Respiratory problems in children with neurological impairment

Children with severe neurological impairment have a high incidence of respiratory problems which are multifactorial and may be related to or dependent on the underlying disability. In addition, common respiratory conditions such as asthma will be represented in this group as in the general paediatric population. In order to maximise quality of life and reduce morbidity and mortality, each child should be carefully assessed and treated, making adjustments where necessary in the treatment regime to take account of the disability.     

Adolescents with special health needs: prevalence, severity, and access to health services

The health care needs of disabled adolescents are examined using data from a nationally representative sample of 15,181 randomly selected adolescents aged 10 to 18 years from the 1984 National Health Interview Survey. More than 6% of adolescents, or nearly 2 million nationwide, suffered some degree of disability or limitation in their usual activities in 1984. The leading causes of disability were mental disorders and respiratory diseases. Adolescents living in poverty and in households where the adult members had completed little formal education exhibited increased risk of disability. Disabled adolescents were shown to have three times as many physician contacts annually and spend nine times as many days hospitalized as their nondisabled counter-parts. One in every seven adolescents with a disability was found to be uninsured--exposing their families to extreme financial risks. The great cost of insurance was cited as the primary reason for absence of coverage for 70% of all adolescents without coverage. Public policy implications of these results are discussed.     

Transition to adult care for young people with cerebral palsy

Cerebral palsy (CP) is associated with a high burden of comorbid respiratory disease subsequent to multiple risk factors associated with increasing levels of disability. Correspondingly, respiratory disease is the leading cause of death in CP, including amongst young people who are transitioning or who have just transitioned between paediatric and adult healthcare services. Therefore, consideration of both preventive and therapeutic respiratory management is integral to transition in patients with CP, as summarised in this review.     

The impact of RSV,adenovirus, influenza,and parainfluenza infection in pediatric patients receiving stem cell transplant,solid organ transplant,or cancer chemotherapy

RVIs are a significant cause of morbidity and mortality in immunocompromised children. We analyzed the characteristics and outcomes of infection by four respiratory viruses (RSV, adenovirus, influenza, and parainfluenza) treated at a pediatric tertiary care hospital in a retrospective cohort of patients who had received cancer chemotherapy, hematopoietic stem cell, or SOT. A total of 208 infections were studied among 166 unique patients over a time period of 1993–2006 for transplant recipients, and 2000–2005 for patients with cancer. RSV was the most common respiratory virus identified. There were 17 (10% of all patients) deaths overall, of which 12 were at least partly attributed to the presence of a RVI. In multivariate models, LRT symptoms in the absence of upper respiratory symptoms on presentation (OR 10.2 [2.3, 45.7], p = 0.002) and adenoviral infection (OR 3.7 [1.1, 12.6], p = 0.034) were significantly associated with poor outcome, defined as death or disability related to RVI. All of the deaths occurred in patients who had received either solid organ or HSCT. There were no infections resulting in death or disability in the cancer chemotherapy group.     

A 2-year follow up of babies enrolled in a European multicentre trial of porcine surfactant replacement for severe neonatal respiratory distress syndrome

The postnatal growth, respiratory status and neurodevelopmental outcome of surviving babies enrolled in the first European multicentre trial of porcine surfactant (Curosurf) replacement for severe neonatal respiratory distress syndrome, were assessed at corrected ages of 1 and 2 years. Follow up rates of survivors were 93% at 1 year and 89% at 2 years. Treated and control groups were similar at both 1 and 2 years in terms of physical growth, the prevalence of persistent respiratory symptoms and the occurrence of major and minor disability. Serum antibodies recognising Curosurf and surfactant-anti-surfactant immune complexes were detected in both treated and control babies, the titres showing no difference between groups. Examination of histological lung sections from non-survivors revealed a higher incidence of severe pulmonary interstitial emphysema in control babies than in those treated with surfactant. Surfactant treatment for severe respiratory distress syndrome reduces neonatal mortality and air leaks and is not associated with an increase in disability 2 years later.     

