Biomedical subjects
Publications and source records attributed to J E Haas.
A neonate with ususual facial features and multiple congenital malformations expired at 4 hours of age. An autopsy revealed severe anomalies of the gastrointestinal system and spinal dysplasia. Cytogenetic evaluation of fibroblasts cultured from a lung biopsy revealed a karyotope of 47,XX,+20.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Childhood polymyositis and dermatomyositis are rare conditions that are not generally associated with malignancy even though an association between adult-onset and malignancy has been reported. A child is presented with typical polymyositis in whom an immunoblastic sarcoma subsequently became manifest; the literature also is reviewed concerning the association between malignancy and these childhood conditions.
Malignant rhabdoid tumor of the kidney, a clinically and pathologically unique tumor of early childhood, has been recently distinguished from nephroblastoma. Skeletal muscle histogenesis has been considered because of its aggressive course and histologic resemblance to rhabdomyosarcoma. Application of an indirect immunoperoxidase technique for myoglobin, an early marker of myogenesis, failed to show the protein in four malignant rhabdoid tumors of kidney. This substantiates the lack of ultrastructural evidence of skeletal muscle differentiation in these tumors.
Anatomic details of the infantile oropharynx and relationships to possible airway obstruction in sudden infant death syndrome (SIDS) are understood incompletely. Tonkin recognized this in 1975, suggesting that enlargement of the tongue might be important in SIDS, within the context of other anatomic and physiologic factors unique to early life. We therefore conducted a morphometric study of the tongue in 100 victims of SIDS and 36 control infants; the latter group consisted of infants with the same range in age and body size who had grown normally and died acutely. Highly significant differences in tongue weight, width, and thickness were demonstrated between the two groups by linear regression and analysis of covariance. Two multivariate techniques, stepwise discriminant analysis and factor analysis, identified marked discordance between somatic and lingual size in SIDS and a statistical uniqueness in tongue thickness. Certain explanations for lingual enlargement in SIDS were ruled out: redistribution of fluid and other effects of death, postmortem interval, duration and mode of feeding, and smallness of control tongues. At present, the functional significance of these observations is unclear. The effects of both normal and increased tongue size on airway patency are, however, widely recognized. Further studies of the enlarged tongue, addressing such topics as in situ anatomy and developmental mechanisms, are necessary to establish the potential for pathophysiologic consequences in SIDS.
This comprehensive study of organ weights in sudden infant death syndrome (SIDS) should be of use to those studying postmortem data in SIDS and may have application to issues of growth and development. Analysis of data from 500 autopsies, performed over 15 years by one individual, revealed mean body weights generally below the 50th percentile for living infants. The weights of the thymus, lungs, liver, and brain were significantly greater than published norms. Thymic weights in SIDS probably represent the normal state more closely than those reported in several other series; elevated lung weights are presumably due to the intense pulmonary congestion and edema commonly encountered in SIDS; the liver edge routinely extends below the costal margins at SIDS autopsies, but the reason for increased weight is unknown, although a hemodynamic alteration seems likely; elevated brain weight has been described previously in SIDS. Other organs showed trends differing from "normal"--heart weights showed a marginally significant increase above published norms; for the adrenal glands, a uniform decrease was apparent, but slopes of linear regressions were low, hampering statistical analysis. Organ weights correlated more closely with body weight than with age. When victims were classified as "possible SIDS," "probable SIDS," or "classic SIDS," differences in organ weights were rarely significant. Analysis of organ weights using Z scores and their standard deviations (sigma-Z, or "pattern variability index") revealed increased variability in SIDS victims. Explanations for these findings include the possibility that some weights previously published as normal are low due to confounding variables. Changes could also be genuine, resulting from disturbances in growth or physiology, or artifactual, possibly the result of agonal or postmortem changes. A database on carefully selected, previously healthy infants who died suddenly and unexpectedly of known causes (i.e., trauma) is much needed.
Explore the source record for details and available documents.