Polyamine levels in breast milk are associated with mothers' dietary intake and are higher in preterm than full-term human milk and formulas.

BACKGROUND: Polyamine intake from milk is considered essential for post-natal maturation of the immune system and small intestine. The present study aimed to determine polyamine content in human milk after preterm delivery and the association with mothers' dietary intake. In comparison, the polyamine levels were compared with those in term breast milk and some corresponding formulas. METHODS: Transitional breast milk was collected from 40 mothers delivering after 24-36 weeks of gestation, and from 12 mothers delivering after full term. Food intake was assessed in mothers delivering preterm babies using a 3-day diary. Polyamines were analysed by high-performance liquid chromatography. RESULTS: The dietary intake of polyamines was significantly associated with breast milk content but weaker for spermine than for spermidine and putrescine. Total polyamine level was higher in preterm than term milk and lower in the corresponding formulas. Putrescine, spermidine and spermine contents [mean (SEM)] in preterm milk were 165.6 (25), 615.5 (80) and 167.7 (16) nmol dL⁻¹, respectively, with the levels of putrescine and spermidine being 50% and 25% higher than in term milk. The content of spermine did not differ. CONCLUSIONS: Dietary intake of polyamines has an impact on the content in breast milk. The difference between human milk after preterm and term delivery might be considered when using donor human milk for preterm infants. The corresponding formulas had lower contents. Further studies are important for determining the relationship between tissue growth and maturation and optimal intake.

Nervonic acid is much lower in donor milk than in milk from mothers delivering premature infants--of neglected importance?

Nervonic acid is important for white matter development and its incorporation increases rapidly in the last trimester, but few studies focus on this fatty acid. Other mother's milk, usually after term delivery, is often used for premature infants, whose mothers cannot breastfeed. The fatty acid (FA) concentrations were analyzed by gas chromatography in 12 samples of donor human milk (DHM) from five mothers, and compared to milk from 42 mothers delivering preterm infants. Fat, lactose and protein contents were compared. Nervonic acid showed sevenfold higher concentrations and LCPUFA 90% higher concentrations in premature milk compared to DHM. Linoleic acid was found in 43% higher concentrations in DHM than in premature milk. The fat and protein contents were lower in DHM. Our results suggest that studies are warranted to investigate if DHM given to premature infants may require supplementation of nervonic acid, and not only LCPUFA, protein and minerals.

Motor, mental and behavioral developments in infancy are associated with fatty acid pattern in breast milk and plasma of premature infants.

The objective of this study was to investigate any association between infants' early development and PUFA concentrations in early breast milk and infants' plasma phospholipids at 44 weeks gestational age. Fifty-one premature infants were included. The quality of general movement was assessed at 3 months, and motor, mental and behavioral development at 3, 6, 10 and 18 months corrected age using Bayley's Scales of Infant Development (BSID-II). Linoleic acid, the major n-6/n-3 FA ratios, Mead acid and the EFA deficiency index in early breast milk were negatively associated with development up to 18 months of age. DHA and AA, respectively, in infants' plasma phospholipids was positively, but the AA/DHA ratio negatively, associated with development from 6 to 18 months of age. Our data suggest that the commonly found high n-6 concentration in breast milk is associated with less favorable motor, mental and behavioral development up to 18 months of age.

[Comparison of incomplete (135 degrees ) and complete prone position (180 degrees ) in patients with acute respiratory distress syndrome. Results of a prospective, randomised trial]. / Vergleich von inkompletter (135 degrees) und kompletter Bauchlage (180 degrees) beim schweren akuten Lungenversagen. Ergebnisse einer prospektiven, randomisierten Untersuchung.

