Lactic acidosis associated with cerebellar vermal atrophy and cardiomyopathy.

The association of fluctuating neurological signs and congestive cardiomyopathy with chronic lactic acidosis is described in a 5 1/2 year-old-boy who ultimately succumbed to congestive heart failure. The autopsy findings included severe atrophy of the anterior cerebellar vermis and a hypertrophied heart with left sided endocardial fibroelastosis. Skeletal and cardial muscle calcification was prominent and probably due to the effect of intracellular metabolic alterations associated with lactic acidosis.

Association of maternal systemic lupus erythematosus with congenital complete heart block.

Infants born to mothers with disseminated lupus erythematosus occasionally have transient manifestations of the maternal disease. In six infants with congenital heart block born to mothers with systemic lupus erythematosus we postulated a causative relation. In one of the infants a post-mortem study of the conduction system suggested faulty embryonic development of the atrioventricular node with an abnormally thick annulus fibrosus and the effects of early inflammatory changes.

Propranolol therapy during pregnancy, labor, and delivery: evidence for transplacental drug transfer and impaired neonatal drug disposition.

The administration of 160 mg of propranolol during pregnancy, labor, and delivery was associated with profound hypoglycemia and respiratory depression in a newborn infant. The neonate's plasma propranolol level rose from 40 ng/ml at the time of birth to 90 ng/ml four hours later. This increase in plasma propranolol concentration might be due to redistribution of the drug in the neonate as well as to different elimination mechanisms than in adults.

Total anomalous pulmonary venous return with associated patent ductus arteriosus. Two cases with successful correction.

A large patent ductus arteriosus associated with total anomalous pulmonary venous return results in a unique hemodynamic pattern, and long-term survival has been rare. Two patients, aged five and 18 years, underwent successful surgical repair of supracardiac total anomalous pulmonary venous return and an associated large patent ductus arteriosus. The association of the Noonan syndrome in the 18-year-old woman is of additional interest.

An analysis of contingencies in the Appalachian coalfields.

The history of the Appalachian coalfields points to the inescapable conclusion that affluence and deprivation alternate according to the demand for coal in the national economy. Although this process has frequently been described, it has never been adequately or comprehensively conceptualized. If, however, the region is viewed in terms of contingencies selecting responses, it is possible to regard the population as operating according to a variable-interval schedule.

Benign, solitary schwannomas of the lesser peritoneal sac.

Two cases of a large schwannoma of the lesser omental sac are described. One arose from the lesser omentum itself. This schwannoma also had roentgenographically visible calcifications, a finding never before reported to have been seen in the abdomen.

Amantadine in drug-induced extrapyramidal signs: a comparative study.

In our non-blind comparative study, amantadine was as effective as the standard medications, benztropine and ethopropazine, in controlling drug-induced extrapyramidal signs. However, statistically significant improvement was noted a week after all the three medications. In addition, extrapyramidal signs were not completely controlled in most patients even after weeks, even though substantial improvement was noted.

Closed aortic valvotomy and simultaneous correction of associated anomalies in infants.

A small infant with severe congenital aortic stenosis presents a difficult therapeutic problem. Both operative and nonoperative treatment are hazardous--especially when other cardiovascular anomalies are present. This report describes a simple, effective technique for dilating the stenotic aortic valve and simultaneously repairing certain associated defects.