Quick Search
  Home Journal Information Current Issue Past Issues Services Contact Us  
Vol 13, No 3
Vol 13, No 3 June 2017 ISSN 1708-8569
 
Review articles
Original articles
Brief reports
Letters to the Editor
   
Review articles:
Newborn screening for Duchenne muscular dystrophy in China: follow-up diagnosis and subsequent treatment
  Qing Ke, Zheng-Yan Zhao, Robert Griggs, Veronica Wiley, Anne Connolly, Jennifer Kwon, Ming Qi, Daniel Sheehan, Emma Ciafaloni, R Rodney Howell, Petra Furu, Peter Sazani, Arvind Narayana, Michele Gatheridge
 
Background: Newborn screening for Duchenne muscular dystrophy (DMD) is currently being initiated in Zhejiang Province, China and is under consideration in other countries, including the United States. As China begins to implement DMD newborn screening (DMDNBS), there is ongoing discussion regarding the steps forward for follow up care of positively identified patients as well as false positive and false negative results.
Data sources: Relevant papers related to DMD-NBS, and NBS in China were reviewed in PubMed.
Results: The current state of DMD-NBS is discussed, along with the steps needed to effectively screen infants for this disease in China, recommendations for establishment of follow up care in patients with positive and negative screens, and measurement of patient outcomes.
Conclusions: Zhejiang Province, China is ready to implement DMD-NBS. Future challenges that exist for this program, and other countries, include the ability to track patients, assist with access to care, and ensure adequate follow-up care according to evidence-based guidelines. In addition, China's large rural population, lack of specialty providers, and difficulty in educating patients regarding the benefi ts of treatment create challenges that will need to be addressed.
  [Abstract] [Full Text] [PDF]  
Prevalence and factors associated with stages of behavior change for physical activity in adolescents: a systematic review
  Heloyse Elaine Gimenes Nunes, Diego Augusto Santos Silva, Eliane Cristina de Andrade Gonçalves
 
Background: To analyze prevalence and factors associated with stages of behavior change for physical activity in adolescents.
Data sources: A search was performed in SciELO, PubMed, PsycINFO, SPORTDiscus and LILACS. The search terms were "behavior change" OR "stages of change" OR "stages of readiness" OR "readiness to change" OR "transtheoretical model" OR "process to change" OR "decisional balance", and "motor activity" OR "physical activity" OR "physical exercise" and "adolescent" OR "students". After the eligibility criteria, 18 articles were selected.
Results: The highest prevalence of adolescents in the precontemplation, contemplation, and preparation stages was found in the Germany, South Korea, and Iran while there were more adolescents in the action and maintenance in South Korea and Brazil. The evidence of association indicated that females are more likely to be in precontemplation, contemplation and preparation. The evidence was inconclusive for age (in relation to the precontemplation, contemplation, action and maintenance stages), economic status (all stages), shift of study (when stratifi ed by gender) and body composition (all stages). No association was found with parental level of education.
Conclusion: The fi ndings can help to guide interventions and further research on the topic of behavior changes for physical activity in adolescents.
  [Abstract] [Full Text] [PDF]  
Original articles:
Successful elimination of extended-spectrum betalactamase(ESBL)-producing nosocomial bacteria at a neonatal intensive care unit
  Borbla Szl, Zsolt Reiger, Edit Urbn, Andrea Lzr, Krisztina Mader, Ivelina Damjanova, Kamilla Nagy, Gyula Tlosi
 
