Gender-matched Normal Children Research Articles

Evaluation of left ventricular systolic strain in children with Kawasaki disease.

The current study aimed to assess left ventricular (LV) longitudinal systolic strains in children with KD using two-dimensional speckle-tracking imaging and to analyze the relationship of LV myocardial deformation to coronary lesions and laboratory markers. The study enrolled 101 children with acute KD. An additional 50 age- and gender-matched normal children were enrolled as control subjects. During different phases of KD, echocardiograms were recorded for 61 children. Two-dimensional strain analysis software was used to track myocardial movement and obtain the strain from each LV segment. The LV longitudinal systolic strain decreased significantly in children with acute KD but increased immediately after intravenous immunoglobulin therapy. At 6-8 weeks after the onset of KD, all LV strains had recovered to normal. The LV systolic strain was not associated with coronary dilation in either acute or convalescent KD. In acute KD, aspartate transaminase, alanine transaminase, erythrocyte sedimentation rate, C-reactive protein (CRP), and hemoglobin (Hb) were found to be associated with coronary dilation, whereas LV systolic strains were found to be correlated with elevated CRP and decreased Hb. Speckle-tracking imaging of LV systolic strain was simple and accurate in evaluating LV function during different phases of KD. No association between LV dysfunction and coronary dilation was observed, but a relationship with CRP and Hb was found. Further studies are recommended to validate the current study results and explore further how these findings can improve clinical practice.

Evaluation of retinal nerve fiber layer thickness in vernal keratoconjunctivitis patients under long-term topical corticosteroid therapy

Purpose: The aim of this study was to evaluate the retinal nerve fiber layer (RNFL) thickness in vernal keratoconjunctivitis (VKC) patients who were under long-term topical corticosteroid therapy.Methods: Thirty-six eyes of 36 VKC patients with clear cornea and normal videokeratography and 40 eyes of 40 age- and gender-matched normal children were included in the study. Clinical and demographic characteristics of the patients were noted and detailed ophthalmological examination was performed. Visual acuity (VA), spherical equivalent (SE), axial length (AL) and RNFL thickness measurements were compared between the groups. To correct ocular magnification effect on RNFL, we used Littmann’s formula.Results: All VKC patients had history of topical corticosteroid use and the mean duration of the topical corticosteroid use was 23.8 ± 9.09 months. There was no significant difference between the groups in terms of intraocular pressure (IOP). VKC group had significantly worse VA, greater SE and AL and thinner mean global, superior and inferior RNFL thickness. There were significant negative correlations between the duration of topical corticosteroid use and the mean global, superior and temporal RNFL thickness in VKC group. After correction of magnification effect, VKC group still had thinner mean global, superior and inferior RNFL thickness, and significant difference between the groups in inferior RNFL thickness did not disappear.Conclusion: Significant RNFL thickness difference between the groups suggests a possible effect of long-term corticosteroid use in VKC patients. Because visual field (VF) analysis in pediatric patients is difficult to perform and IOP may be illusive, RNFL thickness measurements in addition to routine examinations in VKC patients may help clinicians in their practice.

Urinary N-acetyl-beta-D glucosaminidase (NAG) level in idiopathic nephrotic syndrome

Urinary N-acetyl-beta-D: glucosaminidase (NAG) is a sensitive biomarker of renal parenchymal disease. The aim of this study was to investigate variations in the levels of NAG excretion among different sub-groups of nephrotic syndrome (first episode, relapsers, and resistant) and its prediction based on proteinuria. Thirty-five patients with idiopathic nephrotic syndrome, aged 1-12 years, as well as 15 age- and gender-matched normal children (controls) were enrolled in the study. Among the 35 patients, ten were classified with first episode nephrotic syndrome (FENS), 17 with relapsing nephrotic syndrome (RNS), and eight with steroid-resistant nephrotic syndrome (SRNS). Urinary NAG/creatinine levels were significantly increased in SRNS patients as compared to FENS and RNS patients (p < 0.001); the FENS and RNS groups had comparable levels. A urinary NAG/creatinine value of ≤108.9 U/g was found to identify steroid-sensitive patients with a sensitivity, specificity, positive predictive value, and negative predictive value of 78.8, 100, 100 and 77.7%, respectively. Significant correlations were found between experimental and predicted values of urinary NAG/creatinine in steroid sensitive nephrotic syndrome (SSNS) (R (2) = 0.9643) and SRNS patients (R (2) = 0.9823). Urinary NAG/creatinine values were found to be higher in SRNS than SSNS patients and have moderate predictive value for steroid responsiveness. This level can be obtained based on urinary protein/creatinine ratio or 24 h urinary protein levels.

