Current Pediatric Reviews - Volume 5, Issue 2, 2009

For early diagnosis of children with neurodevelopmental disorders, neurological assessment during early infancy and follow-up examinations are essential. However, to make the neurological assessment effective, some knowledge regarding the examination procedures and interpretation of the results is required. There have been many articles on the neurological assessment of early infants, but ones that clearly delineated its diagnostic significance have been surprisingly few. Based on our own experience and the literature, in this review we will discuss the diagnostic significance of neurological assessment of early infants, including of primitive reflexes (crossed extensor reflex, suprapubic extensor reflex, heel reflex, Galant response, asymmetric tonic neck reflex, plantar grasp response, and Babkin reflex), involuntary movements (ankle clonus and tremor), and Vojta's postural reactions. Neurological assessment of these reflexes is simple and useful for early detection of infants at high risk for a neurodevelopmental disorder that might later develop.

Asphyxiated neonates often have hypotension, shock and poor tissue perfusion. Various inotropic medications are commonly used to provide the cardiovascular support to improve the blood pressure and to treat shock. However, there is a paucity of literature on the systemic and regional hemodynamic effects of these inotropic medications in asphyxiated neonates, necessitating cautious translation of findings from other clinical populations and animal studies. This review discusses the current available information, from both clinical studies and animal models of neonatal asphyxia, on commonly used mediations including dopamine, dobutamine, epinephrine, milrinone, levosimendan and hydrocortisone.

In a retrospective study we examined frequency of perinatal asphyxia occurrences and the post mortem changes in newborn infants. Legal outcome of medical malpractice was also analyzed among our cases. There were 66 cases with perinatal asphyxia between 1992 and 2001. During neonatal period 27 infants died. There was one crime case and two civil law cases, which were started against the medical treatment during delivery. The improvements in the quality of prenatal care and the increased number of routine intensive fetus examinations, the in utero transport and the rate of cesarean section, may result in a decreasing perinatal mortality.

We report a case with severe persistent pulmonary hypertension (PPHN) associated with congenital cystic adenomatoid malformation of the lung (CCAM) treated with sildenafil PO. The case was unresponsive to the High-Frequency Oscillatory Ventilation (HFOV), with a oxygenation index (OI) of 50 before sildenafil. Substantial improvement in OI was followed by extubation and survival. No side-effects were noted.

Velocardiofacial syndrome (VCFS), the most common chromosomal microdeletion syndrome in humans, is caused by a heterozygous deletion of chromosome 22q11.2. With an incidence of 1/2000-1/6000, it is associated with a vast array of abnormalities such as congenital heart disease, palatal dysfunction, immune deficiency, hypoparathyroidism and cognitive impairment. In addition to the numerous medical and developmental problems, retrospective studies in the last decade have reported a markedly high incidence (∼ 40%) of schizophrenia, bipolar disorder and depression in late adolescence and adulthood in individuals with the deletion. This risk of schizophrenia spectrum disorders in VCFS approaches that of a monozygotic twin of a patient with schizophrenia, or that of an individual with both parents with schizophrenia. These observations provide the strongest known link between psychosis and an identified genetic condition. In recent years, schizophrenia has been viewed as a neurodevelopmental disorder. This neurodevelopmental theory suggests that neurocognitive and neuroanatomical abnormalities often precede the development of overt psychosis. Genetic factors are thought to be contributory to these neurodevelopmental anomalies that are thought to ultimately culminate in schizophrenia spectrum disorders. However, gene identification has largely been unsuccessful, due to the complex nature of the inheritance of the genes and the probability that multiple genes of individual modest effect are involved. Identification of predisposing genetic markers would enable identification of a high-risk group that would enable the prospective study of the factors contributing to psychosis. The presence of a known genetic abnormality that causes neurodevelopmental deficits, confers a high-risk of psychosis and since the signs, symptoms and response to treatment of schizophrenia secondary to VCFS are thought to be no different to that in primary schizophrenic illness, it has been suggested that VCFS represents an ideal model for the study of the factors contributing to schizophrenia. This article delineates the current understanding of the psychological and psychiatric findings, brain morphometric abnormalities and genetic studies in VCFS and their relevance to schizophrenia spectrum disorders.

