Evaluation of screening procedures for congenital cataracts.

AIM: To evaluate the efficacy of two different Swedish screening procedures for early detection of congenital cataracts in comparison with no screening. METHODS: Children born between January 1992 and December 1998 in Swedish regions with an established eye-screening routine procedure, diagnosed with congenital cataract, and operated on before 1 y of age, were included in a retrospective study. Age at referral and age at time of the operation were compared between regions using different screening procedures: screening in the maternity wards (Region 1), at the well-baby clinics (Region 2) and one region without any screening (Region 3). RESULTS: Seventy-two children were included in the study. Concerning early diagnosis and surgery, Region 1 differed significantly from Regions 2 and 3, which were more similar and were combined for further analysis. The difference in detected cases was greatest at 21 d of age (55% vs 18%; p < 0.001), but persisted even at 100 d of age (78% vs 64%; p < 0.02). Region 1 screening resulted in more and earlier cases detected than the other two regions (22 vs 15 per 100,000 births). In 72% of all cases, surgery was performed in response to referrals from either the maternity wards (36%), or the well-baby clinics (36%). However, half of the cases from the well-baby clinics were detected too late, i.e. at > 100 d. CONCLUSION: Eye screening in the maternity ward is preferable to well-baby clinic screening and to no screening at all, since it leads to early detection. Screening should also be performed routinely at well-baby clinics within the period when successful treatment is possible.

Malassezia pachydermatis fungaemia in a neonatal intensive care unit.

UNLABELLED: Malassezia pachydermatis, a non-obligatory lipophilic yeast, has occasionally been implicated in nosocomial fungaemias. This study investigated a cluster of eight cases of M. pachydermatis infection and colonization in a neonatal intensive care unit over a 6 mo period. All patients were preterm with very low birthweight and suffered from various underlying diseases. Prolonged use of indwelling catheters and parenteral lipid formulations were important predisposing factors for their infection. All M. pachydermatis strains were susceptible to amphotericin B, fluconazole and itraconazole but resistant against flucytosine. CONCLUSION: Molecular typing by random amplification of polymorphic DNA showed distinct banding profiles for each blood isolate. Since no epidemiological association among the strains could be shown, the reason for this cluster of nosocomial fungaemias remains unclear.

Budesonide delivered by dosimetric jet nebulization to preterm very low birthweight infants at high risk for development of chronic lung disease.

UNLABELLED: We investigated the effect of an aerosolized corticosteroid (budesonide) on the oxygen requirement of infants at high risk for developing chronic lung disease (CLD) in a randomized, double-blind study. The study objective was to attain a 30% decrease in FiO2 levels in the budesonide treatment group after 14 d of therapy. Thirty very low birthweight (VLBW) infants (median (range)) gestational age 26 wk (23-29) and birthweight 805 g (525-1227) were randomized. Inclusion criteria were mechanical ventilation on day 6 of life, or if extubated on nasal continuous positive airway pressure with FiO2 > or = 0.3. The budesonide (Pulmicort) dose was 500 microg bid, or placebo. The aerosol was delivered with a dosimetric jet nebulizer, with variable inspiratory time and breath sensitivity. Inhalations were started on day 7 of life. Twenty-seven patients completed the study. A significant lowering of the FiO2 levels at 21 d of life was not detected. Infants who received budesonide were more often extubated during the study period (7/8 vs 2/9) and had a greater relative change from baseline in their oxygenation index (budesonide decreased 26% vs placebo increased 60%). Subsequent use of intravenous dexamethasone or inhaled budesonide in the treatment group was significantly less. All patients required O2 supplementation on day 28 of life. At 36 wk postconceptual age, 61% of infants in the budesonide group needed supplemental O2 as opposed to 79% in the placebo group. No side effects on growth or adrenal function were observed. CONCLUSION: We conclude that inhaled budesonide aerosol via dosimetric jet nebulizer started on day 7 of life for infants at high risk for developing CLD decreases the need for mechanical ventilation similar to intravenous dexamethasone, but without significant side effects.

Children with blindness due to retinopathy of prematurity: a population-based study. Perinatal data, neurological and ophthalmological outcome.

A population-based group of 27 children with total blindness due to retinopathy of prematurity (ROP), born in Sweden from 1980 to 1990, was examined. They constituted all but two of the total of 29 children with total blindness due to ROP known to the national register of visually impaired children when reviewed from 1980 to 1 January 1995. All children had a gestational age of less than 31 weeks and most had had a complicated perinatal period. The retinal disease was discovered late, most often after it had already progressed to bilateral retinal detachment. Repeated vitreoretinal surgery had been performed in most children, but postoperative visual function did not improve. Three-quarters of the group had major neurological impairment (mental retardation, cerebral palsy, or epilepsy) at age 4 to 14 years. There was an impression that extensive ophthalmological efforts delayed neurodevelopmental assessments and examinations as well as adequate habilitation.

