Health‐related quality of life and sleep among Chinese children after living donor liver transplantation

LDLT is a well‐established treatment for most terminal liver diseases in children. Survival rates have improved, yet few studies have considered HRQoL or sleep problems in LDLT recipients. In this cross‐sectional study, we enrolled 51 children who had undergone LDLT in Renji Hospital. PedsQL^? 4.0 Generic Core Scales, PedsQL^? 3.0 Transplant Module, and Pediatric Sleep Questionnaire were used to assess outcomes. Of all participants, 11.8% (6/51) reported low total HRQoL scores. Participants’ scores on most HRQoL subscales were comparable to the scores of healthy children. However, compared with solid organ transplant recipients, LDLT recipients scored significantly lower in About My Medicines II (t = 3.092, p = 0.002) and Worry (t = 2.760, p = 0.006). Sleep problems (41.2%) were common among participants. Hierarchical regression analyses showed that SRBD accounted for significant variance in HRQoL on total generic HRQoL (R² = 0.446, p < 0.001), psychosocial health (R² = 0.372, p = 0.001), physical health (R² = 0.345, p = 0.003), total transplant‐specific HRQoL (R² = 0.514, p < 0.001), About My Medicines I (R² = 0.365, p = 0.013), My Transplant and Others (R² = 0.334, p = 0.005), Pain and Hurt (R² = 0.544, p < 0.001), Worry (R² = 0.401, p = 0.001), Treatment Anxiety (R² = 0.526, p < 0.001), How I Look (R² = 0.221, p = 0.040), and Communication (R² = 0.343, p = 0.012). In conclusion, sleep problems are non‐negligible in children after LDLT and predicted significant variance on HRQoL.     

Relation of Left Ventricular Hypertrophy to Microalbuminuria and C-Reactive Protein in Children and Adolescents with Essential Hypertension

Microalbuminuria (MA) and C-reactive protein (CRP) levels are predictors of increased risk for left ventricular hypertrophy (LVH). Whether the strength of association between CRP and LVH is comparable to that of MA in hypertensive children is unknown. CRP and MA were measured in 64 children and adolescents with essential hypertension (HTN). In the entire population, CRP and MA showed positive relations with body mass index (BMI) (r = 0.30, p = 0.04 and r = 0.32, p = 0.04, respectively), systolic blood pressure (SBP) (r = 0.63, p = 0.03 and r = 0.58, p = 0.03, respectively), and LVH (r = 0.86, p < 0.001 and r = 0.81, p < 0.001, respectively). Patients with LVH (n = 23) had significantly higher BMI (p = 0.32), increased SBP (p = 0.031), and higher levels of CRP (p < 0.001) and MA (p < 0.001) compared with those without LVH. Multiple linear regression analysis demonstrated that CRP (r = 2.11, p < 0.001), MA (r = 1.94, p < 0.003), BMI (r = 0.53, p = 0.02), and SBP (r = 0.48, p = 0.04) were significantly associated with LVH. By analysis of covariance, CRP and MA were significantly different between patients who had LVH and those without LVH after adjustment for age, gender, BMI, SBP, SBP index, and diastolic blood pressure (p < 0.001 for the two markers). In conclusion, the strength of association between LVH and CRP is comparable to that of MA in children and adolescents with essential HTN.     

Characteristics of Streptococcus pyogenes strains isolated from Chinese children with scarlet fever

