Mean Standard Score Research Articles

Chest circumference in infancy predicts obesity in 3-year-old children.

The purpose of this study was to investigate the associations between chest circumference in infancy and rapid growth or obesity at 3 years of age. We conducted a retrospective observational study of 1353 children in Kumamoto, Japan. Data collected included chest circumference, head circumference, weight, and body mass index. The area under the receiver operating characteristic curves for chest circumference were analyzed to determine the ability of this index to identify obesity at 3 years of age. Chest circumference at 3-4 months of age and increases in chest circumference during the first 3-4 months of life had higher mean standard deviation scores for rapid growth than for slow or no change in growth (p<0.05). Chest circumference and the increase in chest circumference were also positively correlated with rapid weight gain, and were associated with obesity at 3 years of age. The area under the curve for chest circumference was significantly different from the area under the curve for weight z-score at 6-9 months but not for weight z-score at 3-4 months. In conclusion, we found that chest circumference is associated with obesity in young children, and is positively correlated with rapid growth. Therefore, chest circumference may be a useful marker for rapid growth, and may help clinicians to identify obese children at 3 years of age.

349 Effects of Postnatal Energy and Macronutrient Intakes on Growth in Extremely Preterm Infants: a Population-Based Study

BackgroundExtremely preterm infants often experience growth failure and adequate nutritional supply may be difficult to achieve. It is still debated to what extent nutrition affects growth at an early stage...

Providing a learning disability in‐reach service for young adult offenders serving a sentence of Imprisonment for Public Protection

Purpose – The purpose of this paper is to report on a three‐year contract to provide learning disability in‐reach to young offenders serving a sentence of Imprisonment for Public Protection (IPP) at HMYOI Aylesbury.Design/methodology/approach – A whole population sample (n=75) was obtained between December 2009 and July 2011, where all had received an assessment of intellectual functioning using either the in‐reach screening protocol, consisting of the Kaufman Brief Intelligence Test – Version 2 (KBIT‐2) and four background questions, the Wechsler Abbreviated Scale of Intelligence (WASI) or the Wechsler Adult Intelligence Scale (WAIS).Findings – It was found that 5.5 per cent of the population had a standard score of less than 70 (significant impairment) and a further 18.6 per cent fell between 70 and 79 (borderline range). The mean standard score of the KBIT‐2 (85.8) was consistent with previous studies of prison populations. The background had a significant but weak association with the KBIT‐2 scores, b...

Involution of multicystic dysplastic kidney: Is it predictable?

ObjectiveTo evaluate the clinical course of multicystic dysplastic kidney (MCDK) and to reveal any criteria indicating spontaneous involution. Material methodsHospital records of patients with MCDK followed in two different institutions in 1994–2009 were reviewed and data were analyzed regarding involution. ResultsRecords of 96 patients were reviewed, of whom 46 were diagnosed antenatally and followed for more than 1 year. Fourteen patients had undergone nephrectomy. There was one case of hypertension which resided with nephrectomy. There was no malignancy. Involution rate was 53.6% (15/28) for right-sided and only 16.7% (3/18) for left-sided kidneys. The initial size of the kidney was found to be another predictive parameter for involution. Initial sizes of 43 (15 involuted and 28 non-involuted) kidneys were documented. Mean standard deviation score for involuting and non-involuting kidneys was −3.19 and 3.12, respectively. The chance of involution for a large kidney on the left was zero; however, involution risk for a small right-sided kidney was 67%. ConclusionReviewing a 15-year period of our patient records conveyed data supporting current literature mainly encouraging non-operative management of MCDK. Further studies are required; however, our two objective indicators, the initial size and side of dysplastic kidney, may contribute to the management.

