Table of Content

15 April 2015 Volume 33 Issue 4

  

Inborn errors of bile acid synthesis

DAI Dongling

. 2015, 33(4):  301.  doi:10.3969 j.issn.1000-3606.2015.04.001

Abstract ( 514 )   PDF (1280KB) ( 901 )  

Related Articles | Metrics

Inborn errors of bile acid synthesis caused by enzyme defects are inherited metabolic rare diseases and mostly belong to the autosomal recessive hereditary diseases. They are clinically manifested as progressive cholestasis liver disease, neurological disorders, and fat-soluble vitamin malabsorption. The progressive cholestasis liver disease is characterized by conjugated hyperbilirubinaemia with raised transaminase, but normal γ-glutamyl transpeptidase (γ-GT), and a biopsy specimen shows giant cell hepatitis. The neurological disorders usually present with childhood-onset or adult-onset spastic paraplegia. Early diagnosis is important because oral administration of bile acids is effective for two disorders above. This article reviews pathophysology, clinical features and various enzyme defects of inborn errors of bile acid synthesis.

The risk factors of childhood functional constipation　

HU Hui, XIAO Yongmei, ZHANG Ting

. 2015, 33(4):  306.  doi:10.3969 j.issn.1000-3606.2015.04.002

Abstract ( 465 )   PDF (1241KB) ( 564 )  

Related Articles | Metrics

Objective　To study the risk factors of childhood functional constipation. Methods　A cross-sectional analysis of risk factors associated with 182 cases of functional constipation in children was conducted from March 2013 to February 2014. Results　The male patients were more than females, mainly in preschool children. The risk factors were did not like fruits and vegetables (52.2%), drank less water (44.0%), did not develop the habit of daily bowel movement (39.5%), defecation fear (36.3%), inadequate food intake (25.8%). There were different risk factors in different age stages. Conclusions　Functional constipation is related to gender, age, diet habits and mental psychology.

Congenital chloride diarrhea: one case report　

ZHANG Wen,LI Xihong

. 2015, 33(4):  309.  doi:10.3969 j.issn.1000-3606.2015.04.003

Abstract ( 410 )   PDF (1285KB) ( 534 )  

Related Articles | Metrics

　Objective　To discuss the clinical diagnosis, treatment and genetic diagnosis of congenital chloride diarrhea (CCD), a rare autosomal recessive disease. Methods One month old boy with persistent diarrhea, hypochloremia, hyponatremia, hypokalemia and metabolic alkalosis, his stool electrolyte testing, clinical treatment and follow-up, as well as his and his parents’ SLC26A3 gene mutation analysis were retrospectively analyzed. Results The fecal electrolyte testing showed that the levels of Cl- and K+ were increased and the level of Cl- was much higher than the sum of Na+ and K+. After replacement therapy with NaCl and KCl, the blood electrolyte recovered to normal. Follow-up 4 years, the boy had a normal growth and development. Mutation analysis on SLC26A3 gene showed there was a homozygous mutation of 239G>A and both his father and mother carried the same heterozygous mutation. This mutation was first discovered in China. Conclusions The sequencing analysis of SLC26A3 mutation may help to diagnosis CCD.

Clinicopathologic features of intestinal dysganglionosis in children　

SHEN Wuming, WU Xiangru, YIN Minzhi, MA Jing, LIANG Xin, SHI Chengren, ZHANG Zhongde

. 2015, 33(4):  312.  doi:10.3969 j.issn.1000-3606.2015.04.004

Abstract ( 532 )   PDF (2168KB) ( 385 )  

