Reduced physical activity level and cardiorespiratory fitness in children with chronic diseases

We aimed to compare physical activity level and cardiorespiratory fitness in children with different chronic diseases, such as type 1 diabetes mellitus (T1DM), obesity (OB) and juvenile idiopathic arthritis (JIA), with healthy controls (HC). We performed a cross-sectional study including 209 children: OB: n = 45, T1DM: n = 48, JIA: n = 31, and HC: n = 85. Physical activity level was assessed by accelerometer and cardiorespiratory fitness by a treadmill test. ANOVA, linear regressions and Pearson correlations were used. Children with chronic diseases had reduced total daily physical activity counts (T1DM 497 ± 54 cpm, p = 0.003; JIA 518 ± 28, p < 0.001, OB 590 ± 25, p = 0.003) and cardiorespiratory fitness (JIA 39.3 ± 1.7, p = 0.001, OB 41.7 ± 1.2, p = 0.020) compared to HC (668 ± 35 cpm; 45.3 ± 0.9 ml kg⁻¹ min⁻¹, respectively). Only 60.4% of HC, 51.6% of OB, 38.1% of JIA and 38.5% of T1DM children met the recommended daily 60 min of moderate-to-vigorous physical activity. Low cardiorespiratory fitness was associated with female gender and low daily PA. Conclusion: Children with chronic diseases had reduced physical activity and cardiorespiratory fitness. As the benefits of PA on health have been well demonstrated during growth, it should be encouraged in those children to prevent a reduction of cardiorespiratory fitness and the development of comorbidities.     

Treatment with human growth hormone in patients with Prader-Labhart-Willi syndrome reduces body fat and increases muscle mass and physical performance

Twelve children with documented Prader-Labhart-Willi syndrome were treated with human growth hormone (24 U/m²/week) during 1 year. The children were divided into three groups: group 1: overweight and prepubertal (n = 6, age 3.8–7.0 years); group 2: underweight and prepubertal (n = 3, age 0.6–4.1 years); group 3: pubertal (n = 3, age 9.2–14.6 years). In group 1, height increased from −1.7 SD to −0.6 SD, while weight decreased from 1.1 SD to 0.4 SD, with a dramatic drop in weight for height from 3.8 SD to 1.2 SD. Hand length increased from −1.5 SD to −0.4 SD and foot length from −2.5 SD to −1.4 SD. Body fat, measured by dual X-ray energy absorptiometry, dropped by a third, whereas muscle mass increased by a fourth. Physical capability (Wingate test) improved considerably. The children were reported to be much more active and capable. In group 2, similar changes were seen, but weight for height increased, probably because muscle mass increase exceeded fat mass decrease. Changes in group 3 were similar as in group 1, even though far less distinct. Conclusion Growth hormone treatment in Prader-Labhart-Willi syndrome led to dramatic changes: distinct increase in growth velocity, height and muscle mass, as well as an improvement in physical performance. Fat mass and weight for height decreased in the initially overweight children, and weight for height increased in underweight children. Received: 25 July 1997 / Accepted in revised form: 3 November 1997     

Relationship between symptoms of attention-deficit/hyperactivity disorder and family functioning: a community-based study

This study examined the relationship between family functioning and attention-deficit/hyperactivity disorder (ADHD) symptoms in an Australian community-based sample. Children were screened for ADHD in their second year of formal schooling. Two hundred and two (202) primary caregivers completed validated measures of family quality of life (QoL), parent mental health, parenting styles and parental relationship quality. Compared with controls, parents of children screening positive for ADHD reported poorer family QoL in the domains of emotional impact (mean difference [MD] −20.1; 95% CI −38.2 to −1.9, p = 0.03) and impact on family activities (MD −17.2; 95% CI −27.9 to −6.5, p = 0.002), less parental warmth (MD −3.4; 95% CI −6.0 to −0.9, p = 0.01) and higher parental depression (MD 6.8; 95% CI 1.8 to 11.7, p = 0.009) and anxiety (MD 6.2; 95% CI 1.7 to 10.6, p = 0.008) after adjusting for socio-demographic characteristics and child conduct symptoms. Parents of children screening positive for ADHD reported higher stress (MD 4.5; 95% CI 1.2 to 7.1, p = 0.007) and more inconsistent (MD 3.0; 95% CI 1.2 to 4.8, p = 0.002) and hostile (MD = 2.2; 95% CI 1.0 to 3.4, p = 0.001) parenting after adjusting for socio-demographic factors only. No difference in parental relationship quality and parental inductive reasoning was identified. Conclusion: These findings suggest a strong association between poor family functioning and ADHD symptoms and carry implications for comprehensive ADHD management and the importance of seeing the child within the family context.     

