Glutamine supplementation for young infants with severe gastrointestinal disease.

BACKGROUND: Endogenous glutamine biosynthesis may be insufficient to meet the needs of infants with severe gastrointestinal disease. Studies using animal models of gastrointestinal disease and controlled trials in adult patients have suggested that glutamine supplementation improves clinical outcomes. OBJECTIVES: To assess the evidence from randomised controlled trials that providing supplemental glutamine reduces mortality and morbidity in infants with severe gastrointestinal disease. SEARCH STRATEGY: The standard search strategy of the Cochrane Neonatal Review Group was used. This included searches of the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 3, 2006), MEDLINE (1966 - August 2006), EMBASE (1980 - August 2006), conference proceedings, and previous reviews. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials that compared glutamine supplementation versus no glutamine supplementation in infants (up to three months old, corrected for preterm birth) with severe gastrointestinal disease (defined as a congenital or acquired gastrointestinal condition that is likely to necessitate providing parenteral nutrition for at least 24 hours). DATA COLLECTION AND ANALYSIS: Data were extracted using the standard methods of the Cochrane Neonatal Review Group, with separate evaluation of trial quality and data extraction by two reviewer authors, and synthesis of data using relative risk, risk difference and weighted mean difference. MAIN RESULTS: Two trials in which a total of 100 infants participated were identified. In one trial, a minority of participants were infants older than three months. These studies were generally of good methodological quality but were underpowered to detect clinically important effects of glutamine supplementation. Meta-analysis did not reveal a statistically significant difference in the risk of death before hospital discharge [typical relative risk 1.57 (95% confidence interval 0.25 to 9.66); typical risk difference 0.02 (95% confidence interval -0.06 to 0.10)], nor in the rate of invasive infection [typical relative risk 1.22 (95% confidence interval 0.55 to 2.70); typical risk difference: 0.04 (95% confidence interval -0.12 to 0.20)]. AUTHORS' CONCLUSIONS: The available data from randomised controlled trials are not sufficient to determine whether glutamine supplementation confers clinically significant benefits for infants with severe gastrointestinal disease. Further trials are needed.

The emerging pattern of hydrops fetalis--incidence, aetiology and management.

OBJECTIVES: To analyse the incidence, aetiology and management of live born cases of hydrops fetalis in a Regional Perinatal Centre. METHODS: We reviewed 35 cases of hydrops delivered over a six year period. RESULTS: Non-immune hydrops accounted for 80% of the cases and the majority of babies required Level 1 intensive care. The mortality rate was 40%. CONCLUSION: The pattern of hydrops is changing. Most of these babies now have non-immune hydrops and approximately two thirds are surviving.

Nutritional factors and visual function in premature infants.

Approximately 5-7% of all infants are born prematurely, and birth before 37 weeks is the most common cause of neonatal mortality, morbidity and long-term disability. Premature infants are poorly equipped for life outside the womb, and oxidant stress has been implicated in the aetiology of visual impairment in these infants, who are often exposed to increased O2 concentrations and high light intensity in neonatal units. The carotenoids lutein and zeaxanthin, which give the macular area of the eye its yellow colour, are located in the retinal pigment epithelium of the eye, and are believed to play a role in protecting it against oxidative and light damage. The macular pigments are of dietary origin, and green leafy vegetables are the primary source of lutein and zeaxanthin. Lutein is one of the five most common carotenoids found in the diet. There is current interest in the macular pigment in relation to age-related macular degeneration, but these pigments may also have a protective role in the retinal pigment epithelium of the newborn infant. Little information is available on blood lutein and zeaxanthin levels in neonates. Levels of lutein in human milk are two to three times higher than those of beta-carotene, whereas their concentrations in the mothers' blood are approximately the same. Human milk is the main dietary source of lutein and zeaxanthin for infants until weaning occurs. The biochemical mechanisms which mediate the transport of the macular carotenoids into the eye are not known, but tubulin has been identified as the major carotenoid-binding protein, and may play a role in the physiology of the macula.

