Lung function measurements in young children with spinal muscle atrophy; a cross sectional survey on the effect of position and bracing.

BACKGROUND: Spinal muscular atrophy (SMA) affects respiratory muscles, which in addition to progressive scoliosis leads to respiratory impairment. Children with developing scoliosis are usually treated with spinal bracing to delay the progress. AIMS: To assess the impact of body position and application of spinal bracing on lung function during tidal breathing in children with SMA. METHODS: Lung function was determined by tidal flow volume loops and passive respiratory mechanics (single breath occlusion technique) in all eight children in southern Norway with SMA type I and II, in both the sitting and supine position. Additional measurements were performed with and without bracing in five children. Muscle strength was assessed by the Brooks scale. RESULTS: Tidal expiratory volume (V(E)) and compliance of the respiratory system (CRS) tended to be higher in the sitting compared to the supine position, but this was not statistically significant. However, applying bracing in the sitting position significantly reduced V(E). The highest values of CRS and V(E) were found in the sitting position without bracing. CONCLUSION: Impairment of tidal respiration must be considered when applying spinal bracing in very young children developing scoliosis with SMA.     

Lung function measurements in young children with spinal muscle atrophy; a cross sectional survey on the effect of position and bracing

BACKGROUND—Spinal muscular atrophy (SMA) affects respiratory muscles, which in addition to progressive scoliosis leads to respiratory impairment. Children with developing scoliosis are usually treated with spinal bracing to delay the progress.AIMS—To assess the impact of body position and application of spinal bracing on lung function during tidal breathing in children with SMA.METHODS—Lung function was determined by tidal flow volume loops and passive respiratory mechanics (single breath occlusion technique) in all eight children in southern Norway with SMA type I and II, in both the sitting and supine position. Additional measurements were performed with and without bracing in five children. Muscle strength was assessed by the Brooks scale.RESULTS—Tidal expiratory volume (V_E) and compliance of the respiratory system (CRS) tended to be higher in the sitting compared to the supine position, but this was not statistically significant. However, applying bracing in the sitting position significantly reduced V_E. The highest values of CRS and V_E were found in the sitting position without bracing.CONCLUSION—Impairment of tidal respiration must be considered when applying spinal bracing in very young children developing scoliosis with SMA.     

Communication between Parents and Deaf Children: Implications for Social-emotional Development

Parent-child communication plays a central role in social growth, as it does in other domains of development. Over 90 % of deaf children, however, have hearing parents who frequently do not have a fully effective means of communicating with them. This paper examines the role of effective parent-child communication in the social and emotional development of deaf children. Evidence concerning relations between early communication and social-emotional development of deaf children is reviewed, and superficial differences in the ways that parents interact with deaf versus hearing children are distinguished from differences that may have more significant and enduring effects. Hearing parents and their deaf children are found to develop alternative, often nonverbal, interaction strategies. Of primary interest is the extent to which those strategies have impact comparable to the strategies of hearing parents with hearing children or deaf parents with deaf children.     

The effect of age on physical fitness of deaf elementary school children

The aim of this study was to measure physical fitness of deaf Dutch elementary school children compared with hearing children and to investigate the influence of age on physical fitness. Deaf children were physically less fit than hearing children. Overall, physical fitness increased with age in deaf children, but no significant differences were found between the age groups of 9-10 years and 11-12 years on most of the Eurofit items. The difference in performance between deaf and hearing children, favoring the latter, increased with age in handgrip strength and the 20-m endurance shuttle run. More attention should be paid to developing and maintaining an adequate level of physical fitness in deaf children.     

