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Biomedical subjects

M E Msall

Publications and source records attributed to M E Msall.

35 records · Page 2Linked to original sources
PubMed1994

The Functional Independence Measure for Children (WeeFIM). Conceptual basis and pilot use in children with developmental disabilities.

Few tools are available to pediatricians for tracking and monitoring disability status in children. We describe the conceptual basis and pilot use of the Functional Independence Measure for Children (WeeFIM). Our pilot use of this instrument in children with limb deficiency, Down's syndrome, spina bifida, cerebral palsy, and extreme prematurity demonstrates that the WeeFIM is a valid measure for tracking disability in preschool age and middle childhood. The WeeFIM measures the impact of developmental strengths and difficulties on independence at home, in school, and in the community. This allows the pediatrician to prioritize interventions for enhancing comprehensive functional outcomes and supporting families.

Adaptation, Psychological↗
PubMed1994

WeeFIM. Normative sample of an instrument for tracking functional independence in children.

A cross-sectional community sample of 417 children, ages 6 months to 8 years without developmental delays or in developmental programs, was seen. The Functional Independence Measure for Children (WeeFIM) was used to assess independence in self-care, sphincter control, transfers, locomotion, communication, and social cognition. Mean total WeeFIM was similar for males and females. There was a significant correlation between the age of the child in months and total WeeFIM scores for children ages 2 to 5 years (n = 222, r = .80, P < .01). There was a progressive increase of functional independence with increasing chronological age across all WeeFIM domains. We conclude that the WeeFIM is a useful instrument for measuring disability in children.

Adaptation, Psychological↗
PubMed1993

Functional status of extremely preterm infants at kindergarten entry.

Functional status was formally assessed in 149 of 153 surviving members of an extremely preterm (< or = 28 weeks) birth cohort born at one tertiary center between 1983 and 1986. The children were observed in the completion of motor, speech and self-care tasks, and administered either the Vineland Daily Living Skills Scale (VDLS) or the Functional Independence Measure for children (WeeFIM). 31 children had major neurodevelopmental impairment. Only 5 per cent were considered to have severe functional limitation. The prevalence of functional limitation varied by definition: 11 children were limited using the WeeFIM instrument and 35 using the VDLS instrument. These findings suggest that the majority of extremely preterm children are functional at kindergarten entry, but will require continuous monitoring of academic skills.

Activities of Daily Living↗
PubMed1993

Predictors of mortality, morbidity, and disability in a cohort of infants < or = 28 weeks' gestation.

This study attempted to identify predictors for mortality, morbidity, disability, and educational handicap at age 4 years in a cohort of 194 infants born at 23 to 28 weeks' gestation at one regionalized tertiary center from 1983 to 1986. Forty-one infants died (21%); standardized neurodevelopmental and functional assessments were conducted on 149 of 153 (97%) survivors at a mean age of 52 months. Five significant predictors of death were identified with logistic regression analysis: gestational age 23 to 26 weeks, intraventricular hemorrhage grades 3 or 4, male gender, five-minute Apgar < or = 3, and absence of prophylactic calf lung surfactant extract. Significant predictors of neurodevelopmental morbidity included sepsis, male gender, and nonwhite race. Significant predictors of disability at age four included neurodevelopmental impairment and severe retinopathy of prematurity. Low socioeconomic status, nonwhite race and male gender were predictive of educational handicap. These findings suggest that outcomes may have distinct pathophysiologies. The role of biomedical events appears strongest for death.

Child, Preschool↗
PubMed1992

Kindergarten readiness after extreme prematurity.

OBJECTIVE: To assess kindergarten readiness among survivors of extreme prematurity and to identify predictors of special education requirements. DESIGN: Historic cohort design. SETTING: Regionalized tertiary pediatric center. PARTICIPANTS: One hundred forty-nine (97%) of 153 children who were alive at follow-up (mean +/- SD age, 52.7 +/- 9.9 months). SELECTION PROCEDURES: Study cohort included infants (gestation, 23 to 28 weeks), born between 1983 and 1986 (N = 194), who were alive at follow-up (N = 153, 79% survival). INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Standardized neurodevelopmental and psychometric evaluations were administered by a multidisciplinary team that was blinded to the neonatal course. Thirty-one children (21%) had major neurodevelopmental impairments. By using the McCarthy Scales of Children's Abilities for children free of major impairments, 61 (63%) had one or more minor neurodevelopmental impairments noted. Half of the surviving children were thought to require special education resources at kindergarten entry. Multivariate logistic regression identified three significant predictors of special education: low socio-economic status, nonwhite race, and male gender. CONCLUSIONS: Social and demographic variables were associated with minor neurodevelopmental impairments and special education requirements among extremely premature children. Continued developmental follow-up and targeted interventions to reduce the risk of educational underachievement appear to be warranted.