Related Articles | Metrics

Objective　To investigate the clinical and pathological features of Hirschprung disease (HD), intestinal neuronal dysplasia (IND) and hypoganglionosis (IH) in children. Methods　The clinical data and pathologic slices from 238 children with intestinal dysganglionosis were retrospectively analyzed. The age, sex, involved intestinal length of children and prognosis were compared. Results　In 238 patients, 138 (58.0%) were diagnosed by rectal mucosal biopsies. There were 122 HD patients whose median age at diagnosis was 9 months and the ratio of male to female was 4.3:1, without involvement of whole colon. There were 45 IND patients whose median age at diagnosis was 14 months and the ratio of male to female was 1.05:1, and the whole colon of 33.3% patients was involved. There were two male IH patients whose ages at diagnosis were 12 years and 18 years respectively, and their whole colon was involved. There were 59 patients with HD complicated by IND whose median age at diagnosis was 13 months and the ratio of male to female was 5.56:1 and the whole colon of 16.9% patients was involved. There were 10 male patients with HD complicated by IH whose median age at diagnosis was 11.5 months and the whole colon of 80.0% patients was involved. The ages at diagnosis, the sex ratio, the rates of whole colon involved, and the cure rates among 5 groups were significantly different (all P<0.01). Conclusions　The rectal mucosal biopsy was the main method in diagnosis of intestinal dysganglionsis in children. Patients with HD had higher incidence and mild condition and favorable prognosis. Patients with IH or patients with HD complicated by IH had lower incidence rates and severe condition and poor prognosis, followed by patients with IND or patients with HD complicated by IND.

The assessment of cardiovascular function in obesity children

WANG Hongping, LI Yun, GAO Yan

. 2015, 33(4):  316.  doi:10.3969 j.issn.1000-3606.2015.04.005

Abstract ( 427 )   PDF (1325KB) ( 360 )  

Related Articles | Metrics

　Objectives　To assess the cardiovascular function in obese children. Methods　A total of 30 obese children and 50 age- and sex-matched normal weight children were enrolled and treadmill test was performed. Exercise tolerance, the time to target heart rate and continuous T-wave alternans (TWA) during treadmill test were compared between two groups. Results 　Compared with normal weight children, the exercise tolerance was decreased and the time to target heart rate was shortened, while the incidence of continuous TWA was increased in obese children and there was significant difference between two groups (P<0.01). Conclusions　Obesity may lead to cardiovascular dysfunction in childhood which should be concerned.

Related factors analysis of obstructive hydrocephalus secondary to intracranial hemorrhage in premature infants

　LI Bing, ZHANG Qian, SHI Zanyang, CHENG Xinru

. 2015, 33(4):  319.  doi:10.3969 j.issn.1000-3606.2015.04.006

Abstract ( 471 )   PDF (1304KB) ( 12728 )  

Related Articles | Metrics

Objective　To explore the risk factors of obstructive hydrocephalus secondary to intracranial hemorrhage in premature infants. Methods　A total of 304 premature infants were selected who were diagnosed as sever intracranial hemorrhage (grade III and IV) by cranial bedside ultrasound admitted to our hospital from Jun. 2013 to Sep. 2014. According to whether the obstructive hydrocephalus was followed, all infants were divided into hydrocephalus group (n=59) and non-hydrocephalus group (n=185). The risk factors of obstructive hydrocephalus secondary to intracranial hemorrhage were analyzed and the lateral ventricle size was measured dynamically. Results　The univariate analysis showed the factors related with obstructive hydrocephalus were as follows: gestational age ≤ 32 weeks, birth weight < 1500g, severe asphyxia, cesarean section, RDS, neonatal infection, heart failure, PDA, acidosis, thrombocytopenia, coagulation abnormalities, and intracranial hemorrhage (grade Ⅲ or Ⅳ) (all P<0.05). Multivariate logistic regression analysis showed that acidosis, thrombocytopenia, coagulation abnormalities, gestational age ≤ 32 weeks, severe asphyxia, intracranial hemorrhage (grade Ⅲ or Ⅳ ) were independent risk factors for obstructive hydrocephalus (OR: 1.76~20.46, all P<0.05). At each time point after birth, the ratio of posterior horn of lateral ventricle was significantly higher in hydrocephalus group than that in non-hydrocephalus group (P<0.05). There were significant differences in the changes of the posterior horn ratio of left or right lateral ventricle with time in hydrocephalus group (P=0.000), increasing at 14 days and reaching the peak at 28 days after birth. Conclusions　The risk factors for obstructive hydrocephalus secondary to intracranial hemorrhage in neonates are important. Regular and dynamical monitoring of ventricle size by cranial ultrasound is needed in infants with sever intracranial hemorrhage.