Phonological awareness of syllables, rhymes, and phonemes in deaf children

Phonological awareness is important for reading development in hearing children, in whom it develops at the three consecutive levels of the syllable, rhyme, and phoneme. Deaf children typically have literacy difficulties, and previous research has been equivocal about whether deaf children can develop phonological awareness. Three experiments are presented that investigate the phonological skills of deaf children (mean age 11 years) at the three linguistic levels of syllable, rhyme, and phoneme. The first experiment showed that deaf children's syllable awareness can be equivalent to that of chronological age-matched hearing controls. In the second experiment, deaf children's ability to make rhyme judgements was above chance, but poorer than that of younger reading-matched hearing controls. The third experiment showed that deaf children could phonologically recode nonsense words at a level above chance, suggesting that they could draw on phonemic skills in certain conditions. We conclude that deaf children can develop phonological awareness, but that their phonological skills lag those of hearing children and may develop in different ways. Differences between our tasks and those used in other studies are discussed.     

Deaf children's understanding of emotions: desires take precedence

Deaf children frequently have trouble understanding other people's emotions. It has been suggested that an impaired theory of mind can account for this. This research focused on the spontaneous use of mental states in explaining other people's emotions by 6- and 10-year-old deaf children as compared to their hearing peers. Within both age-groups deaf children referred to others' beliefs as often as their hearing peers and their references to desires even exceeded those of hearing children. This relative priority for the expression of desires is discussed in terms of possible communicative patterns of deaf children. The specific problems that deaf children meet in their daily communication might explain their abundance of desire-references: plausibly, they give a high priority to stress their own desires and needs unambiguously.     

Estimating the burden of congenital rubella syndrome in Costa Rica, 1996-2001

BACKGROUND: The epidemiology of rubella in Costa Rica changed during recent decades, shifting the susceptible groups to the reproductive age. This study estimates the burden of congenital rubella syndrome (CRS) from 1996 to 2001 in this country. METHODS: Three methods to calculate CRS incidence were used. A retrospective search ("Observed cases") was conducted using hospital discharge records of children born from 1996 to 2001 with selected codes of ICD9 and ICD10 consistent with CRS and children <3 months of age with a positive serologic test for rubella IgM antibody at the National Children's Hospital (NCH). Cases were classified as either suspected, compatible or confirmed CRS and congenital rubella infection. "Expected" incidence of CRS was calculated using reported cases of rubella (women 15-45 years of age) and fertility rates, assuming CRS probability of 0.9 during the first trimester of pregnancy and 0.5 of asymptomatic rubella cases. "Estimated" CRS cases were calculated using incidence rates reported from modeling analysis during epidemic and endemic years. RESULTS: Of the 577 discharge charts reviewed and the 66 children reported as rubella IgM(+), 40 compatible CRS cases, 45 confirmed, and 4 with congenital rubella infection cases were identified. The range of annual incidence rate of CRS (per 1000 live births) was as follows: "Observed" = 0.00-0.33, "Expected" = 0.00-0.35 and "Estimated" = 0.5-1.5. Compared with the estimated number of CRS cases, only 27.2% of CRS cases were detected from the retrospective search and 10.1% would be expected when calculated using rubella reported cases. CONCLUSIONS: The under-detection of CRS cases using rubella reported cases in women of reproductive age and retrospective search of CRS reinforces the importance of suspecting CRS in the presence of a single compatible manifestation. Laboratory confirmation is indispensable to implement CRS elimination strategies and should be done in every suspected case.     

The nerve deaf child--intrauterine rubella or not?

We describe a laboratory test which is useful in determining whether deafness is due to intrauterine rubella. The study group consisted of 8 deaf patients with proved intrauterine rubella, 12 patients with familial deafness, and 17 controls. Blood was taken at time 0 from all of them. All the deaf children and 5 controls were immunised and further blood samples taken at 2 and 6 weeks. Each blood sample was analysed for the lymphocyte response to rubella in vitro and for the presence of rubella haemagglutination inhibition. All the rubella-affected patients had antibody titres to rubella at time 0 and there was no notable change after immunisation. None of these patients had a significant lymphocyte response to rubella antigen at any time. Eight of the familial deaf patients and 13 of the controls had positive antibody titres. With the exception of one patient whose results were equivocal, all the familial deaf patients and controls in whom antibodies were detected had lymphocyte responses to rubella. In those with undetectable antibodies the lymphocytes failed to respond in vitro to rubella antigen.     

