Showing papers in "Archives of Disease in Childhood-fetal and Neonatal Edition in 2001"

Pulse oximetry, severe retinopathy, and outcome at one year in babies of less than 28 weeks gestation

Abstract: Aim—To determine whether diVering policies with regard to the control of oxygen saturation have any impact on the number of babies who develop retinopathy of prematurity and the number surviving with or without signs of cerebral palsy at one year. Methods—An examination of the case notes of all the 295 babies who survived infancy after delivery before 28 weeks gestation in the north of England in 1990‐ 1994. Results—Babies given enough supplemental oxygen to maintain an oxygen saturation of 88‐98%, as measured by pulse oximetry,for at least the first 8 weeks of life developed retinopathy of prematurity severe enough to be treated with cryotherapy four times as often as babies only given enough oxygen to maintain an oxygen saturation of 70‐90% (27.2% v 6.2%). Surviving babies were also ventilated longer (31.4 v 13.9 days), more likely to be in oxygen at a postmenstrual age of 36 weeks (46% v 18 %), and more likely to have a weight below the third centile at discharge (45% v 17%). There was no diVerence in the proportion who survived infancy (53% v 52%) or who later developed cerebral palsy (17% v 15%). The lowest incidence of retinopathy in the study was associated with a policy that made little use of arterial lines. Conclusions—Attempts to keep oxygen saturation at a normal “physiological” level may do more harm than good in babies of less than 28 weeks gestation. (Arch Dis Child Fetal Neonatal Ed 2001;84:F106‐F110)

Educational and behavioural problems in babies of 32-35 weeks gestation.

Abstract: Aim—To identify incidence of school and behaviour problems at age 7 years in children born between 32 and 35 weeks gestation,and investigate perinatal risk factors. Method—The study population consisted of all children born at 32‐35 weeks gestation to mothers resident in Oxfordshire in l990. General practitioners, parents, and teachers were asked about health, behaviour, and education by postal questionnaire. Teachers rated children on level of function in six areas using a five point scale. They also completed the Strengths and DiYculties behaviour questionnaire. Perinatal risk factors were identified for children with poor school performance using a univariate and multivariate analysis. Results—Teacher responses were obtained for 117 (66%) of the 176 children in the cohort. Twenty nine (25%) required support from a non-teaching assistant, five (4%) had required a statement of special educational needs, and three (3%) were at special school. Poor outcome was reported for 32% in writing, 31% in fine motor skills, 29% in mathematics, 19% in speaking, 21% in reading, and 12% in physical education. On the behaviour questionnaire, 19% of the cohort achieved an abnormal hyperactivity score (population norm 10%). Multivariate analysis showed perinatal variables that remained significant, independent of other variables; they were discharge from the special care baby unit > 36 weeks postconceptional age (odds ratio 4.15; 95% confidence interval 1.43 to 12.05) and male sex (odds ratio 3.88; 95% confidence interval 1.42 to 10.6). Conclusion—Up to a third of children born between 32 and 35 weeks gestation may have school problems. As there are larger numbers in this gestational category compared with smaller babies, this finding has implications for educational services. (Arch Dis Child Fetal Neonatal Ed 2001;85:F23‐F28)

Development and initial validation of the EDIN scale, a new tool for assessing prolonged pain in preterm infants

Abstract: OBJECTIVE—To develop and validate a scale suitable for use in clinical practice as a tool for assessing prolonged pain in premature infants. METHODS—Pain indicators identified by observation of preterm infants and selected by a panel of experts were used to develop the EDIN scale (Echelle Douleur Inconfort Nouveau-Ne, neonatal pain and discomfort scale). A cohort of preterm infants was studied prospectively to determine construct validity, inter-rater reliability, and internal consistency of the scale. RESULTS—The EDIN scale uses five behavioural indicators of prolonged pain: facial activity, body movements, quality of sleep, quality of contact with nurses, and consolability. The validation study included 76 preterm infants with a mean gestational age of 31.5weeks. Inter-rater reliability was acceptable, with a κ coefficient range of 0.59-0.74. Internal consistency was high: Cronbach's α coefficients calculated after deleting each item ranged from 0.86 to 0.94. To establish construct validity, EDIN scores in two extreme situations (pain and no pain) were compared, and a significant difference was observed. CONCLUSIONS—The validation data suggest that the EDIN is appropriate for assessing prolonged pain in preterm infants. Further studies are warranted to obtain further evidence of construct validity by comparing scores in less extreme situations.

Breast milk feeding and cognitive ability at 7-8 years.

