Auditory neuropathy associated with postnatally acquired cytomegalovirus infection in a very preterm infant.

Neurosensory hearing loss is a well-known complication of antenatally acquired cytomegalovirus (CMV) infection. We here report an infant who developed auditory neuropathy after a postnatally acquired CMV infection. Infection probably occurred through ingestion of infected breast milk. Following a cochlear implant, there is normal language perception and a mildly delayed language expression at age 4. We speculate that the long-term effects of perinatal CMV infections are more dependent on the postconceptional age at which infection occurs than on whether the infection occurs antenatally or postnatally. An early acquired neonatal CMV infection in very preterm infants may therefore have long-term neurological sequelae, including auditory deficits.

Severe complications in a child with achondroplasia and two FGFR3 mutations on the same allele.

We describe a unique case of achondroplasia with associated complications, including severe respiratory problems. Molecular analysis of the fibroblast growth factor receptor type 3 (FGFR3) gene in this patient showed the common p.G380R mutation and a second novel p.L377R mutation. An allele-specific PCR demonstrated that these mutations were on the same allele (cis). Both mutations were not present in the parents and appear to have occurred de novo. To our knowledge, this is the first report in the literature on an achondroplasia patient with two FGFR3 mutations on the same allele.

Systematic review of the optimal fluid for dilutional exchange transfusion in neonatal polycythaemia.

OBJECTIVES: Several studies have shown the efficacy of dilutional exchange transfusion (DET) in reducing haematocrit (Ht) and relieving clinical symptoms in neonatal polycythaemia. We conducted a systematic review to determine the efficacy of crystalloid versus colloid solutions used in DET in an effort to identify the best solution to replace red blood cells. METHODS: The Cochrane Library, MEDLINE, and EMBASE were searched for relevant randomised controlled trials. Quality assessment and data analysis were performed using the methods and software of the Cochrane Collaboration. Relative risk (RR) and weighted mean difference (WMD) were calculated as measures of effect for categorical and continuous outcome data, respectively. Ninety five percent confidence intervals (95% CI) were calculated and a fixed effect model was used for meta-analysis. RESULTS: Six studies with a total of 235 newborns matched our inclusion criteria. When comparing crystalloid and colloid replacement solutions for DET, there was a clinically unimportant difference in Ht at 2-6 h and at 24 h in favour of colloidal solutions (WMD 2.29% (95% CI 1.28 to 3.31) and 1.74% (95% CI 0.80 to 2.68), respectively). This difference in post DET Ht was more evident when normal saline was compared to plasma but absent when normal saline was compared to 5% albumin. CONCLUSION: There is little difference in effectiveness between plasma, 5% albumin, and crystalloid solutions. Since normal saline is cheap, readily available, and does not carry the potential risk of transfusion associated infection, normal saline is the optimal dilutional fluid for exchange transfusion in polycythaemic neonates.

Postoperative condition after the use of remifentanil with a small dose of piritramide compared with a fentanyl-based protocol in patients undergoing craniotomy.

BACKGROUND AND OBJECTIVE: The use of remifentanil requires other analgesics for postoperative pain relief compared to fentanyl in patients undergoing craniotomy. This could possibly reduce the postoperative advantages of this short-acting opioid. METHODS: We compared remifentanil and fentanyl-based anaesthesia in a randomized observer and patient blinded trial on patients, undergoing an elective craniotomy. Twenty patients received anaesthesia using remifentanil with a small dose of piritramide (0.1 mg kg(-1)) after closure of the dura mater. Twenty patients underwent a fentanyl-based protocol. In both groups, anaesthesia was induced with thiopental and rocuronium, and maintained with 0.6-1 minimum alveolar concentration (MAC) isoflurane in a nitrous oxide/oxygen mixture 2:1 and rocuronium. Patients received 1 g of paracetamol rectally postoperatively. A visual analogue scale (VAS) for pain, the Glasgow Coma Score, a modified Aldrete Score, arterial carbon dioxide tension (PaCO2) and piritramide consumption were evaluated every half an hour postoperatively. RESULTS: No significant differences were found for pain, Aldrete or Glasgow Coma scores or for PaCO2 between the groups when controlled for age, although the pain and Glasgow Coma Scores were consistently higher and PaCO2 lower in the remifentanil group. Furthermore, 11 out of 20 patients in the remifentanil group requested extra piritramide as opposed to 7 out of 20 in the fentanyl group (P = 0.11). CONCLUSIONS: Despite the intraoperative use of piritramide in the remifentanil group, patients experienced more pain postoperatively. A significant influence of age on pain intensity was found. The use of remifentanil with a small dose of piritramide of 0.1 mg kg(-1) has no evident advantage over the use of fentanyl considering the postoperative conditions after craniotomy.