Endocrine late sequelae in long-term survivors of childhood non-Hodgkin lymphoma

Aim of this study was to investigate the long-term endocrine effects of treatment of childhood non-Hodgkin lymphoma (NHL). A single-center cohort of 84 survivors (22 females) was included in this retrospective study. Median age was 21 years (9-40 years) and time after cessation of therapy 12 years (4-30 years). Height, weight, percentage fat, lean body mass (LBM), bone mineral content (BMC), bone mineral density of total body (BMD(TB)) and bone mineral density of lumbar spine (BMD(LS)) were measured. Thyroid-stimulating hormone (TSH), free thyroxin (fT4), insulin-like growth factor-1 (IGF-1), inhibin B and anti-müllerian hormone (AMH) levels were measured. Results were compared with Dutch controls. Height was lower in survivors [mean standard deviation score (SDS) -0.36, P = 0.002], but further analysis showed that shorter stature was already present at diagnosis (mean SDS -0.28, P = 0.023). Body mass index, percentage fat, BMC, BMD(TB) and BMD(LS) were not different from controls. LBM was lower in survivors (mean SDS -0.47, P = 0.008). TSH, fT4 and IGF-1 were normal in all survivors. Three of 20 adult females had low AMH levels and 23 of 42 adult males had low inhibin B levels. Twelve years after cessation of treatment, NHL survivors did not develop adiposity, osteoporosis or thyroid disease. Male survivors may be at risk for infertility.

Lung function in young adults with congenital diaphragmatic hernia; a longitudinal evaluation

Survival rates of patients with congenital diaphragmatic hernia (CDH) have improved to up to 80%. Little is known about long-term consequences of the disease and its treatment. We evaluated lung function and respiratory symptoms longitudinally in a previously studied cohort of CDH patients and age-matched non-CDH patients who underwent similar neonatal intensive care treatment. We tested 27 young adults [mean (SD) age: 26.8 years (2.9)] with CDH and 30 non-CDH patients. Dynamic and static lung volumes, midexpiratory flows, and diffusion capacity were expressed as mean (SD) standard deviation scores. Prevalence of respiratory symptoms was evaluated with the European Community Respiratory Health Survey. All data are expressed as mean (SD). FEF(25-75) in CDH patients had slightly deteriorated since childhood (CDH: -0.7 (1.4) vs. -1.6 (1.5), P < 0.001; non-CDH patients: 0.2 (1.4) vs. -0.3 (1.6), P = 0.038, ns). Diffusion capacity decreased in both groups (CDH: DL(CO) c 0.2 (1.1) vs. -1.5 (1.1), P <0.001; non-CDH: DL(CO) c 0.1 (0.4) vs. -1.1 (1.1), P < 0.001). Lung volumes were normal in both groups. The prevalence of asthma was higher than in the normal population (27.6% in patients and 30% in controls, P < 0.001). Airflow obstruction and diffusion capacity deteriorated mildly from childhood into adulthood in survivors of CDH. The improved survival of patients with more severe forms of diaphragmatic hernia calls for long-term follow-up of lung function.

Abstract 2554: Long-term Neuropsychological Outcomes after Childhood Arterial Ischemic Stroke (AIS)