Outcome in cerebral palsy: life-expectancy

The life-expectancy of people with perinatally acquired cerebral palsy (CP) can be similar to that of the general population, or substantially reduced. The most important factors associated with reduced survival are disabilities of motor, cognitive or visual functions. Prematurity and low birth weight are associated with lower rates of disability and better survival. In contrast, those born light for gestational age have higher rates of severe disability than those of normal birth weight. Very severely disabled children who were born very heavy for gestational age have the worst survival. In recent years, survival has improved for children with severe disabilities and adults with gastrostomies, but not for the majority of people with CP. A 2 year old with severe CP has about a 40% chance of living to age 20, in contrast to a child with mild CP, for whom the chance is 99%. CP, respiratory diseases, epilepsy and congenital malformation are the most commonly recorded causes of early death.     

Antenatal steroids and the developing brain

Randomised clinical trials show that two injections of corticosteroid into the mother before preterm delivery reduce respiratory distress syndrome, neonatal mortality, and intraventricular haemorrhage. However, repeated courses of antenatal steroid are not backed by such evidence of safety and efficacy. Animal studies have shown that maternal corticosteroid delays myelination and reduces the growth of all fetal brain areas particularly the hippocampus. Corticosteroids may reduce or enhance hypoxic-ischaemic injury to the developing brain depending on timing and dosage. Clinical trials of maternally administered corticosteroid show no evidence of increased disability on follow up but numbers are small. Postnatal trials of dexamethasone when brain maturity is still preterm show a significant increase in later disability in the dexamethasone treated groups. There is evidence from randomised trials, retrospective data, experiments on pregnant mice, and the chemical make up of the preparations that betamethasone may be safer and more protective of the immature brain than dexamethasone. Single course corticosteroid treatment before preterm delivery must still be recommended as a life saving and cost effective intervention, but clinicians may wish to change from using dexamethasone to betamethasone. In view of the animal and postnatal data, clinicians should be cautious with repeated courses of antenatal corticosteroids and repetition may be unnecessary for lung maturity.     

Randomised trial of high frequency oscillatory ventilation or conventional ventilation in babies of gestational age 28 weeks or less: respiratory and neurological outcomes at 2 years

BACKGROUND: The long term outcome of children entered into neonatal trials of high frequency oscillatory ventilation (HFOV) or conventional ventilation (CV) has been rarely studied. OBJECTIVE: To evaluate respiratory and neurodevelopmental outcomes for children entered into the United Kingdom Oscillation Study, which was designed to evaluate these outcomes. METHODS: Surviving infants were followed until 2 years of age corrected for prematurity. Study forms were completed by local paediatricians at routine assessments, and parents were asked to complete a validated neurodevelopmental questionnaire. RESULTS: Paediatricians' forms were returned for 73% of the 585 surviving infants. Respiratory symptoms were common in all infants, and 41% had received inhaled medication. Mode of ventilation had no effect on frequency of any symptoms. At 24 months of age, severe neurodevelopmental disability was present in 9% and other disabilities in 38% of children, but the prevalence of disability was similar in children who received HFOV or CV (relative risk 0.93; 95% confidence interval 0.74 to 1.16). The prevalence of disability did not vary by gestational age, but boys were more likely to have overall disability. Developmental scores were unaffected by mode of ventilation (relative risk 1.13; 95% confidence interval 0.78 to 1.63) and were lower in infants born before 26 weeks gestation compared with babies born at 26-28 weeks. CONCLUSIONS: Initial mode of ventilation in very preterm infants has no impact on respiratory or neurodevelopmental morbidity at 2 years. HFOV and CV appear equally effective for the early treatment of respiratory distress syndrome.     

Perinatal risk factors of adverse outcome in very preterm children: a role of initial treatment of respiratory insufficiency?

Aim: To investigate risk factors of adverse outcome in a cohort of very preterm children treated mainly with nasal continuous positive airway pressure (CPAP) during the neonatal course. Methods: In Denmark, preterm children are treated with nasal CPAP as a first approach to respiratory support. A national prospective study of all infants with a birthweight below 1000 g or a gestational age below 28 wk born in 1994-1995 was initiated to evaluate this approach. Of the 269 surviving children 164 (61%) were not treated with mechanical ventilation in the neonatal period. A follow-up of the children at 5 y of age was conducted. Data from the neonatal period and the 5-y follow-up were analysed. Results: In multivariate analyses including 250 children, a severely abnormal neonatal brain ultrasound scan was predictive of cerebral palsy (OR = 19.9, CI 95%: 6.1-64.8) and intellectual disability (OR = 6.2, CI 95%: 2.3-16.5). A high Clinical Risk Index for Babies (CRIB) score (OR = 2.4, CI 95%: 1.1-5.5) and chronic lung disease (OR = 2.8, CI 95%: 1.2-6.9) were predictive of intellectual disability. In univariate analyses mechanical ventilation was associated with cerebral palsy (OR = 4.3, CI 95%: 1.7-10.8) and intellectual disability (OR = 2.2, CI 95%: 1.2-4.2), but the associations became insignificant in multivariate analyses including chronic lung disease and a severely abnormal ultrasound scan.

