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Total anomalous pulmonary venous connection is a cyanotic congenital heart defect that presents with a multitude of clinical presentations. We report a case of obstructive total anomalous pulmonary venous connection (TAPVC) which presented in a way that has not previously been described. An infant with mixed type obstructive TAPVC was found to have pulmonary interstitial emphysema and portal venous gas on a scout radiograph obtained prior to computed tomography scan. The presence of portal venous gas indicated a connection between the pulmonary venous system and the portal venous system infradiaphragmatically.

The newborn can experience two types of differential cyanosis (DC). The common type of DC occurs when oxygen saturation in the right hand is greater than in the foot. The second type of DC, reversed differential cyanosis (RDC), occurs when oxygen saturation is lower in the right hand than in the foot. This phenomenon is observed in transposition of the great arteries (TGA) with patent ductus arteriosis (PDA) and elevated pulmonary vascular resistance or in TGA with PDA and preductal aortic interruption or coarctation. This report describes a case of RDC not previously described involving an infant with supracardiac total anomalous pulmonary venous connection (TAPVC). In supracardiac TAPVC, RDC results from streaming of highly saturated superior vena cava (SVC) blood into the right ventricle, out the main pulmonary artery, through a PDA, and to the descending aorta, with streaming of more desaturated blood from the inferior vena cava (IVC) into the left atrium across the atrial septal defect (ASD)/foramen ovale. Therefore, as part of a neonatal examination to rule out congenital heart disease (CHD), simultaneous pre- and postductal oxygen saturations should be documented. The presence of RDC should initiate immediate full cardiac evaluation for CHD. Supracardiac TAPVC should be included in the differential diagnosis if RDC is observed.

This is the first report of dexamethasone-induced left ventricular outflow obstruction causing decreased coronary artery perfusion and apical transmural cardiac infarct that resulted in hemopericardium and rapid cardiac tamponade. The differential diagnosis of hemorrhagic pericardial effusion must now include dexamethasone-induced hypertrophic cardiomyopathy.

Plasma amino acids were measured in eight very low birth weight infants (less than or equal to 1000 gm) before and after infusion of parenteral alimentations with Freamine III. Significant elevation in serum threonine, valine, isoleucine, methionine, serine, proline, glycine and ornithine was noted after twenty four hours of infusion. On the other hand, significant decreases in taurine and tyrosine levels were noted. Our study suggests that current solution is not optimal for premature neonates and the amount of protein administered during the first week in infants weighing less than or equal to 1000 gm should be decreased from the recommended 2.5-3.0 gm/kg/day.

Oxygen consumption (VO2) and CO2 production (VCO2) were measured continuously for 24 h in 10 premature infants during their ongoing nursing care. Using a flow-through technique, the total VO2 and VCO2 over a given period of time were determined from the area under the O2-and CO2-concentration-time curve of the mixed expired gas. Following chest physiotherapy, heel stick and i.v. needle insertion, there was a significant (p less than 0.01) increase in VO2 and VCO2. When measured for 24 h, the total daily increase of VO2 attributed to these procedures ranged from 2.1 to 11.7% of total daily VO2, equivalent to an estimated energy loss of 0.6-4.1 kcal/kg/day.

