Self-perceived health status and mental health outcomes in young adults born with less than 1000 g.

AIM: To assess self-perceived health status and mental health outcomes of former extremely low-birth-weight (ELBW) infants at young adulthood compared with community norms and to analyse predictors of poor outcome. METHODS: Fifty-five ELBW adults, 18 men (33%), with median (range) gestational age of 28.7 (25.0-34.0) weeks and birth weight of 930 (680-990) grams, born in Switzerland, were included. They self-rated their health status and mental health at a mean (range) age of 23.3 (21.8-25.9) years. Health status was measured by the Medical Outcomes Study Short Form-36 questionnaire and mental health by the Brief Symptom Inventory. RESULTS: The mean scores for both outcome measures were in the normal range. However, the study group self-rated significantly higher physical health status and lower mental health status compared with the community norms, and scores for self-perceived mental health tended to be worse in the former. ELBW adults reported more problems in socio-emotional role functioning compared with the community norms. Female sex was associated with poorer and bronchopulmonary dysplasia with better mental health status. CONCLUSION: Health status and mental health of former ELBW adults were overall satisfying. However, the comparison with the community norms revealed differences, which may be important for parental and patient counselling and developing support strategies.

How useful is routine amniotic fluid and neonatal surface swab microbiology at Caesarean section?

BACKGROUND: Our aim was to evaluate the clinical impact of routine amniotic fluid and neonatal surface swab microbiology at Caesarean section. MATERIALS AND METHODS: Microbiology data from 1 537 neonates delivered by Caesarean section were analysed in the light of clinical outcome. RESULTS: 1 340 (87%) neonates had non-pathogenic bacteria or negative culture results from both amniotic fluid and surface swab samples. Of the 197 (13%) neonates with pathogenic bacteria, 22 (1.4%) were diagnosed with infection, but only in 6 (0.4%) were the bacteria presumed to be responsible for the infection. Amniotic fluid and surface swab culture had sensitivities of 54% and 35%, and positive predictive values of 14% and 17%, respectively, for detecting a neonate at risk of infection. CONCLUSION: Amniotic fluid and neonatal surface swab microbiology at Caesarean section contributes little if anything to postnatal management and can be safely dropped from operative routine.

[The early postnatal weight gain as a predictor of retinopathy of prematurity]. / Der frühe postnatale Gewichtsverlauf als Prädiktor einer Frühgeborenenretinopathie.

BACKGROUND: Premature infants are often stressed by the current retinopathy of prematurity (ROP) screening procedure. Additionally, only < 10 % of the screened infants will develop a ROP stadium requiring laser therapy. Therefore the present screening strategy is unsatisfactory. Furthermore, the current guidelines do not take into account postnatal factors. A new method considering postnatal factors is the weight, insulin-like growth factor, neonatal ROP (WINROP) algorithm. This approach is based on the early postnatal weight gain. The aim of this study was to assign the WINROP-algorithm to a preterm population in Switzerland and to analyze its ability for prediction. PATIENTS AND METHODS: In this retrospective study, all preterm infants with a gestational age (GA) < 32 weeks and/or a birth weight (BW) ≤ 1500 g taken care of in the Department of Neonatology at the University Hospital Zurich from January 2003 to December 2008 were included. The weekly postnatal weight gain was analyzed by means of the modified WINROP-algorithm. RESULTS: Altogether 376 preterm infants were analyzed. In 58 infants a "high-risk" alarm was released, thereof eight preterms developed a severe ROP and four of them needed laser therapy. CONCLUSIONS: The high predictive value of the WINROP-algorithm was confirmed in our population of Swiss preterms. This instrument has the potential to simplify the current ROP screening procedure. Accordingly, the amount of ophthalmoscopies could be reduced significantly.

Interobserver variability in assessment of cranial ultrasound in very preterm infants.

