Table of Content

15 October 2015 Volume 33 Issue 10

  

Analysis of etiology and biochemical markers of acute liver failure in children

　FU Haiyan, WANG Xiaoming, WANG Yali, ZHANG Jianxiao, LI Jingping, ZHAO Xin, LIU Junying, YIN Runkai, CHEN Rui, YANG Limin

. 2015, 33(10):  841.  doi:10.3969 j.issn.1000-3606.2015.10.001

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Objective　To explore the etiology and biochemical markers of acute liver failure (ALF) in children. Methods
　 The cause and the biochemical markers of ALF in children who were treated in December 2014 to January 2011 were analyzed
retrospectively. Results　A total of 67 children were enrolled, including 31 females and 36 males. According to the cause
of the disease, the children were divided into non-genetic metabolic group, genetic metabolic group, and cryptogenic group. In the non-genetic metabolic group (29 cases, 43.28%) there were 12 cases of drug-induced ALF, 5 cases of Reye syndrome, 3 cases of hemophagocytic syndrome, 3 cases of herpes simplex virus infection, 2 cases of autoimmune hepatitis, one of case mushroom poisoning one case of hepatitis A virus infection, one case of cytomegalovirus infection and one case of sepsis respectively. In the genetic metabolic group (14 cases, 20.90%) there were 6 cases of Wilson’s disease, 2 case of glycogen storage disease, 2 of cases progressive familial intrahepatic cholestasis, 2 cases of neonatal intrahepatic cholestasis caused by citrin deficiency, one case of very long-chain acyl coenzyme A dehydrogenase deficiency and one case of primary carnitine deficiency. In the cryptogenic group there were 24 cases (35.82%). The serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), albumin, blood glucose level and AST/ALT were statistically significantly different in genetic metabolic group from in non-genetic metabolism disease group and cryptogenic group, (P<0.05). The genetic metabolic group had the lowest levels of serum ALT, AST, albumin and glucose while the genetic metabolic group had the highest ratio of AST/ALT. Conclusions　The etiology of ALF in children are complex. Genetic metabolic disease should be considered when the child with ALF has no significantly elevated ALT, extremely high ratio of AST/ALT, combined with hypoproteinemia and hypoglycemia.

Clinical analysis of severe liver damage in children

DAI Dongling, WEN Feiqiu, CAI Huabo, ZHOU Shaoming

. 2015, 33(10):  846.  doi:10.3969 j.issn.1000-3606.2015.10.002

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　Objective　To explore the clinical characteristics, treatment and prognosis of severe liver damage in children. Methods　Clinical data of 55 children with severe liver damage were retrospectively analyzed. Results　 In 55 children (31 boys and 24 girls) aged from 28 days to 12 years, forty-five children had acute liver injury mainly caused by infectious diseases (21 cases, 53.3%), blood tumor diseases (5 cases, 11.1%), hereditary metabolic diseases (4 cases, 8.9%), and unexplained diseases (10 cases, 22.2%), ten children had chronic liver injury with decompensated cirrhosis. Most of severe liver damage in children was caused by antipyretic drugs, traditional Chinese medicine and cold medicine, including 31 cases of acute liver injury and 4 cases of chronic liver injury. In children with acute liver injury, clinical symptoms included gastrointestinal symptoms (32 cases, 71.1%), jaundice (26 cases, 57.8%), hemorrhage (9 cases, 20.0%), multiple organ dysfunction (13 cases, 28.9%) and hepatic encephalopathy (6 cases, 13.3%). In children with chronic liver damage, clinical symptoms included abdominal distension and ascites (10 cases), jaundice (9 cases), gastrointestinal bleeding (7 cases), hepatic encephalopathy (3 cases) and multiple organ dysfunction (1 case). In 55 children, 39 children were died and the total mortality was 70.91%. In 14 cases of multiple organs dysfunction syndromes, 13 cases (92.9%) were died. All three cases of hepatic encephalopathy were died. Conclusions　Infectious diseases are the leading cause of sever liver damage in children. The most common inciting factors are antipyretic drugs, traditional Chinese medicine and cold medicine. Children with severe liver damage have a high mortality. Rational use of medicine and the concept of the prevention first should been strengthened.

