Relationship Between Occlusion and Lisping in Children With Cleft Lip and Palate

Introduction: The literature suggests that individuals with history of cleft lip and palate who present with midfacial growth deficiency are at higher risk of presenting lisping. The relationship between distortions during production of linguoalveolar fricative sounds and the severity of malocclusion, however, has not been established for the population with cleft. Objective: To study the association between lisping and dental arch relationship. Methodology: Speech samples and dental arch casts were obtained from 106 children with operated unilateral cleft lip and palate (UCLP) during the stage of mixed dentition and before orthodontic treatment. Videotaped productions of the phrase/u saci saiw sedu/were rated by speech-language pathologists for the identification of lisping during [s]. Dental arch casts were rated by orthodontists using the Goslon Yardstick and the Five-Year Index to establish dental arch relationship. Results: Multiple logistic regression showed no significant association between lisping and dento-occlusal index (p = .802) and age (p = .662). Substantial interjudge agreement during auditory-perceptual ratings was found (kappa = .63). Almost perfect agreement was found between orthodontists while establishing the dental arch relationship (kappa = .81). Discussion: This study failed to reveal an association between lisping and dental arch relationship in children with operated UCLP. Multiple variables may play a role in determining occurrence of lisping, warranting further investigation.

Craniofacial morphology and sleep apnea in children with obstructed upper airways: Differences between genders

Objective: To correlate sleep apnea with craniofacial characteristics and facial patterns according to gender. Methods: In this prospective survey we studied 77 male and female children (3-12 years old) with an upper airway obstruction due to tonsil and adenoid enlargement. Children with lung problems, neurological disorders and syndromes, obstructive septal deviation, previous orthodontic treatment, orthodontic surgeries or oral surgeries, or obesity were excluded. Patients were subjected to physical examinations, nasal fiberoptic endoscopy, teleradiography for cephalometric analysis, and polysomnography. Methods: Cephalometric analysis included the following skeletal craniofacial measurements: facial axis (FA), facial depth (FD), mandibular plane angle (MP), lower facial height (LFH), mandibular arch (MA), and vertical growth coefficient (VERT) index. Results: The prevalence of sleep apnea was 46.75% with no statistical difference between genders. Among children with obstructive sleep apnea (Apneia Hypopnea Index - AHI >= 1) boys had higher AHI values than girls. A predominance of the dolichofacial pattern (81.9%) was observed. The following skeletal craniofacial measurements correlated with AHI in boys: FD (r(s) = -0.336/p = 0.020), MP (r(s) = 0.486/p = 0.00), and VERT index (r(s) = -0.337/p = 0.019). No correlations between craniofacial measurements and AHI were identified in girls. Conclusions: Craniofacial morphology may influence the severity of sleep apnea in boys but not in girls. (C) 2012 Elsevier B.V. All rights reserved.

Ambulatory blood pressure monitoring in children with obstructive sleep apnea and primary snoring

Objective: To evaluate the systemic blood pressure (BP) during daytime and nighttime in children with sleep breathing disorders (SBD) and compare parameters of BP in children with diagnosis of obstructive sleep apnea syndrome (OSA) to those one with primary snoring (PS). Methods: Children, both genders, aged from 8 to 12 years, with symptoms of SBD realized an overnight polysomnography followed by a 24 h recording of ambulatory BP. Results: All subjects presented with a history of snoring 7 nights per week. Children who have apnea/hipoapnea index >= four or a apnea index >= one presented a mean BP of 93 +/- 7 mmHg and 85 +/- 9 mmHg diurnal and nocturnal respectively whereas children who have a apnea/hipoapnea < four or a apnea index < one presented 90 +/- 7 mmHg and 77 +/- 2 mmHg. Eight children out of fourteen, from OSA group, lost the physiologic nocturnal dipping of the blood pressure. Among OSA children 57% were considered non-dippers. Two (16%) have presented absence of nocturnal dipping among children with primary snoring. The possibility of OSA children loosing physiologic blood pressure dipping was 6.66 higher than the possibilities of patients from PS group. Discussion: Our results indicate that children with sleep apnea syndrome exhibit a higher 24 h blood pressure when compared with those of primary snoring in form of decreased degree of nocturnal dipping and increased levels of diastolic and mean blood pressure, according to previous studies in literature. OSA in children seems to be associated to the development of hypertension or other cardiovascular disease. (C) 2012 Elsevier Ireland Ltd. All rights reserved.

