Kaufman Assessment Battery For Children Research Articles

Normalverteilung der Intelligenz von VLBW-Frühgeborenen mit 5 Jahren: sehr geringe Korrelation mit sehr geringem Geburtsgewicht

Preterm infants with very low birth weight < 1500 g (VLBW) have a higher risk of developmental disorders. In addition to the common estimation of the mean intelligence values, we studied the distribution of intelligence at preschool age in VLBW infants and the risk factors influencing this distribution. A prospective cohort study of 277 VLBW infants < 32 weeks born in 1991-1995 and treated according to a standardized regimen in one Perinatal Center was carried out, including measurement of intelligence (Kaufman-Assessment Battery for Children) at age 5. Statistical methods employed were: explorative data analysis, correlation, chi (2)- and t-tests; the tested variables were: small for gestational age (< third percentile), perinatal acidemia (umbilical arterial pH < 7.10), perinatal hypoxia (BE < - 10), hypothermia (< 36 degrees C), hypoglycemia after the first day of life (< 30 mg / dL), bronchopulmonary dysplasia (FiO (2) > 0.21 > or = 36 weeks), intraventricular hemorrhage, ventricular dilation, periventricular leukomalacia, seizures, abnormal acoustic evoked potentials, and hyperexcitability at discharge. The distribution of intelligence in 137 VLBW infants < 32 weeks (60 % follow-up rate) was similar to a symmetrical Gaussian bell curve. The intelligence increased very slightly with birth weight (Pearson correlation: 0.172; p = 0.045) and was significantly lower in children with hypoglycemia after the first day of life (- 13.35; 95 % confidence interval: - 20.08 to - 6.63; p = 0.002), hyperexcitability at discharge (- 16.28; 95 % confidence interval: - 25.26 to - 7.31; p = 0.005), and bronchopulmonary dysplasia (- 7.00; 95 % confidence interval - 11.71 to - 2.29; p = 0.039). At preschool age, the intelligence of VLBW infants is normally distributed and correlates only slightly with the very low birth weight. Hypoglycemia after the first day of life and bronchopulmonary dysplasia are risk factors for lower intelligence. Hyperexcitability at discharge seemed to represent a promising prognostic factor for a later intelligence reduction.

Intellectual outcome, motor skills and BMI of children with congenital hypothyroidism: a population‐based study

To evaluate intellectual outcome, motor skills and anthropometric data of children with congenital hypothyroidism (CH). Children with permanent CH who were born in 1999 in Bavaria were eligible for this prospective, population-based study. Cognitive performance was evaluated by the Kaufman Assessment Battery for Children and motor skills were assessed by the motor test, Motoriktest für vier-bis sechsjahrige Kinder (MOT) 4-6. Eighteen of 21 eligible children participated (86%). Median age of the children was 5.5 years (range 4.9-5.8). Treatment with levothyroxine was started after a median of 7.2 days (range 4-15) with a median dose of 12.0 microg/kg (range 7.2-17.0). Mean intelligence quotient (IQ) of the children was 100.4 (standard deviation [SD] 10.1): no children had IQ values below the normal range. Reactivity and speed of movement were significantly reduced in children with CH. Children with an initial thyroid-stimulating hormone (TSH) value of >200 mU/L performed significantly worse than children with TSH value of <or=200 mU/L (p=0.003). Children with CH had a significant increased risk for overweight (risk ratio [RR] 3.91, 95% confidence interval [CI] 1.22-11.75, p=0.005) compared to the Bavarian reference children. In this study, children with CH had an increased prevalence of overweight compared to reference data. Partially impaired motor skills could be shown, whereas the intellectual development was normal.