BACKGROUND: Cranial ultrasound (cUS) findings help doctors in the clinical management of preterm infants and in their discussion with parents regarding prediction of outcome. cUS is often used as outcome measure in clinical research studies. Accurate cUS performance and interpretation is therefore required. AIMS: The aims of this study were (i) to assess the interobserver variability in cUS interpretation, and (ii) to evaluate whether level of cUS expertise influences the interobserver variability. METHODS: Fifty-eight cUS image series of preterm infants born below 32 weeks of gestation collected within the Swiss Neonatal Network were sent to 27 observers for reviewing. Observers were grouped into radiologists, experienced neonatologists and less experienced neonatologists. Agreement between observers was calculated using Kappa statistics. RESULTS: When cystic periventricular leukomalacia, intraventricular haemorrhage and periventricular haemorrhagic infarction were combined to one outcome, agreement among all observers was moderate. When divided into subgroups, kappa for the combined outcome was 0.7 for experienced neonatologists, 0.67 for radiologists and 0.53 for inexperienced neonatologists. Marked difference in interobserver agreement between experienced neonatologists and radiologists could be found for haemorrhagic periventricular ifraction (HPI). CONCLUSIONS: Our results suggest that interobserver agreement for interpretation of cUS varies from poor to good varying with the type of abnormality and level of expertise, suggesting that widespread structured training should be made available to improve the performance and interpretation of cUS.

[Ethical problems regarding extremely preterm infants]. / Ethische Probleme bei extrem unreifen Frühgeborenen.

In the industrialized countries during the last 20 years, ever smaller preterm infants have been re-animated after birth, subjected to mechanical ventilation, fed parenterally and treated with various drugs. The smaller and more premature the infant, the longer the intensive care, the greater pain and suffering for the infants concerned and the worse the long-term results. Although mortality decreased, chronic problems of the lungs, eyes and brain (cerebral palsy) increased in the surviving infants. Therefore specialists as well as the general public began to discuss the limits of using intensive medicine. Investigations among neonatologists and nursing staff all over Europe resulted in a wide spectrum of opinions where the line should be drawn, e.g. concerning gestational age. A majority is however in favour of stopping intensive care measures when these are not in the best interest of the infant, i.e. when treatment becomes painful and inescapable death is only postponed. In order to reach such a difficult medico-ethical decision in a structured, understandable and well-supported way, special procedures were developed, which have stood their test in practice.

[Ultrasonically established cystic periventricular leukomalacia (PVL): incidence and associated factors in Switzerland 1995-1997]. / Sonographisch nachgewiesene zystische periventrikuläre Leukomalazie (PVL): Inzidenz und assoziierte Faktoren in der Schweiz 1995-1997.

BACKGROUND: Cystic periventricular leukomalacia (PVL) is an ischemic brain lesion that mainly affects preterm infants and causes severe neurological damage. Diagnosis is made by cranial ultrasonography. Objectives of this study were to determine the incidence, to identify associated factors and to evaluate the frequency of neurological abnormality at discharge. PATIENTS AND METHODS: Infants with PVL in Switzerland were systematically registered (Swiss Pediatric Surveillance Unit, Swiss Neonatal Network) over three years (1995 to 1997). They were compared to a control group matched for gestational age. RESULTS: Over the three year period 40 infants with PVL defined as at least 2 cysts with diameter = 2 mm in the periventricular region were registered (35 of them were preterm babies). In comparison with the matched controls the infants with PVL had received significantly less frequently antenatal corticosteroids (44 vs 78%, Event Rate Ratio 0.57, 95% Confidence Interval 0.38-0.68), they had lower umbilical cord arterial pH and lower Apgar scores; there was a trend to arterial hypotonia and hypocapnia associated with PVL. The infants of the study group needed more often mechanical ventilation or nasal CPAP (92% versus 67%; ERR 1.38, CI 1.07-1.77) and had more often intracranial hemorrhage (39 versus 14%; ERR 2.8, CI 1.13-6.96). 56% of the infants with PVL were considered abnormal at the neurological examination at discharge compared to 28% in the control group (p < 0.02). CONCLUSION: The incidence of PVL in Switzerland is 1.2% for preterm infants with a birth weight less than 1500 g. Cranial ultrasonography on infants at risk for PVL is important because 44% of the infants with PVL didn't show neurologic abnormalities at discharge.

The influence of a clear layer on near-infrared spectrophotometry measurements using a liquid neonatal head phantom.