Theory-of-mind development in oral deaf children with cochlear implants or conventional hearing aids

BACKGROUND: In the context of the established finding that theory-of-mind (ToM) growth is seriously delayed in late-signing deaf children, and some evidence of equivalent delays in those learning speech with conventional hearing aids, this study's novel contribution was to explore ToM development in deaf children with cochlear implants. Implants can substantially boost auditory acuity and rates of language growth. Despite the implant, there are often problems socialising with hearing peers and some language difficulties, lending special theoretical interest to the present comparative design. METHODS: A total of 52 children aged 4 to 12 years took a battery of false belief tests of ToM. There were 26 oral deaf children, half with implants and half with hearing aids, evenly divided between oral-only versus sign-plus-oral schools. Comparison groups of age-matched high-functioning children with autism and younger hearing children were also included. RESULTS: No significant ToM differences emerged between deaf children with implants and those with hearing aids, nor between those in oral-only versus sign-plus-oral schools. Nor did the deaf children perform any better on the ToM tasks than their age peers with autism. Hearing preschoolers scored significantly higher than all other groups. For the deaf and the autistic children, as well as the preschoolers, rate of language development and verbal maturity significantly predicted variability in ToM, over and above chronological age. CONCLUSIONS: The finding that deaf children with cochlear implants are as delayed in ToM development as children with autism and their deaf peers with hearing aids or late sign language highlights the likely significance of peer interaction and early fluent communication with peers and family, whether in sign or in speech, in order to optimally facilitate the growth of social cognition and language.     

Incidence and Outcome of Acute Cardiorenal Syndrome in Hospitalized Children

Objectives

To determine the incidence, etiology and outcome of Cardiorenal syndrome (CRS) in hospitalized children.

Methods

A prospective cohort study was carried out in 242 children between 6 mo to 18 y of age hospitalized with primary cardiac, renal or any systemic disorder at a tertiary care center in India. The primary outcome was the development of CRS. Univariate and multivariate logistic regression analysis were performed to determine the risk of mortality secondary to CRS.

Results

Among 242 children, 67 (27.7%) children developed CRS and the rest 175 (72.3%) did not. Among those with CRS, 40.3%, 20.9%, and 38.8% had CRS-1, 3 and 5, respectively. Cardiac diseases leading to CRS were myocarditis (40.7%) followed by congenital heart disease (25.9%), rheumatic heart disease (18.5%), and dilated cardiomyopathy (7.4%); renal disease associated with CRS was acute glomerulonephritis (100%) and major systemic disorders leading to CRS were septicemia (53.8%), malaria (23.1%), scrub typhus (7.7%), and acute gastroenteritis (3.8%). The occurrence of CRS was associated with an increased risk of mortality (OR 6.3, 95% CI: 2.8, 14.1; p 0.000). A subgroup analysis revealed that children with CRS having acute kidney injury stage 2 and 3 also had a higher risk of mortality (p 0.001).

Conclusions

The incidence of CRS is quite high in children with cardiac, renal or systemic diseases and is associated with a significant risk of mortality. Children presenting with these illnesses should be monitored for the occurrence of CRS so that early intervention may reduce mortality.

     

Working capacity of deaf and visually and mentally handicapped children

Using bicycle ergometers, physical working capacity was determined in 61 deaf, 22 visually handicapped, and 67 mentally retarded children 8 to 17 years of age. Compared to the normal population, the working capacities per kilogram body weight of the deaf children, particularly the girls, were superior to the other groups and to the normal population. The visually handicapped had lower working capacities, while those of the retarded children were similar to the normals.The work efficiency was similar in both groups. For the boys and younger girls, the maximum oxygen uptakes per kilogram body weight were similar in the deaf and visually handicapped, results that were contrary to the working capacity studies. The difference could be explained by postulating that the deaf children did not work to maximal values though lactic acid and respiratory quotient data suggested that they did. The maximum heart rates of the deaf children were lower than the visually handicapped.     