BACKGROUND: Ventilation in the prone position is carried out for improvement of pulmonary gas exchange in patients with acute respiratory distress syndrome (ARDS). We compared the effects of an incomplete prone position (IPP, 135( degrees )) with a complete prone position (CPP, 180( degrees )) in patients with ARDS. PATIENTS AND METHODS: For this trial 59 patients with ARDS were randomly assigned and were positioned in a "cross-over" design: patients of group A were placed in IPP for 6 h and then immediately positioned in CPP for another 6 h. Patients in group B were positioned in reverse order. Blood gases, hemodynamic measurements, quasistatic respiratory compliance and assessments of side effects were performed before begin, 30 min and 6 h after first positioning, then 30 min and 6 h after second positioning and 2 after repositioning. RESULTS: Turning patients in IPP and CPP resulted in a significant increase in the arterial oxygenation index (p(a)O(2)/F(I)O(2)), but this effect was more pronounced in the CPP (before: 142+/-46 mm Hg, 6 h: 253+/-107 mm Hg) than in the IPP (before: 139+/-54 mm Hg, 6 h: 206+/-75 mm Hg), and compliance was improved only in CPP. The improvement in arterial oxygenation persisted 2 h after repositioning in the supine position in both groups. The oxygenation responder rate was lower during the IPP (70.3%) in comparison with the CPP (84.0%, p<0.05). The incidence of side effects tended to be increased during the CPP. CONCLUSION: Incomplete prone position improves oxygenation in ARDS patients, but less effectively than a "classic" CPP. In these patients the use of a CPP should be preferred.

Brief report: the Brazelton Neonatal Behavioral Assessment Scale detects differences among newborn infants of optimal health.

OBJECTIVE: To determine whether the Brazelton Neonatal Behavioral Assessment Scale (NBAS) can detect behavioral differences in newborn infants of optimal health and, if such differences appear, also detect gender differences among those neonates. METHODS: Participants were a group of healthy Swedish neonates, 20 boys and 18 girls. The infants were assessed by the NBAS under standardized conditions at 48-72 hours of age, at the midpoint between two meals. RESULTS: All items except those in the dimensions Autonomic System and Motor System had a wide interquartile range. The trend was that girls had higher median item profiles, which means a higher level of functioning than boys. Four out of seven median values in the dimension Social Interactive Organization, as well as the median value in the self-quieting item in the dimension State Regulation, were significantly higher for girls. The interquartile range of the items seemed wider for boys than for girls. CONCLUSIONS: The results indicate behavioral variability among healthy neonates. Gender differences were also observed with girls showing higher levels of functioning than boys.

Plasma metabolites after a lipid load in infants with congenital heart disease.

Growth retardation is common in infants with congenital heart defects. The aim of this study was to investigate whether growth retardation or type of heart defect in infants with congenital heart defects is related to disturbances in lipid metabolism. Sixteen infants with ventricular septal defects and six infants with transposition of the great arteries were given an intravenous load of lipid emulsion (Intralipid 20 mg/ml) corresponding to 0.5 g fat/kg body weight for 5 min after fasting for 8 h. Blood samples were drawn immediately before the infusion and 3, 20, 60, 120 and 240 min after the infusion was completed. Plasma concentrations of triglycerides (TG), free fatty acids (FFA), ketones, lactate, pyruvate, alanine, glycerol and glucose were determined. The fatty acid patterns in the TG and FFA fractions were measured using gas chromatography. Severe growth retardation in infants with defects of these kinds was correlated to higher fasting and maximum levels of linoleic acid in plasma FFA. The maximum levels of linoleic acid in the TG fraction were positively correlated to weight SD score, and maximum glycerol levels were higher in the most growth-retarded infants, indicating faster intravascular lipolysis. Linoleic acid in the TG fraction was still elevated at 120 and 240 min after the lipid load. Some differences between the cyanotic and VSD groups could be noted. These indicate decreased metabolic capacity to utilize released FFA in the cyanotic group. Infants with cyanotic heart defects also had higher lactate and alanine levels compared to infants with VSD. Our results support the hypothesis that lipid metabolism is disturbed in infants with congenital heart defects.

Glucose metabolism and insulin secretion in children with cyanotic congenital heart disease.

The aim of the study was to reveal differences in carbohydrate metabolism in children with cyanotic congenital heart diseases (CHD). Thirteen children with diseases of these kinds were investigated with regard to glucose tolerance and insulin secretion and comparisons were made with healthy controls of the same age. Investigations included an intravenous glucose tolerance test, insulin response to the glucose load in plasma and insulin secretion rate. The results reveal lower fasting glucose levels and signs of a higher insulin secretion rate in the relatively few patients in the CHD group where C-peptide measurements were performed, but no differences in glucose tolerance. The reasons for the differences are unclear, but the chronic increases in circulating catecholamines in combination with the impaired nutritional status of these children with CHD are probably the most important factors. We conclude that these divergences in carbohydrate metabolism should be emphasized in the care of children with CHD.

Skeletal muscle energy substrates, metabolic products and enzyme activity in infants with symptomatic ventricular septal defect.

