William A. Blanc

An experimental study of acute neonatal enterocolitis—the importance of breast milk☆

Abstract Formula feeding in conjunction with hypoxia produces enterocolitis in newborn rats. Breast feeding under the same circumstances is completely protective. Enteric overgrowth of potentially pathogenic bacteria in only the formula-fed rats indicates that the gut flora plays an important role in the pathogenesis of enterocolitis. Breast milk through induction of passive enteric immunity and control of intestinal flora protects the rat and may protect at-risk premature infants from acute enterocolitis.

Thromboses due to catheterization in infants and children

A recent 3.5-fold increase in thrombosis of large vessels in autopsies is believed to be related to direct mechanical injury and alteration of flow in catheterized vessels. Although a useful diagnostic and therapeutic tool, the catheter may cause serious complications. In 75 per cent of the cases the “catheter thromboses” were accompanied by infarcts of major organs; in 12 per cent the sequelae of catheterization were considered to be the cause of death.

Myocardial infarcts in infants and children. I. A necropsy study in congenital heart disease

Myocardial infarction is not rare in infants and children. It is common in congenitalheart disease even in the absence of gross coronary anomaly or pathology. Infarcts were found in 33 of 44 hearts in a series including cases of total anomalous venous return, aortic valvular stenosis, pulmonary valvular stenosis, and complete transposition of the great arteries. All hearts of patients older than 3 months showed infarcts, usually located in the subendocardial myocardium and in the papillary muscles of the hypertrophied ventricle. Decreased coronary perfusion and desaturation of systemic blood probably affect both ventricles. The relative coronary insufficiency associated with increased myocardial mass may be responsible for the preferential localization of infarcts in one ventricle: the left in aortic stenosis, the right in pulmonic stenosis and anomalous pulmonary venous return. Only minor coronary artery lesions were found in the series and these did not correlate with the side or severity of infarction. The papillary muscle infarcts may affect the normal function of both atrioventricular valves. A review of electrocardiographic data showed that about half of the infarcts might have been diagnosed clinically.

Hemangioma with Kaposi's Sarcoma-Like Features: Report of two Cases

We describe two children with vascular neoplasms that resembled Kaposis sarcoma in places. Both presented with intraabdominal masses and severe thrombocytopenia. At autopsy the tumors extensively infiltrated the peritoneum and retroperitoneum and surrounded or invaded numerous organs including the kidneys, pancreas, adrenal glands, gastrointestinal tract, mesentery, and lymph nodes in both cases, and spleen or bone marrow in one case each. The neoplasms were histologically identical and displayed two patterns: dilated vascular spaces (angiomatous areas) lined by flat endothelial-like cells and areas of spindle cells forming slitlike vascular spaces similar to those described in Kaposis sarcoma. Tumor cells in both cases expressed markers for endothelial cells. The clinical and histologic character of these neoplasms differentiates them from Kaposis sarcoma, hemangioendothelioma, and from conventional juvenile hemangioma.

Malformations and chromosome anomalies in spontaneously aborted fetuses with single umbilical artery

Presence of a single umbilical artery was noted 13 times among a series of 879 consecutively collected, spontaneously aborted embryos and fetuses. This rate of 1.5% is approximately twice that reported from the largest series of term births. Eight of the 13 fetuses had additional serious malformations. Of nine successfully karyotyped fetuses, six had chromosome anomalies. All six occurred in fetuses with severe malformations in addition to the single umbilical artery. The presence of single umbilical artery with other major anatomic defects may be a significant predictor of the presence of chromosome anomalies in live births. Early recognition of offspring with chromosome anomalies will be useful in counseling the family and in managing the care of the infant.

Abnormalities of the coronary circulation in pulmonary atresia and intact ventricular septum

To identify developmental aspects and establish morphologic criteria useful for clinical recognition of sinusoids and coronary artery abnormalities in pulmonary atresia and intact ventricular septum, we studied 17 autopsy hearts with this lesion. Hearts from age- and weight-matched infants served as controls. There were two stillborn fetuses, 12 full-term neonates, and 3 infants. None of the eight hearts with normal or large-sized right ventricular chambers had sinusoids or coronary artery abnormalities. Five of nine hearts with small right ventricular chambers had sinusoids; all five lacked trabecular and outlet portions of the ventricle. Four of these five contained major coronary artery abnormalities: atrophy of one or both coronaries proximal to a communication between a sinusoid and the coronary artery. We conclude (1) there is a strong association between the presence of sinusoids and coronary anomalies, supporting the developmental concept of abnormal persistence of embryonic right ventricular sinusoid--coronary artery communications in pulmonary atresia and intact ventricular septum; (2) the absence of trabecular and outlet portions of the small right ventricle indicates a strong possibility of coronary artery abnormalities; and (3) this initial report of a fully-developed lesion in a mid-gestation fetus documents that the coronary artery problem may arise early in fetal life.

Ultrastructure of cultivated fibroblasts in cystic fibrosis of the pancreas

Cultivated fibroblasts from patients with cystic fibrosis of the pancreas and from control individuals were investigated by electron microscopy. In both groups, the cytoplasmic granules which by light microscopy were metachromatic appeared in electron micrographs to consist of membrane-bound bodies (lysosomes) containing a variable amount of material compatible with ultrastructural features of phospholipids and mucopolysaccharides. The only difference was quantitative: Cells from patients contained larger and more numerous lysosomes than control cells. On the basis of such findings it is proposed that many tissues in cystic fibrosis are affected by a common defect which impairs cell transport mechanisms and membrane permeability and which can be enhanced in tissue culture. As a result, excessive amounts of metabolites would accumulate in the cells, stimulating lysosomal activity.

Hemangioma of the Umbilical Cord

Tumors of the umbilical cord are extremely rare. Only two types are recognized: hemangioma and teratoma. A case of hemangioma of the cord prompted a review of the world literature that uncovered 17 other cases, bringing the total number of known cases up to 18. Our case is the first to be diagnosed by ultrasonography and was associated with polyhydramnios.

Electrocardiographic changes associated with papillary muscle infarction in congenital heart disease

The electrocardiograms of 18 infants and children with congenital heart disease (4 with aortic stenosis and 14 with total anomalous pulmonary venous connection) and necropsy evidence of infarction of the papillary muscle or free wall, or both, were reviewed. In the presence of left ventricular hypertrophy, a Q wave in lead V3R followed by a small R wave is strong evidence of infarction of the anterior left papillary muscle or anterior left ventricular free wall, or both. When associated with serially diminishing amplitude of the R wave in lead V3R this is diagnostic of infarction. In the presence of right ventricular hypertrophy a Q wave in lead V3R may be due to the hypertrophy. The finding of a serially diminishing R wave in this lead, however, is evidence of infarction of the anterior right papillary muscles or anterior right ventricular free wall. Hence, with left ventricular hypertrophy or right ventricular hypertrophy a Q wave in lead V3R assumes diagnostic significance for infarction when accompanied by a diminishing R wave amplitude over the right precordial leads.

Ganglion cells in metastatic Wilms' tumor. Review of a histogenetic controversy.

ABSTRACT Ganglion cells were found in pulmonary metastases of a Wilms tumor following chemotherapy and irradiation of the lungs. This unusual finding prompted a review of similar observations and a consideration of possible explanations for the unexpected presence of neural elements in a tumor which is generally believed to originate from metanephric blastema.