Background: Childhood AIS affects 2-6/100,000 children per year and is associated with significant morbidity and mortality. Although previous reports have demonstrated lower IQ’s in children following AIS as compared to healthy children, understanding of cognitive function more broadly in childhood AIS remains limited. Methods: In order to address this gap in knowledge, we collected data on demographics, stroke type, stroke size, stroke location, and long-term neuropsychological outcome, (defined as testing that occurred at least 10 months after stroke diagnosis). Patients were enrolled in an institution-based prospective cohort study of childhood AIS, comprising 63 children diagnosed with AIS from March 2006-August 2011. Neuropsychological assessment employed age-appropriate measures. In addition to IQ, other cognitive domains were tested, including academics, visual-motor skills, memory, and language. Single-tailed one-sample t-tests were performed to compare the childhood AIS group to the normative mean for neuropsychological exams. Furthermore, ANOVA analyses were used to compare neuropsychological outcome among subgroups of interest. Results: 42 patients in our cohort had neuropsychological testing available at least 10 months after stroke diagnosis: 21 males and 21 females, with a mean age of 7 years at time of stroke (SD= +/-5.43). Mean neuropsychological testing was at 36 months after AIS (SD= +/- 30.72). Mean IQ for childhood AIS patients was 93.8 (SD= +/-17.75) and was significantly lower than the population mean (p &lt; .05). Cognitive profile testing revealed significantly decreased functioning in the visual-motor (mean standard score = 84.1 [SD = +/-17.32], p &lt;.001) and language domains (mean standard score = 82.9 [SD= +/-25.15], p = .002). By contrast, academic and memory profiles were not significantly reduced (standard score means of 95.1 and 97.0, respectively). In sensitivity analyses that excluded patients known to have pre-morbid (i.e., pre-AIS) neuropsychological abnormalities, visual-motor and language domains remained significantly reduced as compared to the population mean (p &lt; 0.001, and p= 0.04, respectively). Stroke type, stroke size, stroke location and arteriopathy were not significant predictors of long-term neuropsychological outcome. Conclusions: Long-term neuropsychological outcome in this cohort confirms that childhood AIS patients have lower IQ’s than healthy children; in addition, results indicate that specific visual-motor and language skills are even more significantly affected. We recommend that children with AIS be comprehensively evaluated for the need for targeted interventions to improve visual-motor and language skills, and that the effectiveness of these interventions be evaluated in future studies.

Micronutrient Intakes Affect Early Growth in Extremely Preterm Infants: Preliminary Results from a Swedish Cohort

Background: Extremely preterm infants generally experience postnatal growth failure. It is still unclear if this is related to micronutrient intakes. Aim: To investigate the effect of micronutrient intakes (calcium, zinc, iron, phosphorus, sodium, potassium, chloride, magnesium, vitamin A, vitamin D, vitamin E, folate and vitamin B12) on growth during the first 28 days of life in extremely preterm infants. Method: From the EXPRESS cohort (all infants born < 27 gestational weeks between 2004-2007 in Sweden), those who survived the first 28 days were included (n=524). Daily parenteral and enteral intakes and anthropometric measurements were retrieved from hospital records. Results: Preliminary analyses of data from 333 infants (mean±SD gestational age 25.2±1.0 weeks, birth weight 753±168g) showed that macronutrient intakes were lower than recommended (energy 98±13kcal/kg/day, protein 2.9±0.4g/kg/day). Infants showed postnatal growth failure: mean standard deviation scores decreased by 2.2 for weight, 2.3 for length and 1.4 for head circumference. Intakes of micronutrients were generally low, e.g. adjusted enteral intakes of calcium were 66.6±21.4 mg/kg/day. The exception was iron, with a high parenteral intake of 2.7±1.6 mg/kg/day, mainly from blood transfusions. Adjusting for protein intake and other confounders, calcium intakes were positively correlated with head growth (r=+0.19, p=0.006) and iron intakes were negatively correlated with length gain (r=-0.18, p=0.009). Conclusions: Low calcium intakes and high iron intakes were associated with poor growth with regard to head circumference and length, respectively. If these results are confirmed, optimized micronutrient intakes may improve early growth in extremely preterm infants.

Long-term impact of infantile short bowel syndrome on nutritional status and growth