Muscle biopsy studies were performed on 26 infants with symptomatic ventricular septal defect (VSD) (mean age 4.7 months) and 10 healthy infants (mean age 7.8 months). Analyses were made of muscle energy substrates, metabolic products, muscle enzyme activity, fibre types and fibre sizes. Relatively few differences were noted between the groups. The most important difference was a reduced ATP level in the VSD group. Glucose 6-phosphate concentrations were also lower in the VSD group. These differences could indicate a low metabolic activity in skeletal muscle in infants with heart failure. Most muscle enzyme activity was comparable with the exception of lactate dehydrogenase (LD), which was lower in the VSD group. Within the VSD group, no differences were revealed in muscle substrate concentrations for muscle enzyme activity in terms of the degree of heart failure. We conclude that low energy levels are probably explained by undernourishment and/or reduced blood flow to skeletal muscle and that the lack of other discrepancies in muscle metabolism indicates a desirable relatively normal motor activity in these infants with symptomatic VSD.

Trauma metabolism and the heart. Uptake of substrates and effects of insulin early after cardiac operations.

In a controlled, randomized study the myocardial uptake/release of individual free fatty acids, glucose, lactate, pyruvate, alanine, and glycerol was studied 1 hour after completion of coronary operations. The effects of insulin were evaluated by means of a hyperinsulinemic "clamp" technique. No significant uptake of free fatty acids was found despite markedly elevated arterial concentrations (mean +/- standard error of the mean, 2.01 +/- 0.19 mmol.L-1), oleic acid, together with palmitic and linoleic acid, constituting 80% of the total plasma free fatty acid level. Insulin treatment (1 unit.kg bw-1.hr-1) prevented a further increase of the plasma free fatty acid level, observed concomitantly in the control group. Insulin affected all individual free fatty acids similarly. Changes in plasma free fatty acid levels occurring during the study and the corresponding myocardial uptake correlated (rS = 0.50 to 0.81). No significant uptake or release of glucose, lactate pyruvate, and glycerol occurred, whereas a myocardial release of alanine was seen. The heart and the concomitant leg uptake/release of glucose (rS = 0.40, p less than 0.05) and lactate (rS = 0.67, p less than 0.01) correlated. A substantial uptake of glucose was achieved and a more positive myocardial balance was obtained for alanine, lactate, and pyruvate with insulin. The changes in heart and the concomitant leg uptake/release correlated (glucose, rS = 0.62, p less than 0.01; lactate, rS = 0.64, p less than 0.01; pyruvate, rS = 0.71, p less than 0.01). It is concluded that the myocardial uptake of substrates during the first hours after coronary surgery is markedly abnormal with no uptake of free fatty acids or carbohydrates. These changes can be explained to some extent by the insulin resistance of trauma metabolism and can be modified by insulin treatment.

Arrhythmogenic right ventricular dysplasia in brother and sister: is it related to myocarditis?

Two cases of arrhythmogenic right ventricular dysplasia (ARVD) in siblings are reported. In the boy, 14 years old, the clinical history, ECG, echocardiography, and histopathological findings were consistent with ARVD. Premature ventricular contractions of left bundle branch block (LBBB) pattern were recorded but no ventricular tachycardia (VT). A high titer against mycoplasma and increased concentrations of immunoglobulins were found. Two years after his first admission he died suddenly. Autopsy revealed severe right ventricular (RV) myocardial damage, with fat cell infiltration and collagenous tissue. His sister presented with sustained VT of LBBB pattern 2 years later, at 12 years of age. Vaccination against rubella and signs of upper respiratory illness had preceded the symptoms. During the following 9 days ECGs and serum enzymes indicated the development of left ventricular (LV) infarction. Echocardiography revealed an enlarged RV and a normal LV. After 6 weeks both RV and LV showed akinetic areas and sacculations. We suggest that myocarditis may be a precipitating factor in ARVD, and perhaps the prerequisite for its manifestation.

Glucose metabolism and insulin secretion in infants with symptomatic ventricular septal defect.

