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06 November 2016, Volume 31 Issue 11 Previous Issue    Next Issue

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Significance of podocyte-related molecules in clinical practice of diagnosis and treatment and some thoughts about it DING Jie，DING Fang-rui 2016, 31(11): 801-805.  DOI: 10.7504/ek2016110601 Abstract ( )   Proteinuria is the most common manifestations of pediatric renal diseases. During the development and process of proteinuria，podocytes have played a very important role. A growing number of podocyte-related molecules have been confirmed to be associated with the molecular mechanisms of proteinuria. However，there are few studies focusing on the role of podocyte-related molecules in the diagnosis and treatment of proteinuria. Due to the lack of real transformation from bench to bed situation，the present paper will discuss the role of podocyte in the monitoring and treatment of proteinuria based on the podocytes’ role in mechanisms of proteinuria.

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Clinical epidemiology of proteinuria MIAO Qian-fan，XU Hong 2016, 31(11): 806-808.  DOI: 10.7504/ek2016110602 Abstract ( )   Proteinuria is usually associated with progressive renal disease，but may sometimes be a benign transient finding. Abnormal proteinuria is defined as protein excretion of ＞150 mg/1.73m2 per day or＞4 mg/m2 per hour. Many studies in deferent centers have reported that quite a few asymptomatic children with proteinuria were detected. Excessive proteinuria can be classified as glomerular proteinuria，tubular proteinuria，secretory protein and overflow proteinuria by causes. It can also be classified as transient proteinuria，postural proteinuria，persistent asymptomatic proteinuria，primary/secondary glomerular proteinuria and primary/secondary tubular proteinuria by clinical symptoms.

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Progress in study of proteinuria mechanism ZHOU Jian-hua 2016, 31(11): 808-812.  DOI: 10.7504/ek2016110603 Abstract ( )   Proteinuria occurs mainly due to increased filtration of plasma proteins through glomerular capillary wall and/or incapability of renal tubular reabsorption of small molecular proteins. Significant advances have been made in study on glomerular filtration barrier in recent years. Now endothelial surface layer and subpodocyte space have been accepted as new layers of whole glomerular filtration barrier，and more and more podocyte molecules   have been found to play a causative role or participate in the occurrence of proteinuria.

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Definition of physiological proteinuria and pathological proteinuria LUO Gang，JIANG Hong 2016, 31(11): 812-815.  DOI: 10.7504/ek2016110604 Abstract ( )   Proteinuria，as an important clinical manifestation of kidney disease，is also an independent risk factor for the progression of renal disease. So the control of proteinuria has become an important target for diagnosis and treatment of kidney disease. Physiological proteinuria，which in the past is different from the pathological proteinuria，is considered to be a benign change. But in clinical practice，there is increasing evidence that some of the physiological proteinuria also has a poor prognosis. In this paper，we put forward the definition criterion of physiological proteinuria，and give the corresponding follow-up advice，in order to avoid the judgment of the simple benign change of physiological proteinuria.

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Proteinuria and chronic renal disease LIU Fei，MAO Jian-hua 2016, 31(11): 816-820.  DOI: 10.7504/ek2016110605 Abstract ( )   The number of patients with chronic kidney disease（CKD） has increased year by year，had many of them further progress to end-stage renal disease（ESRD），requiring maintenance dialysis or transplantation，which causes huge social burden，so CKD has become a more and more serious public health issue worldwide. Proteinuria and/or albuminuria is the important independent risk factor of CKD，which have close relationship with CKD，and have been the hotspot in research of the pathogenesis，progression and therapy of CKD.

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Progress of urinary protein measurement LI Hai-xia*，LIU Yi，DING Jie 2016, 31(11): 820-823.  DOI: 10.7504/ek2016110606 Abstract ( )   Proteinuria measurement is used to identify and monitor kidney damage. Accurate assessment of proteinuria is essential to clinical practice. This review focuses on the current status and question of proteinuria measurement. We also addressed standardization of preanalytical，analytical，and postanalytical issues including urine sample collection，urine protein and albumin measurement，and reporting of test results.