Short-term bowel adaptation has been documented, but data on long-term effects are scarce. The aim of the present study was to evaluate the long-term consequences of infantile short bowel syndrome (SBS). A cross-sectional assessment (2005-7) of growth, nutritional status, defecation pattern and health status in individuals with a history of infantile SBS, born between 1975 and 2002, were performed. Data were compared with reference values of healthy controls and presented as means and standard deviations or median and ranges. A total of forty subjects (sixteen male and twenty-four female; mean age 14·8 (SD 6·8) years) had received parenteral nutrition during a median of 110 (range 43-2345) d, following small bowel resection. The mean standard deviation scores (SDS) for weight for height and target height (TH) of the children were normal; mean SDS for height for age was - 0·9 (SD 1·3). The median BMI adults was 19·9 (range 17-26) kg/m2; mean SDS for height for age was - 1·0 (range - 2·5 to 1·5). Height in general was significantly shorter than TH, and 53 % of children and 78 % of adults were below TH range. Most subjects had normal body fat percentage (%BF). SDS for total body bone mineral density were generally normal. The SDS for bone mineral content (BMC) of the children were - 1·0 (SD 1·1). Mean energy intake was 91 % of the estimated average requirements. The frequencies of defecation and bowel complaints of the subjects were significantly higher than in healthy controls. In conclusion, infantile SBS results in shorter stature than was expected from their calculated TH. BMC was lower than reference values, but the subjects had normal weight for height and %BF.

Tocilizumab-effects on growth impairment in systemic juvenile idiopathic arthritis

Background The safety and efficasy of anti-IL-6 receptor monoclonal antibody, tocilizumab (TCZ) has been reported in children with systemic juvenile idiopathic arthritis (s-JIA). Objectives Growth analysis during the study was performed. Methods Forty-five s-JIA patients (8.1±4.2 years) who completed phase-III study of TCZ were enrolled. Mean standard deviation score (SDS) for height, changes in SDS from baseline (ΔSDS), correlation between ΔSDS and several factors such as age, disease duration, corticosteroid dose exposure were evaluated. Yearly height velocity analysis was made with 28 patients who had data for 1 year prior to TCZ administration and received TCZ for more than 1 year. Results Thirty-eight of 45 (84%) obtained clinical response at week 144. The baseline SDS-height was -2.7±2.0 with inverse correlation with disease duration. Significant improvement was seen in height velocity SDS changes from 1 year prior to 1 year posterior to baseline (n=28, -6.0±4.0 to-2.5±3.9, p =0.0064). Reduction in corticosteroid exposure was significantly associated with improvement in height velocity SDS ( p =0.0027). Standardized height velocity continued to improve over 3 years of TCZ, whereas average daily prednisolone equivalent dose showed inverse correlation (n=17, Figure). Conclusions Growth impairments evidenced by SDS-height were more prominent in patients with longer standing disease. Catch up growth was observed in patients who required less or no corticosteroid during TCZ treatment. References Yokota S, et al. Lancet. 2008 Mar 22;371(9617):998-1006. Disclosure of Interest None Declared

Hydrocephalus status in spina bifida: an evaluation of variations in neuropsychological outcomes

Object The effect of hydrocephalus status on neuropsychological outcomes in children with spina bifida (SB) has not been carefully evaluated. The authors hypothesized a stepwise progression of outcomes related to hydrocephalus status (shunt-treated, arrested, or no hydrocephalus) and that motor, spatial, and executive function tasks would be more sensitive to hydrocephalus status than vocabulary and reading tasks. Methods Two hundred eight children (mean age 11.2 years) with SB were grouped according to hydrocephalus status: shunt-treated hydrocephalus (166 children), arrested hydrocephalus (18 children), and no hydrocephalus (24 children). Sixty-one typically developing children were included as a control group (mean age 12.05 years). All children were tested across neuropsychological content domains, including verbal and nonverbal IQ, reading and mathematical achievement, explicit memory, visuospatial function, executive function, and motor skills. Results There was a stepwise progression of outcomes. Averaging across tasks, performance scores of children with SB and no hydrocephalus (mean standard score 92.60) were higher than those of children with SB and arrested hydrocephalus (mean standard score 86.86), and scores of children in the latter group were higher than those of children with SB and shunt-treated hydrocephalus (mean standard score 82.30). All 3 groups scored lower than the control group (mean standard score 105.94). Fine motor tasks best differentiated the arrested-hydrocephalus and shunt-treated groups. Verbal and executive function tasks, often associated with socioeconomic status, best differentiated the group of children with SB and no hydrocephalus from the control group. Conclusions With the exception of fine motor skills and small differences in memory and spatial domains, children with SB and arrested or shunt-treated hydrocephalus have similar neuropsychological profiles. Performance of all 3 groups of children with SB was below that of the control group, which also reflects the lower socioeconomic status of the children with SB.