Background: Extended-spectrum beta-lactamase (ESBL)-producing Gram-negative bacteria are highly dangerous to neonates. At our Neonatal Intensive Care Unit (NICU), the presence of these bacteria became so threatening in 2011 that immediate intervention was required.
Methods: This study was conducted during a nearly two-year period consisting of three phases: retrospective (9 months), educational (3 months) and prospective (9 months). Based on retrospective data analysis, a complex management plan was devised involving the introduction of the INSURE protocol, changes to the antibiotic regimen, microbiological screening at short intervals, progressive feeding, a safer bathing protocol, staff hand hygiene training and continuous monitoring of the number of newly infected and newly colonized patients. During these intervals, a total of 355 patients were monitored.
Results: Both ESBL-producing Enterobacter cloaceae and Klebsiella pneumoniae were found (in both patients and environmental samples). In the prospective period a significant reduction could be seen in the average number of both colonized (26/167 patients; P=0.029) and infected (3/167 patients; P=0.033) patients compared to data from the retrospective period regarding colonized (72/188 patients) and infected (9/188 patients) patients. There was a decrease in the average number of patient days (from 343.72 to 292.44 days per months), though this difference is not significant (P=0.058). During the prospective period, indirect hand hygiene compliance showed a significant increase (from the previous 26.02 to 33.6 hand hygiene procedures per patient per hospital day, P<0.001).
Conclusion: Colonizations and infections were rolled back successfully in a multi-step effort that required an interdisciplinary approach.
  [Abstract] [Full Text] [PDF]  
Impact of relocation and environmental cleaning on reducing the incidence of healthcare-associated infection in NICU
  Qiu-Fang Li, Hong Xu, Xiao-Ping Ni, Rong Lin, Hui Jin, Ling-Ya Wei, Dan Liu, Lin-Hai Shen, Jie Zha, Xin-Fen Xu, Bo Wu
 
Background: Hospital environment remains a risk for healthcare-associated infections (HAIs). This was a prospective study to evaluate the comprehensive impact of relocating a neonatal intensive care unit (NICU) to a new facility and improved environmental cleaning practice on the presence of methicillin-resistant Staphylococcus aureus (MRSA) on inanimate surfaces and the incident rate of HAIs.
Methods: New environmental cleaning measures were adopted after the NICU was moved to a new and better-designed location. The effect of moving and the new environmental cleaning practice was investigated by comparing the positive number of MRSA on ward surfaces and the incidence density of HAIs between the baseline and intervention periods.
Results: Only 2.5% of environmental surfaces were positive for MRSA in the intervention period compared to 44.0% in the baseline period (P<0.001). Likewise, the total incident rate of HAIs declined from 16.8 per 1000 cot-days to 10.0 per 1000 cot-days (P<0.001).
Conclusion: The comprehensive measures of relocating the NICU to a new facility design with improved environmental cleaning practice are effective and significantly reduce the incidence of HAIs.
  [Abstract] [Full Text] [PDF]  
Prediction of signifi cant hyperbilirubinemia in term neonates by early non-invasive bilirubin measurement
  Manish Jain, Akash Bang, Anju Tiwari, Shuchi Jain
 
Background: Neonatal jaundice is a common problem. We evaluated the utility and best cut-off values of 24- and 48-hour transcutaneous bilirubin indices (TcBI) in predicting subsequent significant hyperbilirubinemia and evaluated various associated maternal and fetal risk factors.
Methods: TcBI at 24 and 48 hours and serum bilirubin levels at 72 hours of age were obtained for healthy, term, appropriate for gestational age neonates. Neonates with prematurity, birth weight <2500 g, ABO or Rh incompatibility, onset of clinical jaundice <24 hours, clinical suspicion of septicemia, positive pressure ventilation at birth, admission in neonatal intensive care unit and contraindications for BiliChek were excluded. Twently-four and 48-hour TcB indices were assessed as predictors of subsequent hyperbilirubinemia, defined as serum bilirubin >17 mg/dL after 72 hours of life and various cut-offs, and were evaluated by calculating sensitivity, specificity and predictive values.
Results: Of 500 newborns, 4.6% had significant hyperbilirubinemia, 27% had TcBI (mg/dL) <5 at 24 hours, and 27.4% had TcBI <8 at 48 hours. None of them had subsequent hyperbilirubinemia (100% negative predictive value). The percentage of newborns with subsequent hyperbilirubinemia increased from 3.4% to 13.2% as their 24-hour TcBI increased from 6 to above 9 mg/dL and from 4.2% to 7.4% as their 48-hour TcBI increased from 8 to above 11 mg/dL. The best cut-off value was TcBI (mg/dL) 7 (odd ratio=4.86, 95% confidence interval: 1.66-15.22) at 24 hours and 10 (odd ratio=2.87, 95% confidence interval: 1.04-8.29) at 48 hours. Area under the receiver operating characteristic curve for 24- and 48-hour measurements was 0.750 and 0.715, respectively. Maternal premature rupture of membranes, deep transverse arrest, post-date pregnancy, and fetal distress were significant risk factors for hyperbilirubinemia.
Conclusions: Twenty-four and 48-hour TcB indices are good predictors of subsequent hyperbilirubinemia. Twenty-four-hour TcBI had better predictive ability than 48-hour TcBI.
  [Abstract] [Full Text] [PDF]  
High-mobility group box-1 and receptor for advanced glycation end products in preterm infants with brain injury
  Hong-Yan Lu, Jiang-Lin Ma, Ji-Yan Shan, Jie Zhang, Qiu-Xia Wang, Qiang Zhang
 
