Journal of Clinical Pediatrics

Awareness of septic acute kidney injury in children #br#

YI Zhuwen,ZHANG Hui

. 2014, 32(6): 501-503.

Abstract ( 326 )

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This paper describes the clinical epidemiologic features, main pathogenesis, early diagnostic biomarkers and managements of septic acute kidney injury in children. It is suggested that pediatric clinicians should increase awareness of the treatment of septic acute kidney injury in children.

The expression and clinical significance of toll-like receptor (TLR) 4 and TLR7 on renal tissue in children with primary nephrotic syndrome

ZHANG Fangmin,HAN Ziming,ZHAO Dean

. 2014, 32(6): 512-516.

Abstract ( 429 )

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　Objective　To study the expression and clinical significance of toll-like receptor (TLR) 4 and TLR7 in primary nephrotic syndrome (PNS) of different pathological types in children. Methods Renal expressions of TLR4 and TLR7 were amined and analyzed retrospectively in renal biopsy specimens from 110 PNS patients and 21 healthy controls by immunohistochemical method. According to the renal pathologic classification of PNS, the TLR4 and TLR7 expression levels in PNS of different types were compared. Results Compared with the renal tissue of healthy controls, the expression level of TLR4 on renal tissue of PNS patients was significantly increased (P<0.01). Among MN, MsPGN and FSGS, the highest expression of TLR4 was observed in MCD (P<0.01). Compared with the renal tissue of healthy controls, the expression level of TLR7 in renal tissue of PNS patients was also significantly increased (P<0.01). Among MCD, MN and FSGS, the highest expression of TLR7 was observed in MsPGN (P<0.01). Conclusions TLR4 and TLR7 expression levels are increased in renal tissue of PNS patients and the expression levels may be correlated with renal pathological types.

Combination of urine neutrophil gelatinase lipocalin associated lipocalin, kidney injury molecular-1 and interleukin-18 in the diagnosis of acute kidney injury in children after cardiopulmonary bypass

LIU Huajie,WANG Ping,SHEN Yunlin,LI Xiaobing,ZHANG Rufang,SUN Lei,KUANG Xinyu,HUANG Wenyan

. 2014, 32(6): 517-523.

Abstract ( 288 )

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　Objective　To investigate the values of urine neutrophil gelatinase associated lipocalin (NGAL), kidney injury molecular-1 (KIM-1) and interleukin-18 (IL-18) in the diagnosis of acute kidney injury (AKI) in children after cardiopulmonary bypass (CPB). Methods Sixty-seven patients who had undergone CPB were recruited from March to June 2013 and assigned to acute kidney injury group (AKI group) or non-acute kidney injury group (non-AKI group) according to the pediatric RIFLE (pRIFLE) criteria. Serum and urine samples were collected from each patient at 30 min, 2 h, 4 h, 24 h, 48 h and 72 h after CPB for serum and urine creatinine, urine NGAL, KIM-1 and IL-18. All the data were evaluated by receiver operator characteristic curve (ROC) analysis and area under curve (AUC) analysis. Results Twenty-three cases (34.3%) had AKI in 67 children after CPB. Among them 15 cases were risk-stage AKI, 4 cases injury-stage AKI, 3 cases failure-stage AKI and 1 cases loss-stage AKI. The levels of urine NGAL/Ucr were higher in AKI group than those in non-AKI group at 4h, 48h and 72h after CPB (P<0.05). The cut-off value of NGAL/Ucr was 1.200 at 4 h after CPB, the sensitivity and specificity for prediction of AKI were 0.864 and 0.561, and the AUC was 0.671 (95%CI: 0.537-0.804). The levels of urine KIM-1/Ucr were higher in AKI group than those in non-AKI group at 48h and 72 h after CPB (P<0.05). The cut-off value of KIM-1/Ucr was 1.162 at 24h after CPB, the sensitivity and specificity for prediction of AKI were 0.773 and 0.512, and the AUC was 0.698 (95%CI: 0.563-0.834). The levels of IL-18/Ucr were higher in AKI group than those in non-AKI group at 4 h after CPB (P<0.05). The cut-off value of IL-18/Ucr was 0.04 at 4 h after CPB, the sensitivity and specificity for prediciton of AKI were 0.773 and 0.561, and the AUC was 0.655 (95%CI: 0.510-0.800). Conclusions It is indicated that urine NGAL, KIM-1 and IL-18 may have important clinical values for early prediction of AKI.