Relationship between high body mass index and irritable bowel syndrome in Chinese adolescents

ZHOU Huiqing,YAO Min

. 2015, 33(10):  850.  doi:10.3969 j.issn.1000-3606.2015.10.003

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Objectives　To explore the impact of high body mass index (BMI) on irritable bowel syndrome (IBS) in Chinese adolescents. Methods　The students were randomly selected from middle school and high school. They completed the epidemiological questionnaire of IBS and had their height, body mass measured. Results　In total, 1173 students were recruited, including 700 middle school students and 473 high school students. The incidence of overweight/obesity was 30.52%. The incidence of overweight/obesity was not significantly different between boys (28.40%) and girls (32.65%) (P=0.114), and there was not significantly different between students from middle school (30.57%) and high school (30.44%) (P=0.963). Two hundreds and twenty-one students suffered from IBS and the incidence of IBS was 18.84% with no significant gender difference. The incidence of IBS was significantly higher in students from middle school (13.43%) than those from high school (26.85%) (P<0.001). The incidence of IBS was not statistically significant between overweight/obese students (22.07%) and normal weight students (17.42%) (P=0.061). The distribution of IBS subtypes had no statistical significance between overweight/obese and normal weight students (χ2=0.91，P=0.823). Conclusions　Overweight/obesity and IBS were extremely prevalent in Chinese adolescents, but the correlation between them remains to be confirmed.

Clinical features of childhood eosinophilic gastroenteritis

ZHONG Xuemei, MA Xin, NING Huijuan, ZHU Dan, ZHANG Yanling

. 2015, 33(10):  853.  doi:10.3969 j.issn.1000-3606.2015.10.004

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Objective　To analyze clinical manifestations and treatment of eosinophilic gastroenteritis (EGE) in children. Methods　The clinical data of 10 children with EGE under the treatment from June 2014 to June 2012 were analyzed retrospectively. Results　There were 10 children (6 males and 4 females) aged from 9 months to 14 years. Clinical symptoms of eight preschool and school age children included abdominal pain (8 cases), vomiting (4 cases), diarrhea (3 cases), ascites (3 cases), rash (3 cases), fever (2 cases) and hypoalbuminemia (2 cases). Clinical symptoms of two infant and toddler were diarrhea and hematochezia. Peripheral blood eosinophils were increased in 10 children. IgE was elevated in 9 children. One child had very high C-reactive protein. Endoscopic examination showed mucosal congestion, swelling, erythema, erosion. Multiple sites of biopsy showed eosinophil count> 20/HP. One child was diagnosed by laparoscopic exploration. The treatment included diet avoidance, omeprazole, anti-allergic drug. Four children were treated with corticosteroids. All children had been followed up for 6 months to 2 years. One child had relapsed. Conclusion　 The clinical manifestations of EGE are various. Endoscopic biopsy is necessary for diagnosis. Laparoscopy also is helpful for diagnosis. Dietary and glucocorticoid therapy were effective.

Clinical analysis of 264 cases of benign infantile convulsions associated with mild gastroenteritis　

　ZHANG Yuanda, JI Chaoyu, LI Rongmin, ZHANG Xiaolong, ZHANG Yu, DONG Qingwei, HAN Lipo

