Table of Content

15 July 2015 Volume 33 Issue 7

  

Application of 320-detector row dynamic volume CT angiography in follow up of coronary artery aneurysms caused by Kawasaki disease

LIU Jun, YU Minghua, XU Kaiyuan, XIANG Feng, ZHOU Qianchao

. 2015, 33(7):  601.  doi:10.3969 j.issn.1000-3606.2015.07.001

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Objective　To assess the values of 320-detector row dynamic volume CT angiography (CTA) and transthoracic echocardiography (TTE) in follow up of coronary artery aneurysm (CAA) caused by Kawasaki disease (KD). Methods　320-detector row CTA and TTE were applied in long-term follow-up of 8 patients with CAA caused by KD. Results　In 8 patients, the mean age at onset was 41.63±22.70 months and the mean follow up time was 43.50±10.99 months. In acute phase, 3 cases of giant coronary artery aneurysms (GCAA) and 5 cases of mid-small CAA were diagnosed by TTE. A total of 16/32 arteries (50%) were involved. At the end of follow-up, 3 cases of GCAA and 2 cases of mid-small CAA were still diagnosed by TTE, and small CAAs were regressed in another 3 cases. A total of 6/32 arteries (18.75%) were involved. Simultaneously at the end of follow-up, a total of 7/32 arteries (21.9%) were involved by 320-detector row CTA. The distribution was consistent with that of TTE. Meanwhile, there were one case of left circumflex artery, one case of GCAA at distal of the right coronary artery, 2 cases of thrombus, 1 case of coronary stenosis and 2 cases of calcification. Conclusions　CAA caused by KD may be persistent for a long time. The thrombus, stenosis, and calcification of coronary can occurr at late phase in GCAA. TTE is sensitive and reliable to detect proximal and middle segment of coronary lesions, but has limitations in detection of distal segment of coronary arteries. 320-detector row CTA has more comprehensively view of each coronary artery lesions and is especially sensitive and reliable to detect coronary thrombosis, calcification and narrowing in proximal and distal coronary arteries after acute phase.

Association between polymorphism of GRIN3A gene and clinical characteristic in children with Kawasaki disease

JIN Xiangqun, XIONG Zhi, ZHU Ying, TONG Lina, WANG Liqiong

. 2015, 33(7):  605.  doi:10.3969 j.issn.1000-3606.2015.07.002

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　Objective　To investigate the association between nucleotide polymorphisms of GRIN3A gene and clinical characteristic of Kawasaki disease (KD) in children in Han population in central Chinese. Methods　A case-control study was performed. A total of 191 children with KD were recruited and 217 healthy children were served as controls. The distribution of SNP was determined by PCR-RFLP. Arterial lesions were detected by echocardiographic. Results　The distribution of three genotypes (CC, CG, GG) in SNP (rs7849782) was statistically difference between KD and control groups (P=0.034), and C allele was associated with KD susceptibility (OR=1.46, 95% CI: 1.10 - 1.92, P=0.007). In children with KD, the polymorphism of SNP loci was significantly associated with oral mucosa lesions and coronary artery lesion (P<0.05), but not associated with conjunctival hyperemia, hand-foot edema, rash, and lymphadenopathy (P>0.05). The polymorphism of SNP loci was also associated with the levels of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (P<0.05). Conclusion　The ploymorphism of SNP loci of GRIN3A gene (rs7849782) was associated with the susceptibility of KD. The C allele was the risk factors. The polymorphism of SNP was associated with oral mucosa lesions and coronary artery lesion, and may affect the levels of ESR and CRP.

Study of the risk factors for syncope in children with orthostatic intolerance

WANG Yuanfei, ZHANG Yunjuan, WANG Jin, DONG Xiangyu, YANG Yinan, NI Qian, LIU Yahong

. 2015, 33(7):  609.  doi:10.3969 j.issn.1000-3606.2015.07.003

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Objective　To investigate the risk factors for syncope in children with orthostatic intolerance (OI). Methods 　The clinical data from 136 children with OI were retrospectively analyzed. The children were divided into syncope group and non-syncope group based on the existence of syncope. Results　Among the 136 children, 77 children (56.62%) had syncope and 59 children (43.38%) did not have syncope. The differences in BMI, prevalence of inducement of prolonged standing, prevalence of accompanied symptoms of blurred vision/ blacking out, cold sweating and pallor, prevalence of the history of motion sickness and blood flow velocity of MCA were statistically significant between two groups (P<0.05). Conclusions　Children with OI who has low BMI, inducement of prolonged standing, accompanied symptoms of blurred vision/ blacking out, cold sweating and pallor, the history of motion sickness, and fast blood flow velocity of MCA, are prone to syncope.