Helicobacter pylori infection: effect on malnutrition and growth failure in dyspeptic children

There are conflicting reports regarding the association of Helicobacter pylori (H. pylori) infection with growth failure. We evaluated the role of H. pylori infection on malnutrition and growth failure in dyspeptic children. The study cases included 108 dyspeptic children and were evaluated by endoscopic gastric biopsy, while 50 healthy children constituted the control group. The study cases were grouped as H. pylori [+] (n = 57) and H. pylori [−] (n = 51) by the presence or absence of microorganism in gastric tissue, respectively. Age, gender, height for age (H/A), weight for height (W/H), body mass index (BMI), weight and height z scores and the daily calorie intake of the children were recorded. Malnutrition and growth failure were evaluated by the Waterlow criteria and height z score, respectively. Then, the H. pylori [+], H. pylori [−] and control groups were compared in relation to the variables defined above. All groups were similar with respect to gender and age. The daily calorie intake was lower in dyspeptic children. Although anthropometric variables were similar in the H. pylori [+] and [−] groups, the control cases had higher W/H compared to both H. pylori [+] (p = 0.030) and H. pylori [−] (p = 0.000) cases, and higher BMI (p = 0.001) and weight z scores (p = 0.014) than those in the H. pylori [−] group. The malnutrition rate was similar in the H. pylori [+] and [−] groups. However, mild acute (p = 0.033) and general malnutrition rates (p = 0.000) were lower in the control cases compared to the study cases. The short stature rate was not different significantly in all three groups. In conclusion, the results of this study do not support the data that H. pylori infection plays an extra role in malnutrition and growth failure in children presenting with dyspeptic complaints. However, as a major cause of dyspepsia, H. pylori infection might be considered to cause malnutrition secondary to decreased calorie intake associated with dyspepsia.     

Course of growth during the first 6 years in children exposed in utero to tobacco smoke

Objectives Postnatal growth in children exposed in utero to tobacco smoke is not well understood. This study investigated growth during the first 6 years in children whose mothers smoked during pregnancy. Materials and methods Weight, length, and head circumference were measured annually for 6 years in 100 children in each group of smoking (study) and nonsmoking (control) mothers. Results Weight and head circumference were significantly smaller in the neonates whose mothers smoked ≥15 cigarettes/day, but the difference disappeared by 3 years of life. Length was significantly smaller in the study neonates at birth, followed by increasing divergence from normality up to 2 years, when the mean difference of children whose mothers smoked ≥15 cigarettes/day from control children was −3.4 cm (p ≤ 0.0001). Subsequently, they manifested catch-up growth ,and the difference from the controls at 3, 4, 5, and 6 years was −2.5 cm (p ≤ 0.0001), −2.2 cm (p = 0.005), −2.1 cm (p = 0.013), and −1.9 cm (p = 0.055), respectively. Discussion The delayed growth was related to smoking per se and appeared to be independent of several confounding factors. At birth, there was a significant negative correlation between the number of cigarettes smoked per day and the growth parameters studied; it remained significant up to the 6 year only for length. Conclusion Length exhibits the most persistent growth delay of the parameters studied, but catch-up growth occurs after the second year of life, and thus, intrauterine exposure to tobacco smoke seems to have no permanent effect on children’s growth.     