It is difficult to test near-infrared spectrophotometry instruments in vivo. Therefore we constructed a liquid phantom which mimics the neonatal head. It consists of a spherical 3.5 mm thick layer of silicone rubber simulating skin and bone and a 0.5 mm thick clear layer of polypropylene imitating cerebrospinal fluid. It acts as container for a liquid solution with Intralipid, 60 micromol l(-1) haemoglobin and yeast. The solution was oxygenated using oxygen and then deoxygenated by the yeast. From the instrumental (Critikon 2020) algorithm, we found that with increasing scattering (0.5%, 1%, 1.5% and 2% Intralipid concentration) the reading was increasingly offset from the expected value of 0 micromol l(-1) by 55.7, 68.6, 76.5 and 80.4 micromol l(-1) (oxyhaemoglobin) and 16.0, 24.4, 29.6 and 31.7 micromol l(-1) (deoxyhaemoglobin). This reduced the range of the oxygen saturation reading from the expected 100% to 31.5, 21.1, 14.3 and 11.5%. Haemoglobin concentration changes were increasingly underestimated by a factor of two to four. For a second algorithm based on the diffusion approximation the offsets were smaller: oxyhaemoglobin 11.4, 17.8, 22.5 and 25.1 micromol l(-1) and deoxyhaemoglobin 1.3, 3.4, 5.2 and 6.0 micromol l(-1). The range of the oxygen saturation reading was higher: 41.3, 29.2, 23.4 and 16.6%. Concentration changes were underestimated by a factor of six to ten. This study demonstrates the need to develop algorithms which take into consideration anatomical structures.

A glycine 375-to-cysteine substitution in the transmembrane domain of the fibroblast growth factor receptor-3 in a newborn with achondroplasia.

Achondroplasia, the most common form of chondrodysplasia, has been associated with mutations in the gene of the fibroblast growth factor receptor-3 (FGFR-3) on chromosome 4p. All 39 achondroplasia alleles studied so far carried point mutations which caused the same amino acid exchange, a substitution of glycine by arginine at position 380 (G380R) in the transmembrane domain of the receptor. We report on a newborn with achondroplasia who does not carry a G380R mutation but has a mutation causing substitution of a nearby glycine with a cysteine (G375C). This observation indicates allelic heterogeneity and confirms the role of mutations in the transmembrane domain of FGFR-3 in the pathogenesis of achondroplasia.

Cerebral blood flow in preterm infants affected by sex, mechanical ventilation, and intrauterine growth.

Sex differences in cerebral blood flow (CBF) values have been demonstrated in adults but not in newborns. This study evaluated the influence of sex, intrauterine growth, and need of mechanical ventilation on resting cerebral blood flow in preterm neonates. Sixty-eight preterm infants with gestational ages of less than 34 weeks and birth weights of less than 1,500 gm were enrolled into the study. Cerebral blood flow was measured by the noninvasive intravenous xenon 133 method 3 times. Measurements were classified into 3 groups: group 1: measurement at 2-36 hours (n = 46); group 2: measurement at 36-108 hours (n = 39); and group 3: measurement at 108-240 hours (n = 41). In all 3 groups, the CBF in girls was significantly lower than in boys (group 1: 11.5 +/- 2.8 ml/100 gm/min vs 14.0 +/- 4.1 ml/100 gm/min; group 2: 13.4 +/- 2.9 ml/100 gm/min vs 16.3 +/- 4.3 ml/100 gm/min; group 3: 12.9 +/- 3.2 ml/100 gm/min vs 15.3 +/- 3.1 ml/100 gm/min). In group 1, the CBF in neonates requiring mechanical ventilation was significantly lower (P < .05) than in patients who were spontaneously breathing (11.5 +/- 3.7 ml/100 gm/min vs 14.2 +/- 3.1 ml/100 gm/min), and the CBF in neonates who were too small for gestational age was significantly higher (P < .005) than in children with appropriate intrauterine growth (16.1 +/- 4.1 ml/100 gm/min vs 11.5 +/- 2.6 ml/100 gm/min). It is concluded that in preterm neonates CBF is substantially affected by sex, intrauterine growth retardation, and the need of mechanical ventilation.

[The medical care of newborn infants in Switzerland]. / Die medizinische Versorgung des Neugeborenen in der Schweiz.

Switzerland has one of the lowest neonatal mortalities in the world (5%) which has barely decreased during the last 10 years. The aim of care for newborn infants has therefore shifted from reducing mortality to reducing morbidity and increasing quality of life. Diseases which were frequent and severe some twenty years ago, such as rubella embryopathy and rhesus incompatibility, have almost disappeared today due to general prophylaxis. On the other hand, new, partly iatrogenic diseases, such as retinopathy of prematurity and bronchopulmonary dysplasia, considerably affect present morbidity of newborn infants. Due to newly developed imaging techniques and genetic, biochemical and immunological methods for screening of risk groups or all pregnant women or newborns, more and more diseases are detected earlier. Therefore the optimal procedure has to be settled early and on an interdisciplinary basis, and include prenatal investigation and possibly treatment, planning of delivery, and early and late postnatal diagnostic and therapeutic measures. The consequence of this development is close cooperation between obstetricians, neonatologists, and pediatric specialists, and a rapidly increasing need for neonatal beds, especially for intensive care. In spite of identification and centralization of women with high risk pregnancy before delivery, every newborn may develop sudden, unpredictable problems of adaptation which need immediate action. Therefore, at every delivery in a clinic or at home the necessary equipment and skilled staff must be available in order to cope with acute problems during adaptation from intrauterine to extrauterine life.

