Current Pediatric Reviews - Volume 3, Issue 3, 2007

Prevention campaigns to avoid risk factors for the occurrence of sudden infant death syndrome (SIDS) during sleep have led to a significant decrease in the number of infants dying suddenly and unexpectedly during sleep. Despite a growing amount of evidence, the understanding of the mechanisms responsible for SIDS is still largely incomplete. We will review the most recent epidemiological, electrophysiological, genetic and pathological research on this topic. From these data, a comprehensive model for SIDS has been proposed: the death would result from the combination of three factors (a prenatal vulnerability, a critical developmental period and an exogenous postnatal stress) and three potential mechanisms (deficiencies in breathing, autonomic and sleep-wake controls). As arousal represents the last chance of survival when an infant is exposed to a life-threatening challenge during sleep, failure to arouse could be involved in the final pathway of SIDS. An infant could be vulnerable to SIDS because of a deficiency in cardio-respiratory or in sleep/wake behaviour controls during sleep. Genetic, metabolic, nutritional or toxic prenatal brainstem injury could be responsible for these deficits. The infant' s vulnerability lies latent until he/she enters the critical developmental period from 2 to 6 months when significant changes in sleep-wake, breathing and autonomic controls occur. The accident has a greater probability of occurring when the infant is exposed to an infection, or an unfavourable environmental factor which enhances the immature cardio respiratory and sleep/wake behaviours of the infant.

Chronic lung disease in the extremely preterm baby is still a major complication in neonatal intensive care. Perinatal (ante- and neonatal) glucocorticoids are widely used to prevent severe infant respiratory distress syndrome and/or to reduce chronic lung disease respectively. A review of the literature regarding the cardiovascular side effects of antenatal and neonatal glucocorticoids is presented here.

Music is a good tool to communicate with children no matter what kinds of disorders they have because therapists can interact with them via music and musical instruments without using a single word. Verbal and physical abilities are limited in patients who are physically, cognitively, and socially disabled. Babies also have a similar limitation. Baby science is interdisciplinary research investigating how infants understand and communicate with the surrounding world. Therefore, medical music therapy based on baby science (baby-science-based music therapy) is useful in the rehabilitation of patients with various neuromuscular and developmental disorders to maintain, restore, and develop their physical, cognitive, social, and communicative abilities. Basic concepts and their application methods are as follows: imitation (neonatal oral imitation and higher imitation), integration of crossmodal sensation, rhythm, language, attention, and habituation and dishabituation. To improve the quality of life of patients with severe motor disability and massive involuntary movements, a special music keyboard connected to switch devices (micro switches for Duchenne muscular dystrophy and spastic cerebral palsy, and push-button switches for Rett syndrome and athetoid cerebral palsy) has been developed. This assistive technology enables patients to play music and participate in social activities even in the advanced stage of their diseases.

Neurotrophic factors are important for neuronal growth, differentiation, survival of neurons and synaptic formation of certain brain cells. Neurotrophic factors from cerebrospinal fluid (CSF) in neurological disorders both acute and rapidly progressive chronic neurological disorders that lead to mental retardation were studied. Their role in the pathogenesis of neurological disorders was assessed to see whether they may serve as prognostic markers. In neonates suffering from severe asphyxia, increased brain-derived nerve growth factor (BDNF) might counteract neuronal damage. The level of beta-neurotrophic factor (NGF) might serve as a marker of asphyxia. Children with cryptogenic infantile spasms had largely normal levels of NGF, but children with symptomatic spasms had low to negligible levels. The therapeutic action of adrenocorticotrophic hormone may be mediated by a potentiation of neural growth factors. Patients with Rett syndrome had low concentrations of CSF NGF compared with controls and autistic patients. NGF might be important for the function of cholinergic neurons in Rett syndrome. Low levels of CSF insulin-like growth factor-1 were found in patients with progressive encephalopathy-hypsarrhythmiaoptic atrophy syndrome with cerebellar atrophy and in white matter diseases and infantile ceroid lipofuscinosis. Neurotrophic factors may be involved in the pathogenesis of some neurodegenerative diseases. Whether neurotrophic therapies prevent or arrest progression of these diseases still awaits confirmation in clinical trials.