Abstract: OBJECTIVE—To examine the association between duration of breast milk feeding and cognitive ability at 7-8 years in a birth cohort of very low birthweight infants. DESIGN—280 survivors from a national birth cohort of 413 New Zealand very low birthweight infants born in 1986 were assessed at age 7-8 years on measures of verbal and performance intelligence quotient (IQ) using the WISC-R. At the same time mothers were questioned as to whether they had elected to provide expressed breast milk at birth and the total duration of breast milk feeding. RESULTS—Some 73% of mothers provided expressed breast milk and 37% breast fed for four months or longer. Increasing duration of breast milk feeding was associated with increases in both verbal IQ (p < 0.001) and performance IQ (p < 0.05): children breast fed for eight months or longer had mean (SD) verbal IQ scores that were 10.2 (0.56) points higher and performance IQ scores that were 6.2 (0.35) points higher than children who did not receive breast milk. These differences were substantially reduced after control for selection factors associated with receipt of breast milk. Nevertheless, even after control for confounding, there remained a significant (p < 0.05) association between duration of breast milk feeding and verbal IQ: children breast fed for eight months or longer had adjusted mean (SD) verbal IQ scores that were 6(0.36) points higher than the scores of those who did not receive breast milk. CONCLUSIONS—These findings add to a growing body of evidence to suggest that breast milk feeding may have small long term benefits for child cognitive development.

Nucleated red blood cells in the fetus and newborn.

Abstract: Although nucleated red blood cells (nRBCs) are rarely found circulating in older children,1 they are commonly seen in the blood of newborns. They are primarily produced in the fetal bone marrow in response to erythropoietin and are stored in the marrow as precursors to reticulocytes and mature erythrocytes. Many acute and chronic stimuli cause increases in the number of circulating nRBCs from either increased erythropoietic activity or a sudden release from the marrow storage pools. This paper reviews the various pathological processes associated with increased production and release of nRBCs. It emphasises the effects of acute, subacute, and chronic asphyxia on nRBC counts.#### Key message 1 Common causes of increased nucleated red blood cells include prematurity, increased erythropoiesis from chronic hypoxia, anaemia, and maternal diabetes, from acute stress mediated release from the marrow stores, and from postnatal hypoxia. Extreme increases may occasionally be idiopathic. #### Key message 2 When increased nRBC counts are seen with acute and subacute asphyxia, the magnitude of the increase is a function of the severity and duration of the asphyxia. However, there is a large overlap between the nRBC values found after acute, subacute, and chronic asphyxia; asphyxia of any duration does not always cause an increased nRBC count, and extreme increases may be found without asphyxia. Nucleated red blood cells are sometimes called erythroblasts, normoblasts, or normocytes. For this review, the term “normoblasts” will be used to refer to the cells when they are in the bone marrow and “nRBCs” when they are in circulating blood. Clinically it is best to express nRBCs as an absolute number of cells per unit volume, either “nRBCs/mm3” or “nRBCs/l”. However, most clinical laboratories and many research publications report nRBCs relative to 100 white blood cells (WBCs). Unfortunately the extreme variability in the number of leucocytes …

Blood transfusion increases radical promoting non-transferrin bound iron in preterm infants

Abstract: BACKGROUND—Blood transfusion has been recognised as a risk factor for the development of retinopathy of prematurity (ROP) or chronic lung disease (CLD) in preterm infants, but the precise mechanism involved is not understood. AIM—To investigate the level of non-transferrin bound "free" iron, which has the potential to promote the generation of reactive oxygen species, and its redox status in the plasma of preterm infants immediately before and after blood transfusion. METHODS—Twenty one preterm infants with a median gestational age and birth weight of 27 weeks and 1021 g respectively were prospectively enrolled in the study. Sixteen of the 21 infants developed ROP and/or CLD. The infants were transfused with concentrated red blood cells at a median age of 32 days. The plasma concentration of total bleomycin detectable iron (BDI) was measured and also the ferrous iron (Fe2+) activity by bleomycin-iron complex dependent degradation of DNA. RESULTS—Even before blood transfusion, BDI was detectable in one third of the blood samples, and all but one sample had ferrous iron activity. After transfusion, both BDI and ferrous iron activity were significantly increased, in contrast with the situation in full term infants. Plasma ascorbic acid (AA) concentration was significantly decreased after blood transfusion, whereas the level of its oxidation product, dehydroascorbic acid (DHAA), and the DHAA/AA ratio were significantly increased compared with before the transfusion. The activity of plasma ferroxidase, which converts iron from the ferrous to the ferric state, was appreciably decreased in preterm infants, as expected from their very low plasma caeruloplasmin concentration. CONCLUSIONS—Plasma non-transferrin bound iron was significantly increased in preterm infants after blood transfusion and existed partly in the ferrous form, because of the low ferroxidase activity and the reduction of ferric iron (Fe3+) by ascorbic acid. This finding was specific to preterm infants and was not observed in full term infants after blood transfusion. Non-transferrin bound "free" iron may catalyse the generation of reactive oxygen species, which may be responsible for the clinical association of blood transfusion with ROP and CLD.