Aim: To analyse the characteristics of Streptococcus pyogenes isolates from Chinese children with scarlet fever. Methods: Minimal inhibitory concentration with nine antibiotics was performed on 145 Streptococcus pyogenes isolates acquired from Beijing and Shanghai in 2007. Their macrolide‐resistant genes (mefA, ermB and ermA– a subclass of ermTR), superantigens (speA and speC), and en‐coding mature M protein gene (emm gene) were amplified by PCR. Results: A total of 97.9% of the isolates exhibited resistance to the macrolides, while 96.6% manifested resistance to tetracycline. All isolates were sensitive to chloramphenicol, penicillin, cefradine, and ofloxacin. Moreover, 94.5% exhibited a cMLSB phenotype, while 90.3% had the ermB gene. Five emm types (emm1.0, emm4.0, emm12.0, emm22.0 and st5240) were discovered, of which 9.7% carried the superantigen speA, 35.9% carried the speC, 42.8% carried both speA and speC and 11.7% carried neither speA nor speC. Finally, 85.5% of emm1.0 and 15.5% of emm12.0 isolates carried speA, while 79.0% of emm1.0 and 75.9% of emm12.0 isolates carried speC. Conclusion: The Streptococcus pyogenes isolates had high resistance rates against macrolides and tetracycline. They mainly expressed the ermB gene type and cMLSB phenotype. Their common emm types are emm1.0 and emm12.0, which have different frequencies of speA and speC.     

Functioning of Family System in Pediatric Oncology During Treatment Phase

The study focuses on parents’ psychological implications caused by the treatment of their children suffering from tumor. It investigates some specific mothers’ resource factors such as their strategies of coping and the perception of their own family functioning in terms of cohesion and adaptability.

The study was performed with 34 mothers of children suffering from acute lymphoblastic leukemia (ALL), during the treatment phase. The used tools were the Coping Orientation to Problem Experienced—New Italian Version, to investigate coping strategies, and the Family Adaptability and Cohesion Evaluation Scale-III, to analyze both real and ideal perception of family functioning.

The data related to coping, show how the involved mothers tend to mainly use the strategies of positive aptitude, orientation toward problem and social support (F = 99.88, df = 4, P < .01). The family functioning, in terms of adaptability, is described as chaotic relating to both the real (χ² = 13.29, df = 3, P = .004) and ideal (χ² = 11.52, df = 2, P = .003) family, whereas in terms of cohesion, it is perceived as chiefly disengaged in the real family (χ² = 12.3, df = 3, P = .006) and as enmeshed in the ideal one (χ² = 12.58, df = 3, P = .006).

Statistically positive correlations were only detected between adaptability and avoidance (r = 0.49, P < .01); adaptability and orientation toward problem (r = 0.36, P < .05); and adaptability and transcendent orientation (r = ?0.04, P < .05).

Despite the critical situation, the mothers have shown optimistic view, care for problem management and capability to ask for help. These coping strategies allow the therapeutic alliance between families and health care workers, so useful for the quality of childcare.     

Serodiagnosis of acute respiratory infections in children in Georgia

Objective To investigate the etiology of acute respiratory infections in hospitalized children. Methods A total of 808 children were studied. Investigation ofRSV, Adenovirus, Parainfluenza, Influenza A and B, C.trachomatis, C.pneumoniae, M. pneumonia andLegionella were performed with an ELISA for IgM, IgG and IgA antibodies detection. Results There were 496 males and 312 females giving a male: female ratio of 1.6∶1. Ages range from 1 month to 15 years. The overall detection rate was 57.9%. The most frequently detected were:parainfluenza 12.6%,adenovirus 11.2%,influenza A 7.3%,RSV 6%,M. pneumoniae 5.4%,C.trachomatis 3.5% and mixed-infections 9.2%. Pneumonia was associated most frequently withadenovirus and mixed-infections; wheezing bronchitis—withadenovirus, RSV andM. pneumoniae; bronchitis —withparainfluenza andadenovirus, diseases of upper respiratory tract—withparainfluenza andadenovirus. Peak of the virus activity was during winter (influenza, parainfluenza, adenovirus, RSV) and autumn (parainfluenza, RSV). Conclusion Viruses are the main causes of ARI in Georgian children. A better understanding of the etiology of ARI in all of the regions of the world may be helpful for timely decision of specific therapy, which can help pediatricians to estimate and manage children with ARI.     