The relationship between meteorological parameters and the prevalence of Moraxella catarrhalis infection in children hospitalized with acute respiratory infection in Suzhou　

SUN Huiming,ZHOU Weifang,JI Wei,YAN Yongdong,CHEN Zhengrong,TAO Yunzheng

. 2014, 32(6): 524-527.

Abstract ( 319 )

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　Objective　To explore the correlation of meteorological parameters with the epidemic of acute Moraxella catarrhalis respiratory infection in hospitalized children in Suzhou. Methods A total of 8143 children with acute respiratory infection were participated in the trial during 2006 to 2010, and the secretions of nasopharynx were collected for bacterium culture. Moraxella catarrhalis was identified according to the routine technique of culture. Meteorological parameters including mean temperature, relative humidity, rainfall amount, duration of sunshine and mean wind velocity were collected monthly during the same period. The relationship between the epidemic of Moraxella catarrhalis and metrorological parameters were analyzed by seasonal decomposition method, the Spearman rank correlation and stepwise regression analysis. Results Moraxella catarrhalis was identified in 4.04% of 8 143 specimens. The prevalence of acute Moraxella catarrhalis respiratory infection was higher during winter and spring. The monthly infection rate of Moraxella catarrhalis was negatively correlated with mean temperature as well as duration of sunshine and wind velocity. Wind velocity was independent risk factor for Moraxella catarrhalis infection. Conclusions Moraxella catarrhalis is a primary pathogen in respiratory tract infection in children in Suzhou. The epidemic of Moraxella catarrhalis is closely related to meteorological parameters.

Evaluations of the clinical pathway in children with Mycoplasma pneumoniae pneumonia　

ZHU Lili,ZHANG Hailin,WEN Shunhang,ZHANG Yan,LI Changchong

. 2014, 32(6): 528-531.

Abstract ( 459 )

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　Objective　To investigate the efficacy of the clinical pathway introduced in children with Mycoplasma pneumoniae pneumonia (MMP). Methods Based on a retrospective study, the length of hospital stay, hospital expenses and curative rate were compared between 145 MMP patients managed according to clinical pathway and other 45 MMP patients. The causes of variation were analyzed in the clinical pathway group as well. Results The length of hospital stay in clinical pathway group [9 (6~10) days] was significantly shorter than that in the control group [10 (7-12.5) days] (P=0.003). The curative rate (93.8%) was significantly higher than that in the control group (84.4%) (P=0.043). The hospital expenses [4 696.5 (3 608.3-5 677.6) CNY] was significantly higher than that in the control group [3175.3 (2490.8-4585.0) CNY] (P<0.001). The variation rate of clinical pathway was 48.3% (70/145 cases) in clinical pathway group. Conclusions The curative rate is improved and the length of hospital stay is shortened after the clinical pathway is introduced in MMP children. However, there is a high variation rate in the clinical pathway. It is necessary to optimize the clinical pathway before it is adapted in clinic.

Follow-up study of 1035 children with recurrent wheezing #br#

WEI Lin,ZHANG Yue,JIN Huidi,SAN Beilan,CHEN Jie,YU Jiong,XU Mengbei

. 2014, 32(6): 532-535.

Abstract ( 373 )

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　Objective　To investigate the long-term outcome of children with recurrent wheeze and to determine the effectiveness of inhaled hormone therapy. Methods One thousand and thirty-five children with recurrent wheezing were followed up for more than 4 years and the data were retrospectively evaluated. Results Of 1035 cases, 751 (72.56%) patients outgrew their wheeze during the follow-up period, whereas the other 284 (27.44%) patients had recurrence wheeze during the last two years. The age of wheezing onset was < 3 years in 542 (52.37%) cases, from 3 to 7 years in 386 (37.29%) cases, and from 7 to 12 years in 107 (10.34%) cases. There was significant difference in clinical control rate among groups with different wheezing ages onset (χ²=45.27, P<0.001). Children with wheezing age onset from 7 to 12 years had the lowest clinical control rate. Among 1035 wheeze children, 343 (79.95%) children in 429 cases who received inhaled hormone therapy for more than one year outgrew their wheeze. Whereas 408 (67.35%) in 606 cases who did not receive inhaled hormone therapy outgrew their wheeze. There was significant difference of clinical control rate between inhaled group and non-inhaled group (P<0.01). Conclusions The age of wheezing onset is < 7 years in 89.66% of children with recurrent wheeze. Most of them can be clinicalycontrolled. The long term inhaled hoemone therapy for children with recurrent wheeze can reduce the risk of developing adulthood asthma.