Background: High-mobility group box-1 (HMGB1) protein acts as an important pro-inflammatory mediator, which is capable of activating inflammation and tissue repair. HMGB1 can bind to its receptor such as advanced glycation end products (RAGE). RAGE, in turn, can promote the production of pro-inflammatory cytokines. Soluble RAGE (sRAGE) is a truncated form of the receptor comprising the extracellular domain of RAGE and can inhibit RAGE-activation. The objective of this study was to investigate whether HMGB1 and RAGE are involved in the development of brain injury in preterm infants.
Methods: In total, 108 infants 34 weeks gestation at birth were divided into 3 groups according to cranial ultrasound scan: mild brain damage (n=33), severe brain damage (n=8) and no brain damage (n=67). All the placentas were submitted for pathologic evaluation. Histological chorioamnionitis (HCA) was defined as neutrophil infiltration of amniotic membranes, umbilical cord or chorionic plate. Expressions of HMGB1 and RAGE proteins were assessed by immunohistochemical analysis. The concentration of HMGB1 and sRAGE in umbilical cord blood were measured by enzyme-linked immunosorbent assay.
Results: The frequency of HCA was 30.12%. HCA was associated with elevated concentrations of HMGB1 and decreased sRAGE in umbilical cord blood. The severe brain injury group demonstrated higher cord blood HMGB1 concentrations (P<0.001) and lower sRAGE concentrations (P<0.001) than both other groups. Brain injury in the premature infants was linked to intense staining for HMGB1/RAGE, particularly in inflammatory cells.
Conclusions: Changes of cord blood HMGB1 and sRAGE of premature infants had direct relationship with the degree of inflammation and severity of brain damage. Monitoring sRAGE and HMGB1 levels may be helpful to predict intrauterine infection and brain injury in premature infants.
  [Abstract] [Full Text] [PDF]  
Randomized controlled trial of topical EMLA and vapocoolant spray for reducing pain during wDPT vaccination
  Navratan Kumar Gupta, Amit Upadhyay, Ajeet Kumar Dwivedi, Astha Agarwal, Vijay Jaiswal, Abhishek Singh
 
Background: Intramuscular vaccination is among the most common source of iatrogenic pain in infants. Vapocoolant sprays are rapid-acting alternative to topical anesthetics. They provide transient anesthesia via evaporation induced skin cooling, and reduce pain due to vaccine injection in children and adults. The objective was to compare the synergistic analgesic effect of eutectic mixture of local anesthetics (EMLA) with breastfeeding (EB group) and vapocoolant spay with breastfeeding (VB group) to that of only breastfeeding (BO group) during whole cell diptheria, pertussis and tetanus (wDPT) vaccination.
Methods: A double blind randomized controlled trial was done to include infants up to 3 months of age who came for their first wDPT vaccination. The primary outcome variable was the duration of cry after vaccination. Secondary outcome variables were Modified Facial Coding Score, Neonatal Infant Pain Scale and latency of onset of cry.
Results: Of the 201 eligible participants, 111 babies were excluded and remaining 90 babies were randomized into three groups of thirty each. The groups did not differ significantly in baseline characteristics. Median (interquartile range, IQR) of duration of cry was lesser [35.86 (21.07-107.75) seconds] in babies receiving EMLA cream with breast feeding (EB group) and in babies receiving vapocoolant spray with breast feeding (VB group) [32.58 (21.25-106.21) seconds] as compared to babies receiving only breast feeding (BO group) [67.5 (27.6-180) seconds] (P=0.147). Difference in median (IQR) of latency of cry was not statistically significant. Modified Facial Coding Score and Neonatal Infant Pain Scale at 1 minute and 3 minutes was significantly lower in the EB and VB group, as compared to the BO group (P<0.05).
Conclusions: Addition of topical EMLA application or vapocoolant spray to breastfeeding during wDPT vaccination does not reduce duration of cry in infants up to 3 months of age. However, they are able to show reduction in pain score and further studies are warranted to assess their effi cacy as pain relief measures in infants and children.
  [Abstract] [Full Text] [PDF]  
Total estimated effective doses from radiologic imaging modalities of children with cancer: a single center experience
  Derya Özyörk, Suna Emir, Hacı Ahmet Demir, Glşah Bayram Kabaçam, Bahattin Tunç
 