Over a period of 18 months, 100 full-term newborns developed an axillary or a rectal temperature greater than or equal to 37.8 C during the first four days of postnatal life. These febrile term newborns represented 1% of all full-term newborns in the normal nursery. Of the febrile newborns, 10% had culture-proven bacterial disease (BD). Fever developed in 54%, 27%, 13%, and 6% on the first, second, third, and fourth days, respectively. In 17 newborns fever developed within the first hour of life; 13 of these had mothers with fever and two others were under a radiant warmer in the birth room. Fever occurring on the third day of postnatal life had a significantly higher chance of being associated with BD than fever occurring at any other time in the first four days of postnatal life. Newborns with temperature greater than or equal to 39 C had a significantly higher incidence of BD than newborns with temperature less than 39 C. The incidence of fever among breast-fed newborns (0.98%) was similar to that of formula-fed newborns (1.01%). Of the 100 febrile newborns, 45 had other symptoms compatible with BD, and eight of these had proven BD (group B Streptococcus in five, group D Streptococcus in one, Shigella D in one, and Propionibacterium species in one). The two other febrile newborns with proven BD had no other symptoms of infection (group B Streptococcus and Escherichia coli). Mean WBC count of febrile newborns with BD was significantly lower than that of febrile newborns without BD. Only three febrile newborns had WBC count less than 5,000/cu mm and two of them had proven BD. Febrile newborns should be evaluated and treated with antibiotics when they have symptoms of infection other than fever or when the fever persists or recurs.

Candida albicans meningitis was diagnosed in a 45-day-old premature infant whose birth weight was 1,616 gm. Symptoms consisted of poor weight gain and poor suckling. The combined use of amphotericin B and 5-fluorocytosine (5-FC) resulted in negative CSF cultures after 12 days of therapy. Amphotericin B was given for 45 days (total 83 mg) and 5-FC for 60 days (total 19 mg). Only one other premature infant has been reported in the literature who had similar treatment. A review of Candida meningitis diagnosed before death in 11 other infants less than 1 year of age is presented.

Chest radiographs on 73 neonates with early-onset group B Streptococcus (GBS) infection were reviewed. Eighty-six percent of the infants were premature (less than 38 weeks); 68% weighed less than or equal to 1,500 g. In infants weighing less than or equal to 1,500 g, the predominant radiographic pattern was hyaline membrane disease (HMD) (80%). There was a significant increase in radiographic HMD in 1,000 to 1,500-g neonates with GBS infection (77%) when compared to a control group of infants without GBS infection (44%). Mortality in 1,000 to 1,500-g infants with GBS infection and radiographic HMD (95%) was also significantly higher than in the control group of infants with hmd HMD and no GBS infection (38%). In larger premature and full-term infants, the radiographic findings were not specific and also were not helpful in distinguishing GBS infection from other newborn respiratory disorders.

We studied the effect of penicillin on early-onset Group B streptococcal disease over a 52-month period in neonates who were at high risk of infection. Shortly after birth, 1187 neonates weighing 2000 g or less had blood samples taken for cultures and were randomized into an early-treatment group (given intramuscular penicillin G within 60 minutes of birth) or a control group. The incidence of early-onset disease was 20 per 1000 live births (24 of 1187); the number of infants in the early-treatment group who had disease (10 of 589) was similar to that in the control group (14 of 598). The fatality rates were similar in both groups (6 of 10 vs. 8 of 14). Cultures from blood obtained with one hour of birth were positive in 21 of the 24 infants with disease; 22 of the 24 were symptomatic within four hours of birth. Thus, infection was well established before the first hour of postnatal life. At autopsy, gram-positive cocci were seen in lung sections of four infants in whom cultures of blood obtained after treatment had been sterile; this indicates that giving routine antibiotic therapy before culture samples are obtained can obscure bacteriologic diagnosis. We conclude that penicillin given at birth to neonates weighing 2000 g or less does not prevent early-onset streptococcal disease or reduce excess mortality associated with disease.

Fifteen studies were performed in ten premature infants whose birth weight (mean +/- SD) was 1,444 +/- 250 gm, gestational age 32.7 +/- 1.0 weeks, and postnatal age 10.7 +/- 3.3 days. Each study consisted of three hours simultaneous measurement of insensible water loss and oxygen consumption under two conditions for the same infant: (1) inside a single-walled incubator and (2) inside a double-walled incubator. The double-walled incubator provided significantly (P < 0.001) higher operative temperature and incubator wall temperature than did the single-walled incubator. Infants inside the double-walled incubator had significantly lower (P < 0.01) IWL (30% reduction) and lower (P < 0.05) VO2 (17% reduction) than inside the single-walled incubator--a net caloric saving of 11.8 kcal/kg/day. This saving of energy expenditure may be important in affecting the growth and outcome of the low-birth-weight infants.