Neurodevelopmental disabilities and special care of 5-year-old children born before 33 weeks of gestation (the EPIPAGE study): a longitudinal cohort study

The increasing survival rates of children who are born very preterm raise issues about the risks of neurological disabilities and cognitive dysfunction. We aimed to investigate neurodevelopmental outcome and use of special health care at 5 years of age in a population-based cohort of very preterm children. We included all 2901 livebirths between 22 and 32 completed weeks of gestation from nine regions in France in Jan 1-Dec 31, 1997, and a reference group of 667 children from the same regions born at 39-40 weeks of gestation. At 5 years of age, children had a medical examination and a cognitive assessment with the Kaufman assessment battery for children (K-ABC), with scores on the mental processing composite (MPC) scale recorded. Data for health-care use were collected from parents. Severe disability was defined as non-ambulatory cerebral palsy, MPC score less than 55, or severe visual or hearing deficiency; moderate deficiency as cerebral palsy walking with aid or MPC score of 55-69; and minor disability as cerebral palsy walking without aid, MPC score of 70-84, or visual deficit (<3/10 for one eye). In total, 1817 (77%) of the 2357 surviving children born very preterm had a medical assessment at 5 years and 396 (60%) of 664 in the reference group. Cerebral palsy was diagnosed in 159 (9%) of children born very preterm. Scores for MPC were available for 1534 children born very preterm: 503 (32%) had an MPC score less than 85 and 182 (12%) had an MPC score less than 70. Of the 320 children in the reference group, the corresponding values were 37 (12%) and 11 (3%), respectively. In the very preterm group, 83 (5%) had severe disability, 155 (9%) moderate disability, and 398 (25%) minor disability. Disability was highest in children born at 24-28 completed weeks of gestation (195 children [49%]), but the absolute number of children with disabilities was higher for children born at 29-32 weeks (441 children [36%]). Special health-care resources were used by 188 (42%) of children born at 24-28 weeks and 424 (31%) born at 29-32 weeks, compared with only 63 (16%) of those born at 39-40 weeks. In children who are born very preterm, cognitive and neuromotor impairments at 5 years of age increase with decreasing gestational age. Many of these children need a high level of specialised care. Prevention of the learning disabilities associated with cognitive deficiencies in this group is an important goal for modern perinatal care for children who are born very preterm and for their families.

Kognitive und motorische Leistungsfähigkeit und Verhalten bei Kindern nach Herz-Lungen-Maschinen-Operation im Neugeborenenalter

Empirical results about the cognitive, motor and psychic development in patients with d-transposition of the great arteries after arterial switch operation remain unclear. Potential reasons are the patients' initial status, improved perinatal care or the quality of surgery. To exclude quality of surgery as the main reason, only children, who were operated by the same surgeon, were included in this study. Thirty children operated as neonates were examined at the age of 4.3 to 12.5 years. The Kaufman-Assessment Battery for Children (K-ABC) was administered to assess general intelligence and acquired abilities, the Movement Assessment Battery for Children (M-ABC) to assess gross and fine motor function and the Child Behaviour Checklist (CBCL/4-18) to assess behaviour problems. General intelligence was significantly lower than the population means, whereas the acquired abilities were within the normal range. Motor function also was below average. Parents reported significantly more behaviour problems in patients compared to the normal population. Risk factors for lower general intelligence were the duration of the operation and postoperative complications. The arterial switch operation performed during the neonatal period is associated with lower general intelligence, motor impairment and behaviour problems. Careful assessment of neurodevelopmental outcome for the detection of developmental delays and early initiation of interventions seem to be necessary.

Confirmatory factor structure of the Kaufman Assessment Battery for Children--Second Edition: Consistency with Cattell-Horn-Carroll theory.

The Kaufman Assessment Battery for Children-Second Edition (KABC-II) is a departure from the original KABC in that it allows for interpretation via two theoretical models of intelligence. This study had two purposes: to determine whether the KABC-II measures the same constructs across ages and to investigate whether those constructs are consistent with Cattell-Horn-Carroll (CHC) theory. Multiple-sample analyses were used to test for equality of the variancecovariance matrices across the 3- to 18-year-old sample. Higher-order confirmatory factor analyses were used to compare the KABC-II model with rival CHC models for children ages 6 to 18. Results show that the KABC-II measures the same constructs across all ages. The KABC-II factor structure for school-age children is aligned closely with five broad abilities from CHC theory, although some inconsistencies were found. Models without time bonuses fit better than those with time bonuses. The results provide support for the construct validity of the KABC-II. Additional research is needed to more completely understand the measurement of fluid reasoning and the role of time bonuses on some tasks.