. 2015, 33(10):  857.  doi:10.3969 j.issn.1000-3606.2015.10.005

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　Objective 　To analyze the potential risk factors for relapse and development of epilepsy in patients with benign infantile convulsions associated with mild gastroenteritis (BICE). Methods　A total of 264 cases of BICE were recruited. According to the frequency of convulsions, the patients were divided into single group (n=134, convulsion once), and multiple group (n=130, convulsions ≥ 2 times). According to convulsion duration, the patients were divided into short-term group (n=186, convulsions duration <5 minutes) , and long-term group (n=78, convulsion duration ≥ 5 minutes). The clinical data obtained during hospitalization and follow-up were analyzed. Results　In multiple group, 9.23% were relapsed and 6.15% developed epilepsy. In single group, 2.99% were relapsed and 0.75% developed epilepsy. There were significantly different in the rate of relapses and development of epilepsy between two groups (P<0.05). In the long-term group, 12.82% were relapsed and 8.97% developed epilepsy. In the short-term group, 3.23% were relapsed and 1.08% developed epilepsy. There were significantly different in the rate of relapses and development of epilepsy between the two groups (P<0.05). Conclusions　There are the risks of relapse and development of epilepsy in BICE patients. Convulsions ≥ 2 times and ≥ 5 minutes may be the risk factors of relapse and development of epilepsy.

Comparative study on the clinical features of rotavirus gastroenteritis between the children with or without convulsion

　MENG Xiangying，CHEN Xuqin，WANG Zhedong，LI Yan，SHI Xiaoyan，ZHANG Bingbing，TANG Jihong，LIU Jie，ZHUANG Yun，WU Qingbin

. 2015, 33(10):  860.  doi:10.3969 j.issn.1000-3606.2015.10.006

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Objective　To investigate the clinical features and prognosis of children with rotavirus gastroenteritis and convulsion. Methods　Clinical data of children with rotavirus gastroenteritis hospitalized from January 2010 to December 2013 were retrospectively analyzed. Subjects were divided into the seizure group and no seizure group according to the presence of seizure in the course and compared between the two groups. Results　There were no significant differences in sex, age, and the average duration of hospitalization between two groups (all P>0.05). The family history, history of seizures, the levels of serum sodium, calcium, lactate, standard bicarbonate concentration (SB), actual bicarbonate concentration (AB), carbon dioxide content (TCO2) and pH were statistically significant between two groups (all P>0.05). During the follow-up period (outpatient telephone follow-up), the recurrence of seizure in two groups was significant different (P<0.05) and only one (0.54%) child in seizure group developed epilepsy. Conclusion　This study showed that rotavirus gastroenteritis with convulsion is a benign clinical course.

Study on the involvement of microtubule-associated protein l light chain 3B in the inflammatory response of Kawasaki disease　

　FENG Siqi, GAO Fang, YI Qijian

. 2015, 33(10):  866.  doi:10.3969 j.issn.1000-3606.2015.10.007

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Objective　To investigate the relationship between microtubule-associated protein l light chain 3B (LC3-II) and the inflammatory response of Kawasaki disease (KD). Methods　Thirty-nine cases of acute KD before intravenous administration of immunoglobulin were enrolled. According to the results of echocardiography, the 39 cases were furtherly divided into KD with coronary artery lesion (CAL, 20 cases) group and KD with non-CAL (NCAL, 19 cases) group. At the same time, 12 healthy children were selected as controls. Serum samples were collected and cultured in vitro by human coronary artery endothelial cells (HCAEC) for 12 h. The LC3-II protein and mRNA expression of HCAEC were detected by Western-blotting and Q-PCR respectively. Results　The LC3-II protein and mRNA expression in CAL group and NCAL group were significantly higher than those in control group, the LC3-II protein and mRNA expression in CAL group was higher than those in NCAL group, and both differences were statistically significant (P<0.05). Conclusions　Autophagy may be involved in the inflammatory response of KD in acute phase, which may be related to endothelial cell lesions of coronary artery in children with KD.