Imaging diagnosis and comparative study of agenesis of the corpus callosum in neonates

　HU Liyan, GUO Fuqing,NI Shujian, LIU Ruike, WANG Chunli

. 2015, 33(4):  323.  doi:10.3969 j.issn.1000-3606.2015.04.007

Abstract ( 435 )   PDF (1698KB) ( 620 )  

Related Articles | Metrics

Objective　To evaluate the accuracy and value of the ultrasonography and magnetic resonance imaging (MRI) in the diagnosis of agenesis of the corpus callosum in the newborns. Methods　Conventional cerebral ultrasound screening was performed in 8086 newborns admitted to NICU in our hospital from January 2012 to June 2014 and agenesis of the corpus callosum was suspected or confirmed in 31 newborns. The diagnostic accuracy of ultrasonography was assessed through the comparison between the results of ultrasonography and MRI. Results　In 31 cases with suspected agenesis of the corpus callosum, ultrasonography showed 14 cases of complete agenesis of which 13 cases had the same diagnosis with MRI except one case of partial agenesis, meanwhile, ultrasonography showed 16 cases of partial agenesis of which 15 cases had the same diagnosis with MRI except one case of complete agenesis. One case of abnormal corpus callosum determined by ultrasonography was diagnosed as partial agenesis by MRI. MRI showed there were other brain malformations in 14 cases. Conclusions　Ultrasonography and MRI has a high consistency in the diagnosis of agenesis of the corpus callosum in neonates, so the former can be used as a routine screening and the latter can be used as a method of accurate diagnosis. A combination of both has an important role in early diagnosis and clinical evaluation.

The relation of blood culture results of sepsis in children with serum procalcitonin levels　

　HU Fangqi, CHENG Xiangao, DING Dan, LI Zongguang

. 2015, 33(4):  326.  doi:10.3969 j.issn.1000-3606.2015.04.008

Abstract ( 557 )   PDF (1306KB) ( 529 )  

Related Articles | Metrics

Objective　To explore the roles of procalcitonin in differentiating bacterial infection from other inflammatory processes and identifying bacterial species. Methods　The blood culture results of 253 hospitalized children diagnosed with sepsis from January 2010 to June 2014 were analyzed retrospectively and its relationship with PCT was also analyzed. Results　In 253 children with sepsis, the blood culture results of 124 children showed positive (49.01%). There were 71 (28.06%) cases of Gram-positive infection and 53 (20.95%) cases of Gram-negative infection. The median level of PCT in 253 patients was 1.6 (0.4-4.5) ng/ml. According to the PCT levels, all patients were divided into low level group (0.05-0.5 ng/ml, n=86), middle level group (0.5-2.0 ng/ml, n=55), high level group (2.0-10 ng/ml, n=75) and extremely high level group (10-300 ng/ml, n=37). The positive rates of bacterial culture were significantly different among different levels of PCT (χ2=69.14, P<0.01). In low level group the positive rate of bacterial culture was the lowest and in other three groups the positive rate was all above 60%. The detection rate of Gram-positive and Gram-negative bacteria was significantly different among different levels of PCT (P<0.01). In high level group the detection rate of Gram-positive bacteria was the highest while in extremely high level group the rate of Gram-negative bacteria was the highest. Conclusions　The level of PCT is helpful for the differentiation of bacterial infection from other inflammatory processes in children with sepsis and abnormally elevated PCT level may indicates Gram-negative infection.

Positive bronchial provocation test and lower small airway function in the cough variant asthma　

WU Yufen, ZHANG Hao, GUO Yanfang, ZHANG Xin, ZHU Xiaohong, PAN Hanyun

. 2015, 33(4):  330.  doi:10.3969 j.issn.1000-3606.2015.04.009

Abstract ( 482 )   PDF (1283KB) ( 634 )  

Related Articles | Metrics

Objective　To investigate bronchial provocation test (BPT) and small airway function in children with cough variant asthma (CVA). Methods　A total of 353 children with chronic cough whose mean age was (7.45±2.58) years from three hospitals of Pudong district were enrolled during May 2012 and February 2014. Conventional pulmonary function tests, BPT and questionnaire survey were performed and the difference in pulmonary function was analyzed between children with positive BPT and negative BPT. Results　In 353 children with chronic cough, there were 200 children (56.66%) diagnosed as CVA with positive BPT. Compared with BPT negative group, the percentages of nighttime cough and severe dry cough in BPT positive group were significantly higher while the percentages of morning/daytime cough and wet cough were significantly lower (P<0.01). Furthermore, the rates of history of atopic dermatitis and rhinitis in BPT positive group were significantly higher than those in BPT negative group (P<0.01). Forced expiratory flow at 75% reflecting the small airway function was significantly lower in BPT positive group than that in BPT negative group (P=0.032). Conclusions　Bronchial hyperresponsiveness and decreased small airway function are the important pathological features of CVA. BPT and spirometry have clinical significances in the CVA diagnosis and the analysis of cause of chronic cough.