Motor and Executive Function at 6 Years of Age After Extremely Preterm Birth

Studies of very preterm infants have demonstrated impairments in multiple neurocognitive domains. We hypothesized that neuromotor and executive-function deficits may independently contribute to school failure. We studied children who were born at < or = 25 completed weeks' gestation in the United Kingdom and Ireland in 1995 at early school age. Children underwent standardized cognitive and neuromotor assessments, including the Kaufman Assessment Battery for Children and NEPSY, and a teacher-based assessment of academic achievement. Of 308 surviving children, 241 (78%) were assessed at a median age of 6 years 4 months. Compared with 160 term classmates, 180 extremely preterm children without cerebral palsy and attending mainstream school performed less well on 3 simple motor tasks: posting coins, heel walking, and 1-leg standing. They more frequently had non-right-hand preferences (28% vs 10%) and more associated/overflow movements during motor tasks. Standardized scores for visuospatial and sensorimotor function performance differed from classmates by 1.6 and 1.1 SDs of the classmates' scores, respectively. These differences attenuated but remained significant after controlling for overall cognitive scores. Cognitive, visuospatial scores, and motor scores explained 54% of the variance in teachers' ratings of performance in the whole set; in the extremely preterm group, additional variance was explained by attention-executive tasks and gender. Impairment of motor, visuospatial, and sensorimotor function, including planning, self-regulation, inhibition, and motor persistence, contributes excess morbidity over cognitive impairment in extremely preterm children and contributes independently to poor classroom performance at 6 years of age.

Placental Lesions as Predictors of Cerebral Palsy and Abnormal Neurocognitive Function at School Age in Extremely Low Birth Weight Infants (&lt;1 kg)

Extremely low birth weight (ELBW) infants (<1 kg) have high rates of neurodisability. Although previous studies have implicated placental lesions in adverse short-term neurologic outcomes in this population, none have assessed their effects in these children once they reach school age. We conducted a secondary analysis of placental pathology in a cohort study of inborn singleton ELBW infants born between 1992 and 1995 and evaluated for cerebral palsy (CP) and abnormal neurocognitive testing at 8 years of age (N = 129). The neurocognitive tests were the Kaufman Assessment Battery for Children (K-ABC) and 6 subtests of the NEPSY: A Developmental Neuropsychological Assessment. We found that placental lesions associated with maternal vascular underperfusion (increased syncytial knots and acute atherosis) were risk factors for CP, while villous edema was associated with low scores on both neurocognitive tests. Histologic chorioamnionitis (HCA) was not predictive of outcome in the population as a whole, but a severe fetal vascular response was associated with a lower NEPSY score in the subpopulation with HCA (N = 69). Placentas with increased syncytial knots, villous edema, and those with neither finding constituted nonoverlapping subgroups with distinct pathologic and perinatal characteristics. Among infants with villous edema (N = 25), those with neurologic impairment had lower gestational ages and more severe degrees of HCA. However, by logistic regression these other factors were not independent risk factors for abnormal neurocognitive testing, and only HCA with a severe fetal vascular response decreased the association of villous edema with low test scores for NEPSY, but not K-ABC.

Sex differences in latent general and broad cognitive abilities for children and youth: Evidence from higher-order MG-MACS and MIMIC models

Sex differences in the latent general and broad abilities underlying the Kaufman Assessment Battery for Children—Second Edition (KABC-II) were investigated for children and youth ages 6 through 18. The data were split into different age groups to account for changes due to differential development. Multi-group higher-order analysis of mean and covariance structures (MG-MACS) and multiple indicator-multiple cause (MIMIC) models were used to analyze these data. Boys consistently demonstrated a significant mean advantage on the latent visual–spatial ability (Gv) factor. A significant mean advantage was also found for boys on the latent crystallized ability (Gc) factor at all ages except for 17 and 18. Girls scored higher on the latent, higher-order g factor, at all ages, although this difference was statistically significant at only two age levels. An additional test, however, did not reveal a significant Age × Sex interaction effect, suggesting only main effects of Sex on Gv, Gc, and g.