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Treatment for proteinuria in children JIANG Xiao-yun*，MA Yuan-lin，YANG Fan 2016, 31(11): 824-829.  DOI: 10.7504/ek2016110607 Abstract ( )   In regular checkup，proteinuria can be found in 10% of children aged 8 to 15 years.  Proteinuria can be divided into physiological and pathological proteinuria. Persistent proteinuria，which is the early symptom of kidney disease，is usually caused by pathological factors. It is also the main factor that leads to kidney disease progress. Any intervention to reduce proteinuria is helpful to slow the progress of children’s kidney disease. In this paper，we will talk about  the treatment of physiological proteinuria， especially non-specific drug treatment，according to recent advances in the treatment of proteinuria in children.

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Value of prenatal ultrasonography in evaluation of postpartum prognosis of fetal simple hydronephrosis GUO Li-hua，ZHANG Qian，SUN Quan，FAN Ying-zhong，CHEN Tao，YAN Yue-qiang，Li Yan 2016, 31(11): 830-833.  DOI: 10.7504/ek2016110608 Abstract ( )   Objective    To explore the clinical value of ultrasonography in evaluation of prognosis of fetal simple hydronephrosis not complicated with ureterectasia in order to find out the best ultrasonic evaluation index， which can predict the outcome of simple hydronephrosis. Methods    Retrospective data on prenatal sonographic parameters were compared between patients who had been clinically suspected prenatally with UPJO of hydronephrosis and later underwent postnatal pyeloplasty for ureteropelvic junction obstruction（n＝49） and patients who were treated conservatively（n＝40）. Results    Significant differences were found between the surgically and conservatively treated patients for meananterior-posterior renal diameter［（23.8±8.8） mm vs. （15.0±5.1） mm］（P＜0.0001） and parenchymal thickness［（5.2±2.9）mm vs. （8.4±2.3） mm］（P＝0.007）. Mean ratio of anterior-posterior diameter to parenchymal thickness was significantly higher in the postnatal pyeloplasty group［（5.8±4.0） vs. （2.2±1.1）］（P＜0.0001）. Conclusion    For prenatal simple hydronephrosis，anterior-posterior diameter ＞15 mm is the best single predictor of the need for surgery. A ratio of ＞2.2 of renal pelvis anterior-posterior diameter to parenchymal thickness suggests UPJO and predicts the probability  of the need for surgery and supports the use of more intensive prenatal and postnatal surveillance.

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Evaluation of empirical antibiotic therapy for suspected neonatal early-onset sepsis PAN Qian，YU Jia-lin 2016, 31(11): 834-837.  DOI: 10.7504/ek2016110609 Abstract ( )   Objective    To evaluate the rationality of empirical antibiotic therapy for suspected neonatal early-onset sepsis. Methods    Retrospective study of antibiotic use on suspected early-onset neonates in hospital was performed. All cases were divided into non-sepsis group and sepsis group by the final diagnosis. Results    Of all the 217 cases，122 cases belonged to non-sepsis while 95 cases were sepsis. First-choice antibiotis were penicillin+latamoxef：in non-sepsis group it accounted for 68.4% and in sepsis group，64.5%. In non-sepsis group inappropriate antibiotic use at the initiation period（within 72 hours of use） was in 16 cases（13.1%），and in continuation period（after 72 hours of use） it was in  77 cases（63.1%）. In sepsis group，it was in 9 cases（9.5%） and 49 cases（51.6%） respectively. Conclusion    Inappropriate antibiotic use in suspected neonatal early-onset sepsis is serious，it is necesary to set up neonatal antimicrobial management team to standardize antibiotic use for EOS in neonatal ward.