Nineteen infants with symptomatic ventricular septal defect (VSD) were examined on, altogether, 26 occasions, when each was given an intravenous glucose tolerance test (IVGTT); concentrations of insulin- and C-peptide in plasma were determined. Comparisons were made with 14 healthy infants of the same age. The VSD infants were growth retarded with lower weight/age and length/age ratios. Glucose tolerance as indicated by glucose fasting levels and response to intravenous glucose tolerance test, including glucose disappearance rate, did not differ between the two groups. In response to the glucose load, insulin in plasma was significantly less in VSD infants. In response to the IVGTT, insulin secretion rate calculated from C-peptide levels in plasma was significantly elevated in the VSD group. We conclude that compared to healthy infants, those with symptomatic VSD have normal glucose tolerance, increased secretion rate of insulin, but decreased levels of circulating insulin in response to an intravenous glucose load. We suggest this is so because binding of insulin to peripheral receptors and/or insulin extraction in the liver somehow increases.

The effect of insulin on the leg and splanchnic balance of glucose and gluconeogenic substrates after cardiac surgery.

The effect of insulin and glucose infusion on the leg and splanchnic balance of glucose and the gluconeogenic substrates lactate, pyruvate, alanine and glycerol was studied in 13 patients directly after cardiac surgery. Insulin was infused continuously at a rate of 1.0 Unit/kg/hr during 1 hour. Sixteen patients served as a control group and received no insulin and glucose infusion. A significant increase in arterial lactate concentration in both the control group (from 1.40 +/- 0.19 to 1.68 +/- 0.24 mmol/l p less than 0.01) and the insulin group (from 1.58 +/- 0.27 to 2.07 +/- 0.22 mmol/l, p less than 0.05) was observed. The arterial pyruvate concentration was significantly increased (from 0.075 +/- 0.019 to 0.105 +/- 0.021 mmol/l, p less than 0.01) and glycerol was significantly decreased (from 0.15 +/- 0.03 to 0.12 +/- 0.03 mmol/l, p less than 0.05) during the insulin and glucose infusion. The alanine concentration was unchanged in both groups. Insulin and glucose infusion was followed by an increased leg uptake of glucose (from 0.15 +/- 0.14 to 1.06 +/- 0.16 mmol/min/100 ml, p less than 0.05), and by a changed splanchnic glucose balance (from -8.2 +/- 2.8 to +4.0 +/- 1.2 mmol/kg/min, p less than 0.01). A net leg release and at the same time a net splanchnic uptake of all gluconeogenic substrates was observed. The insulin and glucose infusion did not significantly change either the splanchnic balance or the leg balance of the gluconeogenic substrates.

Echocardiographic findings in children treated for malignancy with chemotherapy including adriamycin.

Thirty-seven children receiving chemotherapy including adriamycin were studied with echocardiography. Abnormal left ventricular function assessed by systolic time intervals (STI) and/or left ventricular shortening fraction (delta LVID) was present in 30%, and dilated left ventricle was in 45% of the case, often accompanied by thinning the left ventricular wall. Three patients developed congestive heart failure, one with fatal outcome. Two of these patients had received chest irradiation along with the chemotherapeutic treatment. These three patients with overt heart failure had gross abnormalities in several echocardiographic variables of which delta LVID seemed most specific.

A long term follow up of 15 patients with arrhythmogenic right ventricular dysplasia.

The clinical course in 15 patients with features consistent with arrhythmogenic right ventricular dysplasia is described. At referral seven patients had abnormal physical findings, nine had abnormal electrocardiograms with non-specific right-sided abnormalities, and seven patients had increased heart size or prominent right ventricles on chest x ray. During long term follow up (mean 8.8 years, range 1.5 to 28 years) 11 patients had abnormal physical findings, 11 had electrocardiographic changes, and nine had increased heart size. Recurrent sustained right ventricular tachycardia was the most common arrhythmia (10 patients). Two patients experienced ventricular fibrillation. Seven patients suffered from over 10 episodes of ventricular tachycardia, nine required cardioversions, and 10 patients had associated serious symptoms such as syncope, severe hypotension, or cardiac arrest. Four patients required operation to correct the arrhythmia and three patients developed right heart failure. Two out of three deaths were sudden. These data suggest that in arrhythmogenic right ventricular dysplasia right ventricular abnormalities may be progressive and that the condition may affect the left ventricle. The course of the ventricular arrhythmias was highly variable and could not be predicted in individual patients. The potential for lethal ventricular arrhythmias is evident and warrants intensive diagnostic efforts to identify patients with adverse prognostic features.

Prediction of symptomatic patent ductus arteriosus in preterm infants using Doppler and M-mode echocardiography.