Minor neurological dysfunction, cognitive development and somatic development at the age of 3 to 11 years in very‐low‐birthweight infants with transient periventricular echodensities

To determine, using strict exclusion criteria, whether transient periventricular echodensities (TPE) in very-low-birthweight infants lead to minor neurological dysfunction and problems in cognitive and somatic development in children without major neurological impairments. 23 children with TPE were matched to 23 children without TPE. Exclusion criteria were small for gestational age, microcephaly at birth, diplegia, asphyxia, psychomotor retardation, intraventricular haemorrhage grade III/IV, major surgical interventions and malformations. The Kaufman Assessment Battery for Children, Draw-a-Man Test and neuropaediatric examination were used for evaluation. There were no differences in demographic data, growth and socio-economic status. Significant differences with lower results in the TPE group were found in fine motor skills and in the Draw-a-Man Test. In the Kaufman Assessment Battery for Children, all subscales were below average in the TPE group, except the sequential processing scale. In the control group, all subscales were within the average range. By using strict exclusion criteria to eliminate other risk factors for minimal neurological dysfunction and poor cognitive development, we were able to focus on the effect of TPE. TPE seem to affect cognitive development and cause minor neurological dysfunction.

The totality of available evidence shows the race IQ gap still remains.

In a previous study (Rushton & Jensen, 2005), we examined 10 categories of technical research and concluded that the mean Black-White IQ difference in the United States is about 80% heritable. We reviewed evidence that (a) the distribution of IQ scores around the world shows averages of 106 for East Asians, 100 for Whites, 85 for U.S. Blacks, and 70 for sub-Saharan Africans; (b) race differences are most pronounced on the more g-loaded subtests (g being the general factor of mental ability); (c) race differences are most pronounced on the subtests whose scores show the most heritability; and (d) racial differences in brain size parallel the IQ differences. We also reviewed corroborating studies of (e) racial admixture, (f) trans-racial adoption, (g) regression to different racial means, (h) 60 related life-history traits, (i) human origins, and (j) the inadequacy of environmental explanations of the racial IQ difference. (In Africa, the 30-point difference is likely only 50% heritable because environmental factors such as malnutrition and disease have so much more impact than they do elsewhere in the world; Lynn, 2006.) Dickens and Flynn (2006, this issue) challenge our hypothesis. They claim that ‘‘no one can really trace the Black-White IQ gap in the United States back to its origins’’ (p. 913) and that in the United States, Blacks have gained ‘‘4 to 7 IQ points on non-Hispanic Whites between 1972 and 2002’’ (p. 913). But to maintain that ‘‘no one can really trace the . . . gap back to its origins,’’ Dickens and Flynn had to sidestep our citation of Shuey’s (1966) review of the literature, which shows that BlackWhite IQ differences in the United States have remained at 15 to 18 points, or 1.1 standard deviations, for nearly a century. For example, she found 23,596 Black draftees in World War I (1917) had an IQ of 83 (vs. 100 for Whites), with a Black overlap of the White mean of 13%. For recent data, we cited the meta-analysis by Roth, Bevier, Bobko, Switzer, and Tyler (2001), which also shows a mean difference of 1.1 standard deviations (range of 0.38 to 1.46 standard deviations, depending on the test’s g loading), based on 6,246,729 individuals from military, corporate, and higher-education samples. Roth et al. found any narrowing of the gap was ‘‘either small, potentially a function of sampling error . . . or nonexistent for highly g loaded instruments’’ (p. 323, italics added). To claim a 4to 7-point gain for Blacks, Dickens and Flynn chose three independent tests showing medium gains (the Wechsler, Stanford-Binet, and Armed Forces Qualification tests) and relegated to their Appendix B four or more tests showing lesser gains. They excluded the Wonderlic Personnel Test, which they acknowledge showed a gain of only 2.4 points for Blacks between 1970 and 2001. (Dickens and Flynn suggest that more ‘‘high quality’’ Whites than Blacks had taken the test.) They excluded the Kaufman Assessment Battery for Children (K-ABC), which Murray (2005) described as showing a loss of 1 IQ point for Blacks between 1983 and 2004. (Dickens and Flynn say the data contained an inflated standard deviation.) They excluded the very g-loaded Woodcock-Johnson test, which Murray (2005; whom they cite) described as showing the conventional gap of 1.05 standard deviations for the third (2001) standardization sample. (Dickens and Flynn say the Blacks were an unrepresentative ‘‘subsubsample.’’) They also excluded the Differential Ability Scale, which in Lynn’s (1996) analysis (which they cite) showed a maximum gain of 1.83 IQ points for Blacks between 1972 and 1986. (Dickens and Flynn say the sample lacked ‘‘quality.’’) To be compelling, however, researchers must take the totality of available evidence into account (Gottfredson, 2005). Even the tests Dickens and Flynn did analyze do not support their conclusion. The alleged gain of 4 to 7 points is from a ‘‘projected’’ trend line based on a small IQ rise per year multiplied by more years than are in the data using unclear procedures (see the additional appendix in the Web site they refer to). Simple arithmetic applied to the data in their Table A1 shows a mean gain for Blacks of only 3.44 IQ points, from 86.44 to 89.88 Address correspondence to J. Philippe Rushton, Department of Psychology, University of Western Ontario, London, Ontario N6A 5C2, Canada, e-mail: rushton@uwo.ca. PSYCHOLOGICAL SCIENCE

