Vertigo in Children and Adolescents: Characteristics and Outcome

Objectives. To describe the characteristics and outcome of vertigo in a pediatric population. Patients. All children and adolescents presenting with vertigo to a tertiary otoneurology clinic between the years 2003–2010 were included in the study. Results. Thirty-seven patients with a mean age of 14 years were evaluated. The most common etiology was migraine-associated vertigo (MAV) followed by acute labyrinthitis/neuritis and psychogenic dizziness. Ten patients (27%) had pathological findings on the otoneurological examination. Abnormal findings were documented in sixteen of the twenty-three (70%) completed electronystagmography evaluations. Twenty patients (54%) were referred to treatment by other disciplines than otology/otoneurology. A follow-up questionnaire was filled by twenty six (70%) of the study participants. While all patients diagnosed with MAV had continuous symptoms, most other patients had complete resolution. Conclusions. Various etiologies of vertigo may present with similar symptoms and signs in the pediatric patient. Yet, variable clinical courses should be anticipated, depending on the specific etiology. This is the reason why treatment and follow up should be specifically tailored for each case according to the diagnosis. Close collaboration with other medical disciplines is often required to reach the correct diagnosis and treatment while avoiding unnecessary laboratory examinations

Thoracic paravertebral block for breast surgery in a pregnant woman -A case report-

Non-obstetrical surgery during the first trimester is stressful to both the mother and the fetus. Anesthesiologists are also stressed, not only because of the effects of surgery itself, but also because of the uncertain influences of anesthesia thrown upon on the fetus. The authors present a case of breast surgery successfully performed on a woman 8 weeks pregnant requiring removal of breast abscess by the application of thoracic paravertebral block without any complications. Thoracic paravertebral block may be a safe anesthetic method for non-obstetric surgery during early pregnancy

Discovery of Rare Variants via Sequencing: Implications for the Design of Complex Trait Association Studies

There is strong evidence that rare variants are involved in complex disease etiology. The first step in implicating rare variants in disease etiology is their identification through sequencing in both randomly ascertained samples (e.g., the 1,000 Genomes Project) and samples ascertained according to disease status. We investigated to what extent rare variants will be observed across the genome and in candidate genes in randomly ascertained samples, the magnitude of variant enrichment in diseased individuals, and biases that can occur due to how variants are discovered. Although sequencing cases can enrich for casual variants, when a gene or genes are not involved in disease etiology, limiting variant discovery to cases can lead to association studies with dramatically inflated false positive rates

Age is the only predictor of small decrease in lung function in children with sickle cell anemia

The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV1 % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: 1) FEV1 % predicted declines over time; and 2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ0 thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study. Study-certified pulmonary function technicians performed spirometry and lung volumes. Each assessment was reviewed centrally. Predicted values were determined for TLC, FEV1 , FVC, and FEV1 /FVC ratio. A total of 197 participants, mean age 11.0 years at first testing (range 4-19.3 years), had a minimum of three spirometry measurements an average of 4.4 years (range 1.08-6.5 years) from baseline to endpoint. In a multivariable model, FEV1 % predicted declines by 0.3% for every additional year of age (95% CI -0.56 - -0.05, p=0.020). Sex, asthma history, hemoglobin, reticulocyte count, white blood cell count, incidence rate of severe acute pain and acute chest syndrome episodes, and hydroxyurea therapy were not associated with a decline in FEV1 % predicted. In a large rigorously evaluated, prospective cohort of an unselected group of children with SCA, FEV1 % predicted declines minimally over an average of 4 years, and none of the examined disease features predict the decline

Systemic aminoglycosides are trafficked via endolymph into cochlear hair cells

Aminoglycoside antibiotics rapidly enter and kill cochlear hair cells via apical mechanoelectrical transduction (MET) channels in vitro. In vivo, it remains unknown whether systemically-administered aminoglycosides cross the blood-labyrinth barrier into endolymph and enter hair cells. Here we show, for the first time, that systemic aminoglycosides are trafficked across the blood-endolymph barrier and preferentially enter hair cells across their apical membranes. This trafficking route is predominant compared to uptake via hair cell basolateral membranes during perilymph infusion

Glycosylation defects underlying fetal alcohol spectrum disorder: a novel pathogenetic model: “When the wine goes in, strange things come out” – S.T. Coleridge, The Piccolomini