Conclusion: The associations between neonatal risk factors and adverse outcome in our cohort were very similar to those found in other cohorts with another initial treatment of respiratory insufficiency. We found no significant adverse effects of mechanical ventilation beyond what could be explained by associations with chronic lung disease and IVH 3-4/PVL.     

Medical management of children with Down syndrome

Down syndrome, trisomy 21, is the most common autosomal trisomy, and commonest identifiable cause of learning disability. Despite current prenatal screening practice birth prevalence continues to be around 1/1000 live births.Children with Down syndrome have an increased risk of congenital abnormalities and a wide range of treatable medical problems. Paediatricians have a key role in ensuring that these are recognized and treated so that the children's progress is not hampered by additional secondary but preventable disability, and health problems do not prevent them reaching their potential.In this article we consider the Paediatrician's role with a suggested approach to medical management throughout childhood, and a review of the most frequently occurring health issues. These include cardiac, respiratory, gastrointestinal and haematological disorders, thyroid dysfunction, hearing and vision problems, cervical spine and other orthopaedic problems, immune and autistic spectrum disorders, growth and sexual development.     

Medical management of children with Down syndrome

Down syndrome, trisomy 21, is the most common autosomal trisomy, and commonest identifiable cause of learning disability. Despite current prenatal screening practice birth prevalence continues to be around 1/1000 live births.Children with Down syndrome have an increased risk of congenital abnormalities and a wide range of treatable medical problems. Paediatricians have a key role in ensuring that these are recognized and treated so that the children’s progress is not hampered by additional secondary but preventable disability, and so that health problems do not prevent them reaching their potential.In this article we consider the paediatrician’s role with a suggested approach to medical management throughout childhood, and a review of the most frequently occurring health issues. These are cardiac, respiratory, gastrointestinal and haematological disorders, thyroid dysfunction, hearing and vision problems, cervical spine and other orthopaedic problems, immune and autistic spectrum disorders, growth and sexual development.     

Progressive subglottic stenosis in a child with Pallister‐Killian syndrome

Pallister‐Killian syndrome (PKS) is rare genetic disorder caused by tetrasomy 12p mosaicism with supernumerary isochromosome 12p that manifests with intellectual disability, craniofacial dysmorphism, and epilepsy. Although PKS presents as a multisystem morphological defect, respiratory system involvement is rare, except for diaphragmatic hernia. We are the first to report a case of PKS with progressive subglottic stenosis. Subglottic stenosis is a potentially lethal condition due to severe respiratory obstruction and difficult intubation; therefore, further accumulation of cases is required to assess the causal link between PKS and subglottic stenosis.     

Intraventricular haemorrhage and posthaemorrhagic hydrocephalus: pathogenesis,prevention and future interventions

Intraventricular haemorrhage (IVH) is still a major complication of preterm birth with serious disability resulting. The fragile blood vessels in the germinal matrix below the ventricular lining and the instability of blood flow to this highly vascular area are the main mechanisms behind IVH. There is good evidence that corticosteroid therapy before preterm delivery reduces mortality and IVH substantially with a trend towards a reduction in disability. There is good evidence that postnatal indomethacin reduces IVH but no evidence that mortality or disability is reduced. There is evidence that stabilizing cerebral blood flow with pancuronium in infants with respiratory distress reduces IVH in the minority of infants with marked fluctuations. There is limited evidence that postnatal vitamin E and ethamsylate reduce IVH but insufficient evidence of reduced mortality or disability. Hydrocephalus following IVH results initially from multiple small blood clots throughout the CSF channels impeding circulation and re-absorption. Transforming growth factor β is released into the CSF and there is mounting evidence that this cytokine stimulates the laying down of extracellular matrix proteins such as laminin and fibronectin which produce permanent obstruction to the CSF pathways. Interventions such as early lumbar punctures, diuretic drugs to reduce CSF production and intraventricular fibrinolytic therapy have been tested and, not only fail to prevent shunt dependence, death or disability, but have significant adverse effects. Surgical interventions such as subcutaneous reservoir and external drain have not been subject to controlled trial. Ventriculoperitoneal shunt is not feasible in the early phase after IVH but, despite the problems with blockages and infections, remains the only option for infants with excessive head expansion over periods of weeks. New treatment approaches aimed at preventing hydrocephalus are needed.     