Visual attention in deaf and hearing infants: the role of auditory cues

BACKGROUND: Successful communication with profoundly deaf children is heavily dependent on visual attention. Previous research has shown that mothers of deaf children--notably those who are deaf themselves--use a variety of strategies to gain their children's attention. This study compares patterns of visual attention in deaf and hearing children to determine how they are affected by the absence of auditory cues, especially when looking to the mother's face. METHOD: The visual attention of 18-month-old infants to their mothers was examined in two groups of deaf children (6 with deaf mothers and 6 with hearing mothers) and two of hearing children (6 with deaf mothers and 8 with hearing mothers). Dyads were observed in free play and 10 minutes of videorecorded interaction was analysed. All looks to the mother were classified as Spontaneous, Responsive (Child turns in response to something done by mother) or Elicited (Mother actively seeks to gain child's attention by, e.g., tapping or waving). The kind of event that attracted the child's attention in Responsive and Elicited episodes was also determined (e.g., object movement, speech, physical contact), as was the focus of the child's attention (e.g., mother's face, mother's body). RESULTS: Responsive looks to the mother were the most frequent for all groups but on only about 25% of occasions were they directed to her face. Elicited and spontaneous looks occurred less often but were frequently directed to the mother's face. Spontaneous looking occurred in all groups but elicited looking very seldom occurred in Hearing-Hearing dyads. Overall, there were fewer looks to the mother in the two groups where mother and child had congruent hearing status, although the proportion of the looks to the mother's face was similar. CONCLUSIONS: Both spontaneous and elicited looks are likely to involve attention to the mother's face. However, while active elicitation of attention is an important part of successful communication with young deaf children, this does not appear necessary for typically developing hearing children who turn to look at their mother's face on hearing her voice. The implications of these findings for differences in the dynamics of communication with young deaf and hearing children are discussed.     

Auditory Perception and the Control of Spatially Coordinated Action of Deaf and Hearing Children

From birth onwards, auditory stimulation directs and intensifies visual orientation behaviour. In deaf children, by definition, auditory perception cannot take place and cannot, therefore, make a contribution to visual orientation to objects approaching from outside the initial field of view. In experiment 1, a difference in catching ability is demonstrated between deaf and hearing children (10-13 years of age) when the ball approached from the periphery or from outside the field of view. No differences in catching ability between the two groups occurred when the ball approached from within the field of view. A second experiment was conducted in order to determine if differences in catching ability between deaf and hearing children could be attributed to execution of slow orientating movements and/or slow reaction time as a result of the auditory loss. The deaf children showed slower reaction times. No differences were found in movement times between deaf and hearing children. Overall, the findings suggest that a lack of auditory stimulation during development can lead to deficiencies in the coordination of actions such as catching which are both spatially and temporally constrained.     

Ophthalmologic abnormalities in children from a Turkish school for the deaf

The aim of this study was to determine the nature and prevalence of ophthalmologic abnormalities in deaf children. Complete ophthalmologic examinations were performed on 104 children aged 7 to 20 years from a Turkish school for the deaf. Of 104 children, 42 children (40.4%) had some form of ophthalmologic abnormality, with the vast majority (31 patients, 29.8%) being refractive errors. The prevalence of hypermetropia, myopia and astigmatism was found to be higher over the general population. Of 104 children, 19 (18.2%) had ocular motility disturbances and one had external adnexal anomaly. Two (1.8%) children had anterior segment and nine (8.6%) children had posterior segment pathologies. Routine ophthalmologic examinations should be carried out in this population because deaf children use sight to compensate for hearing problems. Ophthalmologists play an important role in the diagnosis and correction of ophthalmologic abnormalities.     