Clinical and genetic features of Barth syndrome in three patients　

. 2015, 33(7):  614.  doi:10.3969 j.issn.1000-3606.2015.07.004

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　Objective　To explore the clinical presentation, diagnosis, treatment, and outcome of the Barth syndrome (BTHS). Methods　Clinical data were collected and analyzed from 3 patients with confirmed genetic diagnosis of BTHS from June 2013 to October 2014. Results　All of the 3 patients were males and two of them were twins. The main clinical manifestations of the 3 patients were cardiomyopathy and heart failure, accompanied by different degrees of trabeculations of the left ventricle. Two of them were diagnosed of left ventricular noncompaction (LVNC). All of the 3 patients presented with motor retardation, muscle weakness, growth delay and significantly increased urinary excretion of 3-methylglutaconic acid (3-MGC). One patient was found to have neutropenia. All 3 patients had TAZ gene mutations which included a novel missense mutation (c.527A>G, p.H176R) detected in the twins and a known nonsense mutation (c.367C>T, p.R123X) identified in the other patient. All of the mutations were inherited from their mothers. During the follow-up, the twins died at 7 months old and 7.5 months old respectively. The other patient was still alive. Conclusion　BTHS is one of the causes of cardiomyopathy in children. In the male patients who presented with muscle weakness, neutropenia, and increased urinary excretion of 3-MGC, especially in those combined with LVNC, BTHS should be screened.

Mycoplasma pneumoniae pneumonia combined with venous thrombosis: report of two cases

YI Yang, XIONG Jianxin, WANG Sheyang

. 2015, 33(7):  618.  doi:10.3969 j.issn.1000-3606.2015.07.005

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　Objectives　To explore the clinical manifestations and treatment of Mycoplasma pneumoniae pneumonia (MPP) combined with venous thrombosis. Methods　The clinical manifestations and treatment of MPP combined with venous thrombosis were retrospectively analyzed. Results　Two male patients were 5 years 2 months and 3 years 6 months old respectively. After treatment with erythromycin, corticosteroids, immunoglobulin, bronchoalveolar lavage and thrombolytic, both cases were cured. Conclusions It is necessary to pay attention to the occurrence of thrombosis in MPP. Early diagnosis and treatment of thrombosis is very important for the prognosis. The prevention of thrombosis needs to be further studied.

The clinical analysis of the factors influencing the therapeutic effect of INSURE technology in premature infants with respiratory distress syndrome　

WANG Yanrui, WANG Mingjie, YU Xiaohe, CAO Chuanding, HUO Huiyi, LIAO Zhengchang, YUE Shaojie

. 2015, 33(7):  621.  doi:10.3969 j.issn.1000-3606.2015.07.006

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Objective　To assess the factors influencing the therapeutic effects of INSURE technology in premature infants with respiratory distress syndrome (NRDS). Methods　The clinical data from 309 infants with NRDS treated by INSURE technology were retrospectively analyzed from Jan. 2000 to Dec. 2012. Results　In 309 infants with NRDS, 302 infants were cured and the cure rate was 97.7%. Twenty-one infants (6.8%) needed the reintubation for mechanical ventilation within 72 h. The difference in reintubation rate was statistically significant among infants with different gestational age (P<0.01). The infants with the gestation age ≤ 28 weeks had a significantly higher reintubation rate. According to whether the reintubation was performed, the infants were divided into success group and failure group. Compared to the success group, there were higher percentage of infants who had gestation age ≤ 28 weeks, birth weight <1000 g and severe NRDS, needed high dose and repeated use of pulmonary surfactant and oxygen therapy, and had higher mortality in the failure group had (all P<0.05). Conclusions　The INSURE technology can be effective in treatment of NRDS. Small gestational age, low birth weight, and severe NRDS are the risk factors for the failure of the INSURE technology.