The expression of JC virus T-antigen in colorectal juvenile polyps in children

ZHANG Lina,PAN Jian,JIN Yu

. 2014, 32(6): 536-539.

Abstract ( 341 )

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　Objective　To investigate the relationship between Jamestown Canyon virus (JCV) infection and colorectal juvenile polyps in children. Methods　PCR and immunohistochemical staining were performed to detect JCV DNA sequences and localize T-Ag expression in 35 colorectal juvenile polyps and 23 normal colorectal mucosa. Results　The positive rate of JCV gene sequences was 31.4% (11 of 35) in the colorectal juvenile polyps and 8.7% (2 of 23) in the normal colorectal mucosa (P<0.05). T-Ag protein was expressed in 22.9% (8 of 35) of the colorectal juvenile polyps, but none in the normal colorectal mucosa (P<0.05). Gender, age, and the number, location, and size of the colorectal juvenile polyps were not significantly different between the children with JCV-positive and JCV-negative polyps (P>0.05). Conclusions　This study suggests a positive association between JCV infection and colorectal juvenile polyps in children, indicating that JCV infection may be a risk factor for colorectal juvenile po-lyps in children.

Determination of alkylglycerol contents in breast milk　

QIAN Linxi,SONG Huanlei,ZHENG Tao,ZHONG Yan,YU Wenjuan,WU Shengmei,CAI Wei,

. 2014, 32(6): 540-543.

Abstract ( 428 )

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　Objective　To determine alkylglycerol (AKG) contents and variation in breast-milk of lactating women. Methods Five cases of healthy lactating women with term delivery were selected from June 2011 to June 2012. Breast-milk samples were collected at 1, 2, 3, 4, 8, 12, 16, 20 and 24 weeks postpartum. Breast milk samples were extracted, saponificated and derivatized. AKGs composition in breast-milk was quantitatively analyzed by GC chromatography. Results Mean 16C:0 AKG content in breast-milk decreased from （17.31±3.59）×10^-3g/L to （11.14±1.83）×10^-3g/L. Mean 18C:0 AKG content decreased from （14.95±6.00）×10^-3g/L to （9.68±2.51）×10^-3g/L. Mean 18C:1 AKG content fluctuated between （4.64±0.91）×10^-3g/L and （3.95±0.68）×10^-3g/L. Conclusions 16C:0, 18C:0 and 18C:1 AKG contents exist in Chinese breast-milk through determination by GC chromatography, and the concentrations vary among different stages of lactation.

Analysis of abnormal Hepatitis B virus serological markers in neonatals　

SU Wei,FENG Huiqing

. 2014, 32(6): 544-546.

Abstract ( 278 )

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Objectives To investigate the outcomes of neonatals with abnormal hepatitis B virus (HBV) serological markers. Methods Twenty-eight neonatals who had abnormal HBV serological markers and whose mothers had positive HBsAg, HbeAg and HbcAb were studied. Among them, 21 neonatals who had positive HBsAg, HbeAg and HbcAb were included in vertical transmission group; 7 neonatals who had positive HbeAg, HbcAb and negative HBsAg were included in suspicious group. The quantities of HBV serological markers were determined by time-resolved immunofluorometric assay (IFMA) and the level of HBV DNA were measured by fluorescence quantitative PCR (FQ-PCR). Results Serum HBeAg and HBsAg levels in suspicious group were lower than those in vertical transmission group (P<0.05). The results showed that HBeAg level (0.55±0.19 PEIU/ml) three months after birth was lower than that (4.02±2.00 PEIU/ml) 7 days after birth in suspicious group (P<0.05). Conclusions Early interference has positive effect in mothers and neonatls with abnormal HBV serological markers.