Child, Preschool↗
PubMed1991

Guidelines for safe transportation of children in wheelchairs.

Advocacy efforts by health care professionals have prompted state legislative changes mandating the use of car seats and seat belts by children. These initiatives have greatly improved the level of safety in transportation of nonhandicapped children. Despite these positive changes, the transportation needs of nonambulatory children have not been addressed. In addition, implementation of Public Law 99-457 will result in larger numbers of young children with motor impairments requiring transportation to preschool early intervention programs. This study sought to describe how safely children in wheelchairs are transported. Observations of subjects were made as they were transported by their families or agencies at a residential summer camp, a preschool program for children with developmental disabilities, and a school for children with cerebral palsy. A safety score system was developed based on laboratory studies conducted on wheelchair restraint systems. This observation tool described the position of the wheelchair in the vehicle, the occupant restraint system, and the wheelchair restraint system. These structured observations revealed inadequate safety measures. Comparisons of safety scores of subjects transported by families with those transported by agencies were not found to have statistically significant differences. The findings of this study demonstrate a gap between minimal safety standards in wheelchair transportation and actual observed practices. In an effort to promote safe transportation practices of children regardless of their developmental differences, we present guidelines for health care providers for monitoring safe wheelchair practices in family, school, and community settings.

Adolescent↗
PubMed1991

Risk factors for major neurodevelopmental impairments and need for special education resources in extremely premature infants.

The purpose of this cohort study was to determine the incidence of and risk factors for major neurodevelopmental impairments among survivors of extreme prematurity. The study cohort comprised 100 infants born between 24 and 28 weeks of gestational age at one tertiary center from 1983 to 1984. Twenty-five infants (25%) died; 75 (75%) survived until follow-up (mean, 60 months). Standardized neurodevelopmental and psychometric assessments were performed in blind fashion on 68 of the 75 surviving children (91% follow-up). Informal assessments (parent, teacher, and physician reports) were obtained instead for seven (9%) children who had relocated outside of the area. Overall, 19 children (25%) had one or more major impairments: mental retardation, 9; cerebral palsy, 4; multiple impairments, 5; and blindness, 1. Despite a high prevalence of impairments, 95% of children (n = 71) were functionally independent [corrected]. Special educational resources were definitely necessary for seven (9%) and possibly needed for 36 (48%) additional children. Univariate analyses revealed four significant risk factors for cerebral palsy: hydrocephalus (relative risk = 12.2), grades III and IV intraventricular hemorrhage (relative risk = 5.8), 5-minute Apgar score lower than 7 (relative risk = 5.7), and bronchopulmonary dysplasia (relative risk = 5.5). Hydrocephalus was the only significant risk factor observed for mental retardation (relative risk = 5.4). Risk factors predicting a need for special education resources included sepsis (relative risk = 24.9), low socioeconomic status (relative risk = 16.3), and nonwhite race (relative risk = 3.0). Thus our data suggest that biomedical factors appear to confer the greatest risk of major impairments; sociodemographic factors appear to have a significant impact on educational risk in extremely premature infants who do not die. Continued follow-up with biomedical and developmental-social interventions appears warranted to decrease the risk of educational underachievement in this population.

Birth Weight↗
PubMed1991

Acquired cervical spine impairment in young adults with cerebral palsy.

Three patients with spastic cerebral palsy and no associated movement disorder--each of whom presented with loss of functional skills and delay in the definitive diagnosis of cervical myelopathy--are reported, in order to increase awareness of the possibility of cervical spine pathology in these adults. The possibility of myelopathy should be investigated when considering the etiology of functional deterioration. A functional neurological examination for all multiply disabled individuals is proposed as a reference for future comparison.