Research of the diagnosis and treatment of community-acquired pneumonia in children　

XING Wenya, ZHAO Huijun

. 2015, 33(4):  334.  doi:10.3969 j.issn.1000-3606.2015.04.010

Abstract ( 411 )   PDF (1308KB) ( 470 )  

Related Articles | Metrics

Objectives　To summarize the application of Guidelines for the Management of Community-Acquired Pneumonia  (CAP) (2013 edition) in the diagnosis and treatment of children with CAP. Methods　The clinical features of 209 children with non-severe CAP at different age stages were retrospectively analyzed. The curative effect of azythromycin was summarized which was used as first-line antibiotics in the CAP treatment. Results　In 209 CAP children, the onset of 94 children was occurred in spring and summer and 115 occurred in autumn and winter. The white blood cell counts (WBC) in 51 CAP children (24.4%) were more than 10.0×109/L and absolute neutrophil counts (ANC) in 91 CAP children (43.5%) were more than 5.0×109/L. C-reactive protein levels in 199 CAP children (95.7%) were increased and MP-IgM showed positive in 94 CAP children (45.0%). 173 CAP children (82.8%) presented unilateral inflammatory lesions in chest X-ray, and the right side was predominant. There was no difference in onset season among different age groups (P>0.05). However, the percentage of WBC ≥ 10×109/L among different age groups were significantly different (P=0.002) and the percentage of WBC ≥ 10×109/L was highest in infants and young children group. The proportion of onset in spring and summer was significantly higher in MP-IgM positive group than that in MP-IgM negative group (P<0.01). The total effective rate of azithromycin used as first-line antibiotics was 98.1% (205/209), and there was no significant difference in total effective rate of azithromycin between MP and non-MP pneumonia groups. Conclusions 　The Guidelines for the Management of CAP (2013 edition) have a reference value in the diagnosis and treatment of CAP in our region where the CAP in children has some different features.

Genotyping analysis of epidemic strains of Mycoplasma pneumoniae in Suzhou area

　LI Dong, FAN Liping, SUN Huiquan, HAO Chuangli, SHAO Xuejun, XU Jun

. 2015, 33(4):  338.  doi:10.3969 j.issn.1000-3606.2015.04.012

Abstract ( 307 )   PDF (1312KB) ( 377 )  

Related Articles | Metrics

　Objective　To analyze the genotype and variation of Mycoplasma pneumonia (MP) strains isolated from children with MP infection in Soochow area. Methods　The nasopharyngeal secretions from hospitalized children with MP infection were collected during January 2012 and December 2013. The nested-multiplex PCR based on MP P1 gene was performed to detect the subtype of MP gene. Results　In 313 samples, 304 (97.12 %) samples were classified as P1-I type and 8 (2.56%) samples were classified as P1-II type and one (0.32%) was V2 variant. Gene sequencing results were consistent with nested-multiple PCR results. Conclusions　Nested-multiplex PCR is a reliable method for genotyping of MP P1 gene. During the study period, P1-I type was the common genotype and only one case of V2 variant was found.

Association between gene polymorphism of BLK gene and clinical characteristic in Kawasaki disease

DONG Qizhong, ZHANG Chao, LI Yueru

. 2015, 33(4):  342.  doi:10.3969 j.issn.1000-3606.2015.04.013

Abstract ( 423 )   PDF (1327KB) ( 295 )  