A comparison of conventional and molecular microbiology in detecting differences in pneumococcal colonization in healthy children and children with upper respiratory illness

Conventional microbiology (CM) and real-time polymerase chain reaction (PCR) were used to determine rate and serotype of pneumococcal nasopharyngeal colonization in healthy children and children with upper respiratory illnesses (URI). One hundred and thirty-six healthy children and 79 children with URI were evaluated. Pneumococcal colonization was detected more often by real-time PCR than CM in healthy children (50% vs. 24%, p ≤ 0.001), while detection rates were comparable by CM and real-time PCR in children with URI (61% vs. 65%, NS). Pneumococcal serotypes were identified 2.3 times more often in healthy children by real-time PCR than CM, p ≤ 0.001 and 1.5 times more often in children with URI by PCR than CM, p = 0.01. Real-time PCR was also more sensitive in detecting multiple strains rather than CM in both healthy (p = 0.001) and children with URI (p ≤ 0.001). Overall real-time PCR proved superior to CM in detection and serotyping of Streptococcus pneumoniae. Future studies should incorporate real-time PCR technology along with CM to fully understand the epidemiology of colonization in health and illness.     

On-Line Automated Border Detection for Echocardiographic Quantification of Right Ventricular Size and Function in Children

Rapid, accurate assessment of right ventricular (RV) size is important for the management of children with congenital heart disease. The usefulness of the Acoustic Quantification system of automated border detection (ABD) and on-line quantification (AQ) for assessment of RV size was tested in 36 children. AQ data were compared to ``corrected AQ' measurements (after correction for cavity areas erroneously included in the region of interest) required for AQ. Furthermore, the influence of necessary changes to gain settings was tested in ``lateral gain control' (LGC) images obtained by removal of ABD overlays. All results were compared to conventional echocardiography (echo), and agreement with magnetic resonance imaging (MRI) RV areas was assessed. Systematic differences (±) limits of agreement with MRI (transverse plane) for conventional echo and AQ (apical four-chamber view) were as follows: end-diastolic −0.8 ± 3.8 (conventional echo) versus −1.7 ± 4.6 (AQ) cm²/m² (p < 0.001); end-systolic −1.3 ± 3.2 versus −4.9 ± 5.8 (AQ) cm²/m² (p < 0.001); fractional area change 7.8 ± 17.0% versus 26.9 ± 31.4% (AQ) (p < 0.001). Differences between conventional echo, LGC, and corrected AQ areas were not statistically significant. The best agreement between MRI and echocardiography was with conventional echo. We conclude that automated border detection of the RV can be performed successfully with the AQ system at a fixed point in the cardiac cycle. For adequate assessment of RV function manual corrections of online AQ results are still required, which results in an important reduction of the time gain of on-line quantification.     

Evaluation of tumour necrosis during chemotherapy with diffusion-weighted MR imaging: preliminary results in osteosarcomas

Background During successful chemotherapy of osteosarcomas tumour size does not diminish significantly because the therapy has limited impact on the mineralized matrix of the tumour. Treatment response is considered successful if, histologically, more than 90% of tumour cells show necrosis. Objective To determine if osteosarcomas change their water diffusion during preoperative chemotherapy in relation to the amount of tumour necrosis. Materials and Methods Eight patients (age 11–19 years) with histologically proven limb osteosarcoma underwent T1-weighted, fat-suppressed T2-weighted and contrast-enhanced T1-weighted spin-echo imaging together with diffusion-weighted EPI sequences (b = 700) at 1.5 T before and after five cycles of standard chemotherapy. Tumour volume and apparent diffusion coefficient (ADC) maps were calculated before and after chemotherapy. The degree of tumour necrosis after chemotherapy was assessed using the histological Salzer-Kuntschik classification (grades 1–6). Results During chemotherapy, the ADC values of osteosarcomas changed significantly. The ADC of untreated tumour was 2.1 ± 0.4 × 10⁻³ mm²/s (mean ± SD) (95% CI 1.6–2.0). The ADC of chemotherapy-treated sarcomas was 2.5 ± 0.4 × 10⁻³ mm²/s (95% CI 1.8–2.2). Necrotic areas, which were confirmed by macroscopic examination, showed ADC values up to 2.7 × 10⁻³ mm²/s. Four patients with little viable tumour tissue within the neoplasm (Salzer-Kuntschik grades 1–2) had an increase in ADC of 0.4 up to 0.7 × 10⁻³ mm²/s. Four patients with larger areas of viable tumour (Salzer-Kuntschik grade 4) showed a lesser increase in ADC of 0.0 up to 0.3 × 10⁻³ mm²/s. The differences in ADC values in tumour tissue before and after chemotherapy were highly significant (P = 0.01). Conclusion During chemotherapy of osteosarcomas, tumour ADC changes are related to the degree of tumour necrosis. Supported by Grant DFG# u103     

