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Early identification and treatment necessary to prevent malnutrition in children and adolescents with severe disabilities.

Children with severe developmental disabilities frequently have nutrition and growth problems that range from moderate to severe. Because of notable continuing medical concerns and lowered growth expectations, parents and physicians may fail to recognize gradual deterioration in nutritional status before severe medical complications occur. The two cases reported in this article illustrate the need for early identification and treatment to prevent the development of notable morbidity secondary to malnutrition. Children and adolescents who have growth parameters consistently below age norms require assessment and monitoring by a registered dietitian to detect feeding problems and intake changes and to provide early intervention to help prevent negative consequences (eg, dehydration, protein-energy malnutrition, decubitus ulcers, increased rate and duration of infections, and altered bowel motility). An initial assessment should consist of measurement of length or height, weight, triceps, and subcapsular skinfolds; dietary and feeding history and a review of medical history; and biochemical testing as indicated by the medical and dietary histories. Monitoring frequency, which is determined by age, severity of condition, and response to treatment, may vary from weekly to bimonthly.

Adolescent↗

Risk of mental retardation among children born with birth defects.

BACKGROUND: A paucity of epidemiologic research exists concerning the co-occurrence of birth defects and mental retardation (MR). Study of this co-occurrence may yield important clues about the causes of both. OBJECTIVE: To examine the co-occurrence of birth defects and MR, taking into consideration the type of birth defect, level of MR, co-occurrence of MR with other developmental disabilities, and individual and maternal factors. DESIGN: A retrospective cohort study of infants born in the California Central Valley with and without a structural birth defect by 1 year of age, and with or without MR by 7 to 9 years of age. SETTING AND PARTICIPANTS: One-year survivors (N = 119 556) born in nonmilitary hospitals in 8 California counties between January 1, 1992, and December 31, 1993, for whom information about birth defects was recorded within the first year of life. MAIN OUTCOME MEASURE: Diagnosis of MR by age 7 years considered as being mild or severe and as occurring without other developmental disabilities (isolated MR) or as occurring with other developmental disabilities, including cerebral palsy, epilepsy, or a pervasive developmental disorder. RESULTS: Children with birth defects were nearly 27 times more likely to have MR by 7 years of age compared with children without a diagnosed birth defect regardless of type of defect (prevalence ratio, 26.8; 95% confidence interval, 22.7-31.7). Among those with birth defects, children with Down syndrome (prevalence ratio, 211.7; 95% confidence interval, 171.3-261.5) and children with sex chromosomal defects (prevalence ratio, 57.4; 95% confidence interval, 23.7-138.6) were at the highest risk for MR. Children with nonchromosomal defects, including central nervous system defects and all types of organ and system defects, were at substantially increased risk for all levels of MR. Risks of MR among children with Down syndrome and nonchromosomal defects were not substantially altered when adjusted for individual and maternal factors. CONCLUSIONS: Children with chromosomal and other structural birth defects are at a substantially increased risk for having MR by 7 years of age compared with children born without a birth defect. Children with birth defects are at an especially increased risk for having severe MR and MR occurring independently of other developmental disabilities.

California↗

Long-term developmental outcomes of children identified through a newborn screening program with a metabolic or endocrine disorder: a population-based approach.

OBJECTIVE: To conduct surveillance of the developmental status of children who screen positive and are diagnosed with a metabolic or endocrine disorder. STUDY DESIGN: The Centers for Disease Control and Prevention linked three data sources in Georgia: (1) Metropolitan Atlanta Developmental Disabilities Surveillance Program (MADDSP), (2) Special Education Database of Metropolitan Atlanta (SEDMA), and (3) State of Georgia Newborn Blood-Spot Screening Program (NBSP). RESULTS: When MADDSP and NBSP were linked (birth cohorts 1981-1991), of an estimated 147 infants who screened positive for a metabolic or endocrine disorder and were at risk for mental retardation if left untreated, only three children were identified with mental retardation. When SEDMA and NBSP were linked (birth cohorts 1981-1995), of an estimated 216 children who screened positive for a metabolic or endocrine disorder, nine children were identified as having a developmental disability less severe than mental retardation, eg, speech and language impairments. CONCLUSIONS: Although children found in MADDSP or SEDMA have a low occurrence of developmental disabilities attributable to these metabolic or endocrine disorders, our finding of cases of developmental disabilities of varying severity attributable to a metabolic or endocrine disorder suggests a need for ongoing population-based monitoring of the long-term developmental outcomes of children identified through newborn screening programs.