Analysis of the factors influencing prognosis of respiratory failure in neonates with gestational age of 34 weeks or more　

ZHU Tianwen, ZHANG Yonghong, CHEN Yan, QIAN Jihong

. 2015, 33(7):  627.  doi:10.3969 j.issn.1000-3606.2015.07.007

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　Objective　To explore the risk factors for the prognosis of respiratory failure in neonates with gestational age ≥ 34 weeks. Methods　A total of 143 hospitalized neonates with respiratory failure who had gestational age ≥ 34 weeks were enrolled from Jan. 2011 to Jun. 2013. According to the outcome, the neonates were divided into good prognosis group and poor prognosis group. The risk factors for the prognosis of respiratory failure were screened by univariate analysis and multivariate binary logistic regression model. Results　Among the 143 neonates, 105 neonates had good prognosis and 38 neonates had poor prognosis. Univariate analysis showed that the mode of delivery, small for gestational age, 5 minutes Apgar score <7, and with primary disease being pneumonia/sepsis were associated with a poor prognosis. The differences were statistically significant (P<0.05). Furthermore, the multivariate binary logistic regression model showed that, small for gestation age (OR=23.87, 95% CI:3.149 - 180.9) and pneumonia/sepsis (OR=2.996, 95% CI:1.514 - 5.928) were the independent factors of poor prognosis. Conclusions　Small for gestation age and primary diseases being pneumonia/sepsis are the independent factors influencing prognosis of respiratory failure in neonates born at a gestational age ≥ 34 weeks.

Research in the mathematical model of transcutaneous bilirubin and total serum bilirubin after phototherapy in neonates

MAO Xujie，LIN Zhenlang，ZHAO Yiwei，WANG Xiao

. 2015, 33(7):  632.  doi:10.3969 j.issn.1000-3606.2015.07.008

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Objective　To establish the mathematical model of transcutaneous bilirubin (TcB) and total serum bilirubin (TSB) after phototherapy in neonates. Methods　Neonates with pathological jaundice were enrolled from October 2013 to June 2014. The neonates were divided into three groups by gestational age: full-term neonates (gestation age of 37-42 weeks), late preterm neonates (gestation age of 34-36+6 weeks), early and mid-preterm neonates (gestation age of 28-33+6 weeks). The neonates received single or double sided phototherapy. During the phototherapy, the forehead and chest were covered by opaque material. The TcB was measured at forehead, mid sternum, perineum area three times each before and after phototherapy. Meanwhile the TSB was tested. Results　 Two hundred and sixty-one neonates with hyperbilirubinemia were enrolled, among whom there were 169 full-term neonates, 63 late preterm neonates and 29 early and mid-preterm neonates. Before phototherapy, there were significantly correlation of TSB with TcB on forehead, mid sternum and perineum (r=0.813, 0.827, 0.754; P<0.001) and the best correlation was with TcB on mid sternum. The linear regression equation was TSB=1.35TcB-5.50. After phototherapy, there were significantly correlateion of TSB with TcB on forehead, mid sternum, and perineum (r=0.751, 0.807, 0.683; P<0.001) and the best correlation was with TcB on mid sternum. The linear regression equation was TSB=1.01×TcB-0.62. Among three groups, the full-term neonates had the best correlation. Conclusions　After phototherapy, the TcB measured on mid sternum which was covered by opaque material is well correlated with TSB. The linear regression model can be established.

Analysis of serum amylase levels in 1920 critically ill children

PENG Hongyan, ZHU Yimin, LU Xiulan, LIU Pingping

. 2015, 33(7):  637.  doi:10.3969 j.issn.1000-3606.2015.07.009

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Objective　To explore the value of elevated amylase in assessment of severity of the disease and its influencing factors in critically ill children. Method　The clinical data from critically ill children hospitalized in pediatric intensive care unit were retrospectively analyzed from November 2009 to June 2014. According to levels of serum amylase, the critically ill children were divided into normal serum amylase group ( ≤ 103 IU/L) and elevated serum amylase group (>103 IU/L). The differences between two groups were compared. Results　A total of 1920 critically ill children were enrolled, most of whom had primary respiratory and neurological diseases. Among them, 1470 children had normal serum amylase (76.6%) and 450 children had elevated serum amylase (23.4%). The elevated serum amylase group had significantly higher rate of organs failure ( ≥ 4), lower rate of PCIS (<70), higher rate of invasive mechanical ventilation and mortality than those in normal serum amylase group (P<0.001). The correlation of serum amylase with lipase blood, urine amylase, blood urea nitrogen and creatinine were significant (r=0.246 to 0.683, P<0.001). Blood amylase was positively correlated with shock index (r=0.111, P=0.002) and negatively correlated with respiratory failure index (r=0.133, P<0.001). Conclusions　Serum amylase could be used to assess the severity of disease. The elevated amylase was closely related to pancreatic exocrine function, renal function, ischemia, and hypoxia. The elevated serum amylase in critically ill children is a warning sign.