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Predictive value of lipopolysaccharide binding protein for the infection of preterm infants with premature rupture of membranes RAO Hong-ping *，LI Yi-juan 2016, 31(11): 838-842.  DOI: 10.7504/ek2016110610 Abstract ( )   Objective    To investigate the predictive value of lipopolysaccharide binding protein （LBP） in early diagnosis of preterm infection with premature rupture of membrane（PROM）. Methods    Totally 93 preterm infants with PROM were enrolled in this study， and were  divided into infection group （45 cases） and non-infection group （48 cases） according to the discharge diagnosis. Their serum LBP levels were detected within 24 hours after birth. High-sensitivity C-reactive protein （hs-CRP）， procalcitonin （PCT） and interleukin-6 （IL-6） levels were compared with LBP. Receiver operator characteristic curve （ROC） was drawn and the area under the curve （AUC） was calculated. Each parameter was evaluated for the diagnosis of early-onset infection in preterm infants with PROM. In addition， according to the severity of the disease infection group was further divided into severe infection group（21 cases） and general infection group（24 cases）. LBP levels were detected in two groups of premature infants to assess the value of LBP in the diagnosis of premature infants with premature rupture of membranes. Results    The serum levels of IL-6， PCT and LBP in the infection group were significantly higher than those in the non-infection group. There was no significant difference on serum levels of hs-CRP between the two groups. The level of LBP in the severe infection group was higher than that in the general infection group， and the difference between the two groups was statistically significant. The AUC of ROC for LBP （0.974） in early-onset bacterial infection of preterm infants with PROM was the highest， PCT （0.694） second， IL- 6 （0.588） third， and hs-CRP （0.478） was the lowest. Conclusion    Serum level of LBP is superior to hs-CRP and PCT in the diagnosis of early-onset bacterial infection in preterm infants with PROM.  LBP can be used as a useful index for the early diagnosis of bacterial infection in preterm infants with PROM. LBP levels can evaluate the severity of premature premature infection in preterm premature rupture of membranes.

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Analysis of 19 cases of Kawasaki disease complicated with coronary artery lesions WANG Wen，LUO Gang，ZHANG Qian-zhong 2016, 31(11): 843-846.  DOI: 10.7504/ek2016110611 Abstract ( )   Objective    To analyze the clinical characteristics of patients with CAL complicated by KD，and to improve the understanding of this disease. Methods    Clinical data of 19 cases diagnosed as KD complicated by CAL from September 2008 to August 2015 in the Pediatric Department of First Hospital of China Medical University were retrospectively analyzed. Results    KD children complicated with CAL was more in male than in female，male∶female was 3.75∶1；children with coronary aneurysm were more in male than in female，male：female was 8∶1；it was more often in less than 1-year old children，accounting for 36.8%. The incidence rate of coronary artery lesions in this group was 16.4%；CAD was in 10 cases，CAA in 5 cases，GCAA in 4 cases，the incidence rate being 8.6%，4.3% and 3.5%，respectively；in terms of CAL time 73.7% was 2-3 weeks after the disease； according to the number of branches involved，both coronary artery involved accounted for 68.4%，and according to the location involved，right coronary artery involvement accounted for 41.5%，left main coronary artery involvement accounted for 39.0%，the left anterior descending coronary artery involvement accounted for 17.1%，and left circumflex artery involvement accounted for 2.4%. The clinical grade of severity in CAL children were：Ⅱ grade in 5 cases，accounting for 26.3%；Ⅲ grade in 8 cases，accounting for 42.1%；Ⅳ grade in 6 cases，accounting for 31.6%. During follow-up，71.4% patients had coronary artery aneurysm or dilatation significantly retracted，the average time of retraction being （1.5±0.7）years，of which 1 case was completely dissipated. Conclusion    The incidence rate of the KD patients with CAL is 16.4%，in which CAD has the highest incidence rate while GCAA the lowest. CAL mostly occurs at 2-3 weeks after the disease. Any branch can be damaged，with the right coronary artery to be most likely involved. As to coronary artery disease severity，grade Ⅲ is of the highest proportion. Most coronary artery aneurysm or dilatation has remarkable retraction within 1-2 years.