Neonatal risk factors for retinopathy of prematurity--a population-based study.

PURPOSE: The aim of the study was to evaluate possible neonatal risk factors for retinopathy of prematurity (ROP) in a population-based group of preterm, very low birth weight, infants. METHOD: The main study group included 202 single-born infants with a birth weight of 1500 grams or less. A group of 57 twins were also described. Selected risk factors were extracted from the neonatal records. RESULTS: Univariate analysis revealed an association between ROP and respiratory distress syndrome (RDS), bronchopulmonary dysplasia (BPD), septicaemia, intraventricular bleeding, and the use of ventilator as well as continuous positive airway pressure. In a stepwise logistic regression analysis, however, only gestational age at birth, birth weight and BPD were significantly associated with ROP. CONCLUSION: Prematurity per se remains the strongest risk factor for ROP.

Neonatal care of very-low-birthweight infants in special-care units and neonatal intensive-care units in Stockholm. Early nasal continuous positive airway pressure versus mechanical ventilation: gains and losses.

Very-low-birthweight (VLBW) infants are usually intubated at birth and mechanically ventilated at neonatal intensive-care units (NICUs). The objectives of this study were to evaluate the use of early nasal continuous positive airway pressure (NCPAP) in a regional cohort and to determine to what extent VLBW infants need transfer to a regional NICU from special-care units (SCUs) that primarily use early NCPAP for respiratory care. We compared the outcome for infants at SCUs and NICUs in Stockholm County, Sweden, from 1988 to 1993. All infants with birthweights of less than 1501 g were included in this study (n = 687). Fifty-nine per cent of the infants (400/687) were supported using only supplemental oxygen or NCPAP. Of these, 170 (25%) received only supplemental oxygen and 230 (34%) were supported only by NCPAP. A total of 350 (51%) infants received early NCPAP. Of these infants, 120 (34%) later required mechanical ventilation. Only 167 (24%) infants received mechanical ventilation from the beginning Failure of NCPAP was significantly associated with the presence of respiratory distress syndrome. A total of 161/412 (39%) infants were transferred from SCUs to NICUs. Of infants < or = 26 weeks' gestation and infants > 26 weeks, 71% and 34% were transferred, respectively. Total mortality was 16%. The mortality for transfers was 20% compared to an overall mortality in SCU and NICU infants of 9% and 15%, respectively. The overall incidence of intraventricular haemorrhage (IVH), grade III-IV was 8%, periventricular leucomalacia (PVL) grade I-IV was 7%, retinopathy of prematurity (ROP) requiring cryotherapy was 4.3% and chronic lung disease (CLD) was 14%. There were significant differences in the incidence IVH, PVL, CLD and ROP between SCU and NICU infants in matched gestational age groups. In conclusion, infants with a gestational age of 27 weeks or more may often be adequately cared for at SCUs without mechanical ventilation by using early NCPAP. However, infants with a gestational age of 26 weeks or less should be transferred to tertiary-care centres preferably before birth, because they will often require mechanical ventilation.

Maternal risk factors for retinopathy of prematurity--a population-based study.

BACKGROUND: The aim of the study was to investigate maternal risk factors for retinopathy of prematurity (ROP) and also to describe risk factors for prematurity per se in our population. DESIGN: A retrospective, population-based study. MATERIAL AND METHODS: The study group included the mothers (identified with the help of a National Birth Register) of 202 single-born preterm infants with a birth weight of 1500 grams or less, from a previous population-based study. From the same register we also received information on 307 mothers of full term pregnancies for description of risk factors for preterm birth. Data from antenatal clinics, labor rooms and neonatal units were studied. RESULTS: Univariate analysis of maternal risk factors for ROP revealed a few positive findings. In a further logistic multiple regression analysis, apart from gestational age at birth and birth weight, only essential hypertension prior to pregnancy was a predicting risk factor (odds/ ratio 8.36, 95% confidence interval 1.62-43.0). CONCLUSIONS: Prematurity per se remains the strongest risk factor for ROP.

Visual impairment in preterm children with periventricular leukomalacia--visual, cognitive and neuropaediatric characteristics related to cerebral imaging.

Thirteen preterm children, aged 4 to 14 years, with visual impairment due to periventricular leukomalacia (PVL) were evaluated for visual function, intellectual level, cognitive profile and motor function. Their visual impairment was characterized by low acuity, crowding, visual field defects and ocular motility disturbances. Their cognitive profile was uneven, often with considerably higher scores on verbal than on visual-spatial tasks. Nine children had normal intelligence, three had mild mental retardation and one had severe mental retardation. In all the children, visual impairment was complicated by visual perceptual difficulties, accounting for their greater visual handicap than would be expected from their visual acuities and strabismus alone. Though CT or MRI revealed bilateral PVL in all the children, six had no motor impairment consistent with cerebral palsy, which is an unexpected finding.