Related Articles | Metrics

Objective　To investigate the association of two single nucleotide polymorphisms (SNPs) (rs2736340 and rs2618476) in BLK gene with Kawasaki disease (KD) and coronary artery lesion in Chinese Han children. Methods　In the case-control study, 179 children with KD were selected and 182 healthy check-up children during the same period were selected as normal controls. The genotypes of two SNPs in BLK gene were detected using PCR-RFLP and the data were analyzed. Results 　There was no difference in distribution of three genotypes (TT, CT, and CC) of SNP rs2736340 between KD group and control group (P=0.093). However, T allele frequency in KD group was significantly higher than that in control group (P=0.021). The distribution of three genotypes (CC, CT, and TT) of SNP rs2618476 between KD group and control group was significantly different (P=0.021). C allele frequency in KD group was significantly higher than that in control group (P=0.006). The two SNPs in BLK gene were not associated with rash, hand-foot edema and coronary artery lesion (CAL), but SNP (rs2618476) was associated with oral mucosa lesions (P=0.018). Conclusions　The SNP (rs2736340) in BLK gene was not associated with KD, but the T-allele was associated with KD. The SNP (rs2618476) was associated with KD in Han Chinese, and was also associated with oral mucosa lesions in KD patients.

Class Ⅰ integron and its correlation with genes coding for ESBLs in ESBLs-producing Escherichia coli from children#br# 　

　JIANG Hongchao, Kui Liyue, HUANG Hailin, SU Min, FAN Mao

. 2015, 33(4):  345.  doi:10.3969 j.issn.1000-3606.2015.04.014

Abstract ( 328 )   PDF (1265KB) ( 307 )  

Related Articles | Metrics

Objective　To study the correlation with Genes Coding for ESBLs and Class Ⅰ Integron in ESBLs-producing Escherichia coli from children. Methods　PCR was used for gene typing detection of 100 strains of ESBLs-producing Escherichia coli strains. While detection of class I integrase gene in the 100 strains ESBLs-producing Escherichia coli and 100 strains of non-ESBLs producing Escherichia coli were separately performed by PCR. It provides the solid base not only to reveal the relationship between class I integron and drug resistance, but also the relationship between class I integron and ESBLs-producing. Results　The most frequently genotyping from ESBLs-producing Escherichia coli in children is CTX-M (84%), followed by TEM-1 (63%). The predominant distribution of genotype in ESBL- producing strains is TEM-1 + CTX-M (45%), followed by CTX-M (34%). Class I integrase gene detected in ESBLs- producing and non- ESBLs producing strain were 100 cases (100%) and 25 cases (25%) separately, the difference was statistically significant (P<0.05); drug resistance in class I integron positive strains were significantly higher than in class I integron negative strains, especially in Ciprofloxacin, Levofloxacin, and Aminoglycoside (86.4%, 88%, and 80%). Conclusions　The distribution of Class I integron in ESBLs-producing Escherichia coli is significantly higher than that in non-ESBLs-producing strains, It is rational that Class I integron highly correlate with strong drug resistance in ESBLs-producing strains.

Tachycardia-induced cardiomyopathy in children: a report of 20 cases　

　ZHANG Wenle, LIU Shuaishuai, JIN Lianhua, PIAO Jinhua

. 2015, 33(4):  348.  doi:10.3969 j.issn.1000-3606.2015.04.015

Abstract ( 328 )   PDF (1285KB) ( 628 )  

Related Articles | Metrics

Objective　To explore the clinical features, treatment, and outcomes of tachycardia induced cardiomyopathy (TIC) in children. Methods　The clinical data of 20 children with TIC hospitalized from January 2007 to October 2014 were retrospectively analyzed. Results　In 20 patients with TIC, there were 11 infants, one toddler, 5 pre-school age children, and 3 adolescent patients were as follows: 15 cases of atrial tachycardia (distributed in each age group), 3 cases of paroxysmal supraventricular tachycardia (2 in infancy and one in adolescence), and 2 cases of ventricular tachycardia (2 in infancy). After the treatment of anti-arrhythmic drugs, sinus rhythm was restored in 11 patients and ventricular rate was controlled in 5 patients while poor effect of drug was found in one patient who received radiofrequency ablation eventually and got cured. Three patients received radiofrequency ablation after admission immediately. Compared with those before treatment, left ventricular ejection fraction (LVEF) and left ventricular diastolic diameter (LVDD) measured by cardiac ultrasonography were significantly improved after treatment (P<0.01). Conclusions　TIC is common in infancy. Atrial tachycardia is the main type of arrhythmia. Generally drug therapy is the first choice in the treatment of TIC but in older children and those refractory to drug therapy the radiofrequency ablation is chosen.

