Neonatal Pneumopericardium

We would like to add a case of pneumopericardium in the newborn to the seven reviewed by Matthieu, et al.1 This case is unusual in that it is only the second reported in which the pneumopericardium cleared spontaneously without aspiration. V. J. was a premature, Negro, female infant born at 26 weeks' gestation to a 14-year-old Gravida I, Para 0 mother. Birth weight was 794 gm. Her 1-minute and 5-minute Apgar scores were 1, and spontaneous respirations did not begin until after 20 minutes of positive pressure oxygen therapy and administration of 5 mEq of sodium bicarbonate solution via the umbilical vein.

ABNORMAL BLOOD GLUCOSE REGULATION IN COCKAYNE'S SYNDROME

Review of all previous reports of Cockayne's syndrome indicates that the disorder is a unique entity readily identified by certain physical findings and radiographic features. A 16-year-old Negro female with the disorder was found to have hyperglycemia and elevated plasma insulin. Increased serum growth hormone levels were not demonstrated.

MULTIPLE HEMANGIOMAS IN AN INFANT WITH CARDIAC HYPERTROPHY

A case of multiple benign hemangiomas in a 10-week-old Negro female is presented. At autopsy, numerous cutaneous and visceral sites of involvement were found. The hemangiomas are believed to arise from a multicentric developmental abnormality but appear capable of limited independent growth and tissue destruction. Postmortem angiograms demonstrate the existence of numerous arteriovenous shunts, which are believed responsible for the marked cardiac enlargement and early congestive heart failure in this case, as well as in previously reported cases of infantile hemangioendothelioma of the liver. Visceral hemangiomatosis should be considered as a possible extra-cardiac cause of infantile cardiac hypertrophy or failure of unknown etiology, especially in the infant with cutaneous hemangiomas. Angiographic techniques may be of help in the diagnosis and determination of extent of visceral hemangiomas.

CONGENITAL NONHEMOLYTIC ICTERUS WITH DAMAGE TO THE CENTRAL NERVOUS SYSTEM

A case of nonhemolytic icterus with damage to the central nervous system, without familial incidence, occurring in a 2-year-old Negro female is presented. Jaundice began in this patient at an early age, and has been associated with severe central nervous system damage. There has been a continuous marked elevation of the indirect-reacting bilirubin in the serum. The liver is histologically normal, and neither hemolytic disorder nor biliary obstruction has been demonstrated. The fecal excretion of urobilinogen is normal. The clinical and laboratory findings are given. The possible relationship of this syndrome to recent advances in the knowledge of bilirubin metabolism is cited.

Hereditary Spherocytosis-Sicklemia in the Negro

1. A case of coexistent hereditary spherocytosis and sicklemia in a Negro female is reported. Hematologic and clinical amelioration followed splenectomy. 2. Study of three generations of the family of the propositus revealed one additional case of hereditary spherocytosis-sicklemia and 10 cases of hereditary spherocytosis in the Negro. 3. Further evidence is adduced that the combination of this heterozygous hemoglobinopathy with the red blood cell defect of hereditary spherocytosis produces an illness which is not grossly different from hereditary spherocytosis associated with normal hemoglobin.