To investigate whether the development of symptomatic patent ductus arteriosus could be predicted, 26 preterm infants dependent on mechanical ventilation were examined daily with Doppler and M-mode echocardiography until 3 days after birth. The presence or absence of a hemodynamically significant ductus shunt, as judged from echocardiographic criteria, was tested for predictive power in terms of sensitivity, specificity and total error rate. Out of the 26 infants 13 developed symptomatic patent ductus arteriosus at a median age of 5 days (range 2-8). These 13 infants developed echocardiographic evidence of a large shunt at a median age of 2 days (range 1-3). The sensitivity of prediction was 18, 46 and 100% at 1, 2 and 3 days after birth. The specificity was 80, 92 and 85% and the total error rate was 52, 32 and 8%. Thus, accurate prediction was possible 3 days after birth.

Total anomalous pulmonary venous connection in siblings. Report on three families.

Three pairs of siblings with total anomalous pulmonary venous connection (TAPVC) are reported. The types of the anomalous venous return (supra- or infracardial connections) varied within the families indicating that genetic regulation deals with the left atrial connection to the intrapulmonary veins. Having failed to establish this connection the intrapulmonary veins can attach themselves to any adjacent venous structure; hence the variety of connections at birth. Our observations also indicate the possibility that specific genetic mechanisms operate in at least some families. Since signs, such as murmurs and cardiomegaly typical of a cardiac anomaly, are not always present in TAPVC, special attention should be paid to any neonatal abnormality in siblings of known cases with TAPVC.

Doppler determination of cardiac output in infants and children: comparison with simultaneous thermodilution.

Ten children, aged six weeks to 13 years, without intracardiac shunts or lesions that could cause turbulent flow in the ascending aorta or aortic regurgitation, underwent cardiac catheterization, including cardiac output measurements by thermodilution. Simultaneously with each of six consecutive thermodilution injections, mean and maximal blood velocities in the ascending aorta were measured by pulsed Doppler echocardiography from the suprasternal notch. Aortic root and aortic orifice diameters were measured with M-mode and cross-sectional echocardiography. One patient had to be excluded from the analysis because of inadequate Doppler recordings. The best agreement with the results of the thermodilution was observed when internal systolic aortic root diameter was combined with mean velocity (r = 0.97, y = 0.90x + 0.28, SEE = 0.31 liters/min). When cardiac output was normalized for body size, there was still a good correlation between the results of these two methods.

Neonatal surgery for coarctation of the aorta. The Gothenburg experience.

Thirty-four neonates (weights 0.97-4.47 kg) were operated on for coarctation of the aorta. They included five premature infants with birth weight less than or equal to 2.1 kg. All 34 were symptomatic, 31 severely so. Coarctation was "simple" in 12 cases and "complex" in 22; 12 with ventricular septal defect and 10 with more complex malformations. The mean age at operation was 11 days. Subclavian flap aortoplasty was used in 27 cases, a lusoric artery in two, and combined end-to-end anastomosis and flap repair in five. The main pulmonary artery was banded in 13 of the 22 complex coarctation syndromes. Absorbable suture was used in the last 22 cases. There was one early postoperative death. Serious ischaemic complications (bowel or limb gangrene) arose in four patients who were in severe heart failure preoperatively. During follow-up averaging 1.9 years there were two recurrences of coarctation. Resection with end-to-end anastomosis combined with subclavian flap aortoplasty when there is bulging ductal tissue or long, narrow isthmus and use of absorbable vascular suture may further lower the incidence of recurrent coarctation.

Total anatomic correction of interrupted aortic arch complex. Experience in 4 infants.

In two cases of interrupted aortic arch (IAA) of type A, one associated with a ventricular septal defect (VSD) and one with an aortopulmonary window, and two of type B, both associated with a VSD, total anatomic repair was performed at respective ages of 6 months and 24, 8 and 3 days. All four operations were performed through a median sternotomy, using profound hypothermia and circulatory arrest. The repair included resection of the patent ductus arteriosus, direct end-to-side anastomosis of the descending to the ascending aorta and closure of the VSD or, in one case, of the aortopulmonary window. The two oldest infants (with type A IAA) survived. Reexamination two years postoperatively demonstrated good width of the aortic anastomosis with no gradient. In the child who had had an aortopulmonary window there was a proximal tight stenosis of the right pulmonary artery, which was corrected at reoperation. Total anatomic correction of IAA through an anterior approach is technically feasible and the aortic anastomosis seems to grow satisfactorily. The management of very sick neonates with IAA remains a great challenge.