Normalization of Maximal Cardiovascular Variables for Body Size in Premenarcheal Girls

Previous studies have indicated the importance of allometric scaling of VO_2max for body size. However, no information is available on adjusting maximal cardiac output (Q _max) and stroke volume (SV_max) for body dimensions. The allometric exponent b was determined for the equation Y=aX ^b (where Y is the physiological outcome and X is the anthropometric variable) for VO_2max, Q _max, and SV_max relative to mass, height, and body surface area (BSA) in 24 premenarcheal girls (mean age 12.2 years) during cycle testing. Values for b were 1.08 and 1.05 for BSA relative to Q _max and SV_max, approximating that of 1.0 using the traditional ratio standard (cardiac index and stroke index). Exponents of body mass relative to VO_2max, Q _max, and SV_max (0.55, 0.55, and 0.59, respectively) eliminated the effects of body size, but the ratio standard (M ^1.0) did not. In this group of subjects, use of the ratio standard BSA was an appropriate means of adjusting maximal values of Q and SV for body size.     

农村儿童事故倾性的现况及影响因素的研究

目的 研究农村儿童事故倾性发生的现况,并探讨其影响因素,为农村儿童事故倾性的预防及干预提供科学依据.方法 按随机整群抽样的方法,采用自制的一般情况调查表和伤害调查表对湖南省某农村1 560名儿童进行问卷调查.采用非条件多因素logistic回归分析探讨事故倾性发生的主要危险因素.结果 共筛查出事故倾性儿童147例,事故倾性的发生率为9.42%.单因素分析结果显示儿童性别(PPPPPPPPPOR=3.683,95%CI:1.028~4.283)、下等(OR=2.099,95%CI:1.587~8.546)、经济状况贫困(OR=2.353,95%CI:1.222~4.533)、教育方式放任或漠不关心(OR=1.914,95%CI:1.029~3.559)和变化无常(OR=4.153,95%CI:1.847~9.338)是儿童事故倾性的危险因素,女孩(OR=0.539,95%CI:0.369~0.788)是儿童事故倾性的保护因素.结论 学习成绩差、家庭经济贫困、教育方式放任或漠不关心和变化无常会增加农村儿童事故倾性的发生;相对于男孩,女孩事故倾性的发生较低.     

Adaptive functioning and its correlates after intestine and liver transplantation

In this cross‐sectional study, we compared levels of adaptive functioning and examined potential correlates of adaptive functioning in 18 pediatric intestine (ITX) and 22 liver (LTX) recipients transplanted between June 2003 and March 2009. Family caregivers completed the ABAS‐II scale and provided socio‐demographic information regarding recipients' age at transplantation, gender, ethnicity, time since transplantation, and caregivers' role, ethnicity, education, and family income. Overall adaptive functioning and all three adaptive functioning subdomain scores were significantly lower in ITX patients compared with LTX patients (p ≤ 0.04) and compared with the general population normative mean (p ≤ 0.003). Significant correlates of adaptive functioning after abdominal transplant included type of transplant procedure (r = ?0.4, p = 0.02), gender (r = 0.4, p = 0.01), and educational level of caregiver (r = 0.5, p = 0.003) and together explained 45% of the variance in overall adaptive functioning. Findings provide new information regarding everyday functioning outcomes of ITX patients, add to existing data regarding non‐medical outcomes for LTX patients, and highlight the need for ongoing monitoring and intervention following transplantation to enhance outcomes.     

