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Objective: Bronchopulmonary dysplasia (BPD) is now the leading cause of lung disease in US infants. In a large regional cohort, we tested the hypothesis that despite innovations in neonatal care, very low birth weight (VLBW) infants (<1500 g) with BPD had poorer developmental outcomes than nonaffected infants during the first 3 years of life, and that BPD predicted poorer outcome beyond the effects of other risk factors., Methods: Three groups of infants (122 with BPD, 84 VLBW without BPD, and 123 full-term) were followed longitudinally to 3 years of age with the Bayley Scales of Mental and Motor Development. Comparison groups of VLBW infants without BPD and full-term infants did not differ in sex, race, or socioeconomic status. Statistical analyses included hierarchical and stepwise multiple regression., Results: Infants with BPD performed more poorly at all ages. By 3 years, cognitive and/or motor development was in the range of retardation (<70 standard score) for 21% to 22% of infants with BPD. In multiple regression analyses controlling for socioeconomic and neonatal risk conditions, BPD had an independent negative effect on motor outcome at 3 years. Neurologic risk, a summary measure of neurologic problems other than intraventricular hemorrhage, and the presence of BPD independently predicted motor delay. By 3 years, social class, race, and neurologic risk predicted mental outcome, suggesting that the specific effects of BPD are primarily on the motor domain., Conclusions: In VLBW infants, BPD predicts poorer motor outcome at 3 years, after control for other risks. Cohorts of infants with BPD also had higher rates of mental retardation, associated with greater neurologic and social risk. These findings underscore the need for intensive prevention and habilitation efforts for this growing group of VLBW survivors, as well as investigation into the potential role of BPD in the higher rates of learning disabilities in VLBW cohorts at school age.

Depression among 148 Latina women who have children with mental retardation was examined. Results showed that their depressive symptomatology was elevated, with almost half reporting negative experiences in excess of a commonly used cut-off for the Center for Epidemiologic Studies Scale (CES-D). Depression scores related to variables pertaining to the child, mother's health and level of acculturation, and aspects of stress and coping. When mothers were categorized in three groups by CES-D scores, discriminant analysis correctly classified 84% of the low and high group mothers. High CES-D membership was predicted by mothers' reporting more family problems, worse health, fewer interactions with English-speaking persons in their daily lives, and more negative feelings about parenting their child with mental retardation.

The present article presents how the bio-ecological assessment system is applied in assessing a child's cognitive intelligence beyond a psychometric intelligence test. The recognition that there are many types of intelligence, such as musical and bodily kinesthetic, is exemplified. The case presented is that of a child with deficient cognitive functioning on the psychometric tests, but advanced functioning on the "Other Intelligence Assessment Measure." Diagnostic impressions are given and implications for report writing and interventions are suggested.

This is an ethnographic study that examines, from the relatives' perspective, the attributes of nurses that are considered important in the caring process of mentally handicapped people in an institutional setting in Hong Kong. Twelve interviews were carried out with a family member who had a relative living in a special unit for severely mentally handicapped people. Empirical studies, mainly from the west, have shown that decisions to place a mentally handicapped family member in residential care is a complex process. Family characteristics, the relatives' characteristics as well as the availability of support, have been found to influence decisions about out-of-home placement. Once this decision has been made, a new caring relationship between nurse, family and relative needs to be established. The final categories that emerged from the data show that families have expectations about the nurses' competence and commitment to the job and about their relationships with the client and the family. Consequently quality is judged, not only by the way in which nurses work with the clients, but also by the way in which they work with the family. Implications for practice and education of nurses working with mentally handicapped people and for nursing administration in Hong Kong are discussed.

To assess the factor validity and reliability for the Japanese version of the Aberrant Behavior Checklist-Community (ABC-Community), 322 subjects with moderate to profound mental retardation (188 males and 134 females, mean age 29.79 +/- 12.45) were rated by the staff at the residential facilities in Wakayama Prefecture, Japan. Rating data were analyzed by factor analysis using the principal factoring method with iteration, followed by oblique rotation. The internal consistency was estimated by Cronbach's alpha. Of these subjects, 43 were rated twice with an interval of 4 weeks to confirm the test-retest reliability and 33 were rated by pairs of raters for interrater reliability. Forty-eight of 58 items loaded most heavily on the same factors as in the original factor solution. Coefficient alpha raged from .85 to .95 across five subscales. The test-retest reliability was high with Spearman's rank correlation coefficient ranging from .84 to .90. Although the correlation coefficients for interrater reliability tests were somewhat lower (.58 to .78), they were all statistically significant (p < .001). The Japanese version of ABC Community was comparative to the original and was useful for assessing behavior problems in Japanese persons with mental retardation.