Comparison of prophylaxis and rescue treatment with Curosurf in neonates less than 30 weeks' gestation: a randomized trial.

OBJECTIVE: The aim of this randomized clinical trial was to evaluate the immediate effects of prophylactic administration of Curosurf and to compare outcomes after prophylactic or expectant management. STUDY DESIGN: Porcine surfactant (Curosurf, 200 mg/kg body weight) was administered intratracheally within 10 minutes of birth to preterm neonates with a gestational age of 26 to 29 weeks (n = 75); rescue-eligible neonates (n = 72) were initially subjected to a sham maneuver. The primary end points of the trial, evaluated at the age of 6 hours, were to obtain (1) a 40% decrease in the ratio between transcutaneous oxygen tension (tcPO2) (kPa) and fraction of inspired oxygen (FIO2), and (2) a 50% decrease in the incidence of radiologically verified respiratory distress syndrome (RDS). After 6 to 24 hours, a similar dose of surfactant was given to the neonates of both the prophylaxis and the rescue-eligible group, if they needed mechanical ventilation with an FIO2 > or = 0.6. RESULTS: At 6 hours the prophylaxis group had, in comparison with the rescue-eligible group, significantly higher tcPO2/FIO2 ratios (mean +/- SD: 39.7 +/- 15.3 vs 28.1 +/- 18.1; P < .001) and less severe RDS by radiological scoring (chi 2 = 14.9; P = .005). Severe RDS was present in 19% of the prophylactically treated neonates versus 32% in the rescue-eligible group (P < .05). The prophylaxis group needed shorter periods of FIO2 > 0.40 than the rescue-eligible neonates (P < .01), and eight neonates of the prophylaxis group (11%) versus 23 of the rescue-eligible group (32%) qualified for rescue treatment with surfactant in the interval 6 to 24 hours (P < .01). There were no differences in the incidence or severity of pneumothorax, pulmonary interstitial emphysema, cerebral hemorrhage, periventricular leukomalacia, patent ductus arteriosus, in the duration of mechanical ventilation or time in supplemental oxygen, or in mortality. CONCLUSIONS: Subgroup analysis revealed (1) that administration of corticosteroids reduced the risk of developing neonatal RDS as effectively as did surfactant prophylaxis at birth, and (2) that prophylaxis was effective especially in neonates with gestational age < 28 weeks or birth weight < 1000 g, in male neonates, and in neonates who had received no antenatal treatment with corticosteroids. Our data indicate that prophylactic treatment with surfactant should be considered in high-risk neonates fulfilling these latter criteria.

Perinatal factors associated with retinopathy of prematurity.

The etiology of retinopathy of prematurity appears to be multifactorial. Introduction of new treatments in neonatal care may add new risk factors. We have analyzed the relationship between 42 perinatal factors and the development of retinopathy of prematurity in 78 infants with a birth weight < 1501 g and/or gestational age < 33 weeks. We have also applied a chronological analysis of the maximum and minimum pO2 and pCO2 values. Retinopathy of prematurity was seen in 37 of 78 infants (47.4%). Nineteen factors were found to be related to the development of retinopathy of prematurity. However, when step-wise logistic regression analysis was used, only birth weight, number of days of oxygen therapy and use of beta-blocking agents by the mother before birth were found to be associated with the development of retinopathy of prematurity. The results suggest that medication with beta blockers immediately before birth should be used cautiously.

Morbidity and mortality in 262 infants with birth weight below 1500 g born in Stockholm County 1988-1992.

The aim of this study was to examine the relationship between neonatal clinical conditions and the development of senso-motoric handicaps. Two hundred and sixty-two infants with a birth weight < 1500 g, born in or referred to the Karolinska/S:t Göran's Children Hospital from September 1988 to April 1992 were studied prospectively. The total survival was 83%. Preliminary results of the Griffiths' test of these infants showed an average cognitive ability of the same level as for full-terms except for those pre-term infants who had suffered from the combination of chronic lung disease and leukomalacia. The incidence of moderate to severe senso-motoric disability is up to the present time 5%.

Allo-immunization during pregnancy. Clinical results from 1983 to 1989 in a Scandinavian university hospital.

From 1983 to 1989, 147,068 pregnancies were analyzed for allo-immunization against erythrocyte antigens. Approximately half of the cases were due to immunization against factor D and the others were due to allo-immunization against other antigens (K, c, E, etc.). In 61 cases exchange transfusion of the newborn was needed and in 115 cases diagnostic amniocentesis was done during pregnancy. Intrauterine transfusions were performed in 10 cases. Fetal and neonatal mortality was 4% in these moderate to severe cases, all due to immunization against D. Immunization against D was due to failure to give immunoglobulin anti-D in about 2/3 of the cases. Systematic prophylactic treatment with anti-D during pregnancy would probably not be cost-effective in this population.