手术治疗腺样体肥大儿童阻塞性睡眠呼吸暂停综合征

目的　探讨腺样体切除术、腺样体扁桃体切除术对腺样体肥大儿童阻塞性睡眠呼吸暂停综合征(OSAS)的治疗作用。方法　对 2 0例腺样体肥大合并OSAS(OSAS组 )儿童手术前后的临床表现、多导睡眠图(PSG)检查结果进行前瞻性比较研究 ,并与同期住院的 1 0例单纯性腺样体肥大儿童 (对照组 )进行对照研究。结果　OSAS组的常见症状发生率与对照组差异无显著性 (P >0 .0 5 ) ;两组体块指数分别为 1 5 .4± 2 .5kg/m2 和1 7.6± 3.1kg/m2 ,差异无显著性 (P >0 .0 5 )。OSAS组与对照组的鼻咽侧位片A/n值、总睡眠时间、睡眠效率及S1、S2、慢波睡眠 (SWS)、快速眼动睡眠期 (REM)所占比例差异均无显著性 (P >0 .0 5 )。OSAS患儿术后呼吸暂停指数 (AI)、呼吸暂停低通气指数 (AHI)、阻塞性呼吸暂停指数 (OAI)较术前降低 (P <0 .0 5或 0 .0 1 ) ,REM所占比例较术前增高 (P <0 .0 5 )。结论　腺样体肥大合并OSAS的临床表现、鼻咽侧位片A/n值、睡眠结构与单纯腺样体肥大患儿无差别 ;腺样体肥大合并OSAS儿童手术治疗效果良好。     

Evaluation of lean body mass in obese children

Multiple skinfold anthropometry (MSA) and bioelectrical impedance analysis (BIA) are useful as clinically non-invasive, inexpensive and portable techniques, although it is not clear if they can be used interchangeably in the same patient to routinely assess her/his body composition. In order to compare BIA, MSA and DXA in the estimation of lean body mass (LBM) of a pediatric obese population, 103 obese [body mass index (BMI) > 97th percentile] children (median age: 11 years; range: 5.4–16.7 years) underwent nutritional evaluation. After an overnight fast, the subjects’ anthropometric measurements were performed by the same investigator: body weight (BW), height, skinfold thickness (four sites); fat body mass (FBM) using Brook or Durnin equations and dual X-ray absorptiometry (DXA). BIA was performed using a bioelectrical impedance analyzer (Analicor-Eugedia, 50 kHz) and Houtkooper’s equation to calculate LBM. Linear regression analysis was performed to evaluate the relationship between the prediction of LBM by MSA, DXA and BIA. The differences between the three techniques were analysed using Student’s t-test for paired observations and the Bland and Altmann method. A considerable lack of agreement was observed between DXA- and BIA-LBM (δ = −4.37 kg LBM; δ−2σ = −11.6 kg LBM; δ+2σ = +2.8 kg LBM); between DXA- and MSA-LBM (δ = −1.72 kg LBM; δ−2σ = −8.2 kg LBM; δ+2σ = +4.8 kg LBM) and between BIA- and MSA-LBM (δ = −2.65 kg LBM; δ−2σ = −10.5 kg LBM; δ+2σ = +5.2 kg LBM). Conclusion: In obese children, DXA, BIA and MSA should not be used interchangeably in the assessment of LBM because of an unacceptable lack of agreement between them. The discrepancies between methods increase with the degree of obesity.     