The characteristics and clinical significance of NOTCH1 mutations in childhood T-cell acute lymphoblastic leukemia

YAN Hui, LIU Lanbo, DING Lixia, LI Benshang, SHEN Shuhong, TANG Jingyan, MO Xi

. 2015, 33(10):  870.  doi:10.3969 j.issn.1000-3606.2015.10.008

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Objective　To clarify the characteristics and clinical significance of the NOTCH1 mutations in childhood T-cell acute lymphoblastic leukemia (T-ALL). Methods　Amplify and sequence the heterodimerization (HD) domain and the proline- glutamicacid-serine-threonine (PEST) domain of the NOTCH1 gene in 28 T-ALL children, in order to explore the frequency, position and type of the mutations as well as their reletions with prognosis. Results　In 28 children with T-ALL, 15 cases (51.57%) had been identified the NOTCH1 mutations, all of which were heterozygous mutations. The lymphoblast counts in peripheral blood and bone marrow in the NOTCH1 mutant group at admission were significantly higher than in the non-mutant group (P<0.05). The 1-year remission rate in the 28 children with T-ALL was 75% (21/28), including 80% (12/15) in mutant group in which 3 patients relapsed and all of them died (1-year mortality 20%) and 69.20% (9/13) in non-mutant group in which 4 patients relapsed but all survived (1-year mortality 0%). Conclusions　The children with T-ALL had a high incidence of NOTCH1 mutations at various sites. In addition, the patients with NOTCH1 mutations had more severe disease at diagnosis, better short-term prognosis and poor outcome with salvage therapy after relapse.

Changes of lymphocyte subsets and their significance in children with hemophagocytic lymphohistiocytosis

　AN Qi, FANG Daihua, XUAN Chengmin, XU Shumin, JIN Mingwei, JI Qiang

. 2015, 33(10):  876.  doi:10.3969 j.issn.1000-3606.2015.10.009

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Objective　To explore the role of the lymphocyte subsets in the peripheral blood in diagnosis, treatment and prognosis of hemophagocytic lymphohistiocytosis (HLH) in children. Method　A total of 30 children with HLH were enrolled in this study and treated according to the HLH-2004 diagnostic guidelines. 20 children with HLH entered complete remission (CR) and 10 children with HLH died. Thirty age-matched healthy children were selected as normal controls. T cell subsets in the peripheral blood were measured by flow cytometry. Results　Compared with control group, CD3+T and CD8+T cells were significantly increased, CD4+T and CD3-CDl6+CD56+ NK cells were significantly decreased, and CD4+/CD8+ cell ratio was significantly decreased in 20 CR children and 10 died children with HLH in acute phase (P<0.05). CD19+B cells was not statistically different in 20 CR children and 10 died children with HLH in acute phase from control group (P>0.05). In acute phase, the lymphocyte subsets were not statistically different between 20 CR children and 10 died children (P>0.05). In 20 CR children, the proportion of CD3-CD16+CD56+NK in CR phase was statistically different than that in acute phase (P<0.05). Conclusions　Children with HLH have obvious changes in peripheral blood lymphocyte subsets and have cellular immunity disorders. Dynamic detection of the changes may help determine the therapeutic effect and prognosis of HLH.

One case report of autoantibody-associated congenital heart block

FENG Zongtai, YANG Xiaolu, YANG Zuming, CAI Yan, WANG Sannan

. 2015, 33(10):  880.  doi:10.3969 j.issn.1000-3606.2015.10.010

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Objective　To explore the pathogenesis, clinical manifestations, diagnosis, and treatment of autoantibody-associated congenital heart block. Methods　The clinical data of one child with autoantibody-associated congenital heart block was retrospectively analyzed. Results　In 24 week gestation, fetal bradycardia had been found by routine fetal echocardiography. After birth, the anti-SSA/Ro antibodies and anti-SSB/La antibodies were positive in both infant and her mother. The diagnosis of autoantibody-associated congenital heart block was confirmed. Intravenous immunoglobulin at 1 g/kg was administrated. At 6 months follow-up, the electrocardiogram suggested type I second degree atrioventricular block. Conclusion　In the fetus or neonates found to have bradycardia and excluded the cardiac structural abnormalities, the autoimmune antibody should been tested and early intervention should been promoted.

