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Biomedical subjects

R Kennedy

Publications and source records attributed to R Kennedy.

At least 19 recordsLinked to original sources

Buschke-Ollendorff syndrome associated with elevated elastin production by affected skin fibroblasts in culture.

Buschke-Ollendorff syndrome (BOS; McKusick 16670) is an autosomal dominant connective-tissue disorder characterized by uneven osseous formation in bone (osteopoikilosis) and fibrous skin papules (dermatofibrosis lenticularis disseminata). We describe two patients in whom BOS occurred in an autosomal dominant inheritance pattern. The connective tissue of the skin lesions showed both collagen and elastin abnormalities by electron microscopy. Cultured fibroblasts from both patients produced 2-8 times more tropoelastin than normal skin fibroblasts in the presence of 10% calf serum. Involved skin fibroblasts of one patient produced up to eight times normal levels, whereas apparently uninvolved skin was also elevated more than threefold. In a second patient, whose involvement was nearly complete, elastin production was high in involved areas and less so in completely involved skin. Transforming growth factor-beta 1 (TGF beta 1), a powerful stimulus for elastin production, brought about similar relative increases in normal and BOS strains. Basic fibroblast growth factor, an antagonist of TGF beta 1-stimulated elastin production, was able to reduce elastin production in basal and TGF beta 1 stimulated BOS strains. Elastin mRNA levels were elevated in all patient strains, suggesting that Buschke-Ollendorff syndrome may result, at least in part, from abnormal regulation of extracellular matrix metabolism that leads to increased steady-state levels of elastin mRNA and elastin accumulation in the dermis.

Blotting, Southern

Scapulothoracic motion in normal shoulders and shoulders with glenohumeral instability and impingement syndrome. A study using Moiré topographic analysis.

Qualitative visual inspection and manual muscle testing are traditional methods of evaluation that may overlook subtle weakness of the axioscapular musculature. A modification of the standard technique of Moiré topographic analysis of spinal deformity was applied to assess axioscapular muscle function in 51 subjects: 22 asymptomatic individuals, 22 with shoulder instability, and seven with impingement syndrome. Static Moiré evaluation demonstrated scapulothoracic asymmetry or increased topography in 14% of asymptomatic subjects, compared with 32% and 57% in the instability and impingement groups, respectively. The dynamic Moiré test demonstrated an abnormal Moiré pattern in 18% of asymptomatic individuals, compared with 64% and 100% in the instability and impingement groups, respectively. Axioscapular muscle dysfunction is common with both instability and impingement syndrome of the shoulder, although it remains to be determined whether this represents a primary or secondary phenomenon.

Adult

Induction of antibodies to the envelope protein of the human immunodeficiency virus by immunization with monoclonal anti-idiotypes.

Anti-idiotypes that possess the internal image of antigen can induce protective humoral immunity toward microbes. Herein we demonstrate antigen mimicry by monoclonal anti-idiotypes of a distinct epitope of the human immunodeficiency virus (HIV) envelope protein that is defined by a synthetic peptide. This peptide, corresponding to amino acid residues 503-535 (peptide 503-535) of HIV-1 IIIB gp160, induced antibodies in three mammalian species that interacted with HIV-1 gp120 and inhibited in vitro syncytium formation caused by HIV-1, IIIB and MN isolates. Three monoclonal anti-idiotypes were generated against rabbit anti-gp120 antibodies specific for peptide 503-535. These anti-idiotypes recognize an interspecies cross-reactive idiotype expressed on mouse, chimpanzee, baboon, rabbit, and human anti-gp120 antibodies specific for peptide 503-535. The interaction with the cross-reactive idiotype is inhibited by synthetic peptide and HIV-1 gp160. Furthermore, rabbits immunized with the monoclonal anti-idiotypes produced antibodies that also bind HIV-1 gp120 and gp160 and recognized the epitope defined by peptide 503-535.

Amino Acid Sequence

Prevalence of maternal HIV infection in Scotland based on unlinked anonymous testing of newborn babies.

Dried blood spot samples from newborn babies have been successfully tested for HIV-1 antibody by the particle agglutination method to assess the prevalence of infection in the mothers. In January, 1990, unlinked anonymous testing of Guthrie cards for HIV antibody was begun in Scotland. 99.6% of Scottish births were tested. 9 mothers spontaneously refused to allow testing of their baby's blood. Samples were coded by district postcodes. Eluates of 65,773 dried blood spots were initially tested for HIV-1 antibody with the Fujirebio technique. Of the 31 positive samples 19 were confirmed to be positive by enzyme-linked radioimmunoassay and western blot (seroprevalence 0.29 per 1000). All these samples came from large metropolitan areas on the east coast. Prevalences were 2.5 per 1000 for Edinburgh city, 1.4 per 1000 for Dundee, and 0.7 per 1000 for Aberdeen. We identified as HIV-positive all babies known to be so in named testing programmes. HIV testing of Guthrie cards can be used to monitor HIV status in mothers who have just given birth. The use of district postcode data in sample identification will allow accurate targetting of prevention strategies and early detection of spread of infection by geographic area.