Language in pediatric epilepsy

This study examined the severity and range of linguistic impairments in young, intermediate, and adolescent youth with epilepsy and how these deficits were associated with illness effects, nonverbal intelligence, psychopathology, and reading. Tests of language, intelligence, achievement, and structured psychiatric interviews were administered to 182 epilepsy youth, aged 6.3-8.1, 9.1-11.7, and 13.0-15.2 years, as well as to 102 age- and gender-matched normal children. Parents provided demographic, seizure-related, and behavioral information on their children. Significantly more epilepsy subjects had language scores 1 standard deviation (SD) below average than the age-matched control groups did. The intermediate and adolescent epilepsy groups also had significantly lower mean language scores compared to their matched controls. The older compared to the younger epilepsy groups had more language impairment and a wider range of linguistic deficits. Longer duration of illness, childhood absence epilepsy, psychiatric diagnosis, and socioeconomic status were associated with linguistic deficits in the young group. Prolonged seizures, lower Performance IQ, and minority status predicted low language scores in the intermediate epilepsy group. In the adolescent group, language impairment was associated with poor seizure control, decreased Performance IQ, and lower socioeconomic status. Linguistic and reading deficits were significantly related in each epilepsy group. The age-related increase in linguistic impairment, different profiles of predictors in each age group, and the relationship of linguistic deficits with poor reading skills have important clinical, developmental, theoretical, and academic implications.

Absence Semiologies

Factors Influencing Clinical Features of Absence Seizures. Sadleir LG, Scheffer IE, Smith S, Carstensen B, Carlin J, Connolly MB, Farrell K. Epilepsia 2008;49(12):2100–2107. PURPOSE: The clinical features of absence seizures in idiopathic generalized epilepsy have been held to be syndrome-specific. This hypothesis is central to many aspects of epilepsy research yet has not been critically assessed. We examined whether specific factors such as epilepsy syndrome, age, and state determine the features of absence seizures. METHODS: Children with newly presenting absence seizures were studied using video electroencephalography (EEG) recording. We analyzed whether a child's epilepsy syndrome, age, state of arousal, and provocation influenced specific clinical features of their absence seizures: duration, eyelid movements, eye opening, and level of awareness during the seizure. RESULTS: Seizures (509) were evaluated in 70 children with the following syndromes: Childhood absence epilepsy (CAE), 37; CAE plus photoparoxysmal response (PPR), 10; juvenile absence epilepsy (JAE), 8; juvenile myoclonic epilepsy (JME), 6; unclassified, 9. Seizure duration was associated with epilepsy syndrome as children with JME had shorter seizures than in other syndromes, independent of age. Age independently influences level of awareness and eye opening. Arousal or provocation affected all features except level of awareness. Specific factors unique to the child independently influenced all features; the nature of these factors has not been identified. DISCUSSION: The view that the clinical features of absence seizures have syndrome-specific patterns is not supported by critical analysis. We show that confounding variables profoundly affect clinical features and that syndromes also show marked variation. Variation in clinical features of absence seizures results from a complex interaction of many factors that are likely to be genetically and environmentally determined. Childhood Absence Epilepsy: Behavioral, Cognitive, and Linguistic Comorbidities. Caplan R, Siddarth P, Stahl L, Lanphier E, Vona P, Gurbani S, Koh S, Sankar R, Shields WD. Epilepsia 2008 Nov;49(11):1838–1846. PURPOSE: Evidence for a poor psychiatric, social, and vocational adult outcome in childhood absence epilepsy (CAE) suggests long-term unmet mental health, social, and vocational needs. This cross-sectional study examined behavioral/emotional, cognitive, and linguistic comorbidities as well as their correlates in children with CAE. METHODS: Sixty-nine CAE children aged 9.6 (SD = 2.49) years and 103 age- and gender-matched normal children had semistructured psychiatric interviews, as well as cognitive and linguistic testing. Parents provided demographic, seizure-related, and behavioral information on their children through a semi-structured psychiatric interview and the child behavior checklist (CBCL). RESULTS: Compared to the normal group, 25% of the CAE children had subtle cognitive deficits, 43% linguistic difficulties, 61% a psychiatric diagnosis, particularly attention deficit hyperactivity disorder (ADHD) and anxiety disorders, and 30% clinically relevant CBCL broad band scores. The most frequent CBCL narrow band factor scores in the clinical/borderline range were attention and somatic complaints, followed by social and thought problems. Duration of illness, seizure frequency, and antiepileptic drug (AED) treatment were related to the severity of the cognitive, linguistic, and psychiatric comorbidities. Only 23% of the CAE subjects had intervention for these problems. CONCLUSIONS: The high rate of impaired behavior, emotions, cognition, and language and low intervention rate should alert clinicians to the need for early identification and treatment of children with CAE, particularly those with longer duration of illness, uncontrolled seizures, and AED treatment.