Comparison of Self-Esteem and Maternal Attitude Between Children with Learning Disability and Unaffected Siblings

Objectives

To compare self-esteem and maternal attitude between children with learning disability and their unaffected siblings.

Methods

This cross sectional study was conducted at a tertiary care hospital in an urban setting. It comprised of 31 pairs of children with a learning disability, their unaffected siblings and input from their mothers. All children were assessed with Rosenberg self-esteem scale. Mothers were asked to fill Index of parental attitude (IPA) and semi structured proforma with demographic data and questionnaire about her children with a learning disability and his/her unaffected sibling.

Results

Self-esteem was found to be lower in children with learning disability. They felt they do not have much to be proud of and have a fewer number of good qualities. They are also inclined to consider themselves as failures. In factors affecting self-esteem, index of parental attitude was found to be unfavorable towards children with learning disability. Mothers felt child was interfering with their activities and was getting on their nerves. In addition, they also felt that they do not understand their child, feel like they do not love their child and wished that child was more like others they know off. More academic failures, academic difficulties and negative school report were also perceived by mother as lowering child’s self-esteem.

Conclusions

Self-esteem was lower in children with learning disability. In factors affecting self-esteem maternal attitude, academic difficulties, academic failure and negative school reports was found to be unfavorable.     

早产儿异基因细胞移植免疫耐受的研究进展

早产儿是一类特殊的群体,与早产相关的严重神经、呼吸、消化系统疾病的致残/致死率居高不下。异基因细胞移植可能是上述疾病治疗和预防的有效手段。目前,异基因细胞移植治疗缺氧缺血性脑病、支气管肺发育不良以及坏死性小肠结肠炎的动物实验已成功开展。而异基因细胞移植治疗早产儿疾病所面临的困难主要是移植物抗宿主反应,而要解决这一问题,需要实现免疫耐受的成功诱导。本文就近年来早产儿异基因细胞移植免疫耐受的研究进展进行综述。     

Managing specific learning disability in schools in India

Specific learning disability (dyslexia, dysgraphia, and dyscalculia) afflicts 5–15% of school-going children. Over the last decade; awareness about this invisible handicap has grown in India. However, much needs to be done to ensure that each afflicted child gets an opportunity to achieve his or her full academic potential in regular mainstream schools. In order to achieve this ideal scenario, all ‘regular’ classroom teachers should be sensitized to suspect, and trained to screen for this disability when the child is in primary school. School managements should become proactive to set up resource rooms and employ special educators to ensure that these children receive regular and affordable remedial education; and be diligent in ensuring that these children get the mandatory provisions both during school and board examinations. Once specific learning disability is recognized as a disability by the Government of India, these children with the backing of the Right to Education Act, would be able to benefit significantly.     

Burden of childhood and adolescence asthma in Nigeria: Disability adjusted life years

BackgroundA better representation of the burden of childhood asthma should rely on both morbidity and mortality and not only mortality. This will reduce the dearth of information on burden of childhood asthma, and enhance evidence-based decision-making. In this study, burden of childhood asthma was estimated, using disability-adjusted-life-years (DALYs), factoring in the disability weights for asthma, age at mortality and life expectancy.MethodsThe study was conducted at the University of Nigeria Teaching Hospital, Enugu. An Interviewer Administered Questionnaire was used to collect information from parents of children with asthma who presented to respiratory clinics regarding level of their asthma control (controlled, partially controlled and poorly controlled asthma), their age distributions, and gender.The prevalence of asthma, prevalence of associated disability, and case-fatality were obtained from previous publications. The DALYs were estimated by adding together the years lost to disability (YLDs) and years lost to life (YLLs) to asthma (DALYs = YLD + YLL). DALYs were dis-aggregated by age group and by whether their asthma were controlled, partially controlled and poorly controlled.ResultsA total of 66 children with asthma were studied. The proportion of the subjects with controlled, partially controlled and poorly controlled asthma were 26 (39.4%), 31 (47%), and 9 (13.6%) respectively. The subjects that had some form of asthma-related disability were 16 (24.3%). Childhood asthma caused 23.6–34.24 YLLs per 1000 population, 0.01–1.28 YLDs per 1000 population and 24.23–34.41 DALY per 1000 population. There was minimal difference in DALYs across the three clinical categories, but this was consistently higher among older children 12–17 years. The estimated national DALYs was 407820.2, reflecting about of 1.6% of the global all age (children and adults) DALYs of 24.8 million.ConclusionThe DALYs due to childhood asthma were high and did not vary much across the clinical categories, but increased with age. This imperatively necessitates the de-emphasis on just clinical responses as an indicator of the efficiency of childhood asthma control interventions but rather a holistic approach should be adopted considering the limitations the child suffers as a component of both life and environmental modification in a deliberate attempt to prevent attacks. The ability of the child to function optimally while on treatment should be considered in the treatment impact review.     