Effectiveness of semantic therapy for word‐finding difficulties in pupils with persistent language impairments: a randomized control trial

Word-finding difficulties (WFDs) in children have been hypothesized to be caused at least partly by poor semantic knowledge. Therefore, improving semantic knowledge should decrease word-finding errors. Previous studies of semantic therapy for WFDs are inconclusive. To investigate the effectiveness of semantic therapy for secondary school-aged pupils with WFDs using a randomized control trial with blind assessment. Fifteen participants with language impairments and WFDs (aged 9;11-15;11) were randomly assigned to a therapy versus waiting control group. In Phase 1 the therapy group received two 15-min semantic therapy sessions per week for 8 weeks with their usual speech and language therapist. Therapy for each participant targeted words from one of three semantic categories (animals, food, clothes). All participants were tested pre- and post-phase 1 therapy on the brief version of the Test of Adolescent Word Finding (TAWF), semantic fluency and the Test of Word Finding in Discourse (TWFD). In Phase 2 the waiting control group received the same therapy as the original therapy group, which received therapy targeted at other language areas. Testing after Phase 2 aimed to establish whether the waiting control group made similar progress to the original therapy group and whether the original therapy group maintained any gains. The original therapy group made significant progress in standard scores on the TAWF (d= 0.94), which was maintained 5 months later. However, they made no progress on the semantic fluency or discourse tests. Participants in the waiting control group did not make significant progress on the TAWF in Phase 1 when they received no word-finding therapy. However, after Phase 2, when they received the therapy, they also made significant progress (d= 0.81). The combined effect of therapy over the two groups was d= 1.2. The mean standard scores on the TAWF were 67 pre-therapy and 77 post-therapy. Four hours of semantic therapy on discrete semantic categories led to significant gains on a general standardized test of word finding, enabling the participants to begin to close the gap between their performance and that of their typically developing peers. These gains were maintained after 5 months. A small amount of therapy can lead to significant gains even with secondary aged pupils with severe language difficulties. However, further studies are needed to find ways of improving word-finding abilities in discourse.

Growth Hormone Plus Childhood Low-Dose Estrogen in Turner Syndrome

Characteristic features of Turner syndrome include short stature and ovarian failure. There is considerable evidence from nonrandomized studies that treatment with recombinant human growth hormone increases adult stature in patients with Turner syndrome. It is commonly used in such patients despite the lack of objective data from randomized, double-blind, placebo-controlled studies. Ovarian failure associated with Turner syndrome is treated with estrogen-replacement therapy. It has been proposed that very low-dose physiologic estrogen replacement during childhood combined with growth hormone therapy could provide an additional benefit on adult height than that achieved with growth hormone therapy alone. The aim of this double-blind, placebo-controlled trial was to investigate the independent effect of growth hormone therapy alone and a regimen of growth hormone combined with early ultra–low-dose estrogen on adult height in girls with Turner syndrome. A total of 149 girls (5.0–12.5 years of age) were randomized to receive 1 of 4 regimens: (1) placebo injection plus childhood oral placebo (n = 39), (2) placebo injection plus childhood oral low-dose estrogen (n = 40), (3) growth hormone injection plus childhood oral placebo (n = 35), and (4) growth hormone injection plus childhood oral low-dose estrogen (n = 35). Doses of growth hormone (0.1 mg per kg of body weight) were injected 3 times per week. Ethinyl estradiol or placebo was administered in oral doses, with adjustment for chronologic age and pubertal status. From the first visit after the 12th birthday, escalating doses of ethinyl estradiol were administered to patients in all groups, according to an escalating dosage regimen. Patients were assessed at 6-month intervals. Growth hormone therapy was continued until patients reached their protocol-specified adult height (defined as the last height measured once the height velocity was less than 1.5 cm per year). For adult height attained at 17.0 ± 1.0 years after a mean study period of 7.2 ± 2.5 years, the mean standard deviation scores for the 4 groups were as follows: −2.81 ± 0.85 for the double-placebo group, −3.39 ± 0.74 for the estrogen-alone group, −2.29 ± 1.10 for the growth hormone-alone group, and −2.10 ± 1.02 for the combined growth hormone-estrogen group (P < 0.001 among the 4 groups). Patients treated with growth hormone attained greater adult height compared with placebo (mean difference in standard deviation score, 0.78 ± 0.13, equivalent to about 5.0 cm; P < 0.001). There was a trend toward a modest synergistic growth benefit in the combined growth hormone-estrogen group compared with the estrogen-alone group; the adult height gain with combined therapy was 0.32 ± 0.17 standard deviation score; P = 0.059. These findings provide objective evidence from a placebo-controlled trial that growth hormone treatment initiated in mid-childhood increases adult height in girls with Turner syndrome. The data also suggest that the combination of ultra–low-dose childhood estrogen replacement and growth hormone therapy may improve the height gain achieved with growth hormone alone.