Childhood absence epilepsy: Behavioral, cognitive, and linguistic comorbidities

Evidence for a poor psychiatric, social, and vocational adult outcome in childhood absence epilepsy (CAE) suggests long-term unmet mental health, social, and vocational needs. This cross-sectional study examined behavioral/emotional, cognitive, and linguistic comorbidities as well as their correlates in children with CAE. Sixty-nine CAE children aged 9.6 (SD = 2.49) years and 103 age- and gender-matched normal children had semistructured psychiatric interviews, as well as cognitive and linguistic testing. Parents provided demographic, seizure-related, and behavioral information on their children through a semi-structured psychiatric interview and the child behavior checklist (CBCL). Compared to the normal group, 25% of the CAE children had subtle cognitive deficits, 43% linguistic difficulties, 61% a psychiatric diagnosis, particularly attention deficit hyperactivity disorder (ADHD) and anxiety disorders, and 30% clinically relevant CBCL broad band scores. The most frequent CBCL narrow band factor scores in the clinical/borderline range were attention and somatic complaints, followed by social and thought problems. Duration of illness, seizure frequency, and antiepileptic drug (AED) treatment were related to the severity of the cognitive, linguistic, and psychiatric comorbidities. Only 23% of the CAE subjects had intervention for these problems. The high rate of impaired behavior, emotions, cognition, and language and low intervention rate should alert clinicians to the need for early identification and treatment of children with CAE, particularly those with longer duration of illness, uncontrolled seizures, and AED treatment.

Amygdala volume and psychopathology in childhood complex partial seizures

Objective The purpose of this study was to compare amygdala volume in children with cryptogenic epilepsy who have complex partial seizures (CPS) with that of age- and gender-matched normal children. The relationship of amygdala volume to seizure variables and presence of psychopathology was also examined in these patients. Methods Twenty-eight children with cryptogenic epilepsy, all of whom had CPS, and gender-matched normal children, all aged 6–16 years, underwent magnetic resonance imaging (MRI) at 1.5 T. Tissue was segmented, and total brain volume and amygdala volumes obtained from manual tracings were computed. Results There were no significant differences in amygdala volume between the CPS and normal groups. Within the CPS group, the children with an affective/anxiety disorder had significantly larger left amygdala volumes, as well as greater amygdala asymmetry, compared with those with no psychopathology. Exploring the association between seizure variables and amygdala volume yielded no significant predictors. Conclusions In pediatric CPS, left amygdala involvement may reflect effects of the neuropathology underlying comorbid affective or anxiety disorders on amygdala development rather than effects of ongoing seizures.