Language impairment in Huntington's disease

Language alterations in Huntington's disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (p<0.0001), oral comprehension (p<0.0001), repetition (p<0.0001), oral agility (p<0.0001), reading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

Measures of quality of life in children with cochlear implant: systematic review

The use of cochlear implant (CI) in children enables the development of listening and communication skills, allowing the child's progress in school and to be able to obtain, maintain and carry out an occupation. However, the progress after the CI has different results in some children, because many children are able to interact and participate in society, while others develop limited ability to communicate verbally. The need for a better understanding of CI outcomes, besides hearing and language benefits, has spurred the inclusion of quality of life measurements (QOL) to assess the impact of this technology. OBJECTIVE: Identify the key aspects of quality of life assessed in children with cochlear implant. METHOD: Through a systematic literature review, we considered publications from the period of 2000 to 2011. CONCLUSION: We concluded that QOL measurements in children include several concepts and methodologies. When referring to children using CI, results showed the challenges in broadly conceptualizing which quality of life domains are important to the child and how these areas can evolve during development, considering the wide variety of instruments and aspects evaluated.

Questionnaire for monitoring auditory and language development in the first year of life

Purpose: To validate a monitoring questionnaire about hearing and language development applied by community health agents in the first year of life. Methods: Seventy six community health agents, previously trained on infant hearing health, administered a questionnaire to the families of 304 children with ages from 0 to 1 year. The questionnaire contains questions regarding hearing and language development and, for all age groups, the question “Does your child hear well?” was presented. The validity of the questionnaire was assessed by analyzing false positive and false negative rates of the identified children. A double-blind study was conducted so that all children assessed by the questionnaire were submitted to hearing evaluation performed by audiologists. Results: Four children (1.32%) were diagnosed with sensorineural hearing loss (two unilateral), and 69 (22.7%) with conductive hearing loss. The monitoring questionnaire showed specificity of 96% and sensitivity of 67%, with a false-negative rate of 33% for not identifying the unilateral hearing loss, and a false-positive rate of 4%. Conclusion: The questionnaire used has shown to be feasible and relevant to actions of the community health agents of the Family Health Strategy program, with high specificity and moderate sensitivity. The use of the validated instrument should be considered to complement Newborn Hearing Screening Programs, in order to identify late onset or acquired hearing loss.

Auditory complaints and audiologic assessment in children with surgically repaired cleft lip and palate

Introduction: At the initial consultation, the speech-language pathologist and audiologist may consider possible diagnostic hypotheses based on the child's history and the parents' complaint. Aim: To investigate the association of hearing complaints with the findings obtained in the conventional audiologic assessment in children with cleft lip and palate. Retrospective study. Methods: We analyzed medical charts of 1000 patients with cleft lip and palate who underwent surgical repair between 1988 and 1995 at a mean age of 6 years 8 months. We excluded charts with records of inconsistent audiological responses and charts with missing data for any of the audiologic evaluations considered. Thus, the sample consisted of 393 records. Results: Two hundred thirty-nine patients presented hearing loss in one or both ears, but only 3.8% reported hearing complaints. The most frequent were otorrhea followed by otalgia. There was no statistical significance between the complaint and gender (p = 0.26) nor between the complaint and hearing loss (p = 0.83). Conclusion: This study showed no association between the hearing complaint and the conventional audiologic assessment

