Biomedical subjects
Publications and source records attributed to T Kastner.
The case of an adolescent with severe mental retardation, blindness, and a complex of behavioral symptoms consistent with mania is reported. Symptoms include an increased activity level, mood liability, irritability, hyposomia, and severe self-injurious behavior. The successful use of verapamil and valproic acid in the treatment of prolonged mania in this child is described.
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Fragile X syndrome is reported to be the most common inherited cause of mental retardation known, but the majority of affected individuals are as yet undiagnosed. The project described in this paper was developed to increase the public and professional awareness of fragile X syndrome in the state of New Jersey. As a result there were increased efforts at diagnostic screening, provision of client and family support services, and prevention. This educational program proved to be a cost-effective method for increasing community awareness of a genetic disease on a statewide level.
Pinworm, a common parasitic infestation, can be a chronic nuisance in community and institutional residences for people with developmental disabilities. Asymptomatic human carriage of the parasite and its eggs often prevents eradication from a communal residential setting. The elimination of pinworm from a system of community residential settings through a public health approach was described.
Carbamazepine-induced hyponatremia has been reported in 21.7% of 61 patients with mental retardation who received the medication for a variety of reasons. We studied 40 patients with mental retardation receiving carbamazepine to determine the prevalence of hyponatremia. Overall, hyponatremia was found in only 5.0% of these patients. Correlations with sodium level and carbamazepine dose, serum drug level, and concomitant neuroleptic and anticonvulsant polytherapy were also examined. Treatment with carbamazepine resulted in a statistically, but not clinically, significant decrease in serum sodium levels in patients receiving anticonvulsant polytherapy. Decreases in serum sodium were not related to carbamazepine dose or blood levels. Only one patient with underlying schizophrenia and psychogenic polydipsia demonstrated clinically significant hyponatremia during carbamazepine therapy.
The incidence of carbamazepine-associated behavioral side effects in 65 individuals with mental retardation and additional seizure and/or psychiatric or behavioral disorders was evaluated. We identified 6 patients (9.2%) who experienced medication side effects, ranging from irritability to mania. Four of the 20 patients (20%) who received carbamazepine purely for treatment of a behavioral or psychiatric disorder experienced medication side effects, whereas none of the 21 patients treated for an isolated seizure disorder experienced similar effects. This difference was statistically significant, p less than .05. The incidence of behavioral side effects of medication was not associated with age, sex, or serum carbamazepine level. The chemical structure and mechanism of carbamazepine use in various disease processes were discussed.
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Controversy exists regarding immunization with pertussis vaccine of high-risk special care nursery graduates. Our study compared the immunization status of 38 high-risk, 30 low-risk, and 57 normal infants utilizing an immunization response form. Comparison of the three infant groups revealed that complete or incomplete immunization varies, depending on group status. Of 38 infants at high risk for developmental delay, 22 (57.9%) were not immunized against pertussis by age one. Physicians cited concerns related to previous neurologic injury and increased liability for vaccine-related injury as major reasons for deferring vaccination. Recommendations for improving current guidelines and practice are outlined.
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Access to medical care for people with developmental disabilities is a growing problem. The authors review the components of a health care delivery system to help meet the special medical needs of this population.
Persons with mental retardation sometimes exhibit behaviors that are difficult to control. Use of neuroleptic medications may be limited by side effects or ineffectiveness. Beta blockers such as propranolol and metoprolol have been shown to decrease aggressive and impulsive behaviors in some patients with mental retardation.
The development of procedural guidelines for serving people with developmental disabilities living in the community and infected with HIV was discussed. The need for ethical decision-making and universal infectious disease recommendations that allow for the provision of these services in the "least restrictive" environment was emphasized.
We admitted an adult with mental retardation and an eating disorder to a pediatric inpatient unit. Child life services were a core feature in the assessment and treatment of the patient. The role of child life services in the care of persons with mental retardation should be expanded.
A group of 138 community-based patients with Down syndrome were examined for evidence of autoimmune thyroid dysfunction at the time of their referral for routine health care services provided as part of a model program. Twenty-eight patients (20.3%) were found to have previously unrecognized hypothyroidism, and 2 patients (1.4%) had previously unrecognized hyperthyroidism. In addition, 66 patients were tested for thyroid autoantibodies, and 26 were found to have positive antimicrosomal and/or antithyroglobulin antibody test results. There was no statistically significant association between age or sex and the mean thyrotropin value or the presence of thyroid autoantibodies. The relationship between the mean thyroxine level and sex was mildly significant. Of the patients with hypothyroidism, 78.5% were female, and most were between the ages of 30 and 50 years. However, a higher-than-expected number of patients with autoimmune hypothyroidism were under age 30 years. These findings highlight the lack of adequate health care services available to persons with Down syndrome who live in the community. All persons with Down syndrome must undergo regular clinical and laboratory screening for the presence of thyroid disease.
Persons with developmental disabilities living in the community have a greater number and variety of health care needs than the average population of the same age and sex. The erroneous assumption that the generic health care system would be able to provide all necessary services to the large number of individuals recently transferred from state residential facilities to the community has proved to be an unexpected disappointment to human service policymakers. In an effort to remedy this situation, a program of health care services was established by the New Jersey Department of Human Services at a community teaching hospital to supplement the existing generic system of medical care. Within four years, the program had rapidly grown to provide care for 729 patients who had come to rely on the center for primary care, specialty medical and dental services, and medical case management. The demographic characteristics of this program are described as well as data on morbidity, service utilization, and special problems encountered when care was provided to this complex and medically underserved population.
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Eighty-three noninstitutionalized patients with Down's syndrome, aged 9 to 55 years, were randomly selected to receive echocardiograms. Forty-one patients had echocardiographic findings indicative of mitral valve prolapse, and 15 of these patients lacked associated auscultatory findings. Because mitral valve prolapse can predispose patients to bacterial endocarditis after bacteremia-producing dental procedures, these findings suggest that if auscultatory findings alone are used, a significant number of patients with Down's syndrome who are at risk for endocarditis may not be currently identified in the course of routine clinical practice.