Background: Recently, awareness of the cumulative radiation exposure for pediatric oncology patients has been increasing, together with increased survival rates and longer life expectancy. The aim of our study was to quantify the amount of ionising radiation from imaging modalities of pediatric oncology patients.
Methods: Eighty-eight patients who were diagnosed with childhood cancer and followed up for 5 years between 2004-2014 in our center were included in the study. Patients' medical fi les were reviewed retrospectively for imaging history in the first 5 years after diagnosis. Total estimated effective doses from radiologic imaging modalities were determined. Also, the basic demographic data, histologic type, stage, and outcomes of disease were collected for all patients.
Results: The individual total estimated effective doses ranged from 8.73 to 167 mSv, with a median of 62.92 mSv. Computed tomography was the greatest contributor of total effective doses. The doses ranged 21.45-113.20 mSv (median: 62.92 mSv) in Hodgkin lymphoma, 12.53-167.10 mSv (median: 52 mSv) in non-Hodgkin lymphoma, 4.13- 172.98 mSv (median: 52 mSv) in neuroblastoma, 31- 149.89 mSv (median: 63.10 mSv) in Wilms' tumor, 11.50- 73.72 mSv (median: 36.90 mSv) in germ cell tumor, 26.46- 125.86 mSv (median: 80.90 mSv) in other solid tumor and 0.02-13.31 mSv (5.25 mSv) in brain tumor subgroup. Twenty-two children (25%) died with progressive disease during the 5-year follow-up period.
Conclusions: Similar to previous studies, the total estimated effective doses in children with cancer have been found various according to diagnosis, stage and clinical course. To clarify the harmfull effects of radiation burden, prospective studies should be conducted in children with cancer.
  [Abstract] [Full Text] [PDF]  
What is the appropriate strategy for diagnosing NAFLD using ultrasonography in obese children?
  Jee Hyun Lee, Su Jin Jeong
 
Background: The aim of this study is to identify obese children who are candidates for a potential diagnosis of non-alcoholic fatty liver disease (NAFLD).
Methods: We enrolled 242 obese children (122 boys and 120 girls) aged 7-16 years who were examined with abdominal ultrasonography in our pediatric obesity clinic. We compared patients in the normal group with those in the NAFLD group (mild disease, moderate to severe disease) and identified the optimal anthropometric parameters among height, weight, body mass index (BMI), waist circumference, hip circumference, waist to height ratio (WHtR), and waist to hip ratio to predict NAFLD using a receiver operating characteristic curve analysis. We also investigated risk factors associated with NAFLD for the anthropometric parameters and the biochemical model using logistic regression.
Results: The high- and low-risk groups for hepatic steatosis relative to a WHtR of 0.56 as the standard point showed significant differences in hepatic steatosis severity grade (P<0.001), BMI (P=0.004), hip circumference (P=0.090), aspartate aminotransferase (P<0.001), alanine aminotransferase (P<0.001), triglycerides (P=0.001), and the triglyceride to high-density lipoprotein (HDL) cholesterol ratio (P=0.006). Risk factors for hepatic steatosis on logistic regression analysis were male sex (odds ratio: 3.68, 95% confidence interval: 1.76-7.70), WHtR >0.56 (2.25, 1.05-4.81), and waist circumference >90th percentile (20.22, 9.21-44.36) in the anthropometric parameter model and elevated alanine aminotransferase levels (boys >25.8 U/L, girls >22.1 U/L) (6.93, 2.52-19.03), hypertriglyceridemia (>110 mg/dL) (3.80, 1.23-11.75), and triglyceride to HDL cholesterol ratio >3 (9.23, 2.95-8.83) in the biochemical parameter model.
Conclusions: A diagnostic approach to hepatic steatosis is recommended as part of the proper screening and stratification of risk factors in obese children. WHtR is a simple and convenient method of effectively identifying obese children who are candidates for hepatic steatosis screening.
  [Abstract] [Full Text] [PDF]  
Drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children: 20 years study in a tertiary care hospital
  Leelawadee Techasatian, Sunee Panombualert, Rattapon Uppala, Charoon Jetsrisuparb
 
Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe lifethreatening skin conditions. The most common cause of these manifestations is medications. Beside discontinued of the culprit drug, systemic corticosteroids were used as a primary treatment option among pediatric population. This study aimed to explore causative drugs (drug group/ latent period), treaments, complications, and treatment outcome (morbidity, mortality, length of hospital stay) of SJS and TEN in children.
Methods: A retrospective chart was reviewed during the period of 1992 to 2012 at Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand. SJS and TEN were clinically diagnosed and confirmed by pediatric dermatologists. Other possible causes other than drug induced SJS and TEN were excluded.
Results: A total of 30 patients was recorded, including 24 (80%) SJS patients and 6 (20%) TEN patients. The mean age was 6.9 years (SD 4.4). Male to female ratio was 1.5:1. Antiepileptic drug group was the most common causative drug (n=18, 60%), followed by antibiotic drug group (n=8, 26.6%), and others (n=4, 13.3%) which included nonsteroidal antiinflammtory drugs (NSAIDs) and chemotherapy drugs. Systemic corticosteroids were used in 29 patients (96.6%). Intravenous immunoglobulin was used in one TEN patient (3.3%). There was a medium correlation between time to treatment (systemic corticosteroids) and the length of hospital stay (Spearman correlation coefficient=0.63, P=0.005). Two TEN patients (6.6%) died.
Conclusions: Carbamazepine was the most common causative drug of SJS and TEN in our study. The severity of skin detachment is not correlated to severity of ocular findings. However, the persistent of ocular complications up to one year is suggested for promptly appropriate ocular treatment in all SJS and TEN patients. Our data suggested that early administration of systemic corticosteroid may reduce the length of hospital stay and should be considered for the treatment of pediatric drug induced SJS and TEN.
  [Abstract] [Full Text] [PDF]  
Relationship between postoperative complications and fi bular integrity in congenital pseudarthrosis of the tibia in children
  Yao-Xi Liu, Hai-Bo Mei, Guang-Hui Zhu, Rong-Guo He, Kun Liu, Jin Tang, Jiang-Yan Wu, Wei-Hua Ye, Xin Hu, Qian Tan, An Yan, Sheng-Xiang Huang, Xiao-Qian Tan, Ting Lei
 
Background: This study aimed to investigate the relationship between postoperative complications and fi bular integrity in congenital pseudarthrosis of the tibia (CPT) in children.
Methods: A retrospective study was performed in 59 patients with Crawford type IV CPT who were treated with combined surgical technique from 2007 to 2011. The patients were divided into two groups, the CPT with fibular pseudarthrosis (group A) and CPT with intact fi bula groups (group B), on the basis of fibula status after the union of CPT. The incidence rates of refracture, ankle valgus, tibial valgus, and limb length discrepancy in the two groups were investigated.
Results: In group A, 14 (36.8%) cases had refracture, 30 (78.9%) had ankle valgus; 27 (71%) exhibited tibial valgus with an average tibial valgus of 7 (6-20), and 24 (63.2%) had limb length discrepancy with an average limb length of 1.26 cm (0.6-4.4 cm). In group B, 2 (9.5%) cases had refracture, 11 (52.4%) had ankle valgus, 8 (42.9%) had tibial valgus with an average tibial valgus deformity of 2.9 (6-13), and 13 (61.9%) had limb length discrepancy with an average limb length of 1.48 cm (0.5-5 cm). Significant difference in refracture and ankle valgus was found between groups A and B (P<0.05).
Conclusions: After the union of CPT, patients with fi bular pseudarthrosis showed higher incidence of refracture and ankle valgus than those with intact fibula. Attention should be paid to the presence of fibular pseudarthrosis when managing CPT.
  [Abstract] [Full Text] [PDF]  
Identifying key genes associated with Hirschsprung's disease based on bioinformatics analysis of RNA-sequencing data
  Wei-Kang Pan, Ya-Fei Zhang, Hui Yu, Ya Gao, Bai-Jun Zheng, Peng Li, Chong Xie, Xin Ge
 