Regressed retinopathy of prematurity: the relationship between clinical risk factors of the newborn period and regressed retinopathy of prematurity severity in a preterm born population of Stockholm county 1976-81.

In a retrospective study, clinical risk factors of the neonatal period were correlated with the severity of regressed retinopathy of prematurity (ROP) in a population of preterm infants (bw less than 1500 g and or gestational age less than 33 weeks). At the age of 5-11 years 134 out of 528 preterm born infants (25.4%) were found to be under ophthalmic care. Reliable information on eye fundus status could be obtained in 105 of them. Regressed ROP was found in 61, the moderate form in 48 (9.1%) and the severe form in 13 (2.5%) patients. Twelve patients (2.3%) had visual acuity of less than 0.3 on the worst eye and two (0.4%) of these patients were blind from ROP. Twenty-four clinical factors of the newborn period were correlated with the severity of regressed ROP. The results suggest that long oxygen exposure in combination with other factors interfering with retinal vasotonus are associated with the degree of the disease developed.

Characterization of beta-lactam-resistant Klebsiella oxytoca isolated in a neonatal intensive care unit.

The occurrence of Klebsiella oxytoca resistant to ampicillin, piperacillin, aztreonam and cefuroxime in a neonatal intensive care unit, including two cases of septicemia, was shown to consist of a spread on three consecutive occasions caused by three different biochemical Klebsiella oxytoca phenotypes. All isolates, except six surface isolates from one infant belonging to phenotype 1, were sensitive to cefotaxime (MIC 0.5-4 mg/l) and ceftazidime (MIC 0.25-1 mg/l). Isolates of phenotypes 1 and 2 produced a beta-lactamase with an isoelectric point of 5.5 and isolates of phenotype 3, a beta-lactamase with an isoelectric point of 7.9. The beta-lactamases of all three phenotypes hydrolysed benzylpenicillin and more slowly cephalothin. All phenotype 1 isolates carried a 2.9 Md plasmid and most isolates also a 36 Md plasmid. All phenotype 2 isolates carried a 4.8 Md plasmid and one isolate also a 30 Md plasmid. The phenotype 3 isolates carried only one 85 Md plasmid.

Arterial blood pressure in the very low birthweight neonate. Evaluation of an automatic oscillometric technique.

The noninvasive oscillometric technique of arterial blood pressure determination was evaluated in fifteen very low birth weight neonates (less than 1,400 g). Measurement of invasive umbilical artery catheter pressure was used as a reference standard. By using a cuff width to arm circumference ratio of 0.33-0.42, mean blood pressure was overestimated by 6.2 (7.2) mmHg (mean and 1 SD), p less than 0.001. More accurate mean blood pressure measurements were recorded with a cuff width to arm circumference ratio of 0.44-0.55. Measurements of systolic and diastolic blood pressure showed similar results.

Pharmacokinetics of free and total flucloxacillin in newborn infants.

Flucloxacillin 50 mg/kg b.w. was administered intravenously (in combination with ampicillin/gentamicin) and orally (with amoxicillin) to 9 newborn infants (gestational age 33-41 weeks) to treat bacterial infections. The concentrations of flucloxacillin in plasma and urine after i.v. injection were analysed according to an open two-compartment model, and the plasma protein binding of flucloxacillin and its distribution to blood cells and plasma water in whole blood were determined. Considerable differences were found from values reported in adults. The terminal half-life averaged 4 h 38 min and was significantly correlated with gestational age. Plasma clearance was low (0.744 ml X min-1 X kg-1), due to the small renal clearance (0.182 ml X min-1 X kg-1), whilst non-renal clearance (0.563 ml X min-1 X kg-1) was approximately the same as in adults. The mean apparent volume of distribution of total drug (Vz) was 0.280 l/kg. The corresponding volume of distribution of unbound drug (Vu1 + Vu2) was 1.74 l/kg, which indicates considerable extravascular drug binding. The plasma protein binding of flucloxacillin (mean 86.3%) was significantly correlated with gestational age and the bilirubin/albumin concentration ratio. Bioavailability after oral administration, when corrected for changes in terminal half-life, was 47.7%, which is only slightly lower than that reported in adults. Since the plasma concentrations after both i.v. and oral administration were well above the MIC-values generally reported for Staphylococcus aureus, and since few side-effects were observed, intravenous injection or, in selected cases, orl administration of flucloxacillin appears to be a reliable therapy for the treatment of infections due to sensitive strains of S. aureus in premature newborn infants.