James B. Arey

The causes of neonatal mortality." An analysis of 501 autopsies on newborn infants

The major disease processes identified in a series of 501 consecutive necropsies onnewborn infants who died within the first 28 days of life are classified. The data are compared with a similar series of 102 autopsies performed elsewhere and reported in 1953, 1 national statistics on neonatal mortality as presented by the United States Public Health Service, 12 and all of the itemized entries on 501 consecutive death certificates for newborn infants who died in philadelphia in 1966. There comparisons show that vital statistics derived from death certificates of infants dying during the neonatal period must be interpreted with great caution. In certain of the major categories, data so derived do not appear to reflect the actual frequency of these processes as causes of death.

Persistent jaundice in infancy

I have collected the opinions of a number of pediatricians and pediatric pathologists regarding the causes of persistent jaundice during infancy. Most would discard the term “inspissated bile syndrome”, but there is less unanimity regarding the meaning of “neonatal hepatitis”, the significance of giant cells in the liver, and the value of needle biopsies. The many causes of infantile jaundice are mentioned, and hepatic physiology during the neonatal period is discussed. Finally, a program is outlined for the study of the infant with persistent jaundice. The value of the determination of the excretion of radioactive rose bengal in differentiating complete biliary atresia from other causes of jaundice is emphasized.

THE INTESTINAL LESIONS IN CYSTIC FIBROSIS OF THE PANCREAS.

The clinical and pathologic data from 30 infants and children with cystic fibrosis of the pancreas without meconium ileus and from 9 infants with meconium ileus have been reviewed. Pancreatic involvement tended to be less severe in young infants than in older children and was usually mild in the few infants with meconium ileus who were studied post-mortem. Severe involvement of the intestinal glands of the small bowel, on the contrary, was common in infants with meconium ileus. The findings suggest that meconium ileus is dependent primarily, if not solely upon alterations in the intestinal glands of the small bowel.

Boric acid poisoning

The autopsy findings in 3 fatal cases of boric acid poisoning are described in detail. The demonstration of intracytoplasmic inclusion bodies in the acinar cells of the pancreas is considered to have diagnostic significance. The literature on boric acid poisoning is reviewed, and the data relative to 83 fatal and 89 nonfatal cases are tabulated. In addition, various aspects of the subject, such as minimal lethal doses, blood and tissue concentrations, procedures for toxicologic analyses, and modes of absorption and elimination of the drug, are discussed.

Cystic lesions of the kidney in infants and children

Summary Simple renal cysts, perinephric cysts, and multilocular cysts of the kidney are rare in infants and children. They manifest themselves by the presence of an abdominal mass which may be interpreted clinically as a Wilmss tumor. The clinical, pathologic, and hereditary features of true polycystic disease of the kidney appear to be distinct from those of renal hypoplasia, and every attempt should be made to differentiate these two diseases. The cystic kidneys which may be associated with intrauterine urethral obstruction may represent instances of renal hypoplasia and should not be interpreted as true polycystic disease of the kidneys. Similarly, the unilateral multicystic kidney, which manifests itself by the presence of an abdominal mass, is a form of renal hypoplasia and does not represent unilateral polycystic disease of the kidneys. There is evidence to indicate that infantile and adult forms of true polycystic disease of the kidneys represent two independent entities. Further study is necessary before this fact can be established unequivocably.

Unexpected death in early life.

Summary An adequate cause of death has been demonstrated in eighty-five of 103 infants and children who either were dead at the time of arrival in the hospital or died within twenty-four hours after admission; only ten in the entire group can be considered as sudden, unexpected deaths occurring in previously healthy infants. Infections were the leading cause of death, being responsible for forty-two of the 103 deaths; minor infections, such as otitis media or minimal pneumonic processes, were not considered an adequate explanation of the death of an infant or child. The importance of obtaining post-mortem cultures of the blood of infants who die suddenly and unexpectedly is emphasized, and the significance of the presence of different types of microorganisms in post-mortem cultures of the blood is discussed. Congenital malformations, involving especially the heart, were responsible for eighteen of the 103 deaths. In over one-half of these patients the presence of a congenital malformation had been recognized for some time prior to death. Mild cellular infiltrates in the interalveolar septa were not included as a cause of death, nor was aspiration of gastric contents into the trachea. There was no evidence that any of the infants died as a result of suffocation. It is suggested that the term “status thymicolymphaticus” might better be omitted from medical writings.

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.

Observations on some causes of cerebral palsy based on post-mortem findings in newborn infants

Summary Intracranial lesions are an important cause of neonatal death. Similar but possibly less severe lesions occur in the central nervous system of a significant number of infants dying during the neonatal period of other causes. The possible role of such lesions in the development of neurological disorders in later life is briefly discussed. Better understanding of the frequency and of the types of cerebral lesions found in newborn infants should contribute to our knowledge of the pathogenesis of cerebral palsy.