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Background/Aims: The postnatal activation of the hypothalamic-pituitary-gonadal axis is more exaggerated in preterm than in full-term-born infants, and may be important for reproductive function. Our objective was to investigate this activation of the hypothalamic-pituitary-gonadal axis in male very-low-birthweight (VLBW) infants. Methods: Twenty-one VLBW boys (gestational age 26.0-30.0 weeks), participating in the NIRTURE trial, were included. Gonadotropin and testosterone levels were measured in serial urine samples collected at 1 and 4 weeks' postnatal age, at 32 weeks' postmenstrual age, at expected date of delivery and at the corrected age of 3 and 6 months. Results: Longitudinal analysis shows that after birth LH and FSH levels peak at a mean postnatal age of 1-4 weeks (mean postmenstrual age of 30-32 weeks) and decrease until 38 weeks' postnatal age (corrected age of 6 months). Testosterone levels decrease with increasing age, and this decrease is faster in infants receiving early insulin therapy. Conclusions: Serial urine sampling for measurement of gonadotropin and testosterone levels provides accurate information about the postnatal activation of the hypothalamic-pituitary-gonadal axis in VLBW boys. FSH and LH levels peak at 1-4 weeks of age. Insulin treatment causes faster decrease in testosterone levels. Copyright © 2012 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Fetal growth retardation is associated with decreased postnatal growth, resulting in a lower adult height. In addition, a low birth weight is associated with an increased risk of developing diseases during adulthood, such as insulin resistance, type 2 diabetes mellitus, hypertension, dyslipidemia, and cardiovascular diseases. Children with persistent postnatal growth retardation, i.e., incomplete catchup growth, can be treated with human GH. The GH/IGF-I axis is involved in the regulation of carbohydrate and lipid metabolism. The question of whether treatment with GH in children born small for gestational age (SGA) has long-term implications with respect to glucose/insulin and lipid metabolism has not been answered yet. In this article, the available data are reviewed. [ABSTRACT FROM AUTHOR]

Early growth restriction followed by nutritional intakes that permit accelerated growth may result in adiposity and metabolic disease in later life. This study compared growth, body composition and nutritional intake between term age and 6 months post-term in 83 appropriate-for-gestational-age preterm infants with growth restriction at term age (AGA GR+), 15 AGA without growth restriction at term age (AGA GR−) and 33 small-for-gestational-age (SGA) preterm infants. AGA GR+ and SGA preterm infants had higher protein intake, higher energy intake and higher gain in weight SDS between term age and 6 months post-term, with similar lean mass (LM) and lower fat mass (FM) at 6 months post-term compared with AGA GR− preterm infants. In conclusion, despite higher energy and protein intake compared with AGA GR− preterm infants during the first 6 months post-term, AGA GR+ and SGA preterm infants restore their LM without excessive FM. [ABSTRACT FROM AUTHOR]

The decision to disclose the presence of a mental illness in the workplace is one of the most difficult struggles facing any mental health professional who is also a consumer. The benefits of such disclosure are many, including the increased availability of support or accommodation as well as the additional knowledge the individual may bring to the professional role. The risks of such disclosure are also great as stigma exsits in the mental health profession to a degree at least equal to society at large. This article addresses some issues that may emerge for people who choose to disclose, the potential results of disclosure, and strategies to deal with negative reactions. The article also discusses ways in which the employer, coworkers and other significant people in the worker's life can support the individual and minimize the risk of disclosure. [ABSTRACT FROM AUTHOR]