The use of in-line intravenous filters in sick newborn infants.

AIM: This study assesses the improvement in outcome for newborn infants by decreasing major complications associated with intravenous fluid therapy by using an in-line filter, and evaluates the economical impact this might have in relation to daily changing of i.v. lines. METHODS: In a prospective controlled study, 88 infants were randomly assigned to receive either filtered (except for lipids, blood and blood products) or non-filtered infusions via a central catheter. Main outcome measures such as bacteraemia, phlebitis, extravasation, thrombosis, septicaemia and necrosis were all scored. The costs attributable to patients during a standard 8-day stay were also recorded. RESULTS: Significant reductions were found in major complications such as thrombi and clinical sepsis (control group (21), filter group (8); p < 0.05). Bacterial cultures of the filters showed a contamination rate on the upstream surface of 15/109 filters (14%). The mean costs of disposables were less in the filter group, showing a reduction from 31.17 euros to 23.79 euros. CONCLUSIONS: The use of this in-line filter leads to a significant decrease in major complications and substantial cost savings.

Evaluation of the iron status of the newborn by soluble transferrin receptors in serum.

The concentration of soluble transferrin receptors in serum has proven to be a reliable predictor of iron status in adults. Its high sensitivity for iron deficiency combined with a small sample size (10 microliters) makes it an interesting parameter for the assessment of iron stores in newborn infants. In the present study we investigated the usefulness of the concentration of soluble transferrin receptors in serum in the assessment of iron metabolism in the newborn. Infants born after an uncomplicated labour were compared to infants in the intensive care unit. The concentration of soluble transferrin receptors in serum was found to be elevated compared to normal adults and independently of iron metabolism. The concentration of soluble transferrin receptors did not correlate with serum iron and ferritin concentrations. In contrast to what was found in other studies, no relationship could be demonstrated between soluble transferrin receptors and birth weight or gestational age. The results of this study have shown that care has to be taken in the interpretation of the concentration of soluble transferrin receptors in serum in newborn infants. It seems to be a parameter which is independent of iron metabolism at least during the first days of life.

Surfactant replacement therapy in neonates with respiratory failure due to bacterial sepsis.

We describe four newborns (gestational ages 29-37 weeks; birthweights 1380-3040 grams) who were mechanically ventilated for respiratory insufficiency because of bacterial sepsis. A beneficial effect of bovine surfactant (Alvofact, dosages 50 or 100 mg/kg) was found, as shown by decreases in mean airway pressures and oxygen demands. No side effects were seen after instillation.

Minor neurological dysfunction and quality of movement in relation to neonatal cerebral damage and subsequent development.

Minor neurological dysfunction (MND) and quality of movement were studied in relation to neonatal cerebral damage and developmental assessments at 3 1/2 years of age in 66 very low-birthweight children without obvious disability. MND was found in 19 children and was significantly related to the quality of movement. The results demonstrate that MND is associated with neonatal cerebral damage at preschool-age, but that the assessment of quality of movement is associated with more complex sensory motor tasks and simultaneous processing. At preschool-age, quality of movement might therefore be a better marker of later learning problems than traditional signs of minor neurological dysfunction.

A case of severe neonatal thrombocytopenia with schizencephaly associated with anti-HPA-1b and anti-HPA-2a.

We report a family with a neonate who was severely damaged by intracranial haemorrhages. These probably occurred before the 20th week of gestation. The neonate had a moderate thrombocytopenia. In the maternal serum anti-HPA-1b and anti-HPA-2a alloantibodies were detected. Third-generation assays were applied to identify the alloantibodies. No other cause for the bleeding was found. Probably the combination of anti-HPA-1b and anti-HPA-2a alloantibodies, directed against the platelet fibrinogen receptor and the von Willebrand receptor, respectively, induced a thrombocytopenia and a thrombocytopathy.

Effects of biological and social factors on the cognitive development of very low birth weight children.

OBJECTIVE: To investigate the effects of biological and social factors on the cognitive development of very low birth weight children, a longitudinal follow-up study was conducted from birth to 3.6 years of age. METHODOLOGY: The study group consisted of a cohort of 79 surviving high-risk, very low birth weight infants. Neonatal cerebral ultrasonographic findings and a neurological score were used as indicators of biological risk. A sociodemographic risk score and the Home Observation for Measurement of the Environment inventory were used as indicators of social risk. Cognitive development was assessed at ages 1 and 2 years by the Mental scale of the Bayley Scales of Infant Development and at age 3.6 years by the Kaufman Assessment Battery for Children. RESULTS: The mean mental index at 1 year of age was 96 (SD 19), at 2 years of age 86 (SD 26), and at 3.6 years of age for intelligence 87 (SD 13) and for achievement 86 (SD 14). In a stepwise multiple regression analysis of biological as well as social factors, the neurological score alone was the best predictor for cognitive development at 1 year of age, explaining 46% of the variance. From 2 years of age onward, the best predictors for cognitive development were the neurological score together with the home environment, explaining 46% of the variance for the Mental Developmental Index at age 2, 34% for intelligence, and 56% for achievement at age 3.6. CONCLUSIONS: Children at high biological risk were able to catch up on their cognitive delay in a highly stimulating home environment. Children at low as well as high biological risk in a less stimulating home environment showed a decline in cognitive development. For these children, early intervention programs might be important in the prevention of cognitive disabilities.