Severe neonatal centronuclear (myotubular) myopathy: an X-linked recessive disorder.

Prenatal onset and rapidly fatal course of centronuclear myopathy are described in four male newborns including two brothers. Diagnosis was established by muscle biopsy within the first week of life in two and at autopsy in the two other patients: Central nuclei, central aggregation of oxydative enzyme activity in the majority of muscle fibers and type 1 fibre hypotrophy were demonstrated. Prenatal manifestation included polyhydramnios, reduced fetal movements and breech presentation. All four newborns developed respiratory insufficiency requiring artificial ventilation immediately after birth. Severe muscular weakness and hypotonia as well as hardly elicitable grasping, deep tendon reflexes and Moro response were noticed. Additional findings included high arched palate, joint contractures, thin ribs, lung hypoplasia, abundant skin and cryptorchidism. In two families, the pedigree contains other affected males, suggesting X-linked inheritance. Seven female carriers were clinically healthy and one of them showed normal muscle histology. Fourteen previously published neonatal cases of centronuclear myopathy are reviewed and compared with our findings. This severe perinatal form of centronuclear myopathy has to be considered in male fetuses and newborns with polyhydramnios and respiratory failure due to muscular weakness or in infants who died of unexplained postnatal asphyxia. Diagnosis should be established by muscle biopsy.

Prenatal diagnosis improves the prognosis of children with obstructive uropathies.

Five neonates with gross obstructive uropathies diagnosed in utero were operated during the first few days of life. Follow-up studies of up to 2 years postoperatively showed normal development of all the children. Radiologic investigations showed no obstruction and normal growth of renal parenchyma in all but one case, thus differing markedly from infants with gross obstructive uropathies who were diagnosed and referred to us some months after birth. The importance of prenatal diagnosis and hence early post partum treatment of obstructive uropathies appears to us proven.

Permanent flaccid paraplegia in children with thoracic spinal cord injury.

From January 1960 to March 1979 25 children with spinal cord injury were admitted to our hospital (10 newborns with birth injury to the spinal cord were excluded). Among 12 patients with complete thoracic lesions four remained permanently flaccid. These four cases who had sustained relatively minor trauma showed marked muscular atrophy of the lower limbs, areflexia, absence of anal and cremasteric reflexes, no response to plantar stimulation and no foot deformities. In contrast to children with spastic traumatic paraplegia, motor nerve conduction velocities and H-reflexes were not measurable in these flaccid patients. Myelography was performed in two, this showing myelomalacia below the level of injury. All four patients had clinically an autonomous bladder and voided by gentle manual pressure. The clinical, neurophysiological and radiological findings are consistent with a lower motor neurone lesion below the level of cord injury, resulting presumably from an extensive longitudinal cord lesion on a vascular basis. Judging from Guttmann's experience, flaccid paraplegia occurs in about 12 per cent of adults with complete thoracic cord lesions. The literature is too scant to give an estimate of this complication in children with traumatic paraplegia.

tcPO2 in pediatric cardiology: application during balloon septostomy, tolazoline administration, and in children with right-to-left shunt.

In pediatric cardiology tcPO2 is useful in monitoring cyanotic children given high-risk therapy such as balloon septostomy or drugs with controversial effects such as tolazoline in persistent fetal circulation. tcPO2 during administration of 100% oxygen enables a rapid, noninvasive differentiation between cyanosis due to intracardiac right-to-left shunt and that due to low cardiac output or pulmonary ventilation or diffusion difficulty. The size of the right-to-left shunt can be roughly estimated from the highest value of tcPO2, this estimation being influenced by anemia, hypothermia, and acidosis, among other factors. A trend of the tcPO2 rise is evident 90 seconds after the beginning of oxygen breathing. If tcPO2 does not rise at least 40 mm Hg over the initial value, a significant right-to-left shunt must be suspected. Interpretation of tcPO2 rise is difficult in dynamic right-to-left shunt, changing with oxygen breathing.