Unlabelled: In a prospective study, 81 preterm infants and 25 healthy term infants were neurologically and neurophysiologically evaluated in the neonatal period. At 5-7 years of age the neurodevelopmental outcome was assessed. The validity and predictive value of the Neonatal Neurological Inventory (NNI) and the Neurobiological Risk Score (NBRS), including an additional gestational age factor (GAF) and an auditory evoked response factor (AERF) were assessed. Three of the 53 surviving preterm infants showed major neurological abnormalities at 5-7 years. Five infants showed neuropsychological abnormalities and 12 infants showed both neurological and neuropsychological abnormalities. An important subgroup of preterm infants could be identified as high-risk using the NNI and NBRS. The low sensitivity and negative predictive value resulted in a number of false-negative results. Logistic regression showed that intraventricular haemorrhage (IVH) and bilirubin levels contributed highly to the prediction of neurological outcome. For neuropsychological outcome these factors were IVH and assisted ventilation. Addition of the GAF and AERF as separate items to the NBRS did not affect the predictive power. Combined addition of the GAF and AERF showed improvement of both validity and predictive value.Conclusion: This study shows that IVH, bilirubin and assisted ventilation contribute most to the validity and predictive value of the NBRS. Furthermore, regarding neurological outcome addition of a GAF in combination with an AERF resulted in a substantial improvement of the validity and predictive value. The shortcomings of the current neonatal risk scores require a careful interpretation of clinical perinatal data regarding the prediction of neurodevelopmental outcome in preterm infants. [ABSTRACT FROM AUTHOR]

Background: Congenital gastrointestinal malformation (CGIM) require neonatal surgical treatment and may lead to disease-specific sequelae, which have a potential psychological impact on parents. The aim of this study is to assess distress and symptoms of post-traumatic stress disorder (PTSD) in parents of patients with CGIM. In this cross-sectional study, seventy-nine parents (47 mothers and 32 fathers) of 53 patients with CGIM completed the Distress Thermometer for Parents (DT-P) and the Self Rating Scale for Posttraumatic Stress Disorders (SRS-PTSD) as part of the multidisciplinary follow-up of their children (aged 5-35 months). Group differences were tested between parents and representative Dutch reference groups with regard to rates of (clinical) distress and PTSD, and severity of overall distress and PTSD, for mothers and fathers separately. Mixed model regression models were used to study factors associated with the risk of (clinical) distress, PTSD and with severity of symptoms of PTSD (intrusion, avoidance and hyperarousal).Results: Prevalence of clinical distress was comparable to reference groups for mothers (46%) and fathers (34%). There was no difference in severity of overall distress between both mothers as well as fathers and reference groups. Prevalence of PTSD was significantly higher in mothers (23%) compared to the reference group (5.3%) (OR = 5.51, p < 0.001), not in fathers (6.3% vs 2.2.%). Symptoms of intrusion were commonly reported by all the parents (75%). Longer total length of child's hospital stay was associated with more severe symptoms of intrusion, avoidance and hyperarousal. Child's length of follow-up was negatively associated with severity of intrusion.Conclusions: Having a child with CGIM has a huge impact on parents, demonstrated by a higher prevalence of PTSD in mothers, but not fathers, compared to parents in the general population. Monitoring of symptoms of PTSD of parents in follow-up is necessary. [ABSTRACT FROM AUTHOR]

The past two decades have witnessed a renewed interest in low frequency radio astronomy, with a particular focus on frequencies above 30 MHz e.g., LOFAR (LOw Frequency ARray) in the Netherlands and its European extension ILT, the International LOFAR Telescope. However, at frequencies below 30 MHz, Earth-based observations are limited due to a combination of severe ionospheric distortions, almost full reflection of radio waves below 10 MHz, solar eruptions and the radio frequency interference (RFI) of human-made signals. Moreover, there are interesting scientific processes which naturally occur at these low frequencies. A space or Lunar-based ultra-low-frequency (also referred to as ultra-long-wavelength, ULW) radio array would suffer significantly less from these limitations and hence would open up the last, virtually unexplored frequency domain in the electromagnetic spectrum. A roadmap has been initiated by astronomers and researchers in the Netherlands to explore the opportunity of building a swarm of satellites to observe at the frequency band below 30 MHz. This roadmap dubbed Orbiting Low Frequency Antennas for Radio Astronomy (OLFAR), a space-based ultra-low frequency radio telescope that will explore the Universe's so-called dark ages, map the interstellar medium, and study planetary and solar bursts in the solar system and search them in other planetary systems. Such a radio astronomy system will comprise of a swarm of hundreds to thousands of satellites, working together as a single aperture synthesis instrument deployed sufficiently far away from Earth to avoid terrestrial RFI. The OLFAR telescope is a novel and complex system, requiring yet to be proven engineering solutions. Therefore, a number of key technologies are still required to be developed and proven. The first step in this roadmap is the NCLE (Netherlands China Low Frequency Explorer) experiment, which was launched in May 2018 on the Chinese Chang'e 4 mission. The NCLE payload consists of a three monopole antenna system for low frequency observations, from which the first data stream is expected in the second half of 2019, which will provide important feedback for future science and technology opportunities. In this paper, the roadmap towards OLFAR, a brief overview of the science opportunities, and the technological and programmatic challenges of the mission are presented. [ABSTRACT FROM AUTHOR]