Behavioral problems and emotional stress in children with bruxism

Bruxism has a multifactorial etiology, and psychosocial factors have been considered to increase the risk of occurrence of this parafunction. The aim of this study was to evaluate the behavior profile of a group of children diagnosed with bruxism. Eighty 7-11-year-old children of both genders (mean age 8.8 years) first recruited as eligible participants. Twenty-nine children (18 males and 11 females) whose parents/guardians reported to present frequent episodes of tooth grinding/clenching while awake or during sleep (at least 3 nights a week) in the previous 3 months were enrolled in the study. The diagnosis of bruxism was established based on the parents/guardians' report about the children's behavior, habits and possible discomforts in the components of the stomatognathic system allied to the presence of signs and symptoms such as pain on the masticatory muscles, masseter muscle hypertrophy, wear facets, fractures of restorations, dental impressions on the cheek mucosa and tongue. As part of the psychological evaluation, the Rutter's Child Behavior Scale-A2 was applied to the parents/caregivers (one for each child) and the Child Stress Scale was applied to the children. Data were analyzed descriptively based on the frequency of each studied variable. Twenty-four (82.76%) children needed psychological or psychiatric intervention; 17 of them presented neurotic disorders and 7 children presented antisocial disorders. Six (20.70%) children presented significant physical and psychological manifestations of stress. The findings of the present study suggest that behavioral problems and potential emotional problems can be risk factors to bruxism in children.

Auditory processing in children and adolescents in situations of risk and vulnerability

CONTEXT AND OBJECTIVE: Children and adolescents who live in situations of social vulnerability present a series of health problems. Nonetheless, affirmations that sensory and cognitive abnormalities are present are a matter of controversy. The aim of this study was to investigate aspects to auditory processing, through applying the brainstem auditory evoked potential (BAEP) and behavioral auditory processing tests to children living on the streets, and comparison with a control group. DESIGN AND SETTING: Cross-sectional study in the Laboratory of Auditory Processing, School of Medicine, Universidade de São Paulo. METHODS: The auditory processing tests were applied to a group of 27 individuals, subdivided into 11 children (7 to 10 years old) and 16 adolescents (11 to 16 years old), of both sexes, in situations of social vulnerability, compared with an age-matched control group of 10 children and 11 adolescents without complaints. The BAEP test was also applied to investigate the integrity of the auditory pathway. RESULTS: For both children and adolescents, there were significant differences between the study and control groups in most of the tests applied, with significantly worse performance in the study group, except in the pediatric speech intelligibility test. Only one child had an abnormal result in the BAEP test. CONCLUSIONS: The results showed that the study group (children and adolescents) presented poor performance in the behavioral auditory processing tests, despite their unaltered auditory brainstem pathways, as shown by their normal results in the BAEP test.

Adrenal insufficiency after glucocorticoid withdrawal in children with rheumatic diseases

?Glucocorticoids (GCs) are often used for the treatment of rheumatic disorders. However, doses are prescribed, which may suppress the hypothalamic-pituitary-adrenal (HPA) axis. After GC withdrawal, recovery of the HPA axis may be delayed putting the patient at risk for adrenal insufficiency. We assessed adrenal function and factors influencing adrenal responsiveness after termination of GC therapy in paediatric patients with rheumatic diseases.

Growth hormone receptor polymorphism and growth hormone therapy response in children: a bayesian meta-analysis

Recombinant human growth hormone (rhGH) therapy is used in the long-term treatment of children with growth disorders, but there is considerable treatment response variability. The exon 3-deleted growth hormone receptor polymorphism (GHR(d3)) may account for some of this variability. The authors performed a systematic review (to April 2011), including investigator-only data, to quantify the effects of the GHR(fl-d3) and GHR(d3-d3) genotypes on rhGH therapy response and used a recently established Bayesian inheritance model-free approach to meta-analyze the data. The primary outcome was the 1-year change-in-height standard-deviation score for the 2 genotypes. Eighteen data sets from 12 studies (1,527 children) were included. After several prior assumptions were tested, the most appropriate inheritance model was codominant (posterior probability = 0.93). Compared with noncarriers, carriers had median differences in 1-year change-in-height standard-deviation score of 0.09 (95% credible interval (CrI): 0.01, 0.17) for GHR(fl-d3) and of 0.14 (95% CrI: 0.02, 0.26) for GHR(d3-d3). However, the between-study standard deviation of 0.18 (95% CrI: 0.10, 0.33) was considerable. The authors tested by meta-regression for potential modifiers and found no substantial influence. They conclude that 1) the GHR(d3) polymorphism inheritance is codominant, contrasting with previous reports; 2) GHR(d3) genotypes account for modest increases in rhGH effects in children; and 3) considerable unexplained variability in responsiveness remains.