The Chinese version of the WAIS-R was factor analyzed for a sample of 130 Chinese adults in Hong Kong who had low intellectual abilities. All subtests except the Vocabulary subtest were included for analyses. Results supported a three-factor solution composed of Verbal Comprehension, Perceptual Organization, and Memory/Freedom from Distractibility Factors, as well as a two-factor solution of classic Verbal-Perceptual dichotomy. Comparison of present two- and three-factor structure with individuals having low or normal IQ in Mainland China and North America revealed satisfactory congruence coefficients. However, our general factor accounted for only a small portion of common and total variance (28.5% and 35.3% respectively). Error variances of our subtests were large when compared to normative samples of Mainland China and US. Results were discussed in terms of clinical interpretation of the WAIS-R subtests, danger of using short-forms to assess IQ and need for normative studies of WAIS-R in Chinese-speaking countries.

Objectives: In this study, data from the Metropolitan Atlanta Developmental Disabilities Study were used to determine the administrative prevalence (i.e., the number of children previously identified for service provision) of mental retardation among 10-year-old children during the years 1985 through 1987., Methods: Children with mental retardation (intelligence quotient [IQ] of 70 or lower) were identified by review of records from multiple sources, with the public schools as the primary source., Results: The overall administrative prevalence of mental retardation was 12.0 per 1000 children. The rate for mild mental retardation (IQ of 50 to 70) was 8.4 per 1000 and the rate for severe mental retardation (IQ lower than 50) was 3.6 per 1000. The prevalence was higher in Black children than in White children (prevalence odds ratio [POR] = 2.7) and in boys than in girls (POR = 1.4). Children with severe mental retardation had more coexisting disabilities than did children with mild mental retardation., Conclusions: The mental retardation prevalence rates reported here, especially the race-specific rates, may reflect social and demographic features unique to the metropolitan Atlanta area and therefore should be used with caution in making comparisons with other populations.

Objectives: This study assessed differences in the prevalence of mild mental retardation, defined as an intelligence quotient (IQ) from 50 to 70, between Black and White children., Methods: A case-control study design was used. Ten-year-old children with mental retardation were identified from multiple sources. Information on race, sex, maternal age, birth order, economic status, and maternal education was abstracted from birth certificates of 330 case children and 563 control children (public school students)., Results: The crude Black-White odds ratio (OR) was 2.6, but it was reduced to 1.8 after the other five covariates were controlled. The disparity was largest among children whose mental retardation was first diagnosed when they were 8 to 10 years old (adjusted OR = 2.5). We found no significant difference in the occurrence of mild mental retardation between Black and White children diagnosed before the age of 6 years (adjusted OR = 1.2). Black children had a higher prevalence of mild mental retardation within all strata of the other five covariates., Conclusions: Five sociodemographic factors accounted for approximately half of the excess prevalence of mild mental retardation among Black children. Possible reasons for the residual difference are discussed.

We have investigated, in the genomic DNA of ten Tunisian patients, the presence of a splice junction mutation at the 5' end of intron 2 in the carbonic anhydrase II gene (CAII) previously described in six CAII-deficient patients presumed to be of Arab origin. All our patients were homozygous for this mutation and were mentally retarded, a characteristic feature of the phenotype of patients with an Arabic background. This mutation is found exclusively in patients with an Arabic background and thus may be confined to this ethnic group.

Adaptation of Hispanic (n = 51) and non-Hispanic (n = 195) white families to having a member with mental retardation was examined using surveys and interviews. We compared these families' demographic characteristics, religious values, and support resources and examined how these factors differentially affected caregiving burden for each group. Surveys and interviews were collected from 51 Hispanic and 195 non-Hispanic white families. Findings indicated that Hispanic families differed from the other families in SES, age of the person with mental retardation, religious values, and in their perceived burden of caring for a family member with mental retardation. Patterns of determinants of caregiving burden did not differ significantly for the two groups.