Data Collection↗

[Microcephalic children without mental retardation].

8 microcephalic children, mean age 3.06 years, with head circumferences -3.5 SD to -6 SD, achieved IQ scores of 79-115 on the Thermann, WPPSI, WISC-R, and/or Columbia tests. They had no dysmorphic features nor congenital anomalies, and their fundi were normal. 7 had congenital microcephaly, associated with proven autosomal dominant heritage in 3, 1 had suspected autosomal recessive heritage, 1 had intrauterine growth retardation (overall smallness), 1 had cerebral palsy, and in 1 there was an unknown prenatal cause. 6 of these children had 1 or 2 developmental disabilities, including neurosensory deafness, cerebral palsy, short attention span, perceptual dysfunctions, and learning disability. Microcephaly, most probably secondary to malnutrition in early infancy, was diagnosed in 1 child without developmental disabilities. However, our data support the concept that some microcephalic children with significantly small head circumference, who function without mental retardation, have a high incidence of other developmental disabilities.

Child↗

Parental and pediatric trainee knowledge of development.

This study evaluated the fund of knowledge about normal development and developmental disabilities of parents and pediatric residents. A 23-item questionnaire was administered to 91 parents of children who were being evaluated at the Division for Disorders of Development and Learning (DDDL) and to 20 pediatric residents at the University of North Carolina (UNC). The physicians-in-training were provided with an additional 26 questions on development, as well as a rating scale of "personal comfort" in discussing three specific developmental disabilities with parents (epilepsy, hyperactivity, and mental retardation). On the series of identical questions, pediatric residents scored significantly better than parents; the mean number correct was 19.4/23 (residents) versus 15.9/23 (parents). However, in both groups there were notable errors. Incorrect responses by 25% or more of parents and/or physicians were labeled "common misconceptions." Sixteen parent and 15 physician common misconceptions were identified in the areas of normal development and developmental disabilities. The pediatric residents revealed greater comfort in discussing the medical problem (epilepsy) than the two developmental problems (mental retardation and learning disabilities/hyperactivity). The study revealed important and similar gaps in the pediatric trainees' and parents' knowledge of development. These deficiencies need to be addressed in the training of pediatric residents in order to help them better understand the needs of families of developmentally disabled and normal children.

Adult↗

Mental retardation following diagnosis of a metabolic disorder in children aged 3-10 years--metropolitan Atlanta, Georgia, 1991-1994.

One of the largest population-based disease intervention programs in the United States is newborn metabolic screening. Since the mid- to late 1970s, newborns have been screened routinely for one or more metabolic disorders. The goal of early identification and treatment of metabolic disorders is prevention of the serious medical and developmental consequences of the disorders (e.g., mental retardation [MR]). Despite this goal, the United States has no mechanism for systematic surveillance of the developmental status of children who screen positive for and subsequently have metabolic disorders diagnosed. To determine the number of selected developmental disabilities attributable to metabolic disorders detected by newborn screening, CDC conducted a preliminary investigation of children with developmental disabilities and metabolic disorders in the metropolitan Atlanta area using data from the Metropolitan Atlanta Developmental Disabilities Surveillance Program (MADDSP). This report summarizes the results of this investigation, which indicate that newborn screening is highly effective in reducing the burden of MR associated with these disorders.

Child↗

Causes of death in remote symptomatic epilepsy.

OBJECTIVE: To determine the causes of death of individuals with developmental disabilities that occur more frequently among those with remote symptomatic epilepsy (i.e., epilepsy occurring in persons with developmental delay or identified brain lesions) than for those without. METHODS: The authors compared causes of mortality in persons with (n = 10,030) and without (n = 96,163) history of epilepsy in a California population of persons with mild developmental disabilities, 1988 to 2002. Subjects had traumatic brain injury, cerebral palsy, Down syndrome, autism, or a developmental disability with other or unknown etiology. There were 721,759 person-years of data, with 2,397 deaths. Underlying causes of death were determined from the State of California's official mortality records. Cause-specific death rates and standardized mortality ratios (SMRs) were computed for those with and without epilepsy relative to subjects in the California general population. Comparisons were then made between SMRs of those with and without epilepsy, and CIs on the ratios of SMRs were determined. RESULTS: Death rates for persons with epilepsy were elevated for several causes. The greatest excess was due to seizures (International Classification of Diseases-9 [ICD-9] 345; SMR 53.1, 95% CI 28.0 to 101.0) and convulsions (ICD-9 780.3; SMR 25.2, 95% CI 11.7 to 54.2). Other causes occurring more frequently in those with epilepsy included brain cancer (SMR 5.2, 95% CI 2.2 to 12.1), respiratory diseases (SMR 1.7, 95% CI 1.2 to 2.5), circulatory diseases (SMR 1.3, 95% CI 1.0 to 1.7), and accidents (SMR 2.7, 95% CI 1.9 to 3.7), especially accidental drowning (SMR 12.8, 95% CI 7.0 to 23.2). CONCLUSIONS: Remote symptomatic epilepsy is associated with an increased risk of death. Seizures, aspiration pneumonia, and accidental drowning are among the leading contributors.