Left ventricle output and mean arterial blood pressure in preterm infants during the 1st day of life

The objective was to assess the contribution of left ventricular output (LVO) in determining low mean arterial blood pressure (MABP) in preterm infants admitted to the neonatal intensive care unit. Doppler echocardiography was prospectively performed on a cohort of 17 consecutive infants with low MABP (<30 mmHg) and on 17 consecutive control subjects (range: 600–1520 g; 27–30.7 weeks gestation). The median haematocrit was 42.5% in the low MABP group versus 49.4% in the control group (P < 0.01). The index of resistance to the LVO (RILV = MABP:LVO ratio) was lower in the low MABP group (98 vs 156 mmHg · l⁻¹ · kg⁻¹ · min⁻¹; P < 0.05). An analysis of the low MABP group regarding LVO revealed that a subgroup of four infants had LVO <10th percentile (185 ml · kg⁻¹ · min⁻¹) with a high RILV (>90th percentile: 226 mmHg · l⁻¹ · kg⁻¹ ·  min⁻¹) for three of the infants. The remaining 13 infants had LVO >10th percentile and a shortening fraction >25th percentile. In this subgroup, a high proportion of infants (9/13 vs 2/17, P < 0.01) had low RILV (<10th percentile: 96 mmHg · l⁻¹ · kg⁻¹ · min⁻¹) and the incidence of haemodynamically significant patent ductus arteriosus was higher than in the control group (10/13 vs 4/17, P < 0.01). Conclusion Left ventricular output, index of resistance to left ventricular output and patent ductus arteriosus status are important to consider in evaluating mean arterial blood pressure during early postnatal life in preterm infants. Low mean arterial blood pressure is frequently associated with normal or high left ventricular output, low index of resistance to left ventricular output and significant patent ductus arteriosus. Received: 10 November 1998 and in revised form: 8 February 1999 / Accepted: 9 February 1999     

Serum levels of phospholipid fatty acids in mothers and their babies in relation to allergic disease

The fatty acid composition of serum phospholipids was analysed by gas chromatography in 26 non-allergic and 32 allergic mothers at the time of delivery. In 47 of them the levels were compared with those in the cord blood of their babies. The children were then followed for 6 years with regard to the development of allergic disease. There was an inverse relationship between the levels of linoleic acid (LA, C18:2n-6) and its metabolic products arachidonic acid (AA, C20:4n-6) (r = −0.63, P < 0.001), and C22:4 (r = −0.50, P < 0.01) in the non-allergic, but not in allergic mothers (r = 0.25 and r = −0.39, respectively). Comparing the fatty acid levels in maternal and umbilical cord serum, a significant correlation was observed between the LA levels in serum of non-allergic mothers and their babies (r = 0.53, P < 0.05). Furthermore, the maternal dihomo-γ-linolenic acid (DHGLA, C20:3n-6) levels correlated with the cord serum levels of AA (r = 0.65, P < 0.01) and C22:4 (r = 0.65, P < 0.01) and with docosahexaenoic acid (DHA, C22:6n-3, r = 0.65, P < 0.01). None of these relationships were seen when comparing the fatty acid levels in the allergic mothers and their babies. In the mothers of children who did not develop any allergic manifestations during the first 6 years of life, the AA levels correlated with C22:4 (r = 0.53, P < 0.001) and eicosapentaenoic acid (EPA, C20:5n-3) (r = 0.56, P < 0.001). Similar findings were recorded within the n-3 series of fatty acids, i.e. the levels of docosapentaenoic acid (DPA, C22:5n-3) correlated with DHA (r = 0.61, P < 0.001). None of these correlations were significant in the 20 mothers whose babies developed allergic disease (r = 0.42, 0.28 and 0.44 respectively). Taken together, the findings indicate that there is an abnormal metabolism relationship between some of the long-chain polyunsaturated fatty acids in allergic mothers, affecting their infants. Furthermore, the findings suggest an association between the fatty acid composition in maternal serum and the appearance of allergic disease in their children during the first 6 years of life. Conclusion The proportions of various long-chain polyunsaturated fatty acids were altered in the serum phospholipids of allergic pregnant mothers and in mothers whose babies developed allergic disease over the first 6 years of life, indicating that atopy is associated with a disturbed fatty acid metabolism. Received: 6 February 1996 and in revised form: 5 August 1997 / Accepted: 5 September 1997     

Effects of a low-calorie diet on resting metabolic rate and serum tri-iodothyronine levels in obese children