Imaging findings in Down syndrome

Down syndrome, or trisomy 21, is the most common chromosomal anomaly and is characterized by intellectual disability and a typical facies. People with Down syndrome can have abnormalities of multiple organ systems. Cardiac and respiratory system involvement is the most common cause of morbidity and mortality, although every organ system can be affected. Patients may present prenatally with findings on screening sonography. If the diagnosis is not made prenatally, it is apparent at birth because of the characteristic facial features and musculoskeletal findings. Children with Down syndrome present to the radiology department at various ages depending on the severity of the specific finding. The purpose of this paper is to review the most common antenatal and postnatal imaging findings of Down syndrome as they manifest throughout the body.     

Developmental outcome in newborn infants treated for acute respiratory failure with extracorporeal membrane oxygenation: present experience

OBJECTIVE: To describe the later health status of newborn infants who received extracorporeal membrane oxygenation (ECMO) for acute respiratory failure in the era after the UK ECMO trial. DESIGN: Prospective follow up study of newborn infants who received ECMO at a single centre between January 1997 and January 2001. SETTING: Departments of ECMO and Paediatric Intensive Care, University Hospitals of Leicester. PATIENTS: All babies who received ECMO within 14 days of birth. INTERVENTIONS: Neurodevelopment screening using the schedule for growing skills-II (SGS-II) assessment tool. MAIN OUTCOME MEASURES: Survival at 12 months of age by disease and functional development at follow up. RESULTS: A total of 145 neonates received ECMO for treatment of respiratory failure. Of these, 108 (75%) were alive at 1 year of age. There were no deaths in children treated for respiratory failure secondary to meconium aspiration syndrome (73/145). Ninety three (86% of survivors) infants attended a follow up visit at 11-19 months postnatal age. Eighty two were classed as normal, seven as having "impairment", and four as having "severe disability". CONCLUSIONS: Most newborn infants with acute respiratory failure treated with ECMO will have a normal neurodevelopment screening assessment at 11-19 months of postnatal age. There is no evidence to suggest that changes in neonatal practice since the UK ECMO trial have led to changes in outcome of infants undergoing ECMO therapy.     

Estimating the number of New Zealand children with a physical disability

Aims:   To estimate the number of New Zealand children who have a physical disability, and to describe this group in relation to sex, age, ethnic group and severity.
Methods:   We used data collected by Statistics New Zealand's Household Disability Survey 2001. Within the estimated 90 000 children aged 0–14 years with a disability as described in Disability Counts 2001, we identified a subgroup using information on the use of equipment suggestive of mobility disability, the numbers estimated to be in receipt of or in need of physiotherapy and/or occupational therapy, and the small number needing ramps or similar modifications.
Results:   An estimated 14 500 children had a physical disability. This equates to an estimated 1.7% of the New Zealand population of children aged 0–14 years. Fifty-seven per cent were boys. An estimated 9600 were New Zealand-European, 3800 Maori and 600 Samoan. Approximately half were of moderate severity as defined by Statistics New Zealand. An estimated 9500 had a multiple disability.
Conclusions:   The rate is somewhat higher than in studies from countries where only locomotor disability is described, but similar to rates where other types of disability such as dexterity disability are included. This may reflect the fact that within our group are children who in other countries would have been described as having a dexterity or a self-help disability. It would be useful if future New Zealand data collection could attempt to separate these subgroups in order to allow better forward planning of health, education and family support services.