Adolescent↗

Development of low- and high-functioning autistic children.

Aspects of developmental sequences and structures were assessed in low- and high-functioning autistic and non-autistic developmentally disabled children. Specific developmental issues examined included sequences, regressions and profiles. Classification into the high- or low-functioning groups was based on a full scale IQ cutoff of 50. In general, there were few differences in the sequences of development among the groups. However, the autistic children were more likely than non-autistic children to display developmental regressions and unevenness across developmental domains. These developmental peculiarities were more pronounced in the low- as compared to high-functioning autistic children. These findings are discussed with regard to issues of developmental processes, classification and autism.

Autistic Disorder↗

Life course impacts of parenting a child with a disability.

We contrasted parents who had a child with a developmental disability, a serious mental health problem, and a normative comparison group with respect to parental attainment and well-being at mid-life. Data are from the Wisconsin Longitudinal Study, collected when the respondents were 18, 36, and 53 or 54, on average. Although similar at age 18, group patterns of attainment and well-being diverged thereafter. Parents of a child with a developmental disability had lower rates of employment, larger families, and lower rates of social participation but were similar to parents without a child with a disability in educational and marital status, physical health, and psychological well-being. Parents whose child had a serious mental health problem had normative patterns of educational and occupational attainment and marriage, but elevated levels of physical symptoms, depression, and alcohol symptoms at mid-life.

Child↗

The adolescent with a learning disability: a developmental perspective.

The psychosocial difficulties that usually accompany learning disabilities are examined from a framework of developmental theory, particularly that of Erik Erikson. The implications of this perspective for treatment of adolescents with learning problems is discussed, and a specific program described.

Achievement↗

Validation of an early language milestone scale in a high-risk population.

Detailed language evaluations were obtained by interviewing the parents of 191 healthy children aged 0 to 3 years, and by testing the children themselves. From these data, normative values were derived for 41 language milestones in the first 36 months of life. These values were used to construct the Early Language Milestone Scale (ELM Scale), a brief language assessment tool suitable for use by general pediatricians. Physician use of the ELM Scale in a population of 119 children considered at high risk for the presence of developmental disability yielded 97% sensitivity and 93% specificity for the ELM Scale as a detector of developmentally delayed children, when compared with more formal developmental measures as applied by a clinical psychologist or speech pathologist. Early language milestones are a sensitive indicator of developmental integrity; delayed achievement of early language milestones strongly suggests the presence of a significant underlying developmental disability. The ELM Scale may be adopted as a valid measure of developmental status among children considred at high risk for the presence of developmental disabilities.

Cerebral Palsy↗

New patterns of community care: coordinated services for dually diagnosed adults in North Carolina.

North Carolina has developed coordinated care systems for people dually diagnosed with both a mental retardation and a different major mental illness. In response to a class action lawsuit, the state has become a leader in treatment of this form of dual diagnosis. Systems of care for this "Thomas S class" operate in each of 41 area programs for mental health, developmental disabilities, and substance abuse. Networks of care among leaders in mental health and developmental disability promote the coordination of health, housing, social, and vocational services. A survey of 100 area program leaders finds extensive cooperation and a variety of services provided and contracted for, both within and beyond each area program, particularly among developmental disability specialists. Cooperation among leaders is associated with service variety and inter-organizational linkages. The extent of relationships among provider organizations is associated with better access to care. Best practice includes a single portal of entry and inter-agency councils.

Community Mental Health Services↗

The influence of medical history on restorative procedure failure rates following dental rehabilitation.