Neonatal meningitis in England and Wales: 10 years on

Abstract: OBJECTIVES To determine the incidence of neonatal meningitis in England and Wales. DESIGN A national postal survey using the British Paediatric Surveillance Unit (BPSU) card scheme supplemented by information from other sources. SETTING England and Wales 1996–1997. SUBJECTS A total of 274 babies less than 28 days of age who were treated for meningitis. RESULTS The incidence of neonatal meningitis in England and Wales has not changed since our previous study in 1985–1987. However, the acute phase mortality has fallen from 19.8% in 1985–1987 to 6.6% in this study. Group B streptococci (42%) and Escherichia coli (16%) remain the most common infecting microorganisms. Eight of 69 (12%) babies with group B streptococci and 4/26 (15%) with E coli died. Antibiotic regimens based on the third generation cephalosporins, notably cefotaxime, were most commonly used (84%). The BPSU scheme identified 72% of cases during the study period. Most cases of viral meningitis were not reported through the BPSU. Less than a third of samples from aseptic meningitis were examined for viruses; 56% of these were positive. CONCLUSIONS Although the incidence of neonatal meningitis remains unchanged, mortality from this infection has fallen significantly. If this improvement is maintained as reflected in the level of sequelae at 5 years of age, then the fear surrounding meningitis during the neonatal period will have been dramatically reduced.

Outcome at 14 years of extremely low birthweight infants: a regional study.

Abstract: OBJECTIVES—To determine the neurosensory outcome at 14 years of age of a regional cohort of extremely low birthweight (ELBW) children, to contrast their prognosis with normal birthweight (NBW) controls, and to determine the predictive value of assessments earlier in childhood. DESIGN—Geographically determined cohort study. SETTING—The state of Victoria, Australia. PATIENTS—Consecutive ELBW survivors of birth weight 500-999 g (n = 88) born during 1979-1980, and 60 randomly selected contemporaneous NBW (birth weight > 2499 g) controls. MAIN OUTCOME MEASURES—Rates of neurosensory impairments and disabilities at 14 years of age, and earlier in childhood. RESULTS—Of 351 ELBW consecutive live births, 88 (25%) survived and 79 (90%) of the survivors were assessed at 14 years of age. Of the 79 ELBW children assessed, eight (10%) had cerebral palsy, five (6%) had bilateral blindness, four (5%) were deaf requiring hearing aids, and 36 (46%) had an intelligence quotient (IQ) < −1 SD compared with the mean for the NBW controls. Overall 11(14%) ELBW children were severely disabled, 12 (15%) were moderately disabled, 20 (25%) were mildly disabled, and 36 (46%) had no disability. In contrast, only one (2%) of 42 NBW children assessed had a severe disability, six (14%) had a mild disability, and the remaining 35 (83%) were not disabled. Comparing psychological test scores for ELBW children with those for NBW controls, rather than test norms, avoided bias in the assessment of disability earlier in childhood. Relative to assessments earlier in childhood, the prediction of disability at 14 years of age was highly significant at each of 2, 5, and 8 years of age, but the accuracy progressively increased with age. CONCLUSIONS—ELBW children have substantially higher rates of neurosensory impairments and disabilities at 14 years of age than NBW controls. Comparison of ELBW children with NBW controls avoids bias in the assessment of disability. Early childhood assessments are highly predictive of disability at 14 years of age.

Randomised controlled trial of oral vitamin A supplementation in preterm infants to prevent chronic lung disease

Abstract: BACKGROUND—Intramuscular supplementation with vitamin A in large doses may reduce the incidence of chronic lung disease. AIM—To investigate whether oral supplementation with vitamin A would reduce the incidence of chronic lung disease in a group of extremely low birthweight infants. METHODS—Infants with birth weight < 1000 g were randomised at birth to receive oral vitamin A supplementation (5000 IU/day) or placebo for 28 days. The primary outcome was oxygen dependency at 28 days of age or death. RESULTS—A total of 154 infants were randomised; 77 received vitamin A (median birth weight (interquartile range) 806 (710-890) g), and 77 received placebo (median birth weight (interquartile range) 782 (662-880) g). Plasma vitamin A concentrations in the supplemented group were significantly higher at 24 hours of age but did not differ significantly at birth, 12 hours of age, 7 days, or 28days of life. There were no significant differences in the proportion of infants who survived, required oxygen at 28 days, required oxygen at 36 weeks postmenstrual age, survived without chronic lung disease at 36 weeks, survived without significant retinopathy, or who survived without significant intraventricular haemorrhage. CONCLUSIONS—Oral supplementation with 5000 IU vitamin A in extremely low birthweight infants does not significantly alter the incidence of chronic lung disease. However, this dose may have been inadequate to achieve optimal serum retinol concentrations.

Refractory hypotension in preterm infants with adrenocortical insufficiency.

Abstract: Five preterm, very low birthweight infants with severe hypotension and adrenocortical insufficiency are described. The profound hypotension was resistant to volume expansion and inotrope treatment, but responded promptly to corticosteroid treatment. A human corticotrophin releasing hormone (hCRH) test performed before corticosteroid treatment showed adequate pituitary response, and the endocrine dysfunction was identified at the adrenal level. Corticosteroid treatment should be considered and could be life saving in severely hypotensive preterm infants who do not respond to conventional treatment with volume expanders and inotropes.