Chronic sleep difficulties commonly coexist with neurodevelopmental and psychiatric problems. Children with special needs may have a variety of sleep disturbances and of these circadian rhythm sleep disorders appear to be the most common. Melatonin supplementation for some circadian rhythm sleep disorders is often an effective treatment because it corrects the associated abnormal melatonin secretion. Melatonin has a benign safety profile and significant potential health benefits. Melatonin has many functions including sleep promoting and chronobiotic properties. Melatonin therapy is only beneficial when persistent sleep difficulties are associated with low melatonin secretion and additional supplementation beyond the therapeutic dose does not result in further sleep promotion. Abnormal neurological modulation of pineal melatonin secretion is commonly present in neurodevelopmental and psychiatric disorders. Melatonin deficiency not only leads to sleep disturbance, but also to multiple health problems. Chronic sleep difficulties of special needs children must not be ignored because they may exacerbate the deficits in development which are already present. In this review, misconceptions about sleep and melatonin therapy, functions of this indoleamine, causes and diagnoses of circadian rhythm sleep disorders, physiological principles underlying treatment, selection of candidates, dose, safety and health benefits are discussed.

Systemic lupus erythematosus (SLE) is an uncommon chronic autoimmune disease in children. In view of its insidious onset and protean manifestations which may involve any body organ system, the diagnosis may become difficult and challenging. We review this condition, and begin by reporting three affected children to highlight its very different modes of presentation. An up-to-date review of the disease is then presented, including diagnosis, complications and recommended therapy including new therapeutic modalities

Bronchiolitis Obliterans (OB) is a potentially life threatening, uncommon, but severe form of chronic obstructive lung disease in children, that results from lower airway injury following a respiratory infection. Clinically, it is characterized by rapid respirations, rales, wheezing and hypoxemia for at least 30 days after the initial injury. Histologically, OB is characterized by luminal obstruction with inflammation, granulation tissue, fibrosis and obliteration of the small airways and bronchiectasis. A number of respiratory viruses such as respiratory syncytial virus (RSV), parainfluenza, and particularly adenovirus are associated with severe lung injury leading to OB. This mini-review will seek to provide an up to date, concise review of the currently available literature and research, in the area of adenovirus associated Bronchiolitis Obliterans in children.

Background: Vaccines have been tremendously successful in preventing many childhood diseases. However, this success has created new challenges: parents are no longer familiar with vaccine-preventable diseases and as a result the object of parental fear has shifted from diseases to vaccines. In this modern era of vaccination, vaccine risk communication plays an increasingly important role in immunization programs. A 1997 Institute of Medicine Risk Communication and Vaccination workshop concluded that: “Although health risk communication has been an active research area for several decades, the science and practice of vaccine risk communication are not yet well developed”. Objective: To review vaccine risk communication interventions conducted and evaluated in the last decade in the United States while identifying opportunities for improvement in this area. Search Strategy: We searched the CSA, PubMed, and PsycINFO databases. The following search terms were used: vaccine( s), vaccination, immunization, communication, parent(s), risk, risk assessment, safety, attitude(s), belief(s). References reported in all articles identified from the database searches were reviewed to locate additional studies. Study Selection: Studies related to vaccine risk communication, including communication interventions involving parents and/or patients, and published in English between 1996 and October 2006 in peer-reviewed journals were included in this review. Data Extraction: First author, year of publication, study design, number of participants, study population, study objectives, findings, and authors' recommendations were extracted independently and in duplicate. Results: Eighteen studies met inclusion criteria; four studies were evaluations of immunization reminder systems that included an educational component. The studies included a variety of outcome measures; most commonly assessed were immunization rates, vaccine or disease knowledge, intervention material recognition, recall and/or comprehension, and parental satisfaction with the intervention materials. While there was considerable variability of findings due to the variation in types of studies, all eighteen reported some favorable finding. Overall, the authors made few recommendations regarding future vaccine risk communication strategies. Conclusions: It is difficult to determine if substantial progress has been made since the Institute of Medicine workshop; the literature is lacking due to the scant number of studies, types of interventions developed, and the limited number of rigorous evaluations. The development and rigorous evaluation of vaccine risk communication strategies is critical to the success of our immunization programs.