Abner H. Levkoff

DiGeorge syndrome associated with combined immunodeficiency

An infant with DiGeorge syndrome—complete with typical facies, neonatal hypocalcemic tetany, right-sided aortic arch, cardiac defects, and absent thymic shadow—had immunologic deficiencies of both cell-mediated and humoral compartments. The cell-mediated deficit was partial in that in vitro lymphocyte responses to phytohemagglutinin were absent while responses to allogeneic cells in one-way mixed leukocyte cultures were normal after three weeks of age. Humoral immune deficiency was manifested by low or absent immunoglobulin levels, failure to produce antibodies following repeated immunization with killed polio vaccine, typhoid-paratyphoid, and peneumococcal polysaccharide antigen, and lack of in vitro lymphocyte responses to pokeweed mitogen. These studies suggest a diagnosis of “DiGeorge variant.” Thymus transplantation at 45 days of age was followed by full recovery of lymphocyte responses to phytohemagglutinin and pokeweed mitogen, an increase of immunoglobulin levels to within normal ranges, and rejection of two skin grafts. These changes were accompanied by the appearance of normal numbers of small lymphocytes and plasma cells, in bone marrow and intestinal lamina propria, respectively. The effect of thymus transplantation on the immunologic recovery in this patient cannot be determined.

Endemic Serratia marcescens infection in a neonatal intensive care nursery associated with gastrointestinal colonization.

Serratia marcescens (SM) produced a prolonged outbreak in a neonatal intensive care unit of high level gastrointestinal colonization (10(9) SM/g feces) which in the early part of the outbreak predisposed to respiratory infection. The early outbreak featured a strain of SM carrying a 54 X 10(6) dalton conjugative plasmid which mediated resistance to gentamicin, tobramycin and beta-lactam agents. The second part of the outbreak involved primarily gastrointestinal colonization with SM strains that were plasmid-free. Acquisition of SM was related to very low birth weight (less than 1500 g). Among very low birth weight neonates, SM colonization was associated with pneumonia, patent ductus arteriosus, congestive heart failure and septicemia. Among neonates greater than 1500 g, SM colonization was associated with bronchopulmonary dysplasia, use of a respirator, patent ductus arteriosus and congestive heart failure. Respirator contamination, respiratory tract colonization and consequent pneumonia were reduced by more frequent changing of respirator tubing. Colonized sinks remained chronically colonized with multiresistant SM.

Birth weights of infants of black and white mothers without pregnancy complications

An analysis of 10,159 normal spontaneous vaginal deliveries was performed to examine racial differences in mean birth weight of infants whose mothers were without antepartum or intrapartum medical complications of pregnancy. The study was limited to black and white infants of low-income mothers who were inborn, singleton, and weighed greater than or equal to 500 gm at birth. High-risk maternal transfer patients and patients with hypertension, toxemia, bacteriuria, pyelonephritis, renal failure, diabetes, anemia, polyhydramnios, oligohydramnios, prolapsed cord, vaginal bleeding, placenta previa, abruptio placentae, prolonged rupture of membranes, maternal fever on admission, amnionitis, sexually transmitted diseases, or fewer than five prenatal care visits were excluded. When statistically significant differences in demographic characteristics were controlled, black infants had an average birth weight 181 gm less than that of white infants.

Lactobezoar and gastric perforation in a neonate.

Summary A lactobezoar and gastric perforation are described in a premature infant with multiple ulcers and pneumatosis of the stomach occurring during the recovery phase of hyaline membrane disease.

Changing pattern of neonatal susceptibility to hemophilus influenzae

In a study of neonatal and maternal sera, bactericidal antibody to Hemphilus influenzae B was demonstrated in only 10 per cent of newborn infants (cord blood) and in 25 per cent of mothers. These data represent a reversal of the findings of Fothergill and Wright in 1933, when 90 per cent of the blood from neonates was shown to have bactericidal antibody to this organism. A shift in neonatal susceptibility to H. influenzae is suggssted by these results.

Brain calcification in severely stressed neonatesreceiving parenteral calcium

Morphologic evidence for calcium salts within the brains of severely stressed neonates at autopsy correlated to the mean daily parenteral dose of calcium gluconate (P less than 0.01). Survival analysis indicated that parenteral administration of calcium contributed a negative effect to predicted survival (P less than 0.05).

Serum ionized calciumvalues in relation to exchange transfusion

Other authors have emphasized that there is no single clinical sign which is pathognomonic for Downs syndrome. ~ The value of ear length measurement in differentiating Downs syndrome from other patterns of multiple malformations is not known. However, we have found this nonspecific diagnostic sign to be the most consistent clinical characteristic, aside from hypotonia, in making the clinical diagnosis of Downs syndrome.

The association of hypocalcemia and recurrent apnea in premature infants

Abstract Serum total calcium was measured from birth to 48 hours of age in 27 neonates under 1,751 grams. Fourteen developed recurrent apnea; 13 had no apnea and had an essentially uneventful nursery course. The minimum mean calcium value for all infants with recurrent apnea was 5.8 mg. per 100 ml. and occurred at 31 hours of age. The minimum mean calcium value for the infants with no apnea was 8.3 mg. per 100 ml. and occurred at 35 hours of age. The babies with recurrent apnea had consistently higher serum phosphorus and lower serum total protein values.

Effect of tolazoline on persistent hypoxemia in neonatal respiratory distress.

Twenty-seven infants with respiratory distress and hypoxemia of noncardiac etiology were treated with tolazoline. Thirteen infants had hyaline membrane disease and 14 had respiratory distress attributable to causes other than hyaline membrane disease. An immediate response to tolazoline, a rise in Pao2 of 15 torr or more within 15 min, was seen in 11 infants in the hyaline membrane disease group and in eight infants of the other group. Six infants in the hyaline membrane disease group and 11 infants in the second group survived. Tolazoline should be considered as a therapeutic adjunct in the management of hypoxemia which persists after optimal homeostatic and ventilatory support.

THE SOUTH CAROLINA STATEWIDE PERINATAL INFORMATION SYSTEM

Clearly, the statewide system is fully integrated into the management of the neonates who constitute the majority of the high risk babies in South Carolina. Development of the system has resulted in statewide practice standards and a management and outcome reporting system. The data base and reports form an essential element in our resident and student training programs. A mechanism has been established that will permit the evaluation of quality of care and identification of areas needing improvement. This should result in improved patient management.Of major importance is the simple data form which reflects, for the most part, clinical outcome. A more complex system would have made the development of a statewide system for South Carolina most difficult. In addition, the information system is not stagnant or constrained to just those areas so far developed. It is in a constant state of revision in order to respond to the needs of the hospitals and the expectations of the programs that support the Informat...