Time to positivity of neonatal blood cultures

Abstract: AIM To determine how long it takes neonatal blood cultures to become positive. METHODS Data were collected retrospectively on 451 positive blood cultures from babies on a tertiary neonatal unit between January 1997 and December 1998. During the study period, the laboratory used the BacT/Alert microbial detection system. RESULTS Complete information was available on 416 blood cultures. Twelve became positive after 72 hours, none of which were considered to be clinically significant. Of the 404 remaining cultures, 86% were positive at 36 hours, 96% at 48 hours, and 98.5% by 60 hours. If definite bacterial pathogens are considered alone, the time to positivity was 90% by 36 hours, 93% by 48 hours, and 98% by 60 hours. If definite and possible bacterial pathogens are considered (coagulase negative staphylococci taken as possible bacterial pathogens), the time to positivity was 89% at 36 hours and 97% at 48 hours. The negative predictive value, for isolation of any organism before 72 hours, of a negative blood culture was 97% at 36 hours and 99% at 48 hours. The negative predictive value for the isolation of definite bacterial pathogens only was 99.7% at 36 hours and 99.8% at 48 hours. CONCLUSIONS A period of 36 hours is enough to rule out sepsis in the asymptomatic neonate, and a three day incubation period is sufficient to detect all clinically important infections using the BacT/Alert microbial detection system.

A randomised controlled trial to compare methods of milk expression after preterm delivery.

Abstract: Objectives—Primary:to compare sequential and simultaneous breast pumping on volume of milk expressed and its fat content. Secondary: to measure the eVect of breast massage on milk volume and fat content. Design—Sequential randomised controlled trial. Setting—Neonatal intensive care unit, North StaVordshire Hospital NHS Trust. Subjects—Data on 36 women were analysed; 19 women used simultaneous pumping and 17 used sequential pumping. Interventions—Women were randomly allocated to use either simultaneous (both breasts simultaneously) or sequential (one breast then the other) milk expression. Stratification was used to ensure that the groups were balanced for parity and gestation. A crossover design was used for massage, with patients acting as their own controls. Women were randomly allocated to receive either massage or non-massage first. Main outcome measures—Volume of milk expressed per expression and its fat content (estimated by the creamatocrit method). Results—Milk yield per expression was: sequential pumping with no massage, 51.32 g (95% confidence interval (CI) 56.57 to 46.07); sequential pumping with massage, 78.71 g (95% CI 85.19 to 72.24); simultaneous pumping with no massage, 87.69 g (95% CI 96.80 to 78.57); simultaneous pumping with massage, 125.08 g (95% CI 140.43 to 109.74). The fat concentration in the milk was not aVected by the increase in volume achieved by the interventions. Conclusions—The results are unequivocal and show that simultaneous pumping is more eVective at producing milk than sequential pumping and that breast massage has an additive eVect,improving milk production in both groups. As frequent and eYcient milk removal is essential for continued production of milk, mothers of preterm infants wishing to express milk for their sick babies should be taught these techniques. (Arch Dis Child Fetal Neonatal Ed 2001;85:F91‐F95)

Neonatal paroxetine withdrawal syndrome

Abstract: Four term neonates presented with symptoms such as jitteriness and necrotising enterocolitis after paroxetine exposure in utero.

Ultrasound diagnosis and neurodevelopmental outcome of localised and extensive cystic periventricular leucomalacia.

Abstract: Aims—To compare the ultrasound (US) evolution and neurodevelopmental outcome of infants with localised (grade II) and extensive (grade III) cystic periventricular leucomalacia (c-PVL). Methods—Over a nine year period, c-PVL was diagnosed in 96/3451 (2.8%) infants in two hospital cohorts. Eighteen were excluded from the study. Thirty nine infants with grade II PVL were compared with 39 infants with grade III PVL. Results—The two populations were comparable for gestational age and birth weight. In infants with grade II PVL, cysts were noted to develop more often after the first month of life (53%) in contrast with grade III PVL (22%) (odds ratio (OR) 3.81 (95% confidence interval (CI) 1.19 to 12.63)). Cysts were also more often unilateral in grade II (54%) than in grade III PVL (0%) (OR indefinite; RR 3.17 (95% CI 2.16 to 4.64)). At 40 weeks postmenstrual age (PMA), cysts were no longer seen on US in 13/38 infants with grade II PVL,with ventriculomegaly being the only visible sequel in nine cases. In grade III PVL, cysts were still present in 25 of the 27 surviving infants. Nine infants with grade II PVL were free of motor sequelae at follow up compared with one infant with grade III PVL (OR 8.07 (95% CI 0.92 to 181.66)). Twenty two out of 29 children with grade II PVL who developed cerebral palsy achieved independent walking compared with 3/26 with grade III PVL (OR 75 (95% CI 11.4 to 662)). Conclusions—In the cohort studied,50% of the infants with c-PVL had a more localised form (grade II). In grade II PVL, the cysts developed beyond the first month of life in more than half of the cases and were often no longer visible, on US, at 40 weeks PMA. In order not to miss this diagnosis, sequential US should also be performed beyond the first month of life. Mild ventriculomegaly noted at term can sometimes be due to grade II c-PVL. Cerebral palsy was slightly less common and tended to be less severe in infants with grade II PVL than in those with grade III PVL.