Cord blood transferrin receptors to assess fetal iron status.

OBJECTIVE: To study iron status at different gestational ages using cord blood serum transferrin receptors (STfRs). METHODS: STfRs, iron, ferritin, total iron binding capacity, haemoglobin, and reticulocytes were measured in 144 cord blood samples. The babies were divided into three groups according to gestation (26 very preterm (24-29 weeks); 50 preterm (30-36 weeks); 68 term (37-41 weeks)). RESULTS: Serum iron, ferritin, and total iron binding capacity were highest at term, whereas reticulocytes were highest in the very preterm. STfR levels were not influenced by gestation. Haemoglobin (r = 0.46; p < 0.0001) and reticulocytes (r = 0.42; p < 0.0001) were the only indices that independently correlated with STfR levels. CONCLUSIONS: STfR levels in cord blood are not directly influenced by gestation and probably reflect the iron requirements of the fetus for erythropoiesis.

First clinical case of small de novo duplication of 19q (13.3-13.4) confirmed by FISH.

Partial trisomy of 19q has been reported in only 13 patients, of which all but one have been due to unbalanced translocations. Only one previous report of a de novo duplication of distal 19q has been described in a fetal chorionic villus sample. There was no description of clinical phenotype in this report. We describe the clinical manifestations and cytogenetic analysis in a child with an inverted duplication of 19q 13.3 to 13.4 confirmed by FISH using a chromosome 19 whole chromosome probe. This case represents the first report of a liveborn with "pure" distal trisomy 19q. Findings defining this uncommon aneusomy are a flat facies, down turned mouth, abnormal ears, and a short neck with redundant skin folds.

[Effect of 2 different dosages of a porcine surfactant on pulmonary gas exchange of premature infants with severe respiratory distress syndrome]. / Einfluss von 2 unterschiedlichen Dosierungen eines porcinen Surfactants auf den pulmonalen Gasaustausch Frühgeborener mit schwerem Atemnotsyndrom.

BACKGROUND: Doses between 20 and 200 mg/kg body weight (bw) of different surfactant preparations have been recommended in clinical trials for the treatment of neonatal RDS; an optimal dose regimen of surfactant replacement therapy has not yet been defined. Aim of the present pilot study was the evaluation of pulmonary gas exchange in infants with severe RDS following the application of either a high (200 mg/kg bw) or a low (100 mg/kg bw) dose of a natural porcine surfactant (Curosurf). METHODS: 15 neonates were randomized to a high dose regimen, 17 infants to a low dose of surfactant. Apart from a lower 1 minute Apgar in the 100 mg/kg bw group, birth weight, gestational age, sex, 5 minute-Apgar and disease severity (arterial to alveolar oxygenation ratio (a/A-ratio): 0.10 +/- 0.03 [high dose], 0.11 +/- 0.06 [low dose], mean +/- SD) were well matched in both groups. RESULTS: Following surfactant instillation there was a rapid improvement in oxygenation in both groups. The a/A-ratio was slightly higher in the 200 mg/kg bw group during the first 12 hours following surfactant replacement, but statistically this was significantly higher only 4 hours after treatment (0.38 +/- 0.11 vs. 0.24 +/- 0.13, mean +/- SD, p < 0.05). CONCLUSION: The dose of 100 mg/kg bw Curosurf resulted in a rapid improvement in oxygenation and ventilatory requirements; only during the first hours following surfactant replacement there was a slight further improvement with the higher dose of 200 mg/kg bw. The impact of different dose regimens on outcome parameters still has to be defined by a larger clinical trial.

Comparison of clinical responses to natural and synthetic surfactants.