The purpose of the study was to examine the effects of weight loss on resting metabolic rate (RMR) and on serum T3 levels in obese children and to investigate whether RMR changes are related to T3 changes. Sixty-four healthy, overweight, children (age: 12.1 ± 1.1 years, body mass index 29.3 ± 4.3 kg/m²) were studied during a 6-week weight reduction programme. RMR (by indirect calorimetry) total T3, total T4, TSH and fat-free mass (FFM) (by anthropometry) were measured at baseline and after 6 weeks of dietary treatment. Weight loss resulted in a 10.1% decline in RMR (P < 0.01) and a 23.4% decrease in serum T3 levels (P < 0.001). RMR was correlated with FFM before (r = 0.78, P < 0.001) and after weight loss (r = 0.76, P < 0.001). The changes in RMR were positively correlated with the changes in FFM (r = 0.48, P < 0.05) but also with the changes in serum T3 levels (r = 0.47, P < 0.05). The initial T3 levels predicted the subsequent fall in T3 that occurred after 6 weeks of dietary treatment (r = −0.60, P < 0.001). Conclusions A significant decrease in serum T3 concentrations and resting metabolic rate occurred as a result of a 6-week weight reduction programme in an obese child population. The decline in T3 levels combined with fat-free mass loss could be responsible for the reduction in resting metabolic rate. Received: 30 June 1998 / Accepted in revised form: 22 October 1998     

The role of primary myogenic regulatory factors in the developing diaphragmatic muscle in the nitrofen-induced diaphragmatic hernia

Purpose  

The nitrofen model of congenital diaphragmatic hernia (CDH) is widely used to investigate the pathogenesis of CDH. However, the exact pathomechanism of the diaphragmatic defect is still unclear. Diaphragmatic muscularization represents the last stage of diaphragmatic development. Myogenic differentiation 1 (MyoD) and myogenic factor 5 (Myf5) play a crucial role in muscularization. MyoD^−/− : Myf5^+/− mutant mice show reduced diaphragmatic size, whereas MyoD^+/− : Myf5^−/− mutants have normal diaphragms. We designed this study to investigate diaphragmatic gene expression of MyoD and Myf5 in the nitrofen CDH model.     

Mannose-binding lectin polymorphisms and recurrent respiratory tract infection in Chinese children

In order to establish the reference value of mannose-binding lectin (MBL) serum level in children and to investigate the correlation between the polymorphisms of MBL2 gene and serum MBL level in healthy Chinese of Han ethnic group and in children of Chinese Han ethnic group with recurrent respiratory tract infections (RRTI), the concentration of oligomerized MBL was measured by enzyme-linked immunosorbent assay, and MBL2 gene polymorphisms were analyzed by restriction fragment length polymorphism of polymerase chain reaction and polymerase chain reaction-sequence specific primer. The median MBL levels in the 470 normal children were 2536 ng/ml, and the P_2.5–P_97.5 was 161–5,070 ng/ml. Our research showed that two promoter polymorphisms at −550, −221 of start codon and coding variants at codon 54 of MBL2 gene affected the protein level significantly and the most frequent genotype in Hans is HYPA/HYPA. Our results also showed that serum MBL level was significantly lower in recurrent respiratory tract infections patients compared with healthy controls (Z, −3.04, P = 0.002). The frequency of the promoter LXP haplotype and the B allele was significantly higher in RRTI patients than in controls (χ ² 4.05, P < 0.05; OR 1.63, 95%CI 1.01∼2.62; χ ² 4.27, P < 0.05; OR 1.94, 95%CI 1.02∼3.68). Conclusion: We have established that the reference value of serum MBL level in Chinese aged between 0 and 6 years (161–5,070 ng/ml), and we found that LXP and the B are risk factors for RRTI.     