The significance of at-risk symptoms for psychosis in children and adolescents

The early detection and treatment of people at risk for psychosis is currently regarded as a promising strategy in fighting the devastating consequences of psychotic disorders. Currently, the 2 most broadly used sets of at-risk criteria, that is, ultra-high risk (UHR) and basic symptom criteria, were developed mainly in adult samples. We review the data regarding the presence and relevance of at-risk symptoms for psychosis in children and adolescents. The few existing studies suggest that attenuated psychotic symptoms (APS) and brief limited intermittent psychotic symptoms (BLIPS) do have some clinical relevance in young adolescents from the general population. Nevertheless, their differentiation from atypical psychotic symptoms or an emerging schizotypal personality disorder, as well as their stability and predictive accuracy for psychosis, are still unclear. Further, standard interviews for UHR criteria do not define a minimum age for the assessment of APS and BLIPS or guidelines as to when and how to include information from parents. APS and basic symptoms may be predictive of conversion to psychosis in help-seeking young adolescents. Nevertheless, the rate and timing, and thus the required observation time, need further study. Moreover, no study has yet addressed the issue of how to treat children and adolescents presenting with at-risk symptoms and criteria. Further research is urgently needed to examine if current at-risk criteria and approaches have to be tailored to the special needs of children and adolescents. A preliminary rationale for how to deal with at-risk symptoms for psychosis in clinical practice is provided.

Challenges in the early detection of psychosis in children and adolescents

The early detection and treatment of persons at-risk for psychosis is currently regarded a promising strategy in fighting the devastating consequences of psychotic disorders. The two current at-risk approaches, i.e., the "ultra high risk" and the "basic symptom" criteria, were mainly developed on adult samples. Initial evidence suggests, however, that they cannot simply be applied to children and adolescents. For ultra high risk criteria, there is indication of some attenuated psychotic symptoms being potentially non-specific in adolescents and of brief limited intermittent symptoms being difficult to clinically classify in children when observable behavioral correlates are missing. For basic symptoms, too, only preliminary indication of their usefulness in children and adolescents exists. Since developmental peculiarities in the assessment of basic symptoms should be considered, a child and youth version of the Schizophrenia Proneness Instrument (SPI-CY) was developed. In conclusion, research on the clinical-prognostic validity of the at-risk criteria and their potential adoption to the special needs of children and adolescents is needed. If a Prodromal Risk Syndrome for Psychosis or Attenuated Psychotic Symptoms Syndrome will be included into DSM-V, it has to be highlighted that its suitability for children and adolescents is only insufficiently known.

Bipolar disorder risk alleles in children with ADHD

Bipolar disorder (BD) and attention deficit/hyperactivity disorder (ADHD) may share common genetic risk factors as indicated by the high co-morbidity of BD and ADHD, their phenotypic overlap especially in pediatric populations, the high heritability of both disorders, and the co-occurrence in families. We therefore examined whether known polygenic BD risk alleles are associated with ADHD. We chose the eight best SNPs of the recent genome-wide association study (GWAS) of BD patients of German ancestry and the nine SNPs from international GWAS meeting a 'genome-wide significance' level of α = 5 × 10(-8). A GWAS was performed in 495 ADHD children and 1,300 population-based controls using HumanHap550v3 and Human660 W-Quadv1 BeadArrays. We found no significant association of childhood ADHD with single BD risk alleles surviving adjustment for multiple testing. Yet, risk alleles for BD and ADHD were directionally consistent at eight of nine loci with the strongest support for three SNPs in or near NCAN, BRE, and LMAN2L. The polygene analysis for the BP risk alleles at all 14 loci indicated a higher probability of being a BD risk allele carrier in the ADHD cases as compared to the controls. At a moderate power to detect association with ADHD, if true effects were close to estimates from GWAS for BD, our results suggest that the possible contribution of BD risk variants to childhood ADHD risk is considerably lower than for BD. Yet, our findings should encourage researchers to search for common genetic risk factors in BD and childhood ADHD in future studies.

Duplication of the sodium channel gene cluster on 2q24 in children with early onset epilepsy

Sodium channel gene aberrations are associated with a wide range of seizure disorders, particularly Dravet syndrome. They usually consist of missense or truncating gene mutations or deletions. Duplications involving multiple genes encoding for different sodium channels are not widely known. This article summarizes the clinical, radiologic, and genetic features of patients with 2q24 duplication involving the sodium channel gene cluster.