Background electroencephalographic (EEG) activities of very preterm infants born at less than 27 weeks gestation: a study on the degree of continuity

Abstract: AIMS—To clarify the features of the background electroencephalographic (EEG) activities in clinically well preterm infants born at less than 27 weeks gestation and to outline their chronological changes with increasing postconceptional age (PCA). METHODS—EEGs of clinically well premature infants born at less than 27 weeks gestation were recorded during the early postnatal period. The infants were separated into three groups according to their PCA at the time of EEG recording (21-22 weeks PCA, 23-24 weeks PCA, and 25-26 weeks PCA). The mean and maximum duration of interburst intervals (IBIs), the mean duration of bursts, and the percentage of continuous and discontinuous patterns in each PCA group were evaluated. RESULTS—There were three infants at 21-22 weeks PCA, seven at 23-24 weeks PCA, and five at 25-26 weeks PCA. Eighteen EEG recordings were obtained. The mean and maximum IBI duration decreased with increasing PCA. The percentage of continuous patterns increased with increasing PCA. Conversely, the percentage of discontinuous patterns decreased with increasing PCA. CONCLUSIONS—In premature infants born at less than 27 weeks gestation, the characteristics of the background EEG activities were similar to those of older premature infants. These changes reflect the development of the central nervous system in this period. At less than 27 weeks gestational age, the characteristics of background EEG activities were found to be as follows:

A randomised control study comparing the Infant Flow Driver with nasal continuous positive airway pressure in preterm infants

Abstract: Objective—To compare the eVectiveness of the Infant Flow Driver (IFD) with single prong nasal continuous positive airway pressure (nCPAP) in preterm neonates aVected by respiratory distress syndrome. Design—Randomised controlled study. Patients—Between September 1997 and March 1999, 36 preterm infants who were eligible for CPAP treatment were randomly selected for either nCPAP or IFD and studied prospectively for changes in oxygen requirement and/or respiratory rate. The requirement for mechanical ventilation, complications of treatment, and eVects on mid-term outcome were also evaluated. Results—Use of the IFD had a significantly beneficial eVect on both oxygen requirement and respiratory rate (p < 0.0001) when compared with nCPAP. Moreover,O2 requirement and respiratory rate were significantly decreased by four hours (p < 0.001 and p < 0.03 respectively). The probability of remaining supplementary oxygen free over the first 48 hours of treatment was significantly higher in patients treated with the IFD than with nCPAP (p < 0.02). IFD treated patients had a higher success (weaning) rate (94% v 72 %) and shorter duration of treatment (49.3 (31) v 56 (29.7) hours respectively; mean (SD)), although the diVerence was not significant. Conclusions—IFD appears to be a feasible device for managing respiratory distress syndrome in preterm infants, and benefits may be had with regard to oxygen requirement and respiratory rate when compared with nCPAP. The trend towards reduced requirement for mechanical ventilation, shorter clinical recovery time,and shorter duration of treatment requires further evaluation in a multicentre randomised clinical trial. (Arch Dis Child Fetal Neonatal Ed 2001;85:F86‐F90)

Treatment of neonatal thrombus formation with recombinant tissue plasminogen activator: six years experience and review of the literature

Abstract: BACKGROUND Thrombosis is a relatively rare event in children. However, many conditions in the neonatal period result in an increased risk of thrombus formation. The major risk factor is the indwelling intravascular catheter. Numerous small studies have reported experience of thrombolytic treatment for neonatal thrombotic disease with a wide range of different thrombolytic agents in various forms of administration, dosage, and duration, but no conclusions on the most effective treatment for neonates has been reached. OBJECTIVE To assess the efficacy and safety of thrombolytic treatment of neonatal catheter related thrombus (CRT) formation with recombinant tissue plasminogen activator (rt-PA). METHOD Over a six year period, 14 neonates with CRT were treated with the same rt-PA protocol (an initial bolus of 0.7 mg/kg over 30–60 minutes followed by infusion of 0.2 mg/kg/h). RESULTS Complete clot dissolution was documented in 11 patients, and partial clot lysis in two patients, leading to a patency rate of 94%. In two cases, local bleeding occurred, resulting in treatment failure in one case. Finally, antithrombin III substitution was required in one case. No other complications such as severe bleeding were recognised. CONCLUSION With the use of close clinical and haematological monitoring on a neonatal intensive care unit combined with serial two dimensional colour echocardiography, the present rt-PA protocol was shown to be a safe and effective method of clot dissolution in neonates.

Evidence of selection bias in preterm survival studies: a systematic review.

Abstract: OBJECTIVE To determine by how much selection bias in preterm infant cohort studies results in an overestimate of survival. DESIGN Systematic review of studies reporting survival in infants less than 28 weeks of gestation published 1978–1998. Studies were graded according to cohort definition: A, stillbirths and live births; B, live births; C, neonatal unit admissions. Proportions of infants surviving to discharge were calculated for each week of gestation. RESULTS Sixty seven studies report data on 55 cohorts (16 grade A, 23 grade B, 16 grade C). Studies that are more selective report significantly higher survival between 23 and 26 weeks of gestation (grade C > grade B > grade A, p CONCLUSION To minimise the potential for overestimating survival around the limits of viability, future studies should endeavour to report the outcome of all pregnancies for each week of gestation (terminations, miscarriages, stillbirths, and all live births).