The effect of different hypoxic duration on the degree of brain white matter injury　

YANG Yinxiang, SUO Lei, DU Qing'an, LUAN Zuo

. 2015, 33(10):  883.  doi:10.3969 j.issn.1000-3606.2015.10.011

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　Objective　To explore the effect of different hypoxic duration on the brain white matter injury. Methods 　Newborn rats were randomly divided into two groups, normal group (n=24) and model group (n=45). The model group was divided into 3 subgroups (n=15) according to the time of hypoxia (50 min, 70 min, and 90 min). The animal model of white matter injury was established by unilateral carotid artery ligation in model group. After different duration of hypoxia, the mortality rate was recorded, the morphological changes of brain pathology was observed by hematoxylin eosin (HE) staining, myelin basic protein (MPB) of white matter was detected by immunofluorescence staining and motor function was evaluated by climbing slope test. Results　The mortality rates significantly increased with prolonged hypoxia. The mortality rate was as high as 60% in 90 min subgroup. The HE staining showed that there were no obvious injury in 50 min subgroup, selective white matter injury on the operative side appeared in 70 min subgroup, and a wide range of infarction of white matter, hippocampus, and cortex appeared in 90 min subgroup. MBP semi-quantitative scores of white matter injury were higher in 70 min subgroup (3.89 ± 0.47) and 90 min subgroup (4.72 ± 0.57) than that in the normal group (0.06 ± 0.24), the difference was statistically significant (P <0.05). In climbing slope test, the subgroups had different degrees of motor dysfunction on affected side with 90 min subgroup being the most serious. Conclusions　White matter injury model could be established by unilateral carotid artery ligation, and different hypoxic duration significantly affects the range and degree of injury.

Effect of thyroid hormone on the expression of deiodinase and myelin basic protein in brain tissue of preterm rabbit with germinal matrix-intraventricular hemorrhage

　LIU Tiantian, LIU Fang, LI Yingmei

. 2015, 33(10):  887.  doi:10.3969 j.issn.1000-3606.2015.10.012

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Objectives　To investigate the effect of thyroid hormone on the expression of deiodinase and myelin basic protein in brain tissue of neonatal rats with germinal matrix-intraventricular hemorrhage (GM-IVH), and its nerve protective effect. Methods 　Sixty premature rabbits of gestational age 29 days were randomly divided into control, model and intervention groups. The premature rabbits in model and intervention groups were given intraperitoneal injection of 50% glycerol to establish GM-IVH animal model and the premature rabbits in control group were given the same dose of saline. The GM-IVH was screened by cranial ultrasound. The premature rabbits with GM-IVH in intervention group were given intraperitoneal injection of triiodothyronine and the other two groups were given the same dose of saline. At 3 days, 7 days and 14 days after birth, the premature rabbits were killed and the brain tissue were quickly separated. The expression of type 2 iodothyronine deiodinase (D2) and type 3 iodothyronine deiodinase (D3) of periventricular zone tissue were detected by western blot method. The expression of myelin basic protein (MBP) were detected by immunohistochemical method. Results　The expression of D2 in brain tissue increased gradually in three groups. At the same time point, the expression of D2 was the lowest in the model group, the highest in the control group, and the middle in the intervention group. The differences of D2 among three groups were statistically significant (all P<0.001). The expression of D3 increased gradually in the control and model groups, while it was the highest in the intervention group at day 7. At the same time point, the expression of D3 in the control group was the lowest, it was higher in intervention group than that in the model group at 3 days, then it was higher in the model group than that in the intervention group. The differences of D3 among three groups were statistically significant (all P<0.001). The expression of MBP increased gradually in the control and intervention groups, and it decreased gradually in the model group. At the same time point, the MBP was the lowest in the model group, it was the highest in the control group, and it was the middle in the intervention group. The differences of MBP among three groups were statistically significant (P<0.001). Conclusions　The expressions of D2 and MBP in the brain Tissues of premature rabbit with the GM-IVH increased while the expression of D3 decreased in some extent after thyroxine intervention.