Relation between deaf children's phonological skills in kindergarten and word recognition performance in first grade

BACKGROUND: The aim of the present study was twofold: 1) to determine whether phonological skills measured in deaf prereaders predict their later phonological and reading skills after one year of reading instruction as is the case for hearing children; 2) to examine whether the age of exposure to a fully specified phonological input such as Cued Speech may explain the inter-individual differences observed in deaf children's phonological and word recognition levels. METHOD: Twenty-one 6-year-old deaf prereaders and 21 hearing children of the same chronological age performed two phonological tasks (rhyme decision and generation tasks); they were re-assessed 12 months later and presented with other phonological tasks (rhyme decision and common unit identification tasks) and a written word choice test. RESULTS: Phonological skills measured before learning to read predicted the written word recognition score the following year, both for hearing and for deaf participants. Age of onset of exposure to Cued Speech was also a strong predictor of phonological and written word recognition scores in beginning deaf readers. CONCLUSIONS: The evidence broadly supports the idea of a capacity for acquiring phonological skills in deaf children. Deaf children who are able to develop an implicitly structured phonological knowledge before learning to read will be better readers when this knowledge becomes explicit under the pressure of reading instruction.     

INFLUENCE OF YORUBA BELIEFS ABOUT ABNORMALITY ON THE SOCIALIZATION OF DEAF CHILDREN: A RESEARCH NOTE

The study examines patterns of communication modes of guidance and discipline and affectional bonds between 176 Yoruba hearing mothers and their deaf children. Results relating to communication support earlier findings about the frustrations inherent in such endeavour. Contrary to other published reports, the Yoruba mothers studied perceived expressive linguistic abilities of deaf children more negatively than receptive abilities. Communication difficulties affected mothers' guidance and discipline, particularly since the culturally preferred modes of discipline rely very heavily on children's age-related language competence. Mothers' verbal claims of affectional bonds were not supported by evidence from other sources close to and including the deaf children.     

语前聋患儿连接蛋白30基因突变

目的分析46例语前聋患儿连接蛋白30（Connexin30,Cx30,GJB6）基因del（GJB6-D13S1830）突变。方法收集46例散发的语前聋患儿及听力正常健康对照30例血标本,提取DNA后利用聚合酶链反应（PCR）分析方法,筛查Cx30基因del（GJB6-D13S1830）突变。结果Cx30基因存在del（GJB6-D13S1830）杂合突变3例,余患儿及听力正常者无此突变。结论Cx30基因del（GJB6-D13S1830）突变可能是导致语前聋的原因之一。     

Chronic rhinosinusitis in young children differs from adults: a histopathology study

Objective

A histopathologic study of children with chronic rhinosinusitis (CRS) was undertaken to compare the sinus mucosa in pediatric and adult CRS.

Study design

CRS has been defined as persistent or recurrent sinusitis symptoms for ≥12 weeks despite conventional medical therapy, with abnormal computed tomography of the maxillary sinuses. Maxillary mucosal biopsies were obtained from pediatric CRS subjects for inflammatory cell and morphologic studies. Archival sinus mucosal tissues from adults with CRS were used as histologic controls. Sinus lavages were performed on children with and without CRS for microbiologic studies.

Results

Sinus mucosal biopsies were obtained from 19 children with CRS (median age, 3.0 years; range 1.4-8.2 years). Pediatric CRS biopsies, as compared with adult CRS controls, had a higher density of submucosal lymphocytes (median 469 versus 294 cells/mm² per 5 high-power fields [HPF]; P = .02), lower density of submucosal eosinophils (medians 13 versus 82 cells/mm² per 5 HPF; P = .01), thinner and more intact epithelium (P = .01 and .07, respectively), thinner basement membranes (P = .002), and fewer submucosal mucous glands (P = .004).

Conclusion

The sinus mucosa of young children with CRS has less eosinophilic inflammation, basement membrane thickening, and mucus gland hyperplasia characteristic of adult CRS.     

Knowledge of display rules in prelingually deaf and hearing children

Deaf children of elementary and secondary school age participated in a study designed to examine their understanding of display rules, the principles governing the expression and concealment of emotion in social situations. The results showed that deaf children's knowledge of display rules, as measured by their reported concealment of emotion, was comparable to that of hearing children of the same age. However, deaf children were less likely to report that they would conceal happiness and anger. They were also less likely to produce reasons for concealing emotion and a smaller proportion of their reasons were prosocial, that is, relating to the feelings of others. The results suggest that the understanding of display rules (which function to protect the feelings of other people) may develop more gradually in deaf children raised in a spoken language environment than it does in hearing children.