Follow-up Study on Occupational Exposure to Organic Solvents and Neurobehavioral Performances of Automotive Painters

Background/Aims: This study assessed occupational exposure to organic solvents and neurobehavioral performance of exposed automotive painters. The mean personal air toluene concentration (PATC), urinary hippuric acid concentration, and standard score (SS) of the modified WHO Neurobehavioral Core Test Battery (NCTB) of exposed workers in 2008 were compared with those obtained in 2004. Methods: The study subjects consisted of 48 automotive painters who had participated in the 2004 study. They were re-examined in 2008 using PATC, urinary hippuric acid concentration, and 7 test items of the NCTB. A questionnaire was used to obtain sociodemographic information. Results: The mean PATC in 2008 was 0.403 ppm, which was significantly lower (P < 0.001). The mean SS for the Digit Span Test, Benton Visual Retention Test, Pursuit Aiming Test, and Total NCTB were significantly higher in 2008 compared to those in 2004 with the exception of the Santa Ana Manual Dexterity Test that showed significantly lower mean SS in 2008 compared to those in 2004. Conclusion: The lower concentration of solvents in the workplace and a shorter duration of exposure have contributed toward an improvement in the neurobehavioral performance of automotive painters in 2008 as compared to those in 2004 with the exception of manual dexterity.

Moderate weight reduction in an outpatient obesity intervention program significantly reduces insulin resistance and risk factors for cardiovascular disease in severely obese adolescents.

Background. Metabolic risk factors like insulin resistance and dyslipidemia are frequently observed in severly obese children. We investigated the hypothesis that moderate weight reduction by a low-threshold intervention is already able to reduce insulin resistance and cardiovascular risk factors in severely obese children. Methods. A group of 58 severely obese children and adolescents between 8 and 17 years participating in a six-month-long outpatient program was studied before and after treatment. The program included behavioral treatment, dietary education and specific physical training. Metabolic parameters were measured in the fasting state, insulin resistance was evaluated in an oral glucose tolerance test. Results. Mean standard deviation score of the body mass index (SDS-BMI) in the study group dropped significantly from +2.5 ± 0.5 to 2.3 ± 0.6 (P < 0.0001) after participation in the program. A significant decrease was observed in HOMA (6.3 ± 4.2 versus 4.9 ± 2.4, P < 0.03, and in peak insulin levels (232.7 ± 132.4 versus 179.2 ± 73.3 μU/mL, P < 0.006). Significant reductions were also observed in mean levels of hemoglobin A1c, total cholesterol and LDL cholesterol. Conclusions. These data demonstrate that already moderate weight reduction is able to decrease insulin resistance and dyslipidemia in severely obese children and adolescents.