Application of fiber bronchoscopy with three-way laryngeal mask airway continuous ventilation in 1-6 month old infants with oxygen-dependent respiratory disease

GUO Jie, JIN Zhipeng, WANG Qi, CUI Lidan

. 2015, 33(7):  641.  doi:10.3969 j.issn.1000-3606.2015.07.010

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Objective　To discuss the values of fiber bronchoscopy with three-way laryngeal mask airway continuous ventilation in 1-6 month old infants with oxygen-dependent respiratory diseases. Methods　From January 2010 to May 2011, 29 cases of 1-6 month old infants with oxygen-dependent respiratory diseases who underwent conventional fiber bronchoscope and 148 cases of 1-6 month old infants with oxygen-dependent respiratory diseases who underwent fiber bronchoscopy with threeway laryngeal mask airway continuous ventilation were enrolled in control and treatment groups respectively. The success rate of fiber bronchoscope and the incidence rate of complications were compared between two groups. Results　In the treatment group, the success rate of fiber bronchoscope was 89.2% which was significantly higher than 58.6% in the control group and the rate of laryngeal edema was 9.1% which was significantly lower than 35.3% in the control group. The rates of endotracheal intubation ventilation and postoperative hemorrhage were lower than those in the control group, but the difference was not statistical ly significant. Conclusion　Fiber bronchoscopy with three-way laryngeal mask airway continuous ventilation is superior to the traditional fiber bronchoscope in 1-6 month old infants with oxygen dependent respiratory system diseases.

The comparison and analysis of risk factors and outcomes of arterial ischemic stroke and hemorrhagic stroke in children

ZHANG Yuanyuan, WANG Huaili

. 2015, 33(7):  645.  doi:10.3969 j.issn.1000-3606.2015.07.011

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Objectives　To study the clinical characteristics, the risk factors and outcomes of arterial ischemic stroke (AIS) and hemorrhagic stroke (HS) in children. Methods　The clinical data from 142 children with AIS or HS were retrospectively reviewed and compared from Nov. 2010 to May 2014. Results　In these children, 92 cases (64.8%) was diagnosed of AIS, amont whom there were 60 males and 32 females and the onset age of stroke was 4.6±3.6 years (1 months to 16 years old), and 50 cases (35.2%) was diagnosed of HS, among whom there were 34 males and 16 females and the onset age of stroke was 2.6±3.7 years (1 months to 13 years old). The difference in age between two groups was statistically significant (P=0.007). The most common presentation of AIS were focal neurological dysfunction including paralysis (73 cases, 79.3%), central facial palsy (30 cases, 32.6%) and speech impairment (19 cases, 20.7%). The most common presentation of HS were diffuse neurological dysfunction including dizziness (29 cases, 58.0%), nausea/vomiting (22 cases, 44.0%) and headache (14 cases, 28.0%). The major risk factors of AIS were arteriopathy (49 cases, 53.3%), infection (47 cases, 51.1%) and minor head injury (16 cases, 17.4%). The major risk factors of HS were vitamin K deficiency (22 cases, 44.0%), intracranial vascular anomalies (8 cases, 16.0%) and haematological disorders (6 cases, 12.0%). Five cases (6.4%) were died, 48 cases (61.5%) became disabled and 9 cases (11.5%) were relapsed in children with AIS while 15 cases (34.1%) were died, 19 cases (43.2%) became disabled in children with HS. The mortality was significantly higher in children with HS than that in children with AIS (P<0.01). Conclusions　In childhood stroke, HS occurs more frequently than is commonly appreciated and it has a poorer prognosis than AIS.