Plasma water as a diagnostic tool in the assessment of dehydration in children with acute gastroenteritis

Acute gastroenteritis is common in childhood. The estimation of the degree of dehydration is essential for management of acute gastroenteritis. Plasma water was assessed as a diagnostic tool in children with acute gastroenteritis and dehydration admitted to hospital. In a prospective cohort study, 101 patients presenting at the emergency department with dehydration were included. Clinical assessment, routine laboratory tests, and plasma water measurement were performed. Plasma water was measured as a percentage of water content using dry weight method. During admission, patients were rehydrated in 12 h. Weight gain at the end of the rehydration period and 2 weeks thereafter was used to determine the percentage of weight loss as a gold standard for the severity of dehydration. Clinical assessment of dehydration was not significantly associated with the percentage of weight loss. Blood urea nitrogen (r = 0.3, p = 0.03), base excess (r =−0.31, p = 0.03), and serum bicarbonate (r = 0.32, p = 0.02) were significantly correlated with the percentage of weight loss. Plasma water did not correlate with the percentage of weight loss. On the basis of the presented data, plasma water should not be used as a diagnostic tool in the assessment of dehydration in children with acute gastroenteritis.     

Association between toll-like receptor 10 (TLR10) gene polymorphisms and childhood IgA nephropathy

Toll-like receptors (TLRs) play an important role in the induction and regulation of the innate immune system and adaptive immune responses. TLR10 gene polymorphisms have been reported to be associated with a range of immune-related diseases. In this study, we investigated the association of TLR10 gene polymorphisms with immunoglobulin A nephropathy (IgAN) in Korean children. To examine the association, we genotyped one promoter single nucleotide polymorphisms (SNP) [rs10004195 (−113T/A)] and three missense SNPs [rs11096957 (Asn241His), rs11096955 (Ile369Leu), and rs4129009 (Ile775Val)] using direct sequencing in 199 IgAN patients and 289 control subjects. Our case–control analysis showed that rs10004195 was associated with IgAN (codominant model, p = 0.016 in TT vs. TA; p = 0.044 in TT vs. AA; dominant model, p = 0.0068). In addition, when comparing the proteinuria level of IgAN patients according to the genotypes of each SNP, we found that in dominant model of rs1004195, the level of proteinuria of patients with TA or AA genotypes (median, 4.01 mg/m²/h) was higher than that of patients with TT genotype (2.00 mg/m²/h, p = 0.033). In conclusion, these results suggest that TLR10 gene may be associated with susceptibility to IgAN in Korean children.     

Effect of central precocious puberty and gonadotropin-releasing hormone analogue treatment on peak bone mass and final height in females

To evaluate the effect of central precocious puberty (CPP) and its treatment with gonadotropin-releasing hormone (GnRH) analogues on final height and peak bone mass (PBM), we measured lumbar bone mineral density (BMD) in 23 girls at final height. Patients were distributed in two groups. Group 1: 14 patients with progressive CPP were treated with GnRH analogues; seven patients received buserelin (1600 μg/daily), subsequently switched to depot triptorelin (60 μg/kg/26–28 days); seven patients were treated with depot triptorelin (60 μg/kg/26–28 days); mean age of treatment was 6.2 years (range 2.7–7.8 years); the treatment was discontinued at the mean age of 10.1 years (range 8.7–11.3 years); final height was reached at the mean age 13.4 years (range 12.0–14.9 years). Group 2: 9 patients (mean age 6.5 years, range 4.8–7.7 years) with a slowly progressing variant of CPP were followed without treatment; final height was reached at the mean␣age␣13.6 years (range 12.5–14.8 years). Lumbar BMD (L₂-L₄ by dual energy X-ray␣absorptiometry) was measured in all patients at final height. In group 1, final height␣(158.9 ± 5.4 cm) was significantly greater than the pre-treatment predicted height (153.5 ± 7.2 cm, P < 0.001), but significantly lower than mid-parental height (163.2 ± 6.2 cm, P < 0.005). Subdividing the girls of group 1 according to the bone age at discontinuation of therapy (i.e. ≤11.5 years, n = 5, or ≥12.0 years, n = 9), the former patients had a final height significantly higher than the latter (163.7 ± 3.9 cm vs 156.5 ± 4.6 cm, P < 0.02). In group 2, final height (161.8 ± 4.6 cm) was similar to the pre-treatment predicted height (163.1 ± 6.2 cm, P = NS) and was not significantly different from mid-parental height (161.0 ± 5.9 cm). BMD values (group 1: 1.11 ± 0.14 g/cm², group 2: 1.22 ± 0.08 g/cm²) were not significantly different from those of a control group (1.18 ± 0.10 g/cm²; n = 20, age 16.3–20.5 years) and the patients' mothers (group 1: 1.16 ± 0.07 g/cm², n = 11, age 32.9–45.1 years; group 2: 1.20 ± 0.08 g/cm², n = 7, age 33.5–46.5 years). In group 1, the girls who stopped therapy at a bone age ≤11.5 years had significantly higher BMD (1.22 ± 0.10 g/cm²) compared to those who discontinued therapy at a bone age ≥12.0 years (1.04 ± 0.12 g/cm², P < 0.05). Conclusion In girls with progressive CPP, long-term treatment with GnRH analogues improves final height. A subset of patients with CPP does not require treatment because good statural outcome (slowly progressing variant). In CPP, the abnormal onset of puberty and the long-term GnRH analogue treatment do not impair the achievement of PBM. In GnRH treated patients, the discontinuation of therapy at an appropriate bone age for pubertal onset may improve both final height and PBM. Received: 5 June 1997 / Accepted in revised form 21 November 1997     