Spearman's law of diminishing returns in hierarchical models of intelligence for children and adolescents

Spearman's “law of diminishing returns” or SLODR refers to a decrease in g saturation as ability level increases. SLODR has been demonstrated in a number of intellectual batteries but several important aspects of the phenomenon are not yet well understood. We investigated the presence of SLODR in the Kaufman Assessment Battery for Children—Second Edition (KABC-II), a popular measure of intelligence for children. We used confirmatory factor analysis to investigate the invariance of two hierarchical factor structures across ability groups; the subtest variance explained by the ability factors across groups; and whether SLODR was produced only by subtests with low loadings on the general ability factor. We found that SLODR was present in the KABC-II, and its presence was not dependent on the hierarchical model of intelligence. Moreover, our findings suggest that SLODR acts on g and not on the broad abilities, although the contribution of g to various broad abilities is lower in the high ability group. Finally, SLODR was not produced by the subtests with the lowest g loadings on the general factor.

Spacial perception and spatial memory in children with benign childhood epilepsy with centro-temporal spikes (BCECTS)

Despite the benign prognosis regarding the response of seizures to treatment, some evidence now exists that patients with benign childhood epilepsy with centro-temporal spikes (BCECTS) may have neuropsychological deficits sometimes leading to academic underachievement. There is, however, no general agreement on the exact profile of functions disturbed. This study was designed to identify significant deficits in spatial perception and memory in children with BCECTS (ages 6–10 years) compared with healthy controls matched for age, sex and socioeconomic status. The neuropsychological test battery administered consisted of the HAWIK-III, the “Tübinger Luria Christensen Neuropsychological Test Set for Children”, the “Kaufman Assessment Battery for Children” and the “Differential Neuropsychological Test”. Twenty-two patients and 22 control subjects completed all tests. Children with BCECTS exhibited significant deficits in higher functions of spatial perception, including spatial orientation, as well as in basal and complex spatial memory. Deficits were independent of the lateralization of the epileptogenic foci and independent of anti-convulsive drug treatment.

Events at early development: Are they associated with early word production and neurodevelopmental abilities at the preschool age?

Background Obstetrical and neonatal complications and/or an adverse parenting environment are risk factors for language impairment, but little is known about their effects on early word production (late talking). Aims To determine obstetrical and neonatal risk factors in children with delayed word production; to assess the influence of the parenting environment on word production; to determine whether the toddler's vocabulary competence is related to his/her social competence; to document the neurodevelopmental outcome at the late preschool age. Study design Prospective follow-up study. Based on word production at 18 months, children were retrospectively classified into consistent or transient late talkers and controls. Subjects Full-term infants, N = 30 (mean GA 40.3 ± 1.1 weeks, mean birth weight 3577 ± 267 g) were assessed at 18 and 24 months; and 5 years and 7 months. Methods Austrian Communicative Development Inventories; Bayley Scales of Infant Development; Griffiths Developmental Scales. Outcome measures Peabody Picture Vocabulary Test; Kaufman Assessment Battery for Children; Touwen's neurological examination. Results and Conclusions Lower Apgar scores and the need for neonatal intensive care were associated with delayed word production. The parents' educational and vocational levels were lower in late talkers. Early vocabulary competence correlated with social competence. With regard to the prediction of lexical and cognitive outcomes, late talkers did not differ from controls. Minor neurological dysfunctions (MND) were seen in nearly half of the late talkers and indicate the necessity to follow these children into school age, when MND might become more obvious and literacy can be assessed.