Value of citrulline, intestinal fatty acid binding protein, and intestinal trefoil factor in the diagnosis of acute gastrointestinal injury in critically ill children

ZHANG Ni, ZHU Wei, ZHU Jiarui, ZHANG Weijun, WANG Aihua

. 2015, 33(7):  650.  doi:10.3969 j.issn.1000-3606.2015.07.012

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Objective　To compare the values of serum citrulline, intestinal fatty acid binding protein (IFABP) and intestinal trefoil factor (ITF) in diagnosis of acute gastrointestinal injury in critically ill children. Methods　A total of 84 critically ill children were enrolled. The serum citrullin, IFABP, and ITF were measured by high performance liquid chromatography and enzyme-linked immunosorbent assay. The testing results and clinical data were analyzed. Results　Compared with non gastrointestinal injury group, the serum citrulline level was significantly lower and IFABP and ITF levels were significantly higher in gastrointestinal injury group (all P<0.05). In critically ill children, the serum citrulline level was negatively correlated with C-reactive protein, procalcitonin and hospitalization time (r=-0.36 to -0.31, P<0.01). Conclusions　The levels of citrulline, IFABP and ITF have diagnostic values for acute gastrointestinal injury in critically ill children. The level of citrulline may reflect the degree of acute gastrointestinal injury in critically ill children.

Clinical analysis of 11 children with hemophagocytic syndrome

WANG Xinbao

. 2015, 33(7):  655.  doi:10.3969 j.issn.1000-3606.2015.07.013

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　Objectives　To explore the clinical presentation, diagnosis and treatment of hemophagocytic syndrome (HLH) in children. Methods　The clinical data from 11 hospitalized pediatric patients with HLH were collected and retrospectively analyzed from 2009 to 2013. Results　In 11 pediatric patients with HLH, 6 patients were Epstein-Barr virus associated-HLH, 1 patient was T-cell lymphoma associated-HLH, 2 patients had unknown reason, 2 patients had mutations in the UNC13D gene coding sequence, c.2459C>T/p.A832V (alanine to valine mutation) and c.3067C>T/p.R1023C (arginine to cysteine mutation) respectively. In 11 patients, 6 patients were improved after treatment and 5 patients were died. Conclusions 　The HLH in children lacks specific clinical presentation and progresses rapidly. It should be diagnosed and treated in time.

Prediction of abnormal neurodevelopment in children with congenital heart disease

LIU Dong

. 2015, 33(7):  658.  doi:10.3969 j.issn.1000-3606.2015.07.014

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　A lot of children with congenital heart disease have an abnormal development of nervous system. A variety of biochemical and physical examination indices can be used to predict adverse outcome of neurodevelopment caused by congenital heart disease and they contribute to early intervention and improvement of neurodevelopmental disorders. In recent years, new progresses in the laboratory tests, neurological physiccal examination and perioperative management of the neurodevelopmental abnormalities caused by congenital heart disease have been made. The predictive factors of abnormal neurodevelopment caused by congenital heart disease were reviewed in this article.

Advances in fetal immune mediated atrioventricular block

YAN Hualin, LI Yifei

. 2015, 33(7):  662.  doi:10.3969 j.issn.1000-3606.2015.07.015

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　Fetal atrioventricular block (AVB) is a type of fetal bradyarrhythmias. The reported incidence of fetal complete atrioventricular block (CAVB) and mortality of perinatal fetuses and neonates are significantly higher in pregnancies of anti-SSA/Ro-positive mothers than that of anti-SSA/Ro-negative mothers. The auto-antibodies in maternal serum that can be transported into fetal circulation through placenta may damage fetal cardiac conductive system and eventually result in fetal AVB. There are evidences that early diagnosis and proper treatment can improve the prognosis and survival rate of affected fetuses. In this article, the pathogenesis, risk factors, prenatal diagnosis, treatment and prognosis of fetal immune mediated AVB is reviewed.

Progress in pathogenesis, classification, and treatment of Angelman syndrome

LIU Yijing, XIAO Nong

. 2015, 33(7):  668.  doi:10.3969 j.issn.1000-3606.2015.07.016

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Angelman syndrome (AS) is a neurodevelopmental disorder characterized by severe developmental delay, intellectual disability, speech impairment, gait ataxia, epileptic seizures, and exuberant behavior with happy demeanor. The majority of patients have poor quality of life and cause great burden to family and society. It is known that AS is caused by four genotypes which have different clinical manifestations. In this article, the recent advances in pathogenesis, the relationship between genotype and symptoms, and the treatment strategies of AS will be reviewed.