Although developmental disabilities are among the major chronic health problems affecting children in the United States, the contribution of developmental disabilities to childhood mortality is unknown. To investigate the magnitude of this contribution, multiple cause-of-death data were examined for US children, aged 1-19 years, for 1980 and 1983-1989. The following conditions were included as developmental disabilities: autism, attention deficit disorder, learning disorders, mental retardation, cerebral palsy, epilepsy, muscular dystrophy, blindness and deafness. Based on underlying cause only, it was found that developmental disabilities were the fifth leading cause of nontraumatic death for children between 1 and 14 years of age and the third leading cause of non-traumatic death for children between 15 and 19 years. When a multiple cause approach was used to define developmental disability-related deaths (i.e. when contributing as well as underlying cause was considered), the number of such deaths nearly doubled. On the basis of both underlying- and multiple-cause analyses, cerebral palsy was the developmental disability most frequently cited as a cause of death. Mental retardation ranked second according to the multiple-cause approach but only fourth according to the underlying-cause approach. The least frequent causes of death (autism, attention deficit disorder, learning disorders, blindness, and deafness) were the ones most likely to be coded as contributing rather than underlying causes. Developmental disability-related mortality rates were highest among children aged 1-4 and 15-19 years, highest among blacks and lowest among racial groups other than blacks and whites, and higher among males than females. Although results of multiple-cause-of-death analyses more accurately reflect the proportion of deaths related to developmental disabilities, even this approach may underestimate the degree to which mortality is associated with a developmental disability.

The Kabuki (Niikawa-Kuroki) syndrome was reported in 1981 by Niikawa et al. and Kuroki et al. in a total of ten unrelated Japanese children with a characteristic array of multiple congenital anomalies and mental retardation. The syndrome is characterized by a distinct face, mild to moderate mental retardation, postnatal growth retardation, dermatoglyphic and skeletal abnormalities. In Japan, the syndrome appears to have an incidence of about 1:32,000 newborns. Outside of Japan, a growing number of patients have been recognized. Clinical data are presented on 29 Caucasian patients; the patients were diagnosed over a relatively short period of time, indicating that the incidence outside of Japan is probably not lower than in Japan. A literature review of 89 patients (60 Japanese and 29 non-Japanese) is given. In 66% of the non-Japanese patients serious neurological problems were present, most notably hypotonia and feeding problems (which were not only related to the cleft palate); this was not reported in the Japanese patients. Inheritance is not clear. Most patients are isolated, sex-ratio is equal. The syndrome can be recognized in patients with cleft (lip/)palate, with mild to moderate developmental delay and in young children with hypotonia and/or feeding problems. In counselling parents, the designation "Kabuki" syndrome seems to be more appropriate than "Kabuki make-up" syndrome.

This prospective analysis assessed the risk of mild mental retardation (MMR) associated with low birthweight (LBW) in the Child Health and Development Studies. Scores of 50-70 on the Raven Progressive Matrices, a relatively culture-free test of cognitive functioning, were used to categorize MMR. At the age of 5, 13.8% of the 195 children with birthweights less than 2500 g (LBW) were MMR, whilst 4.2% of the 2293 children with normal birthweights (> 2955 grams) were MMR. After adjusting for confounders (maternal age, race, education, prenatal alcohol use, maternal conditions, and congenital anomalies), the relative risk of MMR for LBW was 3.4 (95% CI 1.2-5.4). For children aged 9-11, 7.7% of 194 LBW children were MMR, compared with 6.2% of the 2546 with normal birthweights; the adjusted relative risk for LBW was 1.2 (95% CI 0.7-2.0). Although a strong association between LBW and MMR was observed for both blacks and non-blacks at the age of 5, the association between birthweight and MMR was apparent only for blacks in the cohort of children aged 9-11. These findings suggest that race, a marker for environmental factors which were not measured in this study, may modify the LBW and MMR relationship.

Objective: To determine normative data on age-related probabilities of children with severe disabilities acquiring mobility or self-feeding skills, or dying during a 5-year follow-up period., Research Design: A 5-year follow-up study of three mutually exclusive subgroups formed on the basis of severe, profound, or suspected levels of retardation and incontinence and the following combinations of feeding and mobility skills., Participants: The sample was made up of 7836 children and adults distributed among the three subgroups being served in California between January 1981 and December 1985., Measurements/main Results: Subjects who were tube-fed and immobile showed very little likelihood of becoming mobile or feeding themselves and had a high probability of death. Individuals who had some mobility experienced a better outcome., Conclusions: After age 6 years, the most probable outcome for children who are immobile and cannot feed themselves is death or no improvement in self-help skills.

A 5-year prospective study of 4,934 children of different ethnic groups has demonstrated a 3-fold increase of postneonatal mortality and childhood morbidity in the offspring of consanguineous Pakistani parents. Most of these families contained more than one consanguineous union, resulting in a mean inbreeding coefficient for their children of 0.0686. It is estimated that 60% of the mortality and severe morbidity of this group of children could be eliminated if inbreeding ceased. However consanguinity is much favoured in this minority group, and health education will have to be carefully and sensitively handled.