Background Growth hormone ( GH) treatment is effective in improving adult height ( AH) in short children born SGA. However, there is a wide variation in height gain, even after adjustment for predictive variables. It is therefore important to investigate new factors which can influence the response to GH. Objective To investigate the efficacy of GH treatment (1 mg/m2/day) in short SGA children on AH. To assess the relation between spontaneous catch-up growth after birth and growth during puberty on the total height gain SDS to AH. Patients Longitudinal GH trial in 170 children. Results Median age at start of GH was 7·1 years and height −3·0 SDS. AH was −1·8 SDS ( TH-corrected AH −1·1 SDS) in boys and −1·9 SDS ( TH-corrected AH −1·3 SDS) in girls. Spontaneous catch-up growth after birth was ≥0·5 SDS in 42% of children. In contrast to expectation, spontaneous catch-up growth was negatively correlated with total height gain SDS during GH ( P = 0·009). During puberty, height SDS declined (−0·4 SDS in boys and −0·5 SDS in girls) resulting in a lower total height gain SDS than expected. Pubertal height gain was 25·5 cm in boys and 15·3 cm in girls, significantly lower compared to AGA children ( P < 0·001). At onset of puberty, BA for boys and girls was moderately advanced ( P = 0·02 and P < 0·001, respectively). Growth velocity was comparable to AGA children during the first two years of puberty, but thereafter significantly lower until reaching AH ( P < 0·001). Conclusion In contrast to our hypothesis, children with greater spontaneous catch-up growth after birth show a lower total height gain SDS during GH. Height SDS declines from mid-puberty, due to a marked early deceleration of growth velocity. [ABSTRACT FROM AUTHOR]

Abstract: For a species to develop in nature basically two things are needed: an enabling technology and a “niche”. In spacecraft design the story is the same. Both a suitable technology and a niche application need to be there before a new generation of spacecraft can be developed. In the last century two technologies have emerged which had and still have a huge impact on the development of technical systems: Micro-Electronics (ME) and Micro-Systems Technology (MST). Many different terrestrial systems have changed dramatically since the introduction of ME and MST and many new systems have emerged. In the same period many nano-satellites have been built and launched and shown that they can perform in space. Still it is not clear what the specific role of these small satellites will be. Where will they go? What will they do? In this paper the authors will try to answer these questions and will refer to the OLFAR space born radio telescope as one of the niche applications for a nano-satellite swarm. [Copyright &y& Elsevier]

Sjögren-Larsson syndrome (SLS) is an autosomal recessive neurometabolic disorder characterized by spasticity, learning disability, and ichthyosis. To our knowledge, there is no detailed report in the literature concerning the functional consequences of SLS. Therefore, we performed a cross-sectional study of motor performance and everyday functioning in 17 patients with this rare disorder. Nine female and eight male patients with SLS (age range 1-35y) were investigated. Data were obtained by structured interview with parents and patients with SLS, a telephone-conducted questionnaire, and physical examination. Motor performance was measured by the Gross Motor Function Measure; everyday functioning was assessed using the Pediatric Evaluation of Disability Inventory and the Vineland Adaptive Behavior Scale. In most patients, spasticity was bilaterally present in hamstrings, hip adductors, and gastrocnemic muscles. All participants above 7 years had contractures in the lower extremities. Limitations were present in all gross motor dimensions, except for lying and rolling. Participants had developmental ages far below their chronological age. This study revealed that patients with SLS have limitations in gross motor performance. Although some patients can reach a certain level of independence, most have activity limitations and restrictions in their participation in society. [ABSTRACT FROM AUTHOR]