Ambient light, ophthalmic artery blood flow velocities and retinopathy of prematurity.

Exposure to bright light was recently proposed as a possible risk factor for the development of retinopathy of prematurity. A semi-longitudinal Doppler ultrasound study was conducted of ophthalmic artery flow velocities at normal and increased ambient light in 22 preterm infants, at post-menstrual ages from 28 to 37 weeks. The aim of this study was to obtain relationships between ophthalmic artery blood flow velocities at various post-menstrual ages and lighting conditions and the occurrence of retinopathy of prematurity. A gradual increase in average blood flow velocities was seen between 28 and 37 weeks. A stepwise increase in flow velocity was seen in all cases when ambient light was increased from moderate to intense. Five of the 22 infants developed retinopathy. No association could be established between ophthalmic artery flow velocities or light-induced changes in flow velocity and the occurrence of retinopathy.

Visual functions in relation with neonatal cerebral ultrasound, neurology and cognitive development in very-low-birthweight children.

In order to determine the relationship between visual functions and neonatal cerebral ultrasound, neurological examinations and cognitive development, a prospective longitudinal study was conducted in 69 high-risk very-low-birthweight children. Visual development was studied at 1 and 2.6 years of corrected age by assessment of visual acuity, binocular visual fields, optokinetic nystagmus and strabismus. Visual impairments were found in 33% at age 1 and in 28% at age 2.6. Visual impairments were related to intraparenchymal damage, as detected by neonatal cerebral ultrasound, as well as to abnormal neurological examinations and lower mean developmental indices. A stepwise multiple regression analysis with neonatal cerebral ultrasound as the dependent variable and visual functions at ages 1 and 2.6 and neurological examinations at ages 1 and 2 as independent variables, however, demonstrated that standardized neurological examinations were better markers of neonatal cerebral damage than visual functions. In cognitive development at ages 1 and 2, the neurological examination at age 1 was the most important variable. In cognitive development at age 3.6, visual functions at age 2.6 were more important. Early visual impairments might thus influence later cognitive development. The effectiveness of appropriate early intervention strategies to stimulate visual and cognitive development in infants with less severe visual impairments should be subject to further study.

Early assessment and neurodevelopmental outcome in very low-birth-weight infants: implications for pediatric practice.

To determine which assessments are useful, at what age, in order to identify handicaps in very low-birth-weight infants, neonatal cerebral ultrasound findings, neurological examinations and the mental scale of the Bayley Infant Scales of Development at 1 and 2 years of age were examined in relation to neurodevelopmental outcome at 3.6 years of age in a cohort of 79 high-risk very low-birth-weight infants. At 3.6 years of age, a minor handicap was found in 9 (11%) and a major handicap in 4 (5%) children. Cerebral palsy was found in 9 (11%) children at 3.6 years of age and could only be diagnosed reliably at 2 years of age. For short-term follow-up, as feedback to the neonatologist, the positive predictive value of intraparenchymal damage, as detected by neonatal cerebral ultrasound, was greater than the positive predictive value of a definitely abnormal neurological examination at 1 year of age. Visual handicaps (n = 4, 5%) and severe hearing deficits (n = 1, 1%) were all detected in the first year of life. A mental handicap was found in 7 (9%) children. It was impossible to predict mental handicaps for the individual child. Only 35% of the children with a mental delay at 2 years of age had a mental handicap at 3.6 years of age, whereas 35% had a normal cognitive outcome. Pediatricians therefore should be cautious in the interpretation of developmental test results in infancy. Long-term follow-up is essential for the child and its parents.

Behaviour problems of very low-birthweight children.

Parent and clinician reports of behaviour problems among very low-birthweight (VLBW) children at 3 1/2 years of age were studied in relation to indicators of neonatal cerebral damage, cognition and social factors. VLBW children had more depressed behaviour and more internalizing problems by parent report, and also scored significantly more often within the clinical range on total problem scores, than children in the comparison group. Neither neonatal cerebral ultrasound nor neurological examinations were directly associated with behavioural outcome. Cerebral damage was related to cognitive development. Cognition directly influenced behaviour problems according to clinician report, while the home environment did so according to parent report. The authors suggest that depressed behaviour of preschool VLBW children might be associated with parental reactions to the birth of a VLBW child, and that their attention problems might be linked indirectly to brain damage via cognitive impairments.

