Biomedical subjects
Publications and source records attributed to M Valdes-Dapena.
The last decade has witnessed a remarkable evolution in fundamental concepts regarding SIDS. It begins to appear now that the phenomenon is more likely the remote consequence of a subtle, physiologic birth defect than a single catastrophic incident. One can only hope that the next 10 years will continue to shed more light on this gradually resolving mystery.
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In the past two decades, two groups of investigators have alleged that infants who die of the sudden infant death syndrome (SIDS) have excess numbers of sclerotic glomeruli in their kidneys. This double blind, case-control study was undertaken to test that assertion. Using microscopic sections of the kidney from 153 autopsies (99 SIDS infants and 54 control infants) in the National Institute of Child Health and Human Development (NICHD) Cooperative Epidemiologic Study of Risk Factors for SIDS, we counted relative numbers of sclerotic glomeruli in four fields of renal cortical tissue in two sections from each infant. Our results indicate that there is no difference between the two groups in regard to the proportion of sclerotic glomeruli.
The ever-changing complexion of iatrogenic perinatal disease dictates the following: 1. Neonatologists and pediatric pathologists must be aware of the current spectrum of these lesions and ever alert to the appearance of new ones. 2. The neonatologist and pediatric pathologist within any given institution should be in regular, systematic communication with each other concerning lesions of this type. 3. There must be a national network for rapid dissemination or exchange of information among institutions regarding these lesions. The Study Group for the Complications of Perinatal Care (SGCPC), established in 1984 as an international, multicenter, multidisciplinary study group, is committed to the prevention of complications of perinatal care by individual and collective effort. Its individual members include perinatologists, neonatologists, pediatric pathologists, and obstetricians. There are institutional members as well. Activities to date include the development of a standardized perinatal autopsy protocol and the initiation of a uniform system for the categorization of perinatal deaths. If the reader is interested in obtaining further information about the organization, contact Trevor Macpherson, MD, in the Department of Pathology at the Magee-Womens Hospital in Pittsburgh, Pennsylvania.
The damaging effects of low and declining autopsy rates on the discipline of pediatric pathology are discussed. A survey of autopsy rates in 25 children's hospitals indicates that current rates are about 51%. Strategies for improving autopsy rates are presented.
We present a case of the Goldston syndrome--that is, cystic renal dysplasia and the Dandy-Walker malformation. The condition was diagnosed by ultrasound in a 635 g fetus in the seventeenth week of gestation. Ultrasound studies showed the fetal head to be somewhat enlarged with slight dilatation of the lateral ventricles and marked dilatation of the fourth ventricle. The kidneys were symmetrically enlarged and multicystic. Autopsy revealed evidence of the oligohydramnios syndrome. The kidneys were typical of the Goldston syndrome as were the microscopic lesions in the liver. To our knowledge this is the first reported case in which this diagnosis was made during intrauterine life.
The faculty of the Department of Pathology at the University of Miami School of Medicine has developed a senior elective program in anatomic pathology for fourth-year students. The program is designed in such a way that the fundamental concepts involved will be easily remembered and clinically useful. The teaching methods, which maximize active learning, include the students' group resolution of challenging unknown autopsy cases, participation in the performance of necropsies, individual presentation of one of their own autopsy cases to the group, and work with several interactive videodisc programs. The program has proved to be both effective and popular. It could be reproduced with relative ease in any academic department of pathology.
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Interesting new information concerning SIDS infants has appeared in the medical literature in recent years. It seems that the unique curve for their ages at death is independent of all other risk factors suggesting that, in most cases, there is probably an important mechanism involving growth and development. Furthermore, some SIDS deaths occur among newborns and comprise as much as 10% of all neonatal mortality, an issue heretofore systematically excluded from consideration. Until now, racial differences in SIDS rates were usually attributed to differences in socioeconomic status. Recent data concerning impoverished Hispanics in this country appear to indicate that cultural factors must play some role as well. Pediatric pathologists have demonstrated a higher incidence of dysmorphic, dysplastic, and minor anomalous lesions as well as cytomegaloviral infection among SIDS babies than among autopsied control infants. These observations suggest that the former have experienced adverse influences prenatally which may make them peculiarly vulnerable to postnatal challenges. One study indicates that virtually all SIDS victims have higher than normal levels of fetal hemoglobin which fact, if confirmed, may permit identification of the potential victim before the fact. Finally, the multicenter NICHD study has shown that only 39.8% of 757 crib death babies had received DTP inoculation as compared to 53.2 and 55% of two control groups of living infants. In fact, one conclusion of the study might be that DTP immunization is likely to protect an infant against crib death.
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A unique occurrence of antenatal thrombosis of the left main coronary artery in a term infant is presented. The clinical features are indistinguishable from those of several other forms of congenital heart disease. The etiology of the thrombosis is unknown.
Multiple unusual diverticula of the bladder were observed in 3 of 4 children with Menkes' syndrome. This abnormality of the bladder in children with the kiky hair syndrome has only recently been recognized. The diverticula are best visualized on cystographic studies. The clinical manifestation which led to roentgen evaluation of the urinary tract in the 3 children was urinary tract infection or urine retention. Though the etiology of these diverticula is unknown, evidence is presented to suggest that they are acquired.
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