Background: Hirschsprung's disease (HSCR) is a type of megacolon induced by deficiency or dysfunction of ganglion cells in the distal intestine and is associated with developmental disorders of the enteric nervous system. To explore the mechanisms of HSCR, we analyzed the RNA sequencing data of the expansion and the narrow segments of colon tissues separated from children with HSCR.
Methods: RNA-sequencing of the expansion segments and the narrow segments of colon tissues isolated from children with HSCR was performed. After differentially expressed genes (DEGs) were identified using the edgeR package in R, functional and pathway enrichment analyses of DEGs were carried out using DAVID software. To further screen the key genes, protein-protein interaction (PPI) network and module analyses were conducted separately using Cytoscape software.
Results: A total of 117 DEGs were identified in the expansion segment samples, including 47 up-regulated and 70 down-regulated genes. Functional enrichment analysis suggested that FOS and DUSP1 were implicated in response to endogenous stimulus. In the PPI network analysis, FOS (degree=20), EGR1 (degree=16), ATF3 (degree=9), NOS1 (degree=8), CCL5 (degree=8), DUSP1 (degree=7), CXCL3 (degree=6), VIP (degree=6), FOSB (degree=5), and NOS2 (degree=4) had higher degrees, which could interact with other genes. In addition, two significant modules (module 1 and module 2) were identified from the PPI network.
Conclusion: Several genes (including FOS, EGR1, ATF3, NOS1, CCL5, DUSP1, CXCL3, VIP, FOSB, and NOS2) might be involved in the development of HSCR through their effect on the nervous system.
  [Abstract] [Full Text] [PDF]  
Brief reports:
Clinical experience in the treatment of children who swallowed multiple magnetic foreign bodies: A report of five cases
  Yue-Bin Zhang, Zhi-Gang Gao, Qi-Xing Xiong, Li-Feng Zhang, Dou-Te Cai, Jia-Bin Cai
 
Background: Several clinical reports have been published on complications resulting from swallowing multiple magnetic foreign bodies. This study aimed to summarize the clinical experience of managing children who swallowed multiple magnetic foreign bodies.
Methods: We reviewed the clinical records of five children who swallowed multiple magnetic foreign bodies and were admitted to our hospital during June 2012 to June 2014. Details of the patients' presentation, imaging studies, complications and treatment were recorded.
Results: All fi ve children suffered from gastrointestinal perforation and intestinal obstruction. The magnetic foreign bodies caused local bowel wall tissue ischemia necrosis and perforation as well as other complications associated with fistula formation. The magnets were finally removed by laparotomy surgery.
Conclusion: If magnetic foreign bodies cannot be removed by endoscopy, an operation is suggested as soon as possible to avoid serious complications.
  [Abstract] [Full Text] [PDF]  
Factors controlling fetal echocardiography determine the diagnostic accuracy of isolated ventricular septal defect
  Jiao Chen, Liang Xie, Han-Min Liu
 
Background: Fetal echocardiography (FECG) is a key screening tool for prenatal cardiac abnormalities. Herein, we examined the ultrasonic factors determining prenatal ultrasonic diagnosis of isolated ventricular septal defect (IVSD).
Methods: The diagnostic role of ultrasonic factors was investigated in patients in middle or late pregnancy, diagnosed with IVSD by FECG and confirmed using postnatal echocardiography.
Results: One hundred and six patients with IVSD were enrolled; the majority had perimembranous VSD. The combined imaging mode of 2 dimentionalechocardiography (2DE) and color doppler flow imaging (CDFI) showed the highest rate (56.6%) of IVSD detection, while CDFI was more efficient than 2DE (32.1% vs. 11.3%). The single-view mode was more efficient than multiple-view mode (75.5% vs. 24.5%). The highest efficient mode to detect IVSD was achieved using combined imaging mode on the single view of the left ventricular outflow tract view (LVOTV) (28.3%). FECG correctly classified 71.7% of fetal IVSD. There was a significant difference of accuracy rate in classifying IVSD among the three different imaging modes (2=7.141, P<0.05). The single imaging mode of CDFI and the mode of CDFI combined with 2DE correctly classified 75.9% and 75.0% of fetal IVSD, respectively. LVOTV was the most accurate view of fetal IVSD classification (85.2%; 2=15.782, P<0.05). There was no difference in accuracies of IVSD classification among multiple-view modes (2=2.343, P>0.05) or between single-view mode and multiple-view mode (2=0.32, P>0.05).
Conclusion: Single LVOTV in CDFI or CDFI combined with 2DE of FECG were the most effective diagnostic modes for fetal IVSD diagnosis.
  [Abstract] [Full Text] [PDF]  
Letters to the Editor:
Prominent myalgia-an important clue in the diagnosis of a muscle disorder
  Puneet Jain, Vineet Sehgal
 