Treatment timing and method of glucocorticoids in abdominal type Henoch-Schönlein purpura

　SHI Xuewen, CHEN Juan, YIN Mingdong, CHEN Xiangqian, CAO Qing

. 2015, 33(4):  352.  doi:10.3969 j.issn.1000-3606.2015.04.016

Abstract ( 416 )   PDF (1305KB) ( 652 )  

Related Articles | Metrics

Objectives　To study the treatment timing and method of glucocorticoids in the treatment of abdominal type Henoch-Schönlein purpura (HSP). Methods　A total of 201 children with abdominal type HSP hospitalized from September 2009 to April 2013 received either conventional glucocorticoids treatment or rapid titration treatment of glucocorticoids based on the same basic therapy. According to the treatment timing and method of glucocorticoids, all patients were divided into 4 groups including early convention group (n=46), late convention group (n=44), early titration group (n=56) and late titration group (n=55). The duration from the appearance of gastrointestinal symptoms to using glucocorticoids less than or equal to three days was defined as early treatment and more than three days was defined as late treatment. The patients in two convention groups (early and late) were treated with methylprednisolone (2-6mg/kg per day).The patients in two titration groups (early and late) received rapid titration of methylprednisolone from an initial low dose of 2mg/kg per day to the final target dose. The gastrointestinal symptoms were evaluated every 12 hours. If the gastrointestinal symptoms were not alleviated, an additional dosage of methylprednisolone was given. On the next day, the total dose of methylprednisolone in previous 24 hours was used as the initial dose. The dosage was increased in such a way till the gastrointestinal symptoms disappeared and this dosage was remained for 3 days. Then the dosage was gradually reduced to the maintenance dose and stopped. If the gastrointestinal symptoms were relapsed, the patients were treated again. A follow-up of 3-6 months was performed. The dosage, recurrence of symptoms and the side effects were compared among four groups. Results　The time of remission, target dose of glucocorticoids, total dose of glucocorticoids, recurrence rate, incidence of severe symptoms and Henoch-Schonlein purpura nephritis were significantly different among four groups (P<0.05). The curative effect was best in early titration group and worst in late convention group. Conclusions　In the treatment of abdominal type HSP, early titration treatment with glucocorticoids can significantly relieve the gastrointestinal symptoms and reduce the total dosage of glucocorticoids.

Plasma myeloperoxidase, malondialdehyde, superoxide dismutase, and total anti-oxidant capability of Henoch-Schönlein purpura in children

LI Yufei, LU Ling

. 2015, 33(4):  357.  doi:10.3969 j.issn.1000-3606.2015.04.017

Abstract ( 426 )   PDF (1318KB) ( 510 )  

Related Articles | Metrics

　Objective　To investigate the changes in plasma myeloperoxidase (MPO), malondialdehyde (MDA), superoxide dismutase (SOD) and total anti-oxidant capability (T-AOC) of Henoch-Schönlein purpura in children. Methods　A total of 80 HSP children hospitalized from November 2013 to April 2014 were selected including 40 cases of arthritis type, 20 cases of abdominal type and 20 cases of nephritis type (HSPN). Meanwhile, 16 healthy check-up children were selected as controls. Plasma MPO, T-AOC levels were detected by chemical colorimetry and MDA levels were detected by thiobarbituric acid colorimetry. Plasma SOD levels were measured by hydroxylamine method. Furthermore, 24-hour urine protein and other hematological indices were routinely tested. Results　The plasma levels of MPO, MDA, SOD and T-AOC were significantly different among control group, arthritis type group, abdominal type group and HSPN group (F=24.22-126.99, all P=0.000). In comparison to other three groups, the levels of MPO and MDA were significantly increased and the levels of SOD and T-AOC were significantly decreased in HSPN group (P<0.05). Compared with control group, the levels of MPO and MDA were significantly higher and the levels of SOD and T-AOC were significantly lower in arthritis and abdominal type groups (P<0.05). The levels of MPO and MDA in HSPN group were significantly positively correlated with 24-hour urine protein (r=0.695, 0.559; both P<0.01). Conclusions 　The imbalance between oxidation and antioxidant system exists in children with Henoch-Schönlein purpura.