The clinical responses to both natural and synthetic surfactants were observed in two District General Hospital Neonatal Units who were centrally randomised as part of two separate multicentre trials (OSIRIS and Curosurf 4). Forty five infants were enrolled consecutively in the OSIRIS trial using synthetic surfactant (Exosurf), while 21 infants were subsequently enrolled in the Curosurf 4 trial using natural surfactant (Curosurf). There were no significant differences between the groups for mean birth weight, gestational age, inspired oxygen (FiO2), or arterial: alveolar oxygen ratio (a/A) prior to surfactant administration. Oxygen requirements fell significantly more rapidly within the first 24 hours for patients treated with Curosurf compared to Exosurf (p < 0.001). Mean duration of > 40% oxygen requirement was significantly shorter in the Curosurf group (2.6 days) compared to 8.0 days in the Exosurf group (p < 0.01). Mean duration of oxygen therapy was also significantly shorter in the Curosurf group (10.2 days) compared to 17.1 days in the Exosurf group (p < 0.05). Ten infants (24%) in the Exosurf group developed intraventricular haemorrhage (IVH) compared to none in the Curosurf group (p < 0.05). As oxygen requirements appear to decrease more rapidly following administration of Curosurf compared to Exosurf a large prospective randomized multicentre trial needs to be performed to compare the effects of these surfactants on both short and long-term outcome.

A 2-year follow up of babies enrolled in a European multicentre trial of porcine surfactant replacement for severe neonatal respiratory distress syndrome. Collaborative European Multicentre Study Group.

The postnatal growth, respiratory status and neurodevelopmental outcome of surviving babies enrolled in the first European multicentre trial of porcine surfactant (Curosurf) replacement for severe neonatal respiratory distress syndrome, were assessed at corrected ages of 1 and 2 years. Follow up rates of survivors were 93% at 1 year and 89% at 2 years. Treated and control groups were similar at both 1 and 2 years in terms of physical growth, the prevalence of persistent respiratory symptoms and the occurrence of major and minor disability. Serum antibodies recognising Curosurf and surfactant-anti-surfactant immune complexes were detected in both treated and control babies, the titres showing no difference between groups. Examination of histological lung sections from non-survivors revealed a higher incidence of severe pulmonary interstitial emphysema in control babies than in those treated with surfactant. Surfactant treatment for severe respiratory distress syndrome reduces neonatal mortality and air leaks and is not associated with an increase in disability 2 years later.

Plasma manganese, selenium and glutathione peroxidase levels in the mother and newborn infant.

Manganese (Mn), selenium (Se) and glutathione peroxidase (GPX) levels were determined in 56 paired samples of maternal and cord blood plasma at birth in both term and preterm pregnancies. Correlations existed between maternal and newborn plasma levels for Se, but not for Mn or GPX. Values of Mn, Se and GPX were all greater in the term than the preterm infant. Levels of Mn were greater in the pregnant than the non-pregnant woman, but levels of Se and GPX were lower during pregnancy.

Surfactant replacement therapy for severe neonatal respiratory distress syndrome: implications for nursing care.

This study, which was part of a European multicentre randomised controlled trial of surfactant administration for severe respiratory distress syndrome, monitored the progress and nursing care of the group of babies enrolled in Belfast. In total 33 preterm babies with birth weights between 700-2000 g were studied. Nineteen babies were treated with surfactant and fourteen acted as controls, receiving conventional therapy with mechanical ventilation alone. All the babies had severe respiratory distress syndrome requiring mechanical ventilation in oxygen concentrations greater than 60% by the age of fifteen hours. The aim of the study was to determine the effect on nursing workload of giving surfactant to babies with severe respiratory distress syndrome. The duration of care, such as the length of time the babies required intensive care and subsequent hospitalisation was calculated for each baby. The characteristics of the treated and control babies were similar but survival in the treated group was significantly greater (79% vs 36%, p less than 0.05). There were no significant differences between the groups for individual nursing requirements. However due to the increase in numbers of surviving surfactant treated babies there was a threefold increase in cumulative nursing workload. Surfactant replacement therapy effectively modifies the course of respiratory distress syndrome but also increases the nursing workload and the need for intensive care. This has implications for staffing and financial support of Neonatal Intensive Care Units once surfactant replacement becomes a routine treatment.