Adult↗
PubMed1991

Multiple minor malformations as a marker for prenatal etiology of cerebral palsy.

A consecutive series of 137 patients with cerebral palsy living in institutions and aged between 18 and 30 years was examined for minor malformations, using a modified Weighted Anomaly Score. After examination, the patients were divided into two groups, based on whether their cerebral palsy was prenatal or postnatal in onset. A further subset of prenatal-onset patients with unidentified etiology was also analysed. Both the prenatal group with known etiology and the subgroup with unknown etiology had significantly more minor malformations than the postnatal group. These results suggest that multiple minor malformations may indicate prenatal etiology of cerebral palsy.

Abnormalities, Multiple↗
PubMed1990

Noninvasive determination of acetaminophen disposition in Down's syndrome.

In this study we evaluated subjects with Down's syndrome for the possibility that direct or indirect gene dosage effects of trisomy 21 alter the fate of acetaminophen. We also investigated the usefulness of noninvasive sampling techniques to obtain parameter estimates for drug disposition in these developmentally disabled individuals. After administration of 5 mg/kg and 20 mg/kg oral doses of acetaminophen, subjects with Down's syndrome resembled control subjects in most pharmacokinetic and metabolic parameters, including apparent half-life, volume of distribution per kilogram body mass, total body clearance per kilogram of body mass, extrapolated saliva concentration at time zero, and the urinary excretion of acetaminophen glucuronide and sulfate conjugates. Glutathione conjugation tended to increase and sulfate conjugation tended to decrease in all subjects as the acetaminophen dose increased from 5 mg/kg to 20 mg/kg. Results based on these samples of very limited size also suggest that acetaminophen metabolism to glutathione-derived conjugates may have been increased in subjects with Down's syndrome. The similarity of estimates of acetaminophen pharmacokinetics and data on metabolic fate between subjects with Down's syndrome and normal volunteers indicates that large effects of trisomy 21 on these processes are unlikely. Also, these results were in agreement with extensive data obtained with invasive techniques, indicating that simple noninvasive methodologies appear to be well suited for studying acetaminophen disposition in populations of developmentally disabled individuals.

Acetaminophen↗
PubMed1990

Symptomatic atlantoaxial instability associated with medical and rehabilitative procedures in children with Down syndrome.

The Canadian College of Medical Genetics recently questioned the rationale for radiographic screening for atlantoaxial instability in Down syndrome. We report a case series of four children with Down syndrome with symptomatic atlantoaxial instability and followed for more than 5 years. In all cases a medical or rehabilitational procedure was associated with significant apnea, cardiopulmonary compromise, or severe neurological impairments. None of these patients were screened radiographically. The need for protocols to detect atlantoaxial instability in children with Down syndrome undergoing medical and rehabilitational procedures is highlighted by these cases.

Atlanto-Axial Joint↗
PubMed1990

Prenatal cocaine exposure and fetal vascular disruption.

The question of the potential teratogenicity of cocaine has been raised by the increasing frequency of its abuse in the United States. In previous studies, an increased incidence has been documented of spontaneous abortion, placental abruption, prematurity, intrauterine growth retardation, and neurologic deficits in the infants of women who abused cocaine. More recently, it has been suggested in studies that fetal vascular disruption accompanying maternal cocaine abuse may lead to cavitary central nervous system lesions and genitourinary anomalies. In this article, 10 children born of women who abused cocaine are described, 9 of whom have congenital limb reduction defects and/or intestinal atresia or infarction. The spectrum of anomalies associated with embryonic and fetal vascular disruption accompanying maternal cocaine abuse is thus enlarged. The specific risk for congenital anomalies accompanying maternal cocaine abuse during an individual pregnancy is unknown. However, data from these patients and the available literature suggest that counseling pregnant women concerning cocaine use should incorporate warnings about the possibility of associated embryonic or fetal vascular disruption.

Abnormalities, Drug-Induced↗
PubMed1989

N-acetylator variability in Down's syndrome: characterization with caffeine.