A developmentally normal, 15-year-old boy presented with complaint of gait abnormalities since 8 years of age. The child complained of difficulty in climbing stairs and running. He could not climb stairs without the support of railing. There was no problem with rising from sitting or lying down position. There was no history of upper limb weakness, difficulty in swallowing or breathing, restricted eye movements or diurnal variation of weakness. These complaints were very slowly progressive. He also complained of frequent myalgias (sometimes disabling), sometimes following exercise, affecting bilateral calf muscles and lower back. There was no stiffness or history of passing dark colored urine; and no significant family history was elicited.
Examination revealed normal spine examination and absence of muscle hypertrophy or atrophy. There was weakness at the hip flexors and abductors (4/5) and neck flexors (3/5). There was no facial, truncal or respiratory muscle weakness. The muscle stretch reflexes were normal. There was no muscle stiffness, myotonia, contractures or ligamentous laxity.
Serum creatine kinase (CK) was elevated (1248 U/L). His echocardiography was normal. The parents refused for muscle biopsy. Targeted sequencing analysis of congenital myopathy/dystrophy genes revealed a novel heterozygous missense mutation (c.13739T>A; p.Phe4580Tyr) in exon 94 of ryanodine receptor 1 gene (RYR1). Rehabilitation was initiated, risk of malignant hyperthermia was explained and genetic counselling was done.
Most cases with clinic-pathological features of central core disease are due to dominant missense mutations in the RYR1 gene. The age of onset and phenotype is variable and include myopathies with permanent weakness and "induced" myopathies with anesthesia and non-anaesthesia related episodes of rhabdomyolysis or hyperCKemia.[1,2] The reported case had very slowly progressive hip girdle weakness, prominent myalgias and mildly elevated serum CK. He did not have facial weakness or any orthopedic manifestation at present.
Myalgias with or without rhabdomyolysis are often associated or may be the only presenting feature of core myopathy. Dlamini et al[3] identified RYR1 mutations/variants in 14 out of 39 unrelated families with unexplained rhabdomyolysis and/or exertional myalgia. Myagias may also be a prominent feature in other muscle disorders (Table).[4] Presentation at an interval after sustained exercise distinguishes RYR1- related myalgia and rhabdomyolysis, for example from the glycogenoses,where early onset of symptoms and a second wind phenomenon are typical.
 
1 Jungbluth H, Sewry CA, Muntoni F. Core myopathies. Semin Pediatr Neurol 2011;18:239-249.
2 Snoeck M, van Engelen BG, Ksters B, Lammens M, Meijer R, Molenaar JP, et al. RYR1-related myopathies: a wide spectrum of phenotypes throughout life. Eur J Neurol 2015;22:1094-1112.
3 Dlamini N, Voermans NC, Lillis S, Stewart K, Kamsteeg EJ, Drost G, et al. Mutations in RYR1 are a common cause of exertional myalgia and rhabdomyolysis. Neuromuscul Disord 2013;23:540-548.
4 Chan EK, Kornberg AJ, Ryan MM. A diagnostic approach to recurrent myalgia and rhabdomyolysis in children. Arch Dis Child 2015;100:793-797.
  [Abstract] [Full Text] [PDF]  
Child motherhood: the incapability remains
  Evrim Kiray Bas, Ali Bulbul, Sinan Uslu, Vedat Bas, Umut Zubarioglu
 