An abnormal head posture (AHP), or torticollis, is a common condition in children, with an estimated incidence of 1.3%. This condition is encountered commonly by primary care family pediatricians. AHP can be congenital or acquired. The cause of the AHP can be ocular, orthopedic and neurologic. The orthopedic causes of AHP include congenital muscular torticollis due to tightness of the sternocleidomastoid muscle, Klippel- Feil anomaly and brachial plexus injury. Neurologic causes of AHP are mainly related to brain tumors, postinflammatory central nervous system conditions, psychomotor delay and focal dystonia. Other less common reasons for AHP are: Sandifer syndrome (hiatal hernia associated with gastro- esophageal reflux) and unilateral hearing loss. Numerous ocular conditions can cause AHP or “ocular torticollis”. Among them: superior oblique muscle palsy, lateral rectus muscle palsy, nystagmus, vertically incomitant horizontal strabismus (A or V patterns), Brown's syndrome, Duane's syndrome, refractive errors and DVD. The AHP can take the form of head tilt, face turn, chin up, chin down or combined, depending on the specific etiology. However, there are many variations and the type of the head posture cannot reliably predict the underlying cause. Since the etiology is not always obvious, these patients must be carefully evaluated, and sometimes a multidisciplinary approach is needed, including examinations by ophthalmologist, neurologist and orthopedist. Ocular AHP is usually an attempt to improve visual acuity or binocularity. Some patients adopt the head posture to avoid diplopia caused by incomitant strabismus, those with nystagmus adopt a head position that brings the eyes to the null point (where the oscillations dampen or markedly diminish). Ocular AHP is usually a binocular phenomenon. Rarely, abnormal head position can be acquired following visual loss in one eye. The majority of these ocular conditions require eye muscle surgery. Different ocular etiologies of AHP require different surgical strategy, for this reason careful etiological diagnosis is important. The purpose of this article is to review the ocular conditions that cause AHP, their relative frequency, indication for surgery and the appropriate surgical treatment.

Neuroblastoma is the third most common pediatric cancer in the United States and is responsible for 15% of childhood cancer-related deaths. Despite major advances in multimodal therapy, the clinical outcome for several patients remains poor. Due to the desperate need for innovativation and improved success in the treatment and management of neuroblastoma, research interests in immunotherapy have been on the rise in recent years. Current immunotherapeutic approaches under investigation include antibodies targeting the neuroblastoma antigen GD2, cytokine stimulation of immune cells, use of immunocytokine conjugates, radioimmunotherapy, and tumor-primed dendritic cells. Immunotherapy could serve as a safe alternative or adjunct to current therapeutic protocols and would presumptively have fewer deleterious effects making it more favorable to patients.

Introduction: The role of psychological interventions in improving parents' psychological functioning has received growing recognition. Objective: To review studies regarding the effectiveness of psychological interventions for parents who have children with cancer based on the best available evidence. Methods: Sixteen studies were reviewed using meta-analytic techniques. The outcome measures are emotional distress and positive psychological functioning as reported by parents using standardized questionnaires. The former refers to anxiety, depression, stress and negative mood. The latter refers to general well-being and the use of positive coping strategies. Effect sizes were calculated by comparing control (or pre-intervention) to intervention (or post-intervention) scores. Results: There were moderate support (d = 0.57) for the effectiveness of psychological interventions in improving positive psychological functioning and minimal support (d = -0.35) in alleviating emotional distress. The cognitive behavioral interventions show moderate effects on parental use of positive coping strategies (d = 0.52) and in reducing stress (d = -0.43). Conclusion: Psychological interventions have a potential in improving parental psychological outcomes. Recommendations The effects of psychological interventions could be enhanced by designing treatments that focus on strength and skills building in participants. Having outcomes measures that relate to theoretical model will allow evaluation and refinement of the interventions.

Objective: To describe energy and nutrient intake levels in children 3 years of age. Design: Cohort study to prospectively collect dietary intake three times over one year. Subjects and Settings: A socioeconomically diverse group of 368 white or black non-Hispanic children were recruited from the Cincinnati metropolitan area. Main Outcome Measure: Mean daily dietary intakes of energy and nutrients were calculated for each subject. Group mean, standard deviation, minimum and maximum intakes, and usual intake percentiles were calculated for energy and nutrients. Results: Compared to the DRIs, 42% of study subjects had excess energy intake. Almost all study participants had intakes of fat, carbohydrate, and protein within the recommended range. The study group met requirements for all micronutrients with the exception of vitamin E (49%). Percentage of participants with estimated intake exceeding the recommendations was 1% or lower with the exception of niacin (85%), folate (45%), vitamin A (48%), and zinc (53%). Conclusion: This study suggests that most nutrient intakes for children 3 years of age are adequate. Of concern are the increased intake of energy and some micronutrients. Sustained excess energy intake from an early age may lead to adult obesity and increased risk for many diseases.