PURPOSE: This study evaluated the association between patient medical history and the outcomes of restorative procedures performed under general anesthesia. METHODS: The dental records of patients who had dental rehabilitation under general anesthesia at Children's Hospital in Boston (1990-1992) and Children's National Medical Center in Washington, DC (1994-1998) were examined. Data regarding restorative outcomes and the association between patient medical history and restorative failures were assessed using chi-square tests with correction for continuity. T-tests were performed on parametric data. RESULTS: Significantly higher stainless steel crown failure rates were found in young patients diagnosed with developmental disabilities when compared to patients without such disabilities (p<0.025, x2 = 5.50). However, there was no difference in the failure rates of SSCs in young patients with significant medical histories compared to patients without significant medical histories. Regarding amalgam and composite restorations, there were no differences in failure rates among patients with and without significant medical histories, including developmental disabilities. CONCLUSIONS: SSC failures were higher in young children with developmental disabilities compared to children without these disabilities.

Adolescent↗

Treatment of sialorrhea with glycopyrrolate: A double-blind, dose-ranging study.

OBJECTIVE: To determine the safety and efficacy of glycopyrrolate in the treatment of developmentally disabled children with sialorrhea. DESIGN: Placebo-controlled, double-blind, crossover dose-ranging study. SETTING: Outpatient facilities in 2 pediatric hospitals. PATIENTS: Thirty-nine children with both developmental disabilities and excessive and bothersome sialorrhea. MAIN OUTCOME MEASURES: Parent and investigator evaluation of change in sialorrhea and adverse effects. RESULTS: Glycopyrrolate in doses of 0.10 mg/kg per dose is effective at controlling sialorrhea. Even at low doses, 20% of children may exhibit adverse effects severe enough to require discontinuation. CONCLUSIONS: Glycopyrrolate is effective in the control of excessive sialorrhea in children with developmental disabilities. Approximately 20% of children given glycopyrrolate may experience substantial adverse effects, enough to require discontinuation of medication. Arch Pediatr Adolesc Med. 2000;154:1214-1218.

Adolescent↗

Screening for developmental language disability in 3-year-old children. Experiences from a field study in a Swedish municipality.

This study evaluates a screening instrument for identification of severe developmental language disability (DLD) in 3-year-old children, which is used as a routine assessment at several child health centres (CHCs) in Sweden. The results are reported in terms of clinical outcome (false- and true-positive rates), kind and extent of DLD, signs of comorbidity and relation between nurses' and parents' observations. More than 60 CHC nurses, all with experience of the screening instrument, assessed in all 2359 3-year-old children (98% of the whole population) by direct observation of their language comprehension, language production and level of co-operation. In addition to the screening parents answered a questionnaire. Children who failed the screening had their hearing assessed and were clinically examined by trained speech and language therapists. Forty-four (34 boys and ten girls) of the 65 referred children were clinically examined. Apart from two false-positive cases most of them were diagnosed as generally and severely language disabled. According to the nurses' observations attention deficit was common among the referred boys, which was later confirmed by the speech therapist in two-thirds of them. Agreement between nurses and parents was poor and only half of the parents were concerned about their child's language development. In the light of this result, continued application of the screening and the use of parent questionnaires is discussed.

Child, Preschool↗

Correlates of specific childhood feeding problems.

OBJECTIVE: The correlates of specific childhood feeding problems are described to further examine possible predisposing factors for feeding problems. We report our experience with 349 participants evaluated by an interdisciplinary feeding team. METHODS: A review of records was conducted and each participant was identified as having one or more of five functionally defined feeding problems: food refusal, food selectivity by type, food selectivity by texture, oral motor delays, or dysphagia. The prevalence of predisposing factors for these feeding problems was examined. Predisposing factors included developmental disabilities, gastrointestinal problems, cardiopulmonary problems, neurological problems, renal disease and anatomical anomalies. RESULTS: The frequencies of predisposing factors varied by feeding problem. Differences were found in the prevalence of the five feeding problems among children with three different developmental disabilities: autism, Down syndrome and cerebral palsy. Gastro-oesophageal reflux was the most prevalent condition found among all children in the sample and was the factor most often associated with food refusal. Neurological conditions and anatomical anomalies were highly associated with skill deficits, such as oral motor delays and dysphagia. CONCLUSIONS: Specific medical conditions and developmental disabilities are often associated with certain feeding problems. Information concerning predisposing factors of feeding problems can help providers employ appropriate primary, secondary and tertiary prevention measures to decrease the frequency or severity of some feeding problems.

Child↗