semi-independent support for individuals with IQs in the 70-85 range. Nonetheless, mental retardation accounts for almost 60% of the estimated total cost. A quarter of a billion dollars per year remains a high economic incremental cost by any reasonable standard and represents a benchmark against which costs of potential prevention strategies may be judged.
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PMID:A revised estimate of the economic impact of fetal alcohol syndrome. 175 79


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