Quality of life in young adults with very low birth weight

Abstract: OBJECTIVES—To assess quality of life (QoL) in a group of young adults born in 1980-1982 with very low birth weight (VLBW) and to compare this with a reference group and a similar cohort born eight years earlier. DESIGN—Telephone interview using a fully structured questionnaire. SETTING—Level 3 neonatal intensive care unit. PATIENTS—VLBW group (n = 92, 90% participation rate), LBW group (n = 119, 86%), normal birth weight/reference group (n = 69, 75%). MAIN OUTCOME MEASURES—Objective and subjective QoL. RESULTS—Objective QoL in the VLBW subgroup who did not report a handicap or chronic health problem was lower than in the reference group (median 0.79 v 0.84, p = 0.02). Objective QoL was 0.81 in the similar LBW subgroup whereas it was only 0.72 in the group of 13 VLBW and nine LBW subjects who reported a handicap or chronic health problem. Interestingly, subjective QoL did not differ between the VLBW subgroup and the reference group (median 0.87 v 0.88,p = 0.5). On comparing the VLBW subgroup in the 1980-1982 cohort with the similar VLBW subgroup in the 1971-1974 cohort, objective QoL had apparently increased. CONCLUSION—The VLBW young adults had a lower objective QoL than the reference group, whereas the subjective QoL was similar. Objective QoL in Danish youngsters has apparently increased over the past eight years.

Evaluation of newborn screening for medium chain acyl-CoA dehydrogenase deficiency in 275 000 babies

Abstract: OBJECTIVE To evaluate newborn screening by tandem mass spectrometry for detection of medium chain acyl-CoA dehydrogenase (MCAD) deficiency, a fatty acid oxidation disorder with significant mortality in undiagnosed patients. DESIGN The following were studied: (a) 13 clinically detected MCAD deficient subjects, most homozygous for the common A985G mutation, whose newborn screening sample was available; (b) 275 653 consecutive neonates undergoing routine newborn screening. Screened infants with blood octanoylcarnitine levels ⩾ 1 μmol/l were analysed for the A985G mutation, had analysis of plasma and repeat blood spot acylcarnitines and urinary organic acids, and had fibroblast fatty acid oxidation or acylcarnitine studies. RESULT Twelve of the 13 patients later diagnosed clinically had newborn octanoylcarnitine levels > 2.3 μmol/l. Twenty three screened babies had initial octanoylcarnitine levels ⩾ 1 μmol/l. Eleven of 12 babies with persistent abnormalities had metabolite and/or enzyme studies indicating MCAD deficiency. Only four were homozygous for the A985G mutation, the remainder carrying one copy. CONCLUSIONS Most patients with symptomatic MCAD deficiency could be detected by newborn screening. Infants actually detected had a lower frequency of A985G alleles than clinically diagnosed cases and may have a lower risk of becoming symptomatic.

Study of maternal influences on fetal iron status at term using cord blood transferrin receptors

Abstract: Aims—To determine eVects of maternal iron depletion and smoking on iron status of term babies using serum transferrin receptors (STfR) and their ratio to ferritin (TfR-F index) in cord blood. Methods—Iron, ferritin, STfR, and haemoglobin (Hb) concentration were measured and TfR-F index calculated in 67 cord /maternal blood pairs. Twenty six mothers were iron depleted (ferritin <10 µg/l) and 28 were smokers. Results—Maternal iron depletion was associated with decreased cord ferritin (113 v 171 µg/l) and Hb (156 v 168 g/l) but no change in STfR or TfR-F index. Smoking was associated with increased cord Hb (168 v 157 g/l) and TfR-F index (4.1 v 3.4), and decreased ferritin (123 v 190 µg/l). Cord TfR-F index and Hb were positively correlated (r = 0.48). Conclusions—Maternal iron depletion is associated with reduced fetal iron stores but no change in free iron availability. Smoking is associated with increased fetal iron requirements for erythropoiesis. (Arch Dis Child Fetal Neonatal Ed 2001;84:F40‐F43)

Hyperinsulinism and Beckwith-Wiedemann syndrome

Abstract: Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth syndrome first described by Beckwith in 1963.1 The incidence of BWS is about 1:13 700 births, with an equal sex distribution.2 It is a clinically and genetically heterogeneous disorder. Table 1 outlines the major clinical features. The existence of milder forms of BWS probably underestimates this incidence.1 Developmental delay in BWS has been associated with chromosomal duplication, prematurity, and hypoglycaemia.3 The long term survival in BWS is favourable1, although surveillance for tumours is required.4 The phenotype of BWS is likely to result from an imbalance of a number of critical genes at chromosome 11p15. At this location, genetic imprinting with the loss of maternally expressed tumour and/or growth suppressor genes—for example, p57KIP2and H19—or duplications and unipaternal disomy of paternally expressed growth promoter genes—for example, insulin-like growth factor II—have been implicated in BWS.2 In BWS, 85% of cases are sporadic and 15% are autosomal dominant.2 Identified causes of autosomal dominant BWS include p57KIP2 mutations and 11p15 duplications and translocations.2 About 30% of the sporadic cases result from p57KIP2 mutations, unipaternal disomy, or 11p15 duplications and translocations, while 70% have no identified cytogenetic or DNA abnormality.2 View this table: Table 1 Features of Beckwith-Wiedemann syndrome The incidence of hypoglycaemia in BWS is about 50%.3 5 In one BWS hypoglycaemia series, 80% were reported as mild and asymptomatic, requiring extra feeds or intravenous dextrose only. In 20%, the hypoglycaemia was prolonged (duration greater than one week) and difficult to control.3Hypoglycaemia has been documented into the third year of life.5 Intellectual impairment was associated with hypoglycaemia.3 Hyperinsulinism and BWS Hyperinsulinism is the cause of both transient and prolonged hypoglycaemia in BWS. A number of case reports provide data on the metabolic aspects of …