Relationship between single nucleotide polymorphism on FTO gene rs9939609 and obesity in children

WU Yanming,
LI Weiguo,SHENG Qiuming,DAI Jinsheng,ZHANG Lingjun,WU Min,GONG Huijun

. 2014, 32(6): 547-550.

Abstract ( 347 )

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Relationship between single nucleotide polymorphism on FTO gene rs9939609 and obesity in children　WU Yanming, LI Weiguo, SHENG Qiuming, DAI Jinsheng, ZHANG Lingjun, WU Min, GONG Huijun (Department of Pediatrics, Shanghai Pudong New Area People’s Hospital, Shanghai 201200, China)#br#Objective To investigate the relationship of single nucleotide polymorphism (SNP) on FTO gene rs9939609 with metabolism index and obesity in children. Methods One hundred and fifty-three children (age 7-11 year) were recruited in this study, 102 of them were obese and 51 of them were overweight. One hundred and sixty children with normal body weight were recruited as control. Height, weight and biochemical indicator of liver function were measured. PCR and direct sequence were applied to detect the polymorphism of rs9939609, and the frequency of the allele was calculated. Results TT or TA/AA genotype frequency on FTO gene rs9939609 was significantly different among overweight group, obesity group and normal control group (χ²=23.01, P<0.001); TA/AA genotype frequency in overweight and obesity group was significant higher than that in the control group（P<0.014）. The frequencies of T and A allele in overweight group, obesity group and control group was 96.25% and 3.75%, 85.29% and 14.71%, 85.78% and 14.22% respectively. There was significant difference of allelic frequency among overweight group, obesity group and control group (χ²=21.72, P<0.001). The frequency of A allele in overweight and obesity group was higher than that in control group (P'<0.014). Subjects with TA/AA allele had significantly higher BMI compared with subjects with TT allele. Conclusions rs9939609 of FTO gene is associated with obesity in children, and allele A on this spot may raise BMI and leads to overweight and obesity.

Investigation of social competence and temperament type in preschool children in Shanghai Chongming district

DING Tao,NI Junyi,XU Jueping,SHEN Jin,YE Guiyun,CHEN Liu,LIU Xiaoqing

. 2014, 32(6): 551-554.

Abstract ( 323 )

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　Objective　To investigate current status of preschool children's social competence and its relation to temperament type. Methods A total of 1 251 children participated in this study. The questionnaire was conducted and the children were assessed using "infant-junior middle school student's ability of social life scale" and "children's temperament scale". Results No significant gender difference was observed in total score of social competence (P>0.05). The total score of live independently was higher in girls as compared to boys (P<0.05). A significant gender difference was observed in classified scores of social competence (P<0.05). Girls with excellent or better-than-normal social competence were more than boys (P<0.05). The distribution of the temperament type in preschool children were the difficult-to-raise type (8.1%), the start-slow type (15.4%), the standard type (69.0%) and the easy-to-raise type (7.5%). There was statistically gender significance (P<0.05) in the distribution of the temperament types. The percentage of difficult-to-raise type was higher in girls than in boys. The percentage of easy-to-raise type was higher in boys than in girls. The total score and classified scores of social competence had significant difference among children with different temperament types (P<0.05). Conclusions There is significant diffe-rence of social competence in children with different temperament types. Corresponding educational measures according to the child's temperament may be benefit to the development in preschool children.

Clinical features and antibiotic resistance of 54 children with invasive pneumococcal disease

ZHANG Xiaolong,LUO Zhengxiu,FU Zhou,LUO Jian,LIU Enmei

. 2014, 32(6): 555-558.

Abstract ( 383 )

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　Objective　To investigate the clinical features of invasive pneumococcal disease (IPD) and the antimicrobial susceptibility of invasive Streptococcus pneumoniae (Sp). Methods The clinical features of 54 children with IPD and the antimicrobial susceptibility of 54 invasive Sp isolates were retrospectively analyzed from 2009 to 2012. Results The ratio of boys to girls in IPD was 1.35:1. Most of the children with IPD were within 2 years old. Few of the children had the onset in summer. Most of the children were diagnosed with septicemia, followed by meningitis. All of the children had middle or high fever. Forty-four children had an increased white blood cell count and 42 children had an increased C-reactive protein. β-lactam antibiotics was most frequently used in clinics, followed by vancomycin. Most of invasive Sp were resistant to penicillin. Multiple drug resistance was common in invasive Sp. Conclusions The incidence of IPD has seasonal difference. The clinical manifestations of IPD are diverse, with septicemia being the most common. The resistant rate to penicillin is high in invasive Sp.