Genetic analysis and literature review of Crigler-Najjar syndrome type Ⅰ　

TAN Yanfang, OUYANG Wenxian, JIANG Tao, LI Shuangjie

. 2015, 33(10):  893.  doi:10.3969 j.issn.1000-3606.2015.10.013

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Objective　To analyze the Uridine diphosphoglucuronyl transferase 1 A1 gene (UGT1A1) mutation in non-hemolytic indirect hyperbilirubinemia. Methods　A female patient was diagnosed with type I Crigler-Najjar syndrome (CNS-I) after excluding hemolysis and hypothyroidism. Phototherapy treatment is effective while oral phenobarbital was not. UGT1A1 were amplified by polymerase chain reaction and DNA was sequenced. Results　The patient was compound heterozygote of c.1070A>G p. (Gln357Arg) and 1091C>T p. (pro364Leu) and diagnosed with CNS-I. Heterozygotes of the mutation were found in her parents. Conclusions　In patients highly suspected of CNS, the genetic tests should be carried out as soon as possible.

The progress of research in association of inflammatory and febrile seizures with secondary epilepsy

Dong Na

. 2015, 33(10):  896.  doi:10.3969 j.issn.1000-3606.2015.10.014

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Febrile seizure (FS) is the most common type of seizures in infants, toddler, and preschool children. Inflammatory mediators as triggers of fever are considered to be involved in the occurrence of such seizures. There is evidence that FS is accompanied by inflammation. The potential role of inflammatory mediators in the development of epilepsy after long term FS has not been fully determined. In this article the inflammatory reaction, febrile convulsion, and the occurrence of secondary epilepsy will be reviewed. The progress in research of the interaction among them at home and abroad will be explored.

The clinical efficacy and adverse reactions of ketogenic diet therapy in children with refractory epilepsy

BAI Shiying, MA Yanhong

. 2015, 33(10):  900.  doi:10.3969 j.issn.1000-3606.2015.10.015

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The ketogenic diet (KD) is a special diet with high-fat, low-carbohydrate and moderate amount of protein. The KD therapy is a safe, potentially effective and important treatment of refractory childhood epilepsy in recent years. It has confirmed clinical efficacy and a generally good prognosis. The adverse reactions of KD treatment are normally less and transient. In this paper, the effective rate, patient compliance, adverse reactions and their causes of KD in treating children with refractory epilepsy were reviewed.

Progress of research in neonatal sepsis from necrotizing enterocolitis　

YANG Kaidi

. 2015, 33(10):  903.  doi:10.3969 j.issn.1000-3606.2015.10.016

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Necrotizing enterocolitis (NEC) is one of the severe gastrointestinal emergencies with high mortality in neonatal period. Studies suggested that sepsis from NEC were associated with higher mortality in NEC. Therefore, it is important to explore the related factors and prevention measures of NEC and improve the prognosis of children with NEC. In recent years, studies have shown that the risk factors of sepsis in NEC included prematurity, low birth weight, long time of total parenteral nutrition supports and long duration of antibiotic exposure. The main pathogen of sepsis was gram-negative bacilli. Probiotics, glutamine and lactoferrin supplementation may reduce the incidence and severity of NEC, and may help further reduce the risk of gut-derived sepsis.

The mechanisms of carbapenem resistance in Klebsiella pneumoniae

QIAO Xiaoyu

. 2015, 33(10):  907.  doi:10.3969 j.issn.1000-3606.2015.10.017

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Klebsiella pneumoniae is well known as a causative agent of both community and nosocomial infections and is generally believed to be the cause of 10% of the nosocomial infection. Recently, with the widespread using of carbapenem, the isolation of carbapenem-resistant strains has been greatly increased, which bring great difficulties and challenges in clinical treatment. In this article, the progresses in the mechanisms of carbapenem, resistance in Klebsiella pneumoniae such as the acquisition of carbapenemases, hyperproduction of AmpC cephalosporinases or extended spectrum beta-lactamases (ESBLs) in combination with loss of the outer membrane protein, efflux pump system, and biofilm were reviewed.