Prediction of early tolerance to enteral feeding in preterm infants by measurement of superior mesenteric artery blood flow velocity

Abstract: AIMS—To evaluate whether serial Doppler measurements of superior mesenteric artery (SMA) blood flow velocity after the first enteral feed could predict early tolerance to enteral feeding in preterm infants. METHODS—When clinicians decided to start enteral feeds, Doppler ultrasound blood flow velocity in the SMA was determined before and after a test feed of 0.5 ml milk. The number of days taken for infants to tolerate full enteral feeding (150 ml/kg/day) was recorded. RESULTS—Fourteen infants (group 1) achieved full enteral feeding within seven days. Thirty infants (group 2) took 8-30 days. There was no difference in the preprandial time averaged mean velocity (TAMV) between the groups at a median age of 3 (2-30) days. In group 1, there was a significant increase in TAMV (p<0.01) above the preprandial level at 45 and 60 minutes, but this did not occur in group 2. An increase in TAMV by more than 17% at 60 minutes has a sensitivity of 100% and a specificity of 70% for the prediction of early tolerance to enteral feeds. CONCLUSIONS—There is a significant correlation between an increase in mean SMA blood flow velocity and early tolerance of enteral feeding. Doppler measurements of SMA blood flow velocity may be useful for deciding when to feed high risk preterm infants.

Positioning long lines: contrast versus plain radiography

Abstract: AIM To assess the value of contrast versus plain radiography in determining radio-opaque long line tip position in neonates. METHODS In a prospective study, plain radiography was performed after insertion of radio-opaque long lines. If the line tip was not visible on the plain film, a second film with contrast was obtained in an attempt to visualise the tip. RESULTS Sixty eight lines were inserted during the study period, 62 of which were included in the study. In 31, a second radiographic examination with contrast was necessary to determine position of the tip. In 29 of these, the line tip was clearly visualised with contrast. On two occasions, the line tip could not be seen because the contrast had filled the vein and obscured the tip from view. Eight of the lines that required a second radiograph with contrast were repositioned. CONCLUSION Intravenous contrast should be routinely used in the assessment of long line position in the neonate.

Consent to autopsy for neonates.

Abstract: OBJECTIVES To determine parents9 views on autopsy after treatment withdrawal. DESIGN Face to face interviews with 59 sets of bereaved parents (108 individual parents) for whose 62 babies there had been discussion of treatment withdrawal. RESULTS All except one couple were asked for permission for postmortem examination; 38% refused. The main reasons for declining were concerns about disfigurement, a wish to have the child left in peace, and a feeling that an autopsy was unnecessary because the parents had no unanswered questions. The diagnosis, the age of the child, and the approach of the consultant appeared to influence consent rates. Of those who agreed to autopsies, 92% were given the results by the neonatologist concerned. Whether or not they had agreed to the procedure, at 13 months no parent expressed regrets about their decision. CONCLUSIONS Autopsy rates in the East of Scotland stand at 62%. Parents9 perceptions are an important element in consent to postmortem examination.

Randomised controlled study of oral erythromycin for treatment of gastrointestinal dysmotility in preterm infants

Abstract: Aim—To evaluate the eVectiveness of oral erythromycin as a prokinetic agent for the treatment of moderately severe gastrointestinal dysmotility in preterm very low birthweight infants. Methods—A prospective, double blind, randomised, placebo controlled study in a tertiary referral centre of a university teaching hospital was conducted on 56 preterm infants (< 1500 g) consecutively admitted to the neonatal unit. The infants were randomly allocated by minimisation to receive oral erythromycin (12.5 mg/kg, every six hours for 14 days) or an equivalent volume of placebo solution (normal saline) if they received less than half the total daily fluid intake or less than 75 ml/kg/day of milk feeds by the enteral route on day 14 of life. The times taken to establish half, three quarters, and full enteral feeding after the drug treatment were compared between the two groups. Potential adverse eVects of oral erythromycin and complications associated with parenteral nutrition were assessed as secondary outcomes. Results—Twenty seven and 29 infants received oral erythromycin and placebo solution respectively. The times taken to establish half, three quarters, and full enteral feeding after the drug treatment were significantly shorter in the group receiving oral erythromycin than in those receiving the placebo (p < 0.05, p < 0.05 and p < 0.0001 respectively). There was also a trend suggesting that more infants with prolonged feed intolerance developed cholestatic jaundice in the placebo than in the oral erythromycin group (10 v 5 infants). None of the infants receiving oral erythromycin developed cardiac dysrhythmia, pyloric stenosis, or septicaemia caused by multiresistant organisms. Conclusions—Oral erythromycin is eVective in facilitating enteral feeding in preterm very low birthweight infants with moderately severe gastrointestinal dysmotility. Treated infants can achieve full enteral feeding 10 days earlier, and this may result in a substantial saving on hyperalimentation. However, until the safety of erythromycin has been confirmed in preterm infants, this treatment modality should remain experimental. Prophylactic or routine use of this medication for treatment of mild cases of gastrointestinal dysmotility is probably not warranted at this stage. (Arch Dis Child Fetal Neonatal Ed 2001;84:F177‐F182)