Retrospective analysis the effect of linezolid on severe Gram-positive bacteria infection in children

JUAITI Seyiti,CUI Yunzhi,DUOLIKUN Muzhapaer

. 2014, 32(6): 559-561.

Abstract ( 276 )

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　Objective　To investigate the clinical effect of linezolid on severe Gram-positive bacteria infection in children. Methods The retrospective analysis was performed in the clinical cases of severe Gram-positive bacteria infection which were insensitive to drugs selected from experience but sensitive to linezolid according to sputum culture and sensitivity test. Results Twenty-four children with severe Gram-positive bacterial infections were treated with linezolid by intravenous infusion (10 mg/kg, q8h) for 10-14 days. All of them were markedly improved. Conclusions When drugs selected from experiences are ineffective, linezolid or in combination with other drugs is a good choice in the treatment of severe Gram-positive bacterial infections.

Clinical analysis of antibiotic associated diarrhea in children with severe bacterial pneumonia　

BAO Liansheng,ZHANG Zhen,ZHANG Duo

. 2014, 32(6): 562-563.

Abstract ( 294 )

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　Obiective　To study the clinical characteristics and therapeutic measures of the antibiotic associated diarrhea (AAD) in children with severe bacterial pneumonia. Methods The information of antibiotic use history in children with severe bacterial pneumonia from January 2013 to June 2013 was retrospectively analyzed. Results The incidence rate of AAD was 70.59% in children with severe bacterial pneumonia. According to the frequency, the third-generation cephalosporins, penicillin and carbapenems were the major causes of AAD. The percentage of the age ≤ 3 y, the duration of antibiotic usage> 7 d and the number of antibiotics ≥2 were significantly higher in AAD children than in non-AAD children (χ²=89.81~119.45, P<0.001). All the patients were improved after cessation or change of antibiotics. Conclusions The incidence of AAD in children with severe bacterial pneumonia is high, which is related to the age, the duration of antibiotic usage and the combination use of antibiotics.

The establishment of a hypoxic-ischemic brain damage model in preterm fetal rabbits

NAN Yan,TANG Zhenhai,WANG Nengli,LIU Yanli,YE Wei,LIN Jin,LING Zhenlang

. 2014, 32(6): 564-569.

Abstract ( 319 )

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　Objective　To establish an appropriate preterm hypoxic-ischemic brain injury animal model. Methods A total of 32 pregnant New Zealand white rabbits at gestational day 25 were selected. The uterine blood supply in pregnant rabbits was blocked for 30, 35, 37, 40 minutes respectively, while in the control group it was not blocked. Then the pregnant rabbits were subjected to cesarean section 24 hours (at embryonic day 26, A group) or 5 days (at embryonic day 30, B group) after the experimental procedure. The general conditions of the newborn rabbits were recorded. The degree of neurobehavioral impairment in newborn rabbits was evaluated. The histological changes of brain tissue were observed. Results　In A group, all newborn rabbits survived with ischemia for 30 minutes, while the stillbirth rates increased from 31.0% to 100% with ischemia from 35 to 40 minutes. In survived nowborn rabbits, the brain water content and the number of apoptotic brain cells were increased with prolonged ischemia. All these differences were statistically significant (all P<0.05). In B group, the stillbirth rates increased to 50.0% and 65.7% respectively with ischemia for 35 or 37 minutes. The birth weight of survived newborn rabbits were significantly lower than that in the control group. The neurobehavioral test scores were significantly lower in ischemic groups than that in the control group. All these differences were statistically significant (all P<0.05). The pathological examination of brain tissue found that the white matter damage in B group was more obvious than that in A group. Conclusions Continuous blockage of uterine blood supply in pregnant rabbits at gestational day 25 causes stillbirth, neurobehavioral damages and white matter injury as well as fetal rabbit intrauterine growth restriction, which can be used for the preparation of preterm hypoxic-ischemic brain injury animal model.