Patterns of change in children with Autism Spectrum Disorders who received community based comprehensive interventions in their pre-school years: A seven year follow-up study

There are few long-term follow-up studies of children with Autism Spectrum Disorders (ASD) who attended intensive intervention programmes in their pre-school years. Thirty-six children with ASD enrolled in relatively intensive, specialist pre-school programmes (minimum of 15 h intervention per week for 2 years at a mean age of 3.4 years) were assessed after 2 years (mean age 5.5 years) and again after a further 5 years (mean age 10.3 years). Cognitive, language and adaptive behaviour skills and severity of autism symptoms were assessed at intake (Time 1) and subsequent follow-ups (Times 2 and 3). Children made significant increases in raw and age equivalent scores in most areas of development assessed, although mean standard scores remained stable or decreased over time. Time 1 IQ, language and adaptive behaviour skills were predictive of outcome at Time 3. Although there were marked individual differences in the rate and patterns of change over time, many children continued to show increases in test scores over the course of the study. This study highlights that whilst overall group improvements may be evident, the rate and nature of these improvements is highly variable across individual children. Further investigation of the specific child characteristics that affect treatment effectiveness is required.

Long-Term Language Levels and Reading Skills in Mandarin-Speaking Prelingually Deaf Children with Cochlear Implants

The aim of this study was to document receptive and expressive language levels and reading skills achieved by Mandarin-speaking children who had received cochlear implants (CIs) and used them for 4.75–7.42 years. The effects of possible associated factors were also analyzed. Standardized Mandarin language and reading tests were administered to 39 prelingually deaf children with Nucleus 24 devices. The Mandarin Chinese version of the Peabody Picture Vocabulary Test was used to assess their receptive vocabulary knowledge and the Revised Primary School Language Assessment Test for their receptive and expressive language skills. The Graded Chinese Character Recognition Test was used to test their written word recognition ability and the Reading Comprehension Test for their reading comprehension ability. Raw scores from both language and reading measurements were compared to normative data of nor- mal-hearing children to obtain standard scores. The results showed that the mean standard score for receptive vocabulary measurement and the mean T scores for the receptive language, expressive language and total language measurement were all in the low-average range in comparison to the normative sample. In contrast, the mean T scores for word and text reading comprehension were almost the same as for their age-matched hearing counterparts. Among all children with CIs, 75.7% scored within or above the normal range of their age-matched hearing peers on receptive vocabulary measurement. For total language, Chinese word recognition and reading scores, 71.8, 77 and 82% of children with CIs were age appropriate, respectively. A strong correlation was found between language and reading skills. Age at implantation and sentence perception scores account for 37% of variance for total language outcome. Sentence perception scores and preimplantation residual hearing were revealed to be associated with the outcome of reading comprehension. We concluded that by using standard tests, the language development and reading skill of Mandarin-speaking children who use CIs from a young age appear to fall within the normal range of their hearing age mates, at least after 4.8–7.4 years of experience. However, to fully evaluate the fine linguistic skills of these subjects, a more detailed study and longer follow-up period are needed.

Age-related stature and linear body segments in children with X-linked hypophosphatemic rickets

Children with X-linked hypophosphatemic rickets (XLH) are prone to severe stunting. A multicenter mixed-longitudinal study was conducted to assess age-related stature, sitting height, arm and leg length in XLH patients on continuous treatment with phosphate and calcitriol. Mean standard deviation scores (SDS) for all body dimensions were markedly reduced and differed significantly among each other at the initial and subsequent evaluations (baseline: stature -2.48 SDS; sitting height -0.99 SDS; arm length -1.81 SDS; leg length -2.90 SDS; each p<0.001). A strong association between stature and leg length (r (2)=0.87, p<0.001) was noted. Leg length SDS decreased progressively during childhood (2-9 years) and adolescence (12-15 years; each p<0.001). Sitting height SDS increased significantly during late childhood, indicating uncoupled growth of the legs and trunk and resulting in an ever increasing sitting height index (i.e. ratio of sitting height to stature; age 2 years 2.0 SDS; age 10 years 3.3 SDS; p<0.001) that was associated with the degree of stunting (r (2)=0.314, p<0.001). Mean serum phosphate levels were positively associated with stature and leg length, but negatively with sitting height index. Based on these results, we can conclude that growth of the legs and trunk is uncoupled in XLH and related to serum phosphate levels.