Temperature instability of Prader-Willi syndrome in an infant

　FENG Ying, XIAO Nong, CHEN Yuxia

. 2015, 33(4):  361.  doi:10.3969 j.issn.1000-3606.2015.04.018

Abstract ( 364 )   PDF (1230KB) ( 487 )  

Related Articles | Metrics

Objective　To study the clinical characteristics of the temperature instability in Prader-Willi syndrome (PWS). Methods　The clinical characteristics of one case of PWS with recurrent fever were retrospectively analyzed. Results　 A 5-month-old boy who had such clinical characteristics as low central muscle tension, sucking weakness, feeding difficulties, cryptorchidism and white skin was diagnosed as PWS by chromosomal microarray analysis and methylation-specific PCR. In the course of the disease, the infant presented recurrent fever. The pathogenic bacteria culture and virus antibody detection showed negative, and the treatment of reducing fever, anti-infection and immune support were ineffective. Conclusions　The cause of the recurrent fever is unknown and central fever is suspected. Whether the temperature instability is the clinical feature of PWS still needs a lot of clinical researches.

The practice and outlook of pediatric trauma life support curriculum

LIN Qiang, DU Qirong, PAN Shuming, ZHAO Jie, HUANG Siping, NIU Lei

. 2015, 33(4):  364.  doi:10.3969 j.issn.1000-3606.2015.04.019

Abstract ( 358 )   PDF (1302KB) ( 381 )  

Related Articles | Metrics

　Objectives　To investigate the effect of pediatric trauma life support (PTLS) curriculum on the improvement of rescue skill for pediatric trauma patients. Methods　PTLS course was introduced to train medical staff engaging in pediatric critical trauma care. The course included theoretical and practical part with main focus on enhancement of students’ decision on scene and rescue skill. Results　Compared with pre-training, the students’ post-training written test scores improved significantly, and the rates of qualification and excellence were improved significantly (P<0.01). All students were qualified for the tests of skill stations and simulation scenario. The excellence rates of the tests were as follows: fixation and transportation 42.62%, basic and advanced airway management 81.97%, shock evaluation and management 70.49%, simulation scenario 72.13%. Both pre-training and post-training written test scores in hospital group were significantly higher than those in pre-hospital group (P<0.01). Conclusions　After PTLS courses, the knowledge, practice and integrated life-saving skills of students are greatly improved and teamwork is strengthened.

Effects of caffeine citrate on neuronal proliferation and apoptosis and long-term learning ability in neonatal rats with hypoxia-ischemic brain damage

XU Falin, WANG Caihong, ZHANG Yanhua, GUO Jiajia, CHENG Huiqing

. 2015, 33(4):  368.  doi:10.3969 j.issn.1000-3606.2015.04.020

Abstract ( 337 )   PDF (1644KB) ( 499 )  

Related Articles | Metrics

Objective　To investigate the effects of caffeine citrate (CC) on the neuronal proliferation and apoptosis and longterm learning ability in neonatal rats with hypoxia-ischemic brain damage (HIBD). Methods　Forty-eight 7-day-old Sprague-Dawley neonatal rats were randomly divided into 3 groups: sham operation group (n=16), HIBD group (n=16), HIBD + caffeine citrate group (CC group, n=16). Rats in HIBD and CC groups received ligation of left common carotid artery followed by 2 hours of hypoxia to establish HIBD model. Rats in CC group were injected intraperitoneally with CC (20 mg/kg) before and at 0 min, 24 h, 48 h, and 72 h after hypoxia-ischemic (HI), and rats in the other two groups were injected intraperitoneally with an equal volume of normal saline at the corresponding time. Meanwhile, from postnatal day 10, each rat was injected intraperitoneally with 5-bromo-2’-deoxyuridine (BrdU) (50 mg/kg) for 5 consecutive times, once every 12 h. On postnatal day 12, BrdU in the hippocampal dentate gyrus and cleaved caspase-3 in the hippocampal CA1 area were detected by immunohistochemistry, and neuronal apoptosis in hippocampal CA1 area were detected by TUNEL staining. On postnatal day 28, long-term learning and memory ability of rats was tested by Y maze. Results　There was significant difference in the number of BrdU-positive cells in brain tissues of rats among three groups (F=101.38, P<0.01). The BrdU-positive cells in HIBD group and CC group were significantly more than those in sham operation group (P<0.05). There was significant difference in the number of cleaved caspase-3-positive cells in hippocampal CA1 area among three groups (F=379.77, P<0.01). The cleaved caspase-3-positive cells in CC group were significantly fewer than those in HIBD group but significantly more than those in sham operation group (P<0.05). The TUNEL-positive cells in hippocampal CA1 area were significantly different among three groups (F=505.92, P<0.01) which was most in HIBD group and fewest in sham operation group and significant difference was found through multiple comparison (P<0.05). The total learning number of avoiding electric shock tested by Y maze was significantly different among three groups (F=32.05, P<0.01) which was most in HIBD group. Correct response rate was significantly different among three groups (F=24.99, P<0.01) which was lowest in HIBD group. Conclusions　Caffeine citrate can improve the ability of long-term learning and memory in neonatal rats with hypoxia-ischemic brain damage, the mechanism of which may be related to reducing the neuronal  apoptosis after hypoxia ischemia.