Growth in X-linked hypophosphatemic rickets

Growth failure appears frequently in children with X-linked hypophosphatemic rickets (XLHR) due to hypophosphatemia, disease severity, body disproportion, and primary bone abnormality. Recombinant human growth hormone (rhGH) increases phosphate tubular reabsorption and phosphate level in blood and, thus, constitutes an attractive but controversial therapy in short children with XLHR, those efficacy was demonstrated in small uncontrolled series. Our aim was to report our experience regarding growth in XLHR. Twenty-seven children with XLHR—20 girls, seven boys—diagnosed at a median (md) of 1.46 years of age, (range 0.39–8.5 years), were studied at 10.12 years of age (1.58–18.56), md (range). All received oral treatment with phosphate and calcitriol. At the first visit, grouped Z-height was −1; (−4.58; 0.54) md (range). After 5 years’ follow-up (0.92–15.6), Z-height was −0.91 (− 4.56; 0.17), not different from that at baseline (P = 0.465). In 16 children entirely controlled in our program upon presentation, a “catch up” phenomenon after the rickets had healed (P = 0.823) or throughout the long-term was not observed (P = 0.995). Eight patients had a Z-height ≤ −2SD at the last visit, and impaired linear growth was associated with age >2 years at diagnosis, male gender and non-adherence to treatment. Four children, all boys, received rhGH, and in two cases with sufficient follow up stature normalized. No rhGH side effects were observed, and phosphate and calcitriol doses remained stable. Linear growth failure appeared in a third of XLHR children. Efforts need to be made to reduce the age of diagnosis and to improve adherence to treatment. Treatment with rhGH should be considered early, after the rickets has been controlled, in those patients with impaired growth or delayed diagnosis.     

Radiation doses to children during modified barium swallow studies

Background There are minimal data on radiation doses to infants and children undergoing a modified barium swallow (MBS) study. Objective To document screening times, dose area product (DAP) and effective doses to children undergoing MBS and to determine factors associated with increased screening times and effective dose. Materials and methods Fluoroscopic data (screening time, DAP, kVp) for 90 consecutive MBS studies using pulse fluoroscopy were prospectively recorded; effective dose was calculated and data were analyzed for effects of behavior, number of swallow presentations, swallowing dysfunction and medical problems. Results Mean effective dose for the entire group was 0.0826 ± 0.0544 mSv, screening time 2.48 ± 0.81 min, and DAP 28.79 ± 41.72 cGy cm². Significant differences were found across three age groups (≤1.0, >1.0–3.0 and >3.0 years) for effective dose (mean 0.1188, 0.0651 and 0.0529 mSv, respectively; P  <  0.001), but not for screening time or DAP. Effective dose was correlated with screening time (P = 0.007), DAP (P < 0.001), number of swallow presentations (P = 0.007), lower age (P = 0.017), female gender (P = 0.004), and height (P < 0.001). Screening time was correlated with total number of swallow presentations (P < 0.001) and DAP (P < 0.001). Conclusion Screening times, DAP, effective dose, and child and procedural factors associated with higher effective doses are presented for children undergoing MBS studies. This work was supported by the Royal Children’s Hospital Foundation, Brisbane.