Fetal alcohol spectrum disorder (FASD) is an umbrella term used to describe the craniofacial dysmorphic features, malformations, and disturbances in growth, neurodevelopment and behavior occurring in individuals prenatally exposed to alcohol. Fetal alcohol syndrome (FAS) represents the severe end of this spectrum. Many pathophysiological mechanisms have hitherto been proposed to account for the disrupted growth and morphogenesis seen in FAS. These include impaired cholesterol-modification of the Sonic hedgehog morphogen, retinoic acid deficiency, lipoperoxidative damage due to alcohol-induced reactive oxygen species combined with reduced antioxidant defences, and malfunctioning cell adhesion molecules. In this report, we propose a completely novel concept regarding the pathogenesis of FAS. Based on our observation that transferrin isoelectric focusing (TIEF) – the most widely used screening tool for congenital disorders of glycosylation (CDG) – was transiently abnormal in a newborn with FAS and a confirmed maternal history of gestational alcohol abuse, we came to believe that FAS exemplifies a congenital disorder of glycosylation secondary to alcohol-inflicted disruption of (N-linked) protein glycosylation. Various pieces of evidence were found in the literature to substantiate this hypothesis. This observation implies, among others, that one might need to consider the possibility of maternal alcohol consumption in newborns with transient glycosylation abnormalities. We also present an integrated pathophysiological model of FAS, which incorporates all existing theories mentioned above as well as our novel concept. This model highlights the pivotal role of disrupted isoprenoid metabolism in the origination of FAS

Early oxygen uptake recovery following exercise testing in children with chronic chest diseases.

addresses: Children's Health and Exercise Research Centre, School of Sport and Health Sciences, University of Exeter, Exeter, Devon, UK.types: Journal Article; Research Support, Non-U.S. Gov'tThis is the author's post-print version of an article published in Pediatric Pulmonology, 2009, Vol. 44, Issue 5, pp. 480 – 488. Copyright © Wiley-Blackwell 2009. The definitive version is available at www3.interscience.wiley.comThe value of exercise testing as an objective measure of disease severity in patients with chronic chest diseases (CCD) is becoming increasingly recognized. The aim of this study was to investigate changes in oxygen uptake (VO2) during early recovery following maximal cardiopulmonary exercise testing (CPXT) in relation to functional capacity and markers of disease severity. Twenty-seven children with CCD (age 12.7 +/- 3.1 years; 17 female) [19 children with Cystic fibrosis (CF) (age 13.4 +/- 3.1 years; 10 female) and 8 with other stable non-CF chest diseases (NON-CF) (age 11.1 +/- 2.2 years; 7 female)] and 27 healthy controls (age 13.2 +/- 3.3 years; 17 female) underwent CPXT on a cycle ergometer. On-line respiratory gas analysis measured VO2 before and during CPXT to peak VO2) (VO2(peak)), and during the first 10 min of recovery. Early VO2 recovery was quantified by the time (sec) to reach 50% of the VO2 (peak) value. Early VO2 recovery was correlated against spirometry [forced expiratory volume in 1 sec (FEV(1)) and forced expiratory flow between 25% and 75% of the forced vital capacity (FEF(25-75))] and aerobic fitness (VO2)(peak)) as a measure of functional capacity. Disease severity was graded in the CF patients by the Shwachman score (SS). Compared to controls, children with CCD demonstrated a significantly reduced VO2(peak) (P = 0.011), FEV(1) (P < 0.001), FEF(25-75) (P < 0.001), and a significantly prolonged early (VO2) recovery (P = 0.024). In the CF patients the SS was significantly correlated with early VO2 recovery (r = -0.63, P = 0.004), FEV(1) (r = 0.72, P = 0.001), and FEF(25-75) (r = 0.57, P = 0.011). In the children with CCD, FEV(1), FEF(25-75), and BMI were not significantly correlated with VO2(peak) or early VO2 recovery. Lung function does not necessarily reflect aerobic fitness and the ability to recover from exercise in these patients. A significant relationship was found between VO2(peak) and early VO2 recovery (r = -0.39, P = 0.044) in the children with CCD, showing that a greater aerobic fitness corresponded with a faster recovery

Alcohol Abuse in Pregnant Women: Effects on the Fetus and Newborn, Mode of Action and Maternal Treatment

Offspring of mothers using ethanol during pregnancy are known to suffer from developmental delays and/or a variety of behavioral changes. Ethanol, may affect the developing fetus in a dose dependent manner. With very high repetitive doses there is a 6–10% chance of the fetus developing the fetal alcoholic syndrome manifested by prenatal and postnatal growth deficiency, specific craniofacial dysmorphic features, mental retardation, behavioral changes and a variety of major anomalies. With lower repetitive doses there is a risk of “alcoholic effects” mainly manifested by slight intellectual impairment, growth disturbances and behavioral changes. Binge drinking may impose some danger of slight intellectual deficiency. It is advised to offer maternal abstinence programs prior to pregnancy, but they may also be initiated during pregnancy with accompanying close medical care. The long term intellectual outcome of children born to ethanol dependent mothers is influenced to a large extent by the environment in which the exposed child is raised