1206 Are Elbv Infants at Risk for Developing Atypical Metabolic Syndrome?

Background: Childhood metabolic syndrome increases the risk of cardiovascular complications in adulthood. Being born small for gestational age is an important risk factor of adulthood metabolic syndrome, however it is unclear whether ELBW children are also at increased risk for metabolic syndrome.Aim: Assessment of lipid metabolism in preschool ELBW infants in correlation with obesity and hypertension.Material and Methods: 40 ELBW children (mean birthweight 869g (SD:121), mean gestational age 27.6 weeks (SD: 1.9)) were evaluated at 6.7 years (range 6.3-7.4 years). Anthropometry, serum triglyceride (TG), LDL and HDL cholesterol levels, IGF-1 serum levels and 24-hour ambulatory blood pressure measurements were performed.Results: TG >= 1.1 mmol/l was found in 16 infants (40%) and HDL cholesterol levels < 1.3 mmol/l in 10 (25%). Combined lipid abnormalities were detected in 6 (15%) children. Systolic blood pressure above the 90th percentile was found in 10 children, and diastolic in 7 children. The mean standard deviation score (SDS) of weight/height ratio was -0.45 (range:-1.8 to 1.2 SDS) and mean SDS of fat fold thickness was -1,04 (range: -2.6 to 1.1 SDS). None of the examined children was obese (mean BMI: 14.9; range 10.9-20.1). Serum IGF-1 levels were within normal limits in all children.Conclusions: Lipids abnormalities and increased blood pressure are common in preschool ELBW infants, although the children examined were slimmer than their peers. It may be speculated that ELBW might be a risk factor for atypical metabolic syndrome not related to obesity.Project supported by Polish Ministry of Science (NN407170734).

Motor-function and exercise capacity in children with major anatomical congenital anomalies: An evaluation at 5 years of age

Background Children with major anatomical congenital anomalies (CA) often need prolonged hospitalization with surgical interventions in the neonatal period and thereafter. Better intensive care treatment has reduced mortality rates, but at the cost of more morbidity. Aim To study motor-function and exercise capacity in five-year-old children born with CA, and to determine whether motor-function and exercise capacity differ according to primary diagnosis. Study design Descriptive study. Subjects One-hundred-and-two children with the following CA: congenital diaphragmatic hernia (CDH) n = 24, esophageal atresia (EA) n = 29, small intestinal anomalies (SIA) n = 25, and abdominal wall defects (AWD) n = 24. Outcome measures Overall and subtest percentile scores of the Movement-Assessment Battery for Children (M-ABC) were used to measure motor skills. Endurance time on the Bruce treadmill test was used to determine maximal exercise capacity. Results Motor-function: Seventy-three children (71.6%) had an overall percentile score within the normal range, 18 (17.6%) were classified as borderline, and 11 (10.8%) had a motor problem. This distribution was different from that in the reference population (Chi square: p = 0.001). Most problems were encountered in children with CDH and EA ( p = 0.001 and 0.013, respectively). Ball skills and balance were most affected. Exercise capacity: Mean standard deviation score (SDS) endurance time = − 0.5 (SD: 1.3); p = 0.001; due to poor exercise performance in CDH and EA patients. Conclusions Children with major anatomical CA and especially those with CDH and EA are at risk for delayed motor-function and disturbed exercise capacity.