Correlation between deletion patterns of SMN and NAIP genes and the clinical features of spinal muscular atrophy in Japanese patients

We conducted molecular analysis of two candidate genes for spinal muscular atrophy (SMA), the survival motor neuron gene (SMN) and the neuronal apoptosis inhibitory protein gene (NAIP), in 16 Japanese patients with SMA and compared the phenotypic features of SMA in these patients with the corresponding genotypes. Exons 7 and/or 8 of SMN were homozygously deleted in 11 SMA type I (Werdnig-Hoffmann disease) patients, two SMA type II patients and one SMA type III patient. Exons 5 and 6 of NAIP were homozygously deleted in six SMA type I patients. No patient had a deletion in NAIP without a deletion in SMN. Mechanical ventilation was required during the first 7 months of life in the SMA type I patients who had a deletion in both SMN and NAIP. Ventilatory support was initiated within 2 years after birth in patients who had a deletion in SMN but not in NAIP. We detected homozygous deletion of exon 5 of NAIP in the unaffected mothers of two SMA type I patients. In these families, the patients exhibited a deletion in both SMN and NAIP. The parents and unaffected siblings of these patients did not have a deletion in SMN. The present findings support the hypothesis that SMN deletion plays an important role in the development of SMA and suggest that combined deletion of both SMN and NAIP may be relevant for determining the disease severity.     

Clinical and biochemical manifestations of syndrome X in obese children

The aim of this study was to investigate whether the clinical and metabolic characteristics of syndrome X had their onset in childhood in otherwise healthy but obese children of Greek origin. A group of 25 obese children and 18 age- and sex matched control subjects, aged 6–14 years, underwent an oral glucose tolerance test (OGTT), assessed for determination of plasma glucose and insulin levels. Insulin sensitivity and insulin resistance were estimated by mathematical models using calculations obtained during the OGTT. Body mass index (BMI) and blood pressure were measured, as well as serum lipoprotein and aminotransferase concentrations, after an overnight fast. The obese children had significantly higher blood pressure (systolic and diastolic) (P<0.001), triglycerides, lipoprotein(a) and alanine aminotransferase levels (P<0.05) and significantly lower HDL-cholesterol and apolipoprotein A-1 values (P<0.001). Plasma glucose levels during the OGTT were similar in both obese children and control subjects, while plasma insulin levels were significantly higher in obese children (P<0.01). In mathematical models, mean values of insulin sensitivity predictors: metabolic clearance rate and insulin sensitivity index were significantly lower in obese children (P<0.001). Predictors of beta-cell function: insulin resistance index and insulin release index were significantly higher in obese children (P<0.001). Conclusion:childhood adiposity was associated with all traditional components of syndrome X. The early recognition of these factors as predisposing elements of the appearance of metabolic syndrome requires the development of strategies to manage excess weight gain during childhood, with the ultimate goal being the prevention of type 2 diabetes and cardiovascular disease in adulthood.Abbreviations ApoA-1 apolipoprotein A-1 - ApoB apolipoprotein B - ALT alanine aminotransferase - AST aspartate aminotransferase - BMI body mass index - HDL-C HDL-cholesterol - HOMA-IR insulin resistance index - HOMA-Secr insulin release index - ISI insulin sensitivity index - LDL-C LDL-cholesterol - Lp(a) lipoprotein (a) - MCR metabolic clearance rate of glucose - OGTT oral glucose tolerance test - TC total cholesterol - TG triglycerides     

cagA,vacA, and iceA genotypes of Helicobacter pylori in Korean children

Background: Several putative virulence factors for Helicobacter pylori have been identified including cagA, vacA, and iceA. The aims of the present study were to study the distribution of cagA, vacA, and iceA genotypes in children with H. pylori gastritis and to examine the association of genotypes with severity of gastritis. Methods: H. pylori DNA was extracted from antral biopsy specimens from 33 children with H. pylori gastritis. Specific polymerase chain reaction assays were used for three genes: cagA, vacA, and iceA. The features of gastritis were graded in accordance with the updated Sydney System. Results: Of the 33 children, 31 (94%) were cagA positive. Twenty‐four (72%) had s1c genotype and nine (27%) had s1a. The m1 genotype was seen in 27 (82%) and m2 was found in five (15%). The iceA1 genotype was detected in 25 (76%). Scores of neutrophil activity, chronic inflammation, and H. pylori density were independent of cagA, vacA and iceA status. Conclusion: The cagA‐positive vacA s1c/m1 iceA1 genotype was predominant in Korean children with recurrent abdominal pain and H. pylori gastritis. The cagA, vacA and iceA genotype were not associated with the severity of gastritis.     