Agglutination Tests

Working group recommendations: research on content and efficacy of geriatric evaluation and management interventions.

Methods of conducting comprehensive geriatric evaluation and management (GEM) are proliferating in a variety of clinical settings. However, rigorous evaluations of efficacy for this new approach to care of older patients have demonstrated a favorable impact on patient outcome in only a few studies. All of these have been controlled single site studies, and replication is needed. If replication studies show similar results, further studies should be undertaken to define the minimum necessary intervention to achieve the desired outcome. Controlled trials are needed to determine if consultative geriatric evaluation and/or primary patient management is effective. Further innovative work is needed in model development for geriatric assessment and management in outpatient settings. Finally, studies of geriatric evaluation and management in other environments, such as home care or the nursing home, are recommended.

Forecasting

Child physical abuse observed. Comparison of families with and without history of child abuse treated in an in-patient family unit.

Twenty-seven families, 14 with a history of child physical abuse and 13 with no such history, were studied over the course of intensive in-patient treatment. The families in the former group differed significantly from those in the latter group in terms of current circumstances and background histories. Families where abuse was admitted benefited significantly more from treatment than families where abuse was suspected but not admitted. The ability of mothers to remember good relationships from childhood and to establish good relationships during treatment was an important prognostic factor for successful treatment.

Adult

Development of an optically scanned consultation-liaison data base.

Every clinical service must record certain data about its patient care activities. In low-volume services, such as Psychiatric Inpatient Services, with perhaps 20-30 admissions per month, and an average patient stay of 2-4 weeks, a large amount of data on each patient can be obtained during the contact time with the patient. On the other hand, very large-volume psychiatric services, such as Emergency and Consultation-Liaison (C-L) Services, may not need or be able to gather such a large amount of data on every patient seen. This article describes the development of a brief, optically scannable, and computerized minimal data base form for patients seen by a very large division of C-L Psychiatry. The system is feasible and easily auditable for completeness and reliability. This data base has already served many important functions beyond providing an administrative statistical summary of services rendered. It is presented as a model for the development of similarly efficient data collection methods for other high-volume psychiatric services.

Computer Systems

The geriatric auditory and vestibular systems.

Age-related declines in auditory and vestibular function are becoming more prevalent and better understood. The personal and societal impact of these disorders is impressive, and present techniques of amplification or balance training can be of great benefit. However, many issues await investigation, and continued integration of ever-increasing technologies promises many new answers, and questions, in the future.

Aging

A severe form of breakdown in communication in the psychoanalysis of an ill adolescent.

The paper focuses on a particularly severe kind of breakdown in communication that can arise in the psychoanalysis of ill adolescents who have experienced a real breakdown in functioning, such as a severe suicide attempt or one or more psychotic breakdowns. Clinical material is presented from the analysis of a suicidal and self-mutilating adolescent as an illustration of the theme. Included is a discussion of different types of breakdown in communication.

Adolescent

Transforming growth factor-beta stimulates wound healing and modulates extracellular matrix gene expression in pig skin. I. Excisional wound model.

The effect of transforming growth factor-beta 1 (TGF-beta 1) on matrix gene expression has been investigated during the process of wound repair, where the formation of new connective tissue represents a critical step in restoring tissue integrity. Split-thickness excisional wounds in the pig were studied by in situ hybridization in order to obtain subjective findings on the activity and location of cells involved in matrix gene expression after the administration of recombinant TGF-beta 1. Data focus on the stimulatory role of this growth factor in granulation tissue formation, on the enhanced mRNA content of collagen types I and III, fibronectin, TGF-beta 1 itself, and on the reduction in stromelysin mRNA, suggesting that increased matrix formation measured after treatment with TGF-beta 1 is due to fibroplasia regulated by the abundance of mRNAs for several different structural, matrix proteins as well as inhibition of proteolytic phenomena elicited by metalloproteinases. These studies reveal elastin mRNA early in the repair process, and elastin mRNA expression is enhanced by administration of TGF-beta 1. Moreover, we show that TGF-beta 1 was auto-stimulating in wounds, accounting, at least in part, for the persistent effects of single doses of this multipotential cytokine.

Animals

Joubert's syndrome associated with congenital ocular fibrosis and histidinemia.

We describe a 16-month-old girl with Joubert's syndrome (JS), congenital ocular fibrosis, and histidinemia. Abnormal respiration, ptosis, and minimal eye movements were observed in the neonatal period. Intraoperative examination of the eyes later demonstrated severely restricted eye movements and abnormal insertions and fibrosis of the extraocular muscles. Computed tomography of the head revealed absence of the corpus callosum and brain stem. Histidine levels were elevated in the blood, urine, and cerebrospinal fluid. The patient was ataxic and developmentally delayed. To our knowledge, the association of JS with congenital ocular fibrosis has not previously been described. This report indicates that jerky eye movements are not an invariable finding in JS.

Ataxia