Inhibition of viral myocarditis by Astragaloside through IL-23/IL-17 signaling pathway　

LIU Danli,LIU Haiying,GAO Shunli

. 2014, 32(6): 570-573.

Abstract ( 517 )

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　Objective　To explore the role of interleukin-23 (IL-23)/interleukin-17 (IL-17) signaling pathway in viral myocarditis (VMC) and evaluate the intervention effect of Aastragaloside. Methods Seventy-five male BALB/c mice were randomly divided into 4 groups, control group (n=15), model group (n=20), low-dose intervention group (n=20) and high-dose intervention group (n=20). Mice in control group were inoculated with 0.1 ml virus cultivation solution intraperitoneally while mice in the other 3 groups were treated with 0.1ml virus cultivation solution containing 1×10² TCID50 coxsackievirus B₃ (CVB₃) to establish VMC model. On the day of inoculation, mice in low- and high-dose intervention groups were intragastrically administered with 0.1 ml of 1% and 9% Astragaloside solution respectively, whereas those in control and model groups were treated with 0.1 ml carboxymethycellulose solution. Astragaloside or carboxymethycellulose was given once a day and continued 15 days. The number of mice death and the performance of mice were recorded in experimental period. All mice were sacrificed on day 15. The heart and blood sample were obtained. Histological cross sections of heart were stained with hematoxylin-eosin and scored for myocardial histopathologic changes under optical microscope. Th17 cells were analyzed by flow cytometry. The mRNA and protein expression levels of myocardial IL-23 and IL-17 were detected by real-time quantitative PCR and Western blotting, respectively. Results The mortality was statistically significant differences among the four groups (P = 0.013), which was the lowest in the control group. The myocardial histopathologic scores, the percen-tage of Th17 cells, as well as expression levels of myocardial IL-23 and IL-17 mRNA and protein were significantly lower in high-dose intervention group than those in model group and low-dose intervention group, but higher than those in control group (P < 0.05). The myocardial histopathologic scores, the percentage of Th17 cells, as well as expression levels of myocardial IL-23 and IL-17 mRNA and protein were significantly higher in model group and low-dose intervention group (P < 0.05). There were no significant difference in the above mentioned indicators between low-dose intervention group and model group (P > 0.05). Conclusions Astragaloside may dose-dependently protect against VMC by inhibiting IL-23/IL-17 signaling pathway.

Analysis of causative gene mutations in one child with primary gout　

LIU Shuping,YAO Yong,YE Jintang,WANG Su-xia,DING Jie,LI Qian,ZHANG Yanqin,WANG Fang

. 2014, 32(6): 574-578.

Abstract ( 397 )

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　Objective　To improve the knowledge of primary gout in children. Methods Clinical data of a 12-year-old girl with primary gout was collected. Analysis of UMOD gene, REN gene and HNF-1β gene was performed using PCR and direct sequencing. Results The girl was admitted for 1-month history of left hallux pain accompanied with elevations of serum uric acid concentration and serum creatinine concentration. Several examinations showed serum uric acid/creatinine ratio was greater than 2.5. The fractional excretion of uric acid was 3.4%-6.6%. The X-ray showed that the proximal phalanxes of halluces were erosion. The diagnosis of renal biopsy was ischemic renal injury and chronic tubulointerstitial nephropathy. Blood uric acid concentrations of parents were normal, and the family history of gout was negative. Two single nucleotide polymorphisms (c.264C>T heterozygous and c.866-71 G>A heterozygous) in UMOD gene, 1 single nucleotide polymorphism (c.373+44C>G heterozygous) in REN gene, and 2 single nucleotide polymorphisms (c.100-50-49ins TCTG heterozygous and c.781-22T>C homozygous) in HNF-1β gene were detected. No pathological mutation was detected in these 3 genes. Conclusions This child is highly suspected to have primary gout caused by familial juvenile hyperuricemic nephropathy.

Clinical features and aCGH analysis of one child with Phelan-McDermid syndrome #br#

ZHANG Hongyun,WANG Xi

. 2014, 32(6): 579-582.