The treatment of neonatal hypoglycemia by constant infusion of glucose at the rate of 8 mg/kg/minute was studied in 22 hypoglycemic neonates. In 18 neonates glucose levels rose above the hypoglycemic range within ten minutes of infusion and in three, within 30 to 50 minutes of infusion. The remaining neonate had hyperinsulinemia and responded only to diazoxide. Constant glucose infusion was found to be useful therapeutically for neonatal hypoglycemia.

Serial measurements of pulmonary function and arterial blood gases during the first 3 postnatal days of life were obtained in 12 infants with meconium aspiration syndrome (MAS). Nine normal neonates with similar weight and gestational age were studied as controls. Infants with MAS has significantly lower pH on day 1, and had greater P(A-a)O2 throughout the study period than that of normal controls. The PCO2 was comparable between the groups. Both dynamic lung compliance (Cdyn) and specific lung compliance (C/VL) were lower in infants with MAS as compared with those of normal infants. The functional residual capacity (FRC) for normal infants on days 1, 2, and 3 were 2.0 +/- 0.3, 2.1 +/- 0.3, and 2.2 +/- 0.3 ml/cm, respectively, and for infants with MAS were 1.8 +/- 0.4, 2.3 +!- 1.1, and 2.2 +/- 0.6 ml/cm, respectively. Radiographic hyperinflation of the lungs was seen in 6 infants with MAS on day 1; 3 were associated with high FRC (greater than 2 SD of normal) and 2 with low FRC, indicating air trapping. The early use of PEEP should be cautious if hyperinflation or air trapping is present.

Nine infants less than 2 months of age with group B streptococcal (GBS) osteomyelitis or septic arthritis, or both, were seen from January 1975 through January 1978. The infants had local joint signs, usually in the absence of systemic signs. The bones and joints involved were equally distributed between proximal humerus and proximal and distal femur. An infant had involvement of the talus. Treatment consisted of two to three weeks of parenteral antibiotics, arthrotomy in infants with arthritis, and bone decompression in infants with osteomyelitis. Clinical follow-up showed normal growth and function of the affected joint. Of the organisms, five were typed: four were type III and one was type Ib. Group B streptococcal osteomyelitis and/or septic arthritis was the second most common late-onset GSB infection, being surpassed only by meningitis.

In the course of a double-blind controlled trial of intravenous indomethacin therapy in premature infants with patent ductus arteriosus, plasma glucose was evaluated in 47 infants before and at 24, 48, and 72 hours following the medication. Twenty-two infants were assigned to the control group and 25 were in the indomethacin group. Significantly lower plasma glucose was noted at 24 (P less than 0.01) and 48 (P less than 0.05) hours in the indomethacin group as compared to the control group. There was a significant inverse correlation between the plasma glucose and the corresponding plasma indomethacin concentration (P less than 0.05) and between the plasma glucose and the corresponding plasma indomethacin concentration-time integral (P less than 0.01) at 24, 48, and 72 hours after drug administration, suggesting that the decreased plasma glucose may be related to indomethacin therapy. The results of this study indicate that endogenous prostaglandin may play a role in glucose homeostasis in premature infants.

Nine infants with early-onset Haemophilus sepsis were seen between January 1973 and July 1977. Of the five isolated strains that were typed, only one was type B. All infants had respiratory distress, metabolic acidosis, and large alveolar-arterial oxygen tension difference gradients. Eight infants weighed less than 1,500 gm and died; one infant weighed 1,701 gm and survived. Roentgenograms in six of eight showed hyaline membrane disease. Pulmonary pathologic specimens in eight infants revealed hyaline membranes in six and polymorphonuclear leukocytes in the alveolar spaces in four. In two infants, small Gram-negative bacilli were noted within proteinaceous exudates in alveolar ducts. The route and time of infection in these infants with early-onset Haemophilus sepsis are unclear. However, the possibility that the infection occurs before birth and that these infants represent septically aborted prematures is suggested by the high incidence of prematurity in infants with early-onset Haemophilus sepsis and early detection of bacteremia in three infants.