The research progress of GATA6 in heart development and isolated atrial septal defect

XU Xicai, LI Fen,ZHOU Wanping, ZHU Diqi, JI Wei, HU Jingjing, CHEN Yiwei, QIAN Yijiao, DAI Ke

. 2015, 33(4):  380.  doi:10.3969 j.issn.1000-3606.2015.04.022

Abstract ( 355 )   PDF (1269KB) ( 705 )  

Related Articles | Metrics

GATA6 transcription factor belongs to the GATA family and contains 2 conserved zinc finger DNA binding domains. GATA6 not only presents in embryonic tissues but also found in heart, lung and pancreas and is essential for the maintenance of their function.The present review focuses on the critical roles of GATA6 in heart development and atrial septal defect to provide theoretical basis for diagnosis and treatment of atrial septal defect.

Research progress of miRNA expression in kidney disease　

ZHANG Pei, XIA Zhengkun, GAO Chunlin

. 2015, 33(4):  383.  doi:10.3969 j.issn.1000-3606.2015.04.023

Abstract ( 381 )   PDF (1263KB) ( 596 )  

Related Articles | Metrics

MicroRNAs (miRNAs) are short non-coding RNAs that regulate gene expression at the post-transcriptional level and then affect important physiological and pathological processes. Many microRNAs are tissue or organ-specific which make it possible for them to become potential biomarkers. Similarly, microRNAs are important for physiological functions of kidney and they are involved in various renal disorders. The review summarizes current information about microRNA effect on various kidney diseases, in order to provide references for the diagnosis and treatment of kidney disease in the future.

Progress in non-invasive ventilation for the treatment of neonatal respiratory disease

ZHANG Xindan, ZHENG Jun, WANG Xiaopeng, MA Junling

. 2015, 33(4):  387.  doi:10.3969 j.issn.1000-3606.2015.04.024

Abstract ( 491 )   PDF (1232KB) ( 653 )  

Related Articles | Metrics

　Mechanical ventilation in newborns can be divided into invasive mechanical ventilation and non-invasive mechanical ventilation. In order to reduce invasive ventilation-induced lung injury, more and more non-invasive ventilation patterns have been used clinically. Early application of nasal intermittent positive pressure ventilation (NIPPV) has become the main mode of non-invasive ventilation in many neonatal intensive care units (NICU). At the same time, the combination with selective pulmonary surfactant can reduce the incidence of chronic lung disease. This article reviews the use and prospect of non-invasive respiratory support modes which include continuous positive airway pressure (CPAP) and bi-level CPAP mode (BiPAP) such as  SiPAP and NIPPV. Some modes which are in the research stage were also reviewed including synchronized nasal intermittent positive pressure ventilation, neurally adjusted ventilatory assist and non-invasive high-frequency oscillatory ventilation.

Advances in Helicobacter pylori infection in children　

ZHANG Shuanghong

. 2015, 33(4):  391.  doi:10.3969 j.issn.1000-3606.2015.04.025

Abstract ( 381 )   PDF (1272KB) ( 565 )  

Related Articles | Metrics

Helicobacter pylori (H.pylori) infection is the most important pathogenic microorganisms associated with the development of chronic gastritis, peptic ulcer and gastric mucosa-associated lymphoid tissue lymphoma (MALT) in children. H.pylori infection may occur in early childhood and last a lifetime even directly affecting the health of adulthood unless H.pylori infection was eradicated. This article aims to review the infection condition, infection pathway and immunological characteristics of H.pylori and its relationship with extragastroduodenal diseases in children.