Abstract ( 379 )

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　Objective　To analyze the clinical features and results of array-comparative genomic hybridization (array CGH, aCGH) in a Chinese girl with Phelan-McDermid syndrome. Methods The clinical symptoms of a child with Phelan-McDermid syndrome were retrospectively analyzed. Routine G-banding was performed to analyze the karyotype, and the aCGH was used to analyze subchromosomal abnormalities. Results The routine karyotype analysis showed a normal female karyotype without abnomalities in chromosome number and structure. aCGH analysis finely mapped the deletion of Chr22q13.2-qter. Phelan-McDermid syndrome was diagnosed for this case. Conclusions Phelan-McDermid syndrome can be diagnosed by the typical and detailed clinical features in combination with the laboratory examinations of subchromosomal abnormalities. aCGH is one of the most valuable methods to analyze subchromosomal abnormalities and to diagnose Phelan-McDermid syndrome.

Four cases report of Jeavons syndrome in childhood

XU Zhefeng,LIU Zhanli,HUANG Xianmei,JIANG Chunming,CHEN Liqiong

. 2014, 32(6): 583-586.

Abstract ( 473 )

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　Objective　To explore the clinical manifestations, electroencephalographic characteristics and therapeutic effect of drugs in children with Jeavons syndrome. Methods The clinical and electroencephalographic characteristics and therapeutic effect of drugs were analyzed in 4 children with Jeavons syndrome. Results Among the four children there were 3 female and 1 male. The age at the onset of the disease was from 1 to 6 years. The typical clinical manifestations of this disease were brief, fast and repeated eyelid myoclonia (EM) with or without absence seizure. The typical electroencephalography (EEG) in two patients showed 3-6 Hz generalized spike and waves and polyspikes burst, and the eye closure and intermittent photic stimulation helped to induce discharges and clinical events. The typictal EEG in the other two patients showed 3.0-3.5 Hz generalized δ slow wave rhythm burst. The drugs of choice for treatment was sodium valproate monotherapy in two cases, levetiracetam in one case, sodium valproate combined with levetiracetam in one case. During the follow-up, seizures were controlled in one case, decreased in frequency in two cases and were still frequent in one case. Conclusions Jeavons syndrome is one of the idiopathic and generalized epileptic syndromes and characterized by EM with or without absence seizure. Video EEG monitoring plays an important role in the diagnosis of this disease. Sodium valproate and levetiracetam were effective for this disease.

The progress in examinations of functional constipation in children #br#

Reviewer：ZHANG Liangjuan,Reviser：WANG Baoxi

. 2014, 32(6): 587-590.

Abstract ( 360 )

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Functional constipation is a common and frequently occurring disease in childhood. In addition to meeting the clinical diagnostic criteria for constipation and performing the general routine examinations, there are the need to have further examinations such as colonic transit time measurement, anorectal manometry and colonoscopy in order to rule out other causes. Therefore, the purpose of this paper is to introduce several latest research progress in examinations related to constipation in children and to provide some gui-dance and references for clinicians.

Hematological abnormality in inherited metabolic diseases　

HUANG Lizhen,WU Jinlin,QU Yi,

. 2014, 32(6): 591-594.

Abstract ( 342 )

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There are a variety of inherited metabolic diseases (IMD), the incidence is low and clinical manifestations are not characteristic. IMD primarily affect not only the nervous system, but also the blood system, which characterized by the abnormalities of blood cells and bone marrow. Because of some clinician's lack of this knowledge, some patients are prone to be misdiagnosed. To promote early diagnosis, this article reviews five groups of IMD (lysosomal storage disease, vitaminopathies, organic aciduria, aminoacidopathies and others) and different hematological abnormal manifestations.

Progress in biological agents for treatment of Kawasaki disease

　LI Zhuoying,YANG Zuocheng

. 2014, 32(6): 595-598.

Abstract ( 505 )

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Kawasaki disease (KD) is a systemic vascular inflammatory lesion in children with acute febrile rash illness. The incidence rate in recent years is increasing. The traditional treatment in some patients is ineffective, and KD has high incidence of cardiovascular complications. Therefore, the selection of an appropriate therapy for KD is an urgent. With the continuous advance of biomedical and pharmacologically research, many biological agents were developed. The traditional treatment has been gradually replaced by the new biological agents which achieved the desired results. Thus this paper reviewed several common biological agents in the clinical treatment of KD.

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