Thrombopoietin Measurement as a Key Component in the Evaluation of Pediatric Thrombocytosis

JAK2, MPL, and CALR mutations, which underlie essential thrombocythemia (ET) in most adults, are infrequent in children. Consequently, additional tests are needed to confirm pediatric ET diagnoses. We report a child with suspected ET and normal JAK2, MPL, and CALR analyses. Serum thrombopoietin (TPO) was markedly elevated, leading to analysis of the TPO gene, TPHO, which contains an upstream open reading frame (uORF) known to repress THPO translation. Sequencing revealed a de novo, germline stopgain mutation in the uORF, explaining the elevated TPO and thrombocytosis. This finding suggests that screening TPO levels and, if elevated, THPO 5′ UTR sequencing could be diagnostic.     

Age Dependency of Stiffness of the Abdominal Aorta and the Mechanical Properties of the Aorta in Kawasaki Disease in Children

Measuring aortic distensibility has been shown to be useful in adults as a noninvasive method in the early detection of atherosclerosis. This study had two purposes: to assess the stiffness of the abdominal aorta by using two-dimensional echocardiography (2DE) in healthy neonates, children, and adults and to assess aortic distensibility in children with Kawasaki disease in acute and subacute phases. The study comprised 168 healthy subjects and 40 patients with Kawasaki disease. We recorded systolic (P _s) and diastolic (P _d) blood pressure and measured aortic diameter (D _d) at both minimum diastolic pressure and maximum systolic expansion (D _s) by 2DE. These measurements were used to determine (1) aortic strain (S) = (D _s−D _d)/D _d, (2) pressure strain elastic modulus (E _p) = (P _s−P _d)/S, and (3) normalized E _p (E _p*) =E _p/P _d. Significant correlations were found between S and age, E _p and age, and E _p* and age. In Kawasaki disease, E _p and E _p* showed negative correlations to day after onset. The aorta was less distensible in infants, became soft in 12- to 16-year-olds, and then stiffened with increasing age among normal subjects. In Kawasaki disease, aortic stiffness was high at the acute phase and normal at the subacute phase. These tendencies may be related to the biological characteristics of smooth muscle cells.     

Sex determining gene on the X chromosome short arm: Dosage sensitive sex reversal

The present review article summarizes current knowledge concerning the sex determining gene on Xp21, termed DSS (dosage sensitive sex reversal). The presence of DSS has been based on the finding that, in the presence of SRY, partial active Xp duplications encompassing the middle part of Xp result in sex reversal, whereas those of the distal or proximal part of Xp permit male sex development. Because Klinefelter patients develop as males, it is believed that DSS is normally subject to X-inactivation, and that two active copies of DSS override the function of SRY, resulting in gonadal dysgenesis because of meiotic pairing failure. It may be possible that DSS encodes a target sequence for repressing function of SRY or that DSS is involved in an X chromosome-counting mechanism. Molecular approaches have localized DSS to a 160 kb region and isolated candidate genes such as DAX-1 and MAGE-Xp, but there has been no formal evidence equating the candidate gene with DSS. In addition to its clinical importance, the exploration of DSS must provide a useful clue to phylogenetic studies of sex chromosomes and dosage compensation.     

