Jay Bernstein

Congenital abnormalities of the urinary system. I. A postmortem survey of developmental anomalies and acquired congenital lesions in a children's hospital.

A N O M A L I E S of the genitourinary tract occur in approximately 10 per cent of the population and account for a third of all congenital malformations?, 2 The incidence, classification, and morphology of urinary tract anomalies have been the subjects of extensive studies} -6 Detailed histologic investigations of renal malformations have provided information about their morphogenesis 7, 8; cystic conditions, in particular, have been studied by serial reconstruction 9 and microdissection? ~ As an initial step in studying congenital abnormalities of the urinary tract and their morphogenesis we have undertaken a survey of postmortem material at Childrens Hospital of Michigan. Particular attention has been directed toward patterns of malformation in a n at tempt to delineate the effect of one abnormal part on other components of the system and to separate developmental anomalies from secondarily acquired lesions.

Congenital abnormalities of the urinary system

Summary A review of the clinical material in 11newborn infants with renal necrosis has disclosed histories that are most often compatible with shock due to severe asphyxia or blood loss. The necrosis was present in both the cortex and medulla, but no underlying malformations were encountered. Focal, ischemic necrosis, and petechial hemorrhage were commonly present in other organs. Two other infants were observed at approximately 1 month of age, after a similar clinical course in the early newborn period. One developed severe hypertension, and both had severe renal scarring in association with medullary necrosis. Primitive structures, reminiscent of renal dysplasia, developed in association with the healing process and were regarded as a reaction to injury.

Extrapulmonic Stenosis of the Pulmonary Veins

A case is reported of a 6-year-old boy who had developed pulmonary hypertension because of obstruction to the pulmonary venous flow by severe stenosis of the pulmonary veins at their junction with the left atrium. The development of collateral circulation through the bronchial vessels led to early, severe hemoptysis. The course was complicated by the development of pulmonary arterial and venous thrombi and hypertensive arteritis.

Renal cortical and renal medullary necrosis in the first 3 months of life

Renal cortical necrosis, renal medullary necrosis, and combined renal cortical-medullary necrosis result from renal ischemia without vascular occlusion. Renal hypoperfusion and ischemic injury in infants have been ascribed to massive blood loss, hemolytic disease, septicemia, and severe hypoxemia. In a postmortem study we identified 82 cases among 1,638 autopsies during the 20 years between 1970 and 1989 in infants 3 months old or less at the time of death. The frequency of renal necrosis in autopsy cases increased significantly during the last 6 years of the study. The distribution of the renal lesion was cortical in 28, medullary in 23, and combined in 31. Forty infants carried diagnoses of congenital heart disease, 17 of asphysial shock, 9 of sepsis, 3 of infectious myocarditis, 9 of major malformations, 4 of anemic shock, 1 of vascular malformation, and 1 of gastroenteritis and dehydration. A significantly higher proportion of babies with congenital heart disease had cortical involvement. Comparison of clinical characteristics revealed a significantly higher frequency of prematurity, respiratory distress syndrome, bleeding diathesis, and possibly sepsis in the children with congenital heart disease, suggesting that these factors are important in the pathogenesis of the renal lesion. Fourteen infants underwent cardiac catheterization; there was no demonstrable association between the renal lesions and the use of radiographic contrast medium. We conclude that severe congenital heart disease itself is a risk factor for life-threatening renal cortical and medullary necrosis.

Superficial connective tissue tumors in early infancy: A study of fibromatosis and lipoblastomatosis

Eleven infants with tumors originating from connective tissue are described. Seven fit the picture of fibromatosis and four, that of lipomatosis. Despite the histologic pattern of malignancy and, frequently, the critical anatomic locations of these growths, the tumors tend to be surprisingly benign. Metastasis is rare, and invasiveness decreases with age. An extremely conservative approach is indicated.

A note on the development of gaucher cells in a newborn infant

Summary The newborn sibling of a child known to have Gauchers disease had, in addition to the stigmas of Mongolism, clinical enlargement of the liver and spleen and thrombocytopenic purpura. The spleen, at postmortem examination, contained cells which justified the diagnosis of Gauchers disease. The case is regarded as an example of the chronic form of the disease, although present in its very early stages. The histologic observations suggest that the accumulated intracellular material was present as cytoplasmic fibrils, thereby accounting for the characteristic fibrillary appearance of Gaucher cells, and that cellular abnormality preceded the accumulation of lipid in the pathogenesis of the disease.