The frequency and effects of hereditary diseases of consanguineous marriage (CM) in 210 965 Han nationality people in different districts of Zhejiang province were investigated. Of 44342 couples in three different regions, 951 (2.14%) were proved to be of CM. The frequency of CM was 3.02% in Mi as against 1.13% in Pi and 2.50% in Ii. There were 19 types and 81.07% of CM occurred in the first cousins, of which 0.95% occurred in semisiblings. The frequency of hereditary disease and the mortality before the age of 20 in the offsprings of CM were significantly higher than those of non-CM (P < 0.01). 104 types of hereditary disease and congenital malformation were found. The frequency of mental retardation was 2/1000. There were 197 high risk families which contained 19-types of hereditary disease.

The authors investigated the prevalence of mental retardation (MR) in 6-14-year-old children in the district of Banská Bystrica. The aim was to assess the prevalence of MR and the ratio and character of genetic factors participation in its genesis. The group was based on the Uniform records of defective children and health records of children from special schools and social care institutes in 1987. From a total of 23,510 children 510 (2.16%) suffered from MR. In the population of gipsy children the authors found 21.5% with MR, while in the population of non-gipsy children the percentage was 0.9%. Genetic examinations were made in 106 children with MR. In 33 of these (31.1%) there was evidence of a genetic aetiology (monogenic disease in 21, chromosomal aberration in 11 and in one instance polyfactorial disease); a non-genetic aetiology was found in 21 children (19.8%). The aetiology was not elucidated in 52 children (49.0%). The authors emphasize the importance of genetic prevention for reducing the number of children with MR but draw attention also to non-medical factors which may play a part in the aetiology of mental retardation in a part of the child population.

This paper researches into transcultural psychiatry from She. Hui and Mongolian Nationalities in an epidemiological fact-finding way. Its results show that different historical cultural background geographical environment and custom of these three nationalities closely related to mental health including the sickness rates the symptoms and the kinds of mental disease.

The Niikawa-Kuroki "Kabuki Make-Up" syndrome is a rare sporadic malformation syndrome, characterised by severe psychomotor and growth retardation, peculiar facies, including long palpabral fissures and large malformed ears and skeletal abnormalities. We report a 2-year-old Moslem Arab boy with 28 of the 32 originally described features of this syndrome and in addition with hyperelastic joints, hypospadias and scaphocephaly which were not previously described in association with the Kabuki make-up syndrome. This is the fifth reported case of this syndrome in a non-Japanese patient, and the second reported case in a patient of Arab descent.

The authors present the clinical and genealogical description of 6 patients with neuroectodermal dysplasia diagnosed by a medical expedition party in Khankin district of the Khorezm region. Unique combination of clinical signs (total alopecia, microcephalia, oligophrenia, hyperhydrosis and hypogenitalism) helped differentiating the syndrome from other well-known hereditary neuroectodermal dysplasias. The nature of the syndrome segregation in the families suggested its autosomal-recessive mode of inheritance.

We report a case of Fukuyama congenital muscular dystrophy with inflammatory infiltrate on muscle biopsy in an American girl of non-Japanese ancestry. The child was hypotonic, had decreased muscle strength in all extremities, and poor head control. Her mental and motor development were delayed. She developed generalized seizures at 19 months of age. Her muscle enzymes were abnormal; cranial computed tomography demonstrated hypoplasia of the cerebellum. Electromyogram was normal. Deltoid muscle biopsy documented scattered basophilic regenerating myofibers and focal atrophic fibers with focal increases of endomysial connective tissue, small endomysial foci of inflammatory cells, and occasional perimysial, perivenular lymphocytic infiltrates. Prednisone therapy produced some decrease in serum muscle enzyme levels.

Six cases of inner city black children with both infantile autism and lead poisoning are reviewed. In three cases, developmental deviance seems to have been present before the possible impact of lead toxicity. In two cases the lead poisoning may have contributed to the onset or acceleration of developmental symptomatology. In one case the temporal sequence remains unclear. Possible patterns of interaction and the implications for clinical practice are discussed.

Thirty-nine patients of 32 families affected by the Cohen syndrome are described with regard to clinical features, ethnic distribution and mode of presentation. Associated diseases, mode of inheritance and the apparent high prevalence of the syndrome among Jewish children are discussed.