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Biomedical subjects

J B Arey

Publications and source records attributed to J B Arey.

11 recordsLinked to original sources
PubMed1976

Yellow pulmonary hyaline membranes.

Yellow pulmonary hyaline membranes were observed at autopsy in 16 newborn infants between 1972 and 1974 in four hospitals of Philadelphia, Pa., and Newark, N.J. Other pediatric pathologists in this country and in Spain have seen the same lesion within the last decade. Chemical analysis of affected lung tissue, histochemistry, and electron microscopy show the yellow color to be due to the presence of bilirubin. No substantial clues concerning the basic etiology or mechanism for the formation of these unique membranes emerge from a detailed review of clinical and postmortem data nor from comparison of these data with those for 68 control infants with the usual acidophilic pulmonary hyaline membranes. We are left, however, with the impression that prolongation of life, relatively elevated levels of serum bilirubin, and protracted assisted ventilation (with either CPAP or PEEP) are intimately related to their formation.

Bilirubin↗
PubMedDate not supplied

The lipidoses: morphologic changes in the nervous system in Gaucher's disease, GM2 gangliosidoses and Niemann-Pick disease.

The present paper presents, in tabular form, most of the inborn errors of lipid metabolism (exclusive of the hyperlipoproteinemias); some may, with further studies, be removed from this category. Three of the lipidoses and their subtypes which are associated with severe neurologic disorders are discussed, i.e., infantile Gaucher's disease, Niemann-Pick disease and the GM2 gangliosidoses. Particular emphasis is placed on the importance of careful biochemical and enzymatic studies of either surgical or autopsy material of any patient suspected of having one of the lipidoses. Only by such studies can an exact diagnosis of virtually all of these inborn errors of lipid metabolism be established. Such a diagnosis is important, since in many instances an antenatal diagnosis is possible by demonstration of the enzymatic defect in cell grown in tissue culture from the amniotic fluid.

Brain↗