Assessment of expressive vocabulary outcomes in hearing-impaired children with hearing aids: do bilaterally hearing-impaired children catch up?

To evaluate expressive vocabulary growth in hearing-impaired preschool children wearing hearing aids. Prospective analysis of the outcomes of children included in the 1994 German 'Goettinger Hoer-Sprachregister' (GHR) series, using a repeated-measures paradigm in six- to nine-month intervals (t1-t3). Twenty-seven children (aged 2.0-4.4 years) with bilateral sensorineural hearing loss (with averages at frequencies of 0.5, 1, 2 and 4 kHz of > 20 to > 90 dB in the better ear) from the 1994 GHR series. The children were diagnosed at a mean age of 31.4 months (standard deviation (SD) 10.6 months) and fitted with a binaural hearing aid at a mean age of 32.3 months (SD 10.5 months). Nonverbal intelligence was average (five missing data entries). Standardized, age-appropriate picture naming tests (the 'Sprachentwicklungstest für 2-jährige Kinder', the Kaufman Assessment Battery for Children subtest vocabulary, and the 'Aktiver Wortschatztest für drei- bis sechsjährige Kinder') were carried out at three time points and results compared with data from children with normal hearing. The test raw scores were converted to T scores (mean = 50; SD = 10). On average, the children scored far below the normative population at t1 (mean = 28.9; SD = 11.3) and slowly improved as they got older (at t3, mean = 34.1; SD = 16.1; p = 0.010). Children with mild or moderate hearing loss improved most notably (mean difference t1-t3; p = 0.001), except for one child of deaf parents. Two of the five mildly hearing-impaired children and two of the eleven moderately hearing-impaired children caught up with their normal hearing peers with regards to expressive vocabulary. Such expressive vocabulary achievements were not seen in any children with > 70 dB hearing loss or in six of the eleven children (55 per cent) with a 40-70 dB hearing loss, despite receiving adequate personal amplification. Testing expressive vocabulary size is a useful clinical tool in assessing linguistic lexical outcome.

Assessing Gifted Children with the Kaufman Assessment Battery for Children — Second Edition (KABC-II)

The Kaufman Assessment Battery for Children-Second Edition (KABC-II; Kaufman &amp; Kaufman, 2004a) is an individually administered intelligence test for children ages 3 to 18. The theory-based test was designed for use in a wide variety of clinical, psychoeducational, and neuropsychological situations, including the assessment of the intellectually gifted. This article provides a context for assessing intellectually gifted children with the KABC-II. General definitional issues in the assessment of gifted children are reviewed, followed by specific conceptions of intellectual giftedness, and details of appropriate assessment procedures. With this foundation of general information on giftedness, the KABC-II's structure, scales, and psychometric properties are described; finally the benefits and limitations of using the KABC-II for assessing gifted children are explored and a comprehensive case study is provided.

Variation of Cognition and Achievement With Sleep-Disordered Breathing in Full-term and Preterm Children