Hearing and language in preschool very low birthweight children.

To get more insight into preschool language and hearing in high-risk very low birthweight (VLBW) children, we conducted a prospective study in a cohort of 79 children. The prevalence of language impairment and hearing loss at age 3-4 years, their relationship to each other as well as to perinatal conditions, neurodevelopmental outcome and the home environment are described. Mild hearing loss was found in 26%, moderate hearing loss in 13% and severe hearing loss in 3% of the children. None of the children was deaf. Abnormal tympanometry was found in 57% of the children. Hearing loss at age 4 years was related to a less optimal neonatal condition and was not related to the obstetrical condition or to neonatal cerebral ultrasound findings. Language impairment was found in 21% of the children at age 3.6 years. Receptive and expressive language was not related to perinatal conditions. There was no relation between the language assessments and the audiological assessments. Cognition and the home environment of the child were the only independent variables in the prediction of language in preschool VLBW children.

Neonatal cerebral ultrasound, neonatal neurology and perinatal conditions as predictors of neurodevelopmental outcome in very low birthweight infants.

To determine the assessments before discharge from the intensive care unit, that will predict outcome most accurately, a prospective longitudinal study in a cohort of 79 high risk VLBW children was conducted from birth to 3.6 years of age. Birthweight, gestational age, obstetrical and neonatal optimality, neonatal neurological examinations and neonatal cerebral ultrasound were studied in relation to outcome. The best predictor for outcome was a simple cerebral ultrasound classification according to the presence or absence of ventriculomegaly and intraparenchymal damage of any cause. Infants with normal neonatal cerebral scans or abnormal scans without ventriculomegaly almost invariably had a normal neurological outcome. In infants with cerebral lesions with ventriculomegaly the incidence of normal neurological outcome decreased to less than 50%. Intraparenchymal damage was associated with cerebral palsy as well as other (mental and sensori) handicaps in over 85% of the cases. Neonatal neurological examinations at preterm age had additional value in predicting neurological outcome especially in the group with ventriculomegaly. Neither birthweight, nor gestational age, obstetrical or neonatal optimality were independent variables in the prediction of outcome in high risk VLBW children at 3.6 years of age.

Streptococcal pharyngitis and epiglottitis in a newborn infant.

We describe a newborn infant with Streptococcus sanguis septicaemia and concomitant upper airway obstruction due to epiglottitis and pharyngitis. This rare infection of the supraglottic region was treated with endotracheal intubation and antibiotics. Full recovery occurred within 4 days.

Visual field and grating acuity development in low-risk preterm infants during the first 2 1/2 years after term.

The effect of early visual experience on visual field size and grating acuity development was studied longitudinally in 36 appropriate for gestational age (AGA) and 26 small for gestational age (SGA) low-risk preterm infants. These were selected out of 194 very low birth weight (VLBW) infants (birthweight less than 1500 g) born in 1985 and 1986. Criteria for inclusion as low-risk were the absence of neurological, respiratory, circulatory and alimentary problems in the neonatal period; no retinopathy of prematurity and no evidence of abnormality on the neonatal cranial ultrasound scans. Binocular field sizes were assessed using kinetic arc perimetry. Binocular grating acuity was tested by means of the prototype version of the acuity card procedure. Results were compared with norms obtained in control fullterms in earlier studies. Infants were tested at 6 weeks, 6, 6, 9 and 12 months of age from the expected term date. Twenty-two of these infants were retested at 2 1/2 years of corrected age. Visual field size and visual acuity estimates of (both AGA and SGA) low-risk, VLBW preterms and control fullterms overlapped at all test ages, except for a slight but significantly faster development of the upper and the lower visual field at 6 weeks corrected age in the preterm group. These results indicate that for clinical purposes visual experience before the expected term date has not only no measurable effect on the normal development of behavioural acuity, but also no accelerating effect on the development of peripheral vision.

Ophthalmic and cerebral blood flow velocities in preterm infants: influence of ambient lighting conditions.

Doppler ultrasound was used to study ophthalmic and middle cerebral artery flow velocities at different ambient lighting conditions in 28 preterm infants in the first week of life. We found significant increases of ophthalmic and middle cerebral artery blood flow velocities when ambient light was increased from moderate to intense. Flow velocities in the ophthalmic artery increased significantly more than in the middle cerebral artery. Doppler ultrasound studies of ophthalmic blood flow velocity may assist in answering the intriguing question whether light-induced changes of ocular perfusion play a role in the development of retinopathy of prematurity.