Impact of Parent Practices of Infant Positioning on Head Orientation Profile and Development of Positional Plagiocephaly in Healthy Term Infants

Aims: The influence of infant positioning on the development of head orientation and plagiocephaly is not clear. This study explored the relationship between infant body and head positioning, with the development of asymmetrical head orientation and/or positional plagiocephaly. Methods: Clinician measurement of head orientation profile and parent-reported infant positioning data were collected for 94 healthy term infants at 3, 6, and 9 weeks of age. Plagiocephaly was measured at 9 weeks with the modified Cranial Vault Asymmetry Index. Results: More severe plagiocephaly was associated with longer supine-sleep-maximum (p = 0.001) and longer supine-lying-total (p = 0.014) at 6 weeks. Prone positioning was not associated with plagiocephaly. Parent-reported head asymmetry during awake and sleep time at 3 weeks identified infants with clinician-measured head asymmetry at 9 weeks. Better symmetry in head turning was associated with more side-lying-total time by 9 weeks (p = 0.013). Conclusions: Our results showed that infant positioning is associated with early head orientation and plagiocephaly development. Early parent-reported asymmetry during awake and sleep time is an important indicator for the need for professional assessment and advice. A Plagiocephaly Prevention Strategy and Plagiocephaly Screening Pathway are provided for clinicians and parents.     

Nasopharyngeal carriage of Haemophilus influenzae

Objective: Nasopharyngeal colonization ofHaemophilus influenzae (H. influenzae) in young children may be important in developing countries.Method: In this study, we screened school going children for carriage ofH. influenzae. A total of 44H. influenzae isolates out of a collection of 162 were characterized for biotypes, capsular serotypes and antibiotic resistance.Results:A significant proportion ofH. influenzae (25/44) isolates were serotype b. High antibiotic resistance was observed against commonly administered antibiotics like ampicillin (79%), chloramphenicol (20%), trimethoprim sulfamethoxazole (84%) and erythromycin (95%). Comparison of antibiotic resistance profile of nasopharyngeal isolates was observed to be correlated with those ofH. influenzae from disease.Conclusion: Multidrug resistant nasopharyngealH. influenzae in young healthy children may act as reservoir. Monitoring of antibiotic resistance among nasopharyngealH. influenzae as a surrogate for invasiveH. influenzae seems an attractive option.     

Clinical implications of a slight increase in the gene dosage of <Emphasis Type="Italic">MYCN</Emphasis> in neuroblastoma determined using quantitative PCR

Introduction  Recently, determining the MYCN status in neuroblastoma (NB) using the quantitative PCR (Q-PCR) and FISH instead of the Southern blotting (SB) has been recommended. In order to assess the implications of the gene dosage of MYCN in NB, the MYCN status was evaluated using Q-PCR on DNA extracted from small areas of NB specimens obtained using laser capture microdissection (LCM). Materials and methods   MYCN gene dosages (MYCN/NAGK) were determined in 63 primary NB block samples, as well as in 243 microdissected tissues from 63 samples using Q-PCR. In 23 of 63 cases, the MYCN gene status was evaluated using FISH. Results  Nine block samples with the amplification of MYCN based on SB showed a remarkable increase of the MYCN gene dosage using Q-PCR. Twelve of 54 block samples with no amplification of MYCN based on SB showed a slight increase of the MYCN gene dosage (3.56 ≧ MYCN/NAGK > 1.84), and 8 of these 12 cases were in the advanced stage. Among these 12 cases, 1 case had several LCM areas with a high copy number of MYCN and several LCM areas which showed no increase of MYCN gene. Another case showed a slight increase in the MYCN gene dosage (3.65 ≦ MYCN/NAGK ≦ 4.82) in all LCM areas. In addition, a large number of cells with the MYCN gain were found using FISH in the block sample. In 2 other cases of 12 cases, although no LCM areas showed an increased gene dosage of MYCN, a small number of cells with MYCN amplification were found using FISH were found in the block sample. Conclusion  A slight increase in the gene dosage of MYCN detected by Q-PCR may indicate that the NB tissue contains a small number of cells with the MYCN amplification or a large number of cells with the MYCN gain, which are associated with the aggressive progression of NB.