Spina bifida is a multifaceted neurological condition with complex neuropsychological sequelae. The cognitive outcome in spina bifida has frequently been attributed to the severity of the hydrocephalus. However, because of complex neuropathology, the influence of hydrocephalus alone does not sufficiently explain the deficits in the cognitive profile in spina bifida. To date, little is known of the role of Arnold-Chiari-Il malformation (ACM) in the cognitive profile of these patients. Aim of the current study is to delineate the specific contribution of the ACM in spina bifida by comparing children with ACM and those without ACM. 46 children between 6 and 15 years of age underwent a neuropsychological assessment covering intelligence and a wide range of cognitive functions, such as visuo-motor processing, attention, memory, word fluency and speed of information processing. Comparisons were made between patients with ACM (ACM+) and those without ACM (ACM-); all children with ACM+ also had hydrocephalus. Confounding effects of global cognitive impairment were excluded, such that groups were matched on verbal 10. Because of complex neuropathology, which is inherent to spina bifida, the method applied was based on a comparison of cognitive profiles of the study group with profiles of patients with cerebellar damage and hydrocephalus found in the literature. Impaired visual analysis and synthesis, verbal memory, and verbal fluency, even after correction for global cognitive impairment, were observed in children with ACM. The hypothesis that in addition to impairment in visual analysis and synthesis, which are related to both hydrocephalus and ACM, specific deficiencies in verbal memory and fluency may be attributed to ACM is supported. [ABSTRACT FROM AUTHOR]

This article deals with the tragic experience of parents with young patients suffering from spina bifida. Spina bifida is a congenital malformation of the nervous system causing neurological, urological and orthopaedic defects, as well as possible mental retardation. Since only limited prognoses of the child's mental and physical development can be given at birth, parents are continually confronted with their child's limitations as they manifest themselves in both the first and each successive phase of life. These confrontations usually give rise to feelings of anxiety, helplessness and insecurity, because people come up against their own limits. The experience can be regarded as tragic. With the aid of a measuring instrument for tragic experience devised by SchererRath (2001) we distinguish between three dimensions: a physical, a personal and a religious dimension. We examine to what extent parents of children with spina bifida have tragic experiences and in how far these vary with different neurological and neuropsychological symptoms, and with demographic, worldview-related and ecclesiastic characteristics. Our findings show that among mothers physical tragic experience varies with the severity of their child's spina bifida inasmuch as it concerns the functional lesion level. It also appears that both mothers' and fathers' religious tragic experience varies with worldview-related saliency, church membership and church attendance. [ABSTRACT FROM AUTHOR]

It has long been recognized that from the first months of life auditory perception plays a crucial role in speech and language development. Only in recent years, however, is the precise mechanism of auditory development and its interaction with the acquisition of speech and language beginning to be systematically revealed. This paper presents the results of a series of studies exploring the relevance of electrophysiological measurements for the objective diagnosis of children with language and speech disorders. In the first, retrospective, study, an inventory was made of clinical neurological, neuropsychological, logopedic and neurophysiological findings obtained from 43 children referred to the Paediatric Neurology Centre for the diagnosis of their speech and language deficits. Neurophysiological abnormalities were found in 95% of these children, among these deviant auditory evoked potentials (AEVPs). The second study demonstrated developmental trends in the waveform of the cortical AEVPs up to age 6 years; the implications for longitudinal studies are discussed. Preliminary results from the third study show emerging differences in auditory event-related potentials (AERPs) for young children who are at risk for developmental dyslexia based on their family history. These findings underscore the clinical usefulness of neurophysiological measures for the diagnosis of speech-language disorders. Clinical protocols are further developed and tested. [ABSTRACT FROM AUTHOR]

The corticospinal tract develops over a rather long period of time, during which malformations involving this main central motor pathway may occur. In rodents, the spinal outgrowth of the corticospinal tract occurs entirely postnatally, but in primates largely prenatally. In mice, an increasing number of genes have been found to play a role during the development of the pyramidal tract. In experimentally studied mammals, initially a much larger part of the cerebral cortex sends axons to the spinal cord, and the site of termination of corticospinal fibers in the spinal grey matter is much more extensive than in adult animals. Selective elimination of the transient corticospinal projections yields the mature projections functionally appropriate for the pyramidal tract. Direct corticomotoneuronal projections arise as the latest components of the corticospinal system. The subsequent myelination of the pyramidal tract is a slow process, taking place over a considerable period of time. Available data suggest that in man the pyramidal tract develops in a similar way. Several variations in the funicular trajectory of the human pyramidal tract have been described in otherwise normally developed cases, the most obvious being those with uncrossed pyramidal tracts.A survey of the neuropathological and clinical literature, illustrated with autopsy cases, reveals that the pyramidal tract may be involved in a large number of developmental disorders. Most of these malformations form part of a broad spectrum, ranging from disorders of patterning, neurogenesis and neuronal migration of the cerebral cortex to hypoxic-ischemic injury of the white matter. In some cases, pyramidal tract malformations may be due to abnormal axon guidance mechanisms. The molecular nature of such disorders is only beginning to be revealed. [ABSTRACT FROM AUTHOR]