A newborn with congenital syphilis presented radiographically with a lack of ossification of the medial portions of several ribs and massive sclerosis and decalcification of scapulae. The uniqueness of syphilitic scapular and rib involvement is discussed. The infant also went on to develop skin lesions which appeared following penicillin therapy and also developed an extrahepatic biliary atresia.

The effect of indomethacin on carbohydrate metabolism was studied in six premature infants with significant patent ductus arteriosus (mean +/- S.D., birth weight 1,066 +/- 244 gm, gestational age 30 +/- 1.6 weeks). All infants were in a glucose steady state between 50 and 100 mg/dl over a 2-hour period before indomethacin administration. There was a significant fall in plasma glucose at 1, 6, 12, and 24 hours following intravenous indomethacin infusion. Since there was no significant change in insulin levels from the baseline, the mechanism of indomethacin-mediated lack of prostaglandin inhibition of insulin release was not substantiated. Based on this study, plasma glucose levels should be followed closely in the first 24 hours following intravenous indomethacin administration.

The metabolic responses to a constant glucose infusion were measured in 30 premature infants, 700 to 1,550 gm. The study included 18 stressed premature infants who needed assisted ventilation, and 12 control premature infants. Metabolic measurements were similar in both groups in the cord and preinfusion samples. In the first postinfusion sample, glucose, cortisol, and glycerol values were higher in stressed than in control premature infants. Hyperglycemia was seen in 13 of stressed and in only one of control premature infants. Stressed infants who became hyperglycemic in the first postinfusion sample were then compared to stressed euglycemic infants. Insulin levels were higher, glycerol levels similar, but cortisol levels lower in stressed hyperglycemic than in stressed euglycemic premature infants. The etiology of hyperglycemia in stressed premature infants cannot be attributed to hypoinsulinemia or to hypercortisolemia, and is not associated with increased glycerol levels. There was no difference in mortality between stressed hyperglycemia and stressed euglycemic infants; stress, rather than hyperglycemia, was related to mortality.

From June 1980 to September 1984, forty-five newborns (weight greater than or equal to 2000 g), initially presumed normal, were seen with bilious vomiting in the first 72 hours and were prospectively followed up. Nine (20%) required surgical intervention, five (11%) had nonsurgical obstruction such as meconium plug or left microcolon, and the remaining 31 (69%) had idiopathic bilious vomiting. Infants with idiopathic bilious vomiting had a benign transient course and resumed feedings by 1 week of age; 30 of the 31 had normal or nonspecific findings on initial plain abdominal roentgenogram. Specific findings on the initial plain abdominal roentgenogram were noted in five infants, and four (80%) of these had a lesion requiring surgical intervention; 56% (5/9) of neonates with surgical lesions had normal or nonspecific findings on the plain abdominal roentgenograms. None developed bowel ischemia or midgut infarction secondary to a volvulus as they were identified by contrast studies shortly after the initial episode of bilious vomiting. Although the majority of "normal" neonates with bilious vomiting do not have a surgical lesion, this study indicates that 56% of surgical cases will be missed if contrast studies are not done.

Room-air oxygen concentration in the respiratory care areas of our Special Care Nursery ranged from 20.9% to 24.8%. Calculated oxygen concentrations in our respiratory care areas, assuming an air turnover of five or 12 air changes per hour, were frequently far below observed concentrations. This indicated that ventilation in our respiratory care areas provided below minimum recommended number of air changes per hour. Therefore, in respiratory care areas in older structures with poor ventilation or with ventilatory system breakdown, environmental oxygen should be monitored.