Cardiac troponin T in cord blood.

Abstract: BACKGROUND Perinatal asphyxia is associated with cardiac dysfunction. This may be secondary to myocardial ischaemia. Cardiac troponin T is the ideal marker for myocardial necrosis. Elevated levels in cord blood may be associated with intrauterine hypoxia and increased perinatal morbidity. AIMS To establish an upper limit of normal for cardiac troponin T concentration in the cord blood of infants. Relations between cardiac troponin T levels and other variables were investigated. METHODS Cord blood samples were collected from 242 infants and analysed. Data on gestation, birth weight, sex, Apgar scores, respiratory status, and mode of delivery were recorded. RESULTS A total of 242 samples were collected, and 215 samples from infants without respiratory distress were used to establish the 95th percentile of 0.050 ng/ml. The gestation of these infants ranged from 31 to 42 weeks and birth weight ranged from 1.4 to 5 kg. There were no relations between cardiac troponin T levels and the other variables in these healthy infants. Twenty seven infants developed respiratory symptoms requiring oxygen and/or ventilation. These infants had significantly higher cord cardiac troponin T levels than their healthy counterparts (median (interquartile range) 0.031 (0.010–0.084)v 0.010 (0.010–0.014) ng/ml respectively; p CONCLUSIONS Cardiac troponin T levels in the cord blood are unaffected by gestation, birth weight, sex, or mode of delivery. Infants with respiratory distress had significantly higher cord cardiac troponin T levels, suggesting that cardiac troponin T may be a useful marker for myocardial damage in neonates.

Red blood cell transfusions in very and extremely low birthweight infants under restrictive transfusion guidelines: is exogenous erythropoietin necessary?

Abstract: Objective—To examine the number and volume of red blood cell transfusions (RBCTs) in very and extremely low birthweight infants under restrictive red blood cell transfusion guidelines without erythropoietin administration, and to compare the results with those reported in similar infants receiving erythropoietin. Methods—From April 1996 to June 1999, all RBCTs given to infants with a birth weight of less than 1500 g were prospectively recorded. Data on RBCT combined with erythropoietin treatment and RBCT guidelines were extracted from four prospective randomised trials of erythropoietin for anaemia of prematurity. Results—When the restrictive RBCT guidelines were followed, the number of RBCTs and volume transfused were similar to those reported during erythropoietin administration. Conclusions—RBCT guidelines may have a similar impact on RBCT in very low birthweight infants to the administration of erythropoietin. The eVect of RBCT guidelines on RBCT frequency should be considered when evaluating the eYcacy of erythropoietin administration to preterm infants. (Arch Dis Child Fetal Neonatal Ed 2001;84:F96‐F100)

Lingering death after treatment withdrawal in the neonatal intensive care unit

Abstract: OBJECTIVE—To explore parents' perceptions of treatment withdrawal and the dying process. DESIGN—Face to face interviews with 59 sets of parents of 62 babies in the East of Scotland three months and 13 months after death. RESULTS—22% of the parents expressed reservations about the length of the dying process, which they reported in these instances had taken from three to 36 hours. Deaths that medical teams had predicted would be quick had, according to the parents' recollections, taken from 1.5 to 31 hours. When a baby died swiftly, this seemed to confirm the wisdom of the decision to stop. When babies lingered, doubts were raised. CONCLUSIONS—Parents need to be adequately prepared for what may happen after treatment withdrawal. The debate should be reopened about the best way to manage protracted deaths in line with parental need.

Routine neonatal examination: effectiveness of trainee paediatrician compared with advanced neonatal nurse practitioner.

Abstract: OBJECTIVE To compare the effectiveness of routine neonatal examination performed by senior house officers (SHOs) and advanced neonatal nurse practitioners (ANNPs). DESIGN A prospective study of all infants referred to specialist orthopaedic, ophthalmology, and cardiology clinics. A standardised proforma was used to record details of the professional performing the neonatal check, any abnormalities discovered, source of ultimate referral to the specialist clinic, and specialist findings. RESULTS 527 eligible infants were recruited. For hip abnormalities, ANNPs displayed greater sensitivity than SHOs (96% v 74%; p v 33%; p CONCLUSION ANNPs are significantly more effective in detecting abnormalities during the neonatal check. This has implications both for future workforce planning and current methods of medical training.