Non-REM-sleep instability in recurrent sleepwalking in pre-pubertal children

Background and purpose We questioned whether or not the sleep of pre-pubertal children with recurrent sleepwalking was different from that recorded in normal children. Patients and methods Twelve pre-pubertal chronic sleepwalkers were compared to age- and gender-matched normal children. All children had a clinical evaluation covering pediatric, sleep, neuropsychiatric and otolaryngological fields. Two standardized sleep questionnaires were administered, and a minimum of two successive polysomnograms were performed with monitoring of sleep electroencephalographic (EEG) and cardiorespiratory variables. The research investigations were performed on nights without sleepwalking to search for the presence of other sleep disorders, including upper airway resistance syndrome (UARS). Sleep was scored using standard atlases, but it was also evaluated for the cyclic alternating pattern (CAP) rate. Results All sleepwalkers presented with either obstructive sleep apnea ( n=2) or UARS ( n=10). Compared to normal children, sleepwalkers had shorter total sleep time but no significant change in wake after sleep onset when considering all arousals >3 s. CAP analysis showed a significantly higher CAP rate than in controls. Conclusions Chronic sleepwalkers have instability of non-rapid eye movement (NREM) sleep detectable only by the calculation of CAP rate. Instability of NREM sleep was seen even on nights without sleepwalking and is probably related to the presence of the associated sleep disorders. We hypothesize that chronic NREM-sleep instability is a risk factor for occurrence of sleepwalking when further sleep disruption is triggered by external events.

Haptic form discrimination. Group comparison of children with normal speech development and former speech development disordered patients

The importance of the neurobiological basis of developmental language disorders includes somatosensory modalities. Twenty-five children were diagnosed as having specific language-impairment at preschool age. All were examined with regard to their manual haptic form discrimination without visual control at a mean age of 8.7 years +/- 7.1 months. This study group was compared to age- and gender-matched normal children of equal non-verbal intelligence (control group). Haptic discrimination was measured with the Seguin formboard on which the children were required to place ten geometrical forms in appropriate holes. Both groups differed significantly in their mean quantitative performances in favor of the control group (P < 0.05). The difference in their mean performance and their mean discrimination times did not reach statistical significance. All results were not age-dependent. The control group on average performed significantly better with their left hands than the study group (P < 0.05). Qualitative analysis revealed a significant difference in haptic discrimination of the pointed forms and was probably caused by inadequate exploration procedures and/or cognitive representation deficits. The results of the children with previous developmental language disorders were interpreted as an expression of an impaired cerebral maturation.

Reduced P3 amplitude of the event-related brain potential: its relationship to language ability in autism.

Several studies have found that P3 amplitude of the auditory event-related potential is smaller in autistic than in normal children. The present study investigated whether this characteristic bears any relationship to the degree of language impairment and/or level of intellectual ability of autistic persons. Seventeen autistic children, ranging from 8-19 years of age, and 17 age- and gender-matched normal children participated. Event-related potentials to phonetic ("Da") and chord (piano) stimuli were recorded from three scalp locations: vertex (Cz), right hemisphere (RH), and left hemisphere (LH), during a discrimination task. A battery of language tests was given to autistic children. Compared to normal subjects, autistic subjects showed a significantly smaller P3 amplitude to phonetic stimuli for Cz and LH recording sites. However, no group difference in P3 amplitude to the phonetic stimulus was found for the RH. Furthermore, no group differences in P3 amplitude were found for the chord stimulus at any recording site. Impaired language ability was related to greater RH P3 amplitude, particularly to the chord stimulus. The possibility of differential hemispheric involvement in the attentional deficits of autistic children is raised.