Little is known regarding the biotransformation of drugs in Down's syndrome. In particular, there are no published studies that examine metabolic pathways such as N-acetylation, which can exhibit genetically-determined variability. The objective of the present investigation was to compare the acetylator phenotypes of white subjects with Down's syndrome with age-matched control subjects, with use of caffeine as the pharmacologic probe. After the ingestion of caffeine-containing beverages, spot urine collections were obtained at 2 and 4 hours in 22 subjects with Down's syndrome and in 22 control subjects (age range of 4 to 49 years). The urinary excretion ratios of 5-acetylamino-6-amino-3-methyluracil (AAMU) to 1-methylxanthine (1X) determined in these 2-hour and 4-hour samples were highly correlated (r = 0.82; p less than 0.001). In addition, more extensive urinary excretion studies performed for an 8-hour period in three subjects with Down's syndrome demonstrated that the coefficient of variability for the ratio of AAMU/1X ranged from 10.1% to 14.2%, which is similar to the reproducibility previously reported for control subjects. A trimodal distribution of acetylator phenotypes was observed, with no differences in average or frequency distribution of ratio values between the subjects with Down's syndrome and the control subjects. This study demonstrates that polymorphic N-acetylator status, as assessed by caffeine metabolism, is similar in subjects with Down's syndrome and in control subjects.

Acetylation↗
PubMed1988

Relationships between laterality of congenital upper limb reduction defects and school performance.

Eighty children (34 males, 46 females) with congenital upper limb reduction defects who attended a regional amputee clinic between 1956 and 1986 were classified as to whether they exhibited learning difficulties in school, as indicated by grade failure or by placement in learning disability classrooms. Children with right-sided defects were more likely to encounter learning difficulties than were children with left-sided defects (Chi-square = 6.8; df = 1; p less than 0.01). Children with right-limb defects also were more likely than children with left-limb defects to experience reading problems (Chi-square = 5.9; df = 1; p less than 0.05). These results suggest the need for neuropsychological and neurophysiological study of children with limb reduction defects.

Arm↗
PubMedDate not supplied

Functional assessment and care of children with neurodevelopmental disabilities.

OBJECTIVES: To determine the relationship between pediatric assessment scores and ratings by parents and teachers regarding the amount of assistance required to complete basic activities of daily living; and to examine the relationship among scores for three commonly used pediatric assessments. DESIGN: Prospective correlational study. 205 children with developmental disabilities. The children ranged in age from 11 to 87 mo and included 72 females and 133 males of diverse socioeconomic and ethnic backgrounds. The children were evaluated by using the Battelle Developmental Inventory Screening Test, Vineland Adaptive Behavior Scales, Functional Independence Measure for Children (WeeFIM instrument), and the Amount of Assistance Questionnaire. RESULTS: The test-retest reliability coefficients for items on the Amount of Assistance Questionnaire were found to range from 0.82 to 0.97. Correlations among subscale scores and amount of assistance ratings were highest for the WeeFIM instrument and Battelle Developmental Inventory Screening Test. The highest correlation was between WeeFIM total rating and total amount of assistance rating (r = 0.91). CONCLUSION: Total WeeFIM instrument ratings and severity of disability were the best predictors of amount of assistance ratings provided by parents and teachers.

Activities of Daily Living↗
PubMedDate not supplied

Multivariate risks among extremely premature infants.

This study was designed to identify risk factors for mortality and morbidity of extremely premature infants born in the surfactant era. The study cohort included 194 infants born at < 29 weeks' gestation at one regional tertiary center between 1983 and 1986. Forty-one infants died. Blinded neurodevelopmental assessments were performed on 149 of 153 (97%) survivors at a mean age of 52 months. Thirty-one (21%) survivors had major impairments: 15 had mental retardation, 8 had multiple impairments, 7 had cerebral palsy, and 1 was blind. Logistic regression analysis identifies five significant risk factors for mortality: grade III or IV intraventricular hemorrhage, birth weight < 800 gm, 5-minute Apgar score < or = 3, male sex, and absence of surfactant therapy. Significant risk factors for any major impairment included sepsis (relative risks [RR] = 6.4), male sex (RR = 3.1), and nonwhite race (RR = 2.8). Hydrocephalus requiring shunting was a significant risk factor for cerebral palsy (RR = 16.4) and neonatal retardation (RR = 16.0). Nonwhite race (RR = 7.3), sepsis (RR = 6.8), and male sex (RR = 3.7) also were significant risk factors for mental retardation. Confirmation of these risk factors should facilitate development of targeted interventions for optimizing long-term outcome.

Blindness↗