Drooling frequently occurs in children with multiple handicaps; application of anticholinergic drugs is a potential strategy to treat drooling. A computer aided search of original studies concerning the treatment of drooling was carried out. The methodological and statistical integrity of the identified studies were assessed with previously defined criteria. The articles were weighed for their separate contribution to the evidence. The search resulted in 64 reports, of which seven studies passed the screening and were subjected to further assessment and discussion by three referees. Because of the small number of reports and the methodological restriction within the studies, no meta-analysis could be performed. No general conclusion could be made about the efficacy of anticholinergic drugs in treatment of drooling in children with multiple handicaps. There was some evidence that three anticholinergic drugs (benztropine, glycopyrrolate, and benzhexol hydrochloride) are effective in the treatment of drooling, but it could not be concluded that one drug is preferable. [ABSTRACT FROM AUTHOR]

Unlabelled: The Sjögren-Larsson syndrome (SLS) is an inborn error of lipid metabolism, characterised clinically by congenital ichthyosis, mental retardation and spasticity. Patients also suffer from severe pruritus. The degradation of leukotriene (LT) B4 is one of the defective metabolic routes in SLS. Zileuton inhibits the synthesis of LTB4 and the cysteinyl leukotrienes. Five SLS patients were treated with zileuton for 3 months. Favourable effects were found on pruritus score (P = 0.006), general well-being, and background activity of electroencephalographic studies. Neuropsychological test results did not change significantly. There was, however, a clinically important trend towards improvement in the speed of information processing. Results of cerebral MRI and proton magnetic resonance spectroscopy did not change during therapy. Urinary concentrations of LTB4 and omega-OH-LTB4 decreased significantly (P=0.02 and P=0.003 respectively), while their concentrations in CSF were normal at baseline and remained so after therapy.Conclusion: Patients with Sjögren-Larsson syndrome might benefit from treatment with zileuton, especially with respect to the agonising pruritus. The findings reported here, point to a crucial role for leukotriene B4 in the pathogenesis of pruritus. [ABSTRACT FROM AUTHOR]

Unlabelled: Drooling beyond the age of 4 years is pathological, particularly if it occurs in children with neurological and developmental impairment and disability. Considering the therapeutic spectrum of botulinum toxin A and in view of the innervation of the salivary glands, we postulated that intraglandular injections into the submandibular glands with botulinum toxin A could reduce the secretion of saliva and consequently decrease drooling. Three patients with cerebral palsy and severe drooling were selected and evaluated over a 4-month period. Under ultrasound guidance, one dose of botulinum toxin A was injected bilaterally into the submandibular glands. Saliva secretion was measured at baseline and repeated four times during the following 4 months. In the three patients, maximal salivary flow rate of the sublingual and submandibular glands was reduced by 51% to 63%. The time of the maximal effect differed among the three children. The parents reported a satisfactory reduction of drooling throughout the whole study period. No objectionable disturbances of oral functions were observed. There was mild transient thickening of saliva in one of the patients.Conclusion: The application of botulinum toxin A to the submandibular gland is a promising technique to reduce salivary flow rate and probably an alternative in the treatment of drooling in children with cerebral palsy. [ABSTRACT FROM AUTHOR]

The article investigates the applicability of magnetic motor evoked potentials (MEP) in a prognostic model for spina bifida. According to the authors, study results have shown that MEP is applicable in newborns with spina bifida. The findings, the authors report, demonstrate that excitable neural tissue is present in or under the spinal lesion, even in severe cases of spina bifida.