The period of adolescence, defined by the World Health Organization as the period between 10 and 19 years of age, is the transition from childhood to adulthood through a number of biological, psychological, and social changes. It has been reported that one in five individuals is an adolescent, and 85% of adolescents live in developing countries.[1] Pregnancies that occur in this period, when the individual has yet to complete the maturation process in both biological and psychological senses, are risky for both the mother and child.
In Turkey, 21.6% of the young population are adolescents,[2] and 5%-12% of pregnancies occur during the adolescent period.[3,4]
We performed a study to investigate postnatal care and nutritional status of infants born to adolescent mothers and contraception methods used by adolescent mothers during postnatal period in our country. The data were collected from 241 adolescent mothers. Health care and nutritional status of the babies and contraception methods used by adolescent mothers were obtained at 24 and 36 months of the postnatal period with interviews conducted by face-to-face interview method. Informed consent was obtained from all individual participants included in the study.
There were a total of 3427 deliveries at our hospital in 2011, of which 7.9% (n=271) were adolescent deliveries. After the consent obtained, 241 adolescent mothers and their infants were included in the study.
Interviews indicated that 38.2% of the mothers (n=92) were unable to care for their babies by themselves (incapable maternity), and 15.4% (n=37) missed vaccination and growth and development control visits for their babies. The rate of formulafeeding was 37.8% (n=91), the rate of exclusively breastfeeding for the fi rst 6 months was 13.7% (n=33).
World Health Organization recommends exclusive breastfeeding in the first 6 months. But this rate was low among the babies of adolescent mothers (13.7%). In other countries, this rate of exclusive breast feeding was found between 19%-31%, in the absence of any data from our country.[5,6] Spear[7] reported a low rate of breastfeeding in the first 6 months (22.6%), and they concluded that the families did not receive adequate information or training.
Similarly, the rate of postnatal follow-up (84.6%) due to was found to be inadequate in our study. This can be ignorance, poor socioeconomic status, lack of accessibility of transport, and social pressure. In Turkey, ensuring feeding with breast milk for a sufficient period, the correct timing of starting supplementary food and vaccination follow-ups at appropriate times require an increase in home visits and local motherhood training.
None of the adolescent mothers used any contraception in the pre-pregnancy period, and 43.2% (n=104) also did not use any contraception in the postnatal period. On the other hand, 12.8% of them were pregnant again (n=31).
The sociocultural characteristics still dominating Turkish society, low educational levels, crowded family environment, and related social pressures can prevent mothers from making decisions regarding her own body and baby. Therefore, it must be kept in mind when evaluating pregnant adolescents that these individuals are children in both the biological and psychological senses, and support must be given accordingly.
In conclusion, this study showed that adolescent pregnancies are a chronic social problem in our country. Furthermore, the health of infants and children, public health, and the economy of the country can be adversely affected by insufficient infant care and nutrition information.
 
1 World Health Organization. The World Health Report 1998. Life in the 21st century: a vision for all. Geneva: WHO, 1998: 97.
2 Hacettepe University Institute of Population Studies. Turkey- Demographic and Health Survey 2008.http://catalog.ihsn.org/index.php/catalog/5517/related_citations?sort_ by=authors&sort_order=asc& (accessed December 23, 2014).
3 Keskinoglu P, Bilgic N, Picakciefe M, Giray H, Karakus N, Gunay T. Perinatal outcomes and risk factors of Turkish adolescent mothers. J Pediatr Adolesc Gynecol 2007;20:19-24.
4 Arkan DC, Kaplanoğlu M, Kran H, Ozer A, Coşkun A, Turgut E. Adolescent pregnancies and obstetric outcomes in southeast Turkey: data from two regional centers. Clin Exp Obstet Gynecol 2010;37:144-147.
5 Olaiya O, Dee DL, Sharma AJ, Smith RA. Maternity care practices and breastfeeding among adolescent mothers aged 12-19 years-United States, 2009-2011. MMWR Morb Mortal Wkly Rep 2016;65:17-22.
6 Akhtaruzzaman M, Hossain MA, Karim MR, Khan RH, Islam MS, Ahamed F, et al. Attitude and practices of mothers on breastfeeding attended at a tertiary hospital in Bangladesh. Mymensingh Med J 2015;24:480-485.
7 Spear HJ. Breastfeeding behaviors and experiences of adolescent mothers. MCN Am J Matern Child Nurs 2006;31:106-113.
  [Abstract] [Full Text] [PDF]  
   
 
 
 
roger vivier bags 美女 美女

Home  |  Journal Information  |  Current Issue  |  Past Issues  |  Journal Information  |  Contact Us
Children's Hospital, Zhejiang University School of Medicine, China
Copyright 2007  www.wjpch.com  All Rights Reserved Designed by eb