Pediatric sleep-disordered breathing (SDB) has a disproportionately high prevalence in children who were preterm infants (preterm children) and is associated with behavioral comorbidity. Exposure to intermittent hypoxemia and sleep disruption may contribute to cognitive impairment. We quantified the association of SDB with cognition and achievement and determined whether preterm children are at a differentially increased risk for SDB-related impairments. Cross-sectional analyses. Urban community. Eight hundred thirty-five children, aged 8 to 11 years.Intervention(s) None. Scores on the Peabody Picture Vocabulary Test-Revised, Kaufman Assessment Battery for Children, and Continuous Performance Test. One hundred sixty-four children had SDB. In unadjusted analyses, children with SDB had poorer scores on almost all tests of cognition and achievement. Group differences were attenuated after adjusting for socioeconomic status; in these analyses, children with SDB scored lower on the Peabody Picture Vocabulary Test-Revised (mean +/- SE score, 100.5 +/- 1.4 vs 103.6 +/- 0.7; P = .04), and the Kaufman Assessment Battery for Children riddles and triangles subscales. Associations were stronger in preterm than in full-term children. Of the sleep measures, snoring history was most strongly correlated with indices of cognition and achievement. Although moderate group differences were observed for almost all cognitive measures, an attenuation of effects was observed once socioeconomic status was considered. The deficits in selective measures of academic abilities, language comprehension, and planning and organizational skills suggest a negative impact of SDB on achievement and cognition. Stronger associations in preterm children suggest the importance of screening for snoring, a good predictor of cognitive function in this population.

Neurodevelopmental outcome and haematological course of a long‐time survivor with homozygous alpha‐thalassaemia: Case report and review of the literature

Homozygous alpha-thalassaemia, also called haemoglobin (Hb) Bart's hydrops fetalis, has been thought to be a lethal condition. Due to prenatal diagnosis and intrauterine blood transfusions, a few patients with Hb Bart's hydrops fetalis have survived. This fact raises the urgent questions of clinical management and appropriate follow-up of these patients, both of which are addressed in this article. We report on a 6.5-y-old patient with homozygous alpha-thalassaemia and review the literature of 13 other survivors published to date. Transfusion requirements were evaluated and the rate of liver iron accumulation was assessed by biomagnetic liver susceptometry before and after institution of iron-chelating therapy. Psychometric evaluation was carried out using Munich's Functional Development Test, the Columbia Mental Maturity Scale, the Kaufman Assessment Battery for Children, and the Peabody Picture Vocabulary Test. Our patient had significant delay of psychomotor development. Psychometric evaluation at the age of 5 y revealed an IQ of 85 and an intellectual level of a 4-y-old child. Early tissue iron overload was seen, but a negative iron balance was achieved after institution of desferrioxamine treatment at dosages used for beta-thalassaemia. Homozygous alpha-thalassaemia should no longer be regarded as a lethal condition. Early intervention during pregnancy and careful haematological as well as neuropsychological follow-up was able to provide long-term survival and good life quality in our patient.

Poor Predictive Validity of the Bayley Scales of Infant Development for Cognitive Function of Extremely Low Birth Weight Children at School Age

The Bayley Scales of Infant Development, Second Edition (BSID II) are commonly used to assess outcomes of extremely low birth weight (ELBW) infants. We sought to assess the predictive validity of the BSID II Mental Developmental Index (MDI) for cognitive function at school age. Of 330 ELBW infants admitted in 1992-1995, 238 (72%) survived to the age of 8 years, of whom 200 (84%) were tested at both 20 months' corrected age (CA) and 8 years. Mean birth weight was 811 g, mean gestational age was 26.4 weeks, 41% were boys, and 60% were black. Measures included the BSID II at 20 months' CA and the Kaufman Assessment Battery for Children (KABC) Mental Processing Composite (MPC) at 8 years' postnatal age. BSID II MDI and MPC scores were compared and the predictive validity calculated for all 200 ELBW children and for the 154 ELBW neurosensory-intact subgroup. Predictors of stability or change in cognitive scores were examined via logistic regression adjusting for gender and sociodemographic status. For all ELBW children, the mean MDI was 75.6 +/- 16 versus a mean KABC of 87.8 +/- 19. For the neurosensory-intact subgroup, the mean MDI was 79.3 +/- 16 and the mean KABC was 92.3 +/- 15. Rates of cognitive impairment, defined as an MDI or KABC of <70, dropped from 39% at 20 months' CA to 16% at 8 years for the total ELBW population and from 29% to 7% for the neurosensory-intact subgroup. The positive predictive value of having an MPC of <70 given an MDI of <70 was 0.37 (95% confidence interval [CI]: 0.27, 0.49) for all ELBW infants, 0.20 (95% CI: 0.10, 0.35) for the neurosensory-intact subgroup, and 0.61 (95% CI: 0.42, 0.77) for the neurosensory-impaired subgroup. The negative predictive values were 0.98, 0.99, and 0.85 for the 3 groups, respectively. Neurosensory impairment at 20 months' CA predicted lack of improvement of cognitive function (odds ratio: 6.9; 95% CI: 2.4, 20.2). Children whose cognitive scores improved between 20 months and 8 years had significantly better school performance than those whose scores stayed at <70, but they did less well than those whose scores were persistently >70. The predictive validity of a subnormal MDI for cognitive function at school age is poor but better for ELBW children who have neurosensory impairments. We are concerned that decisions to provide intensive care for ELBW infants in the delivery room might be biased by reported high rates of cognitive impairments based on the use and presumptive validity of the BSID II MDI.

The Conundrum of Prediction

The study by Hack et al1 in this issue of Pediatrics underscores the complexities inherent in prediction of developmental outcome. More specifically, in this study many children who scored >2 SDs below average on the Bayley Scales of Infant Development-II (BSID-II)2 at 20 months' corrected age subsequently did better on the Kaufman Assessment Battery for Children (K-ABC)3 given at 8 years. Greater stability in scores was found in those children with Mental Developmental Index (MDI) scores of 70 also did well on the later assessment. As a result, high negative predictive values, but low positive predictive values, were found. There are numerous advantages to the study: large sample size, consideration of environmental and biomedical variables, long-term serial evaluation, analyses with and without … Reprint requests to (G.P.A.) Division of Developmental and Behavioral Pediatrics, Southern Illinois University School of Medicine, PO Box 19658, Springfield, IL 62794-9658. E-mail: gaylward{at}siumed.edu

52 Longitudinal Neurodevelopmental Outcome at 18 Months and 5 Years in Infants Born &lt;32 Weeks of GA

Background: Children born preterm have a high prevalence of neurologic and developmental disabilities in the first two years of live. Their predictibility for developmental problems at 5 years of age is unclear. Aims: 1)to assess the neurological and developmental outcome at 18 months and 5 years of age in a regionally defined cohort of preterm infants born less than 32 weeks GA. 2)To investigate the effects of prematurity and growth impairment on later motor and cognitive development. Methods: Perinatal data were collected retrospectively on a cohort from January 1996 through December 1996. Growth, neurological examination and development were evaluated at 18 months and 5 years of age. Psychomotor development at corrected age 18 months was assessed using the Bayley Scales of Infant Development (MDI/PDI). At 5 years, cognitive function was assessed with the Kaufman Assessment Battery for Children (K-ABC). Results: Of 54 surviving children, 45 (83%) underwent neurological and developmental assessment by age 18 months. The rates of CP, minor neurological impairment, hearing impairment, visual impairment were 7%, 20%, 7%, 2% respectively. The mean MDI and PDI scores were 94.5±24.2 and 87.8±21.2 respectively. The neonatal factors significantly associated with neurodevelopmental disability at 18 months of age were BPD (p<0.01) and neonatal infection (p<0.05). Severe cranial ultrasounds abnormalities (PVL, IVH grade 3/4) were present only in one infant. Children with MDI<85 had significantly smaller had circumference at birth and still at 18 months of corrected age (both p<0.05). At 5 years of age, the followed 38 children born in 1996 (70% of survivors), scored within the normal range on all subscales of the K-ABC, but children with BW < 1000g exhibited lower general cognitive ability (MCP: 82.1±12.9,vs 95.2±14.8, p=0.05) and scored significantly below the norm in the sequential processing scale (SP: 82.4±6.1,vs 97.9±12.9, p<0.01). Neonatal factors like a smaller head circumference at birth, a higher incidence of neonatal infection and poor post-natal growth correlated negatively with cognitive development (p<0.01, p<0.05, p<0.05 respectively). Children with MCP<85 had already significantly lower MDI scores at 18 months than children who scored within the normal limits (83±20.9 vs 102±19.5, p<0.05) Conclusion: This study provides outcome data for a geographically defined cohort. BPD and neonatal infection were associated with neurodevelopmental disability at 18 months of age. Poor postnatal growth and delayed development at the age of 18 months were predictive of cognitive impairment at 5 years of age.