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Change and significance of osteocalcin in children with Kawasaki disease GAO Jing-ying*，LI Ya-rui，NIU XU-xu 2016, 31(11): 847-850.  DOI: 10.7504/ek2016110612 Abstract ( )   Objective   To observe the change of different forms of osteocalcin in children with Kawasaki disease（KD） and explore the relationship of osteocalcin with Kawasaki Disease. Methods   A total of 34 hospitalized children with Kawasaki disease between March 2015 and December 2015 were selected，and all of them came from Children’s Hospital of Shanxi Province.  According to the course，each child was divided into acute stage and subacute stage. At the same time，20 healthy children were chosen as the control. The levels of plasma N-MID osteocalcin and undercarboxylated osteocalcin were measured. Erythrocyte sedimentation rate（ESR） and C-reactive protein（CRP） were analyzed and the changes of osteocalcin，ESR and CRP were compared. Results    The levels of plasma undercarboxylated osteocalcin in KD patients were significantly lower than control［（14.2±0.3） ng/mL for acute stage，（14.4±0.3） μg/L for subacute stage，（16.3±0.4） μg/L for control，P＜0.01）］. The levels of plasma N-MID osteocalcin in KD patients did not differ from control［（27.3±0.5） μg/L for acute stage，（27.5±0.5） μg/L for subacute stage，（28.3±0.8） μg/L for control，all P＞0.05）］. The levels of CRP in acute stage were significantly higher than in subacute stage［（79.1±10.3）mg/L for acute stage vs.（2.7±0.5） mg/L for subacute stage，P＜0.001）］. The levels of ESR in acute stage were higher than in subacute stage［（58.5±4.1） mm/h for acute stage vs. （49.1±4.7） mm/h for subacute stage，P＜0.05）］. In acute stage， the correlation analysis showed that the levels of plasma undercarboxylated osteocalcin were negatively correlated with CRP and ESR（P＜0.05）. Conclusion    Plasma undercarboxylated osteocalcin might participate in the pathogenesis and development of KD. The measurement of plasma undercarboxylated osteocalcin could be valuable in the diagnosis of KD in children.

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Clinical study of infective endocarditis in 33 children DAI Ke*，XU Xi-cai，JI Wei，FU Li-jun，GUO Ying，LIU Ting-liang，HUANG Mei-rong，CHEN Yi-wei，LI Fen 2016, 31(11): 851-854.  DOI: 10.7504/ek2016110613 Abstract ( )   Objective    To explore the clinical features of infective endocarditis and the factors related to efficacy. Methods    The clinical data of 33 patients with infective endocarditis admitted to our hospital from november 2013 to november 2015 were retrospectively analyzed，including the pathogenic characteristics，underlying diseases，vegetation distributions，treatment program and prognosis. Results    The pathogen detection rate of those cases was 75.76%，and the rate of being with underlying disease was 93.94%. The vegetation laid mainly in the heart valves（63.64%），followed by implant artificial material，the impact place of residual shunt and blood flow. For all the cases，the combination of the surgery with the full course of antibiotic therapy had good effect. Conclusion    The positive blood culture in patients of infective endocarditis is high. The disease is more common in children with basic heart diseases. The combination of the early radical surgery and the full course of antibiotic therapy are the key to the treatments.

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Clinical and molecular genetic analysis of 77 children with 46，XY ambiguous genitalia CHEN Li-fen*，WANG Wei，WANG Ying，LU Wen-li，XIAO Yuan，NI Ji-hong，WANG De-fen，DONG Zhi-ya 2016, 31(11): 855-860.  DOI: 10.7504/ek2016110614 Abstract ( )   Objective    To study the clinical and molecular genetic features of 46，XY ambiguous genitalia and to analyze the relationship between them. Methods    A total of 77 individuals with 46，XY karyotype and sporadic ambiguous genitalia were enrolled for the study in Ruijin Hospital. Genomic DNA were extracted from patients’ peripheral blood leukocytes. To search for DNA point mutations in 15 candidate genes which were potentially associated with sexual development，we amplified the full coding regions and intron-exon boundaries of these genes using PCR which were subsequntly sequenced using next generation sequencing （NGS） by Illumina-Miseq. Results    We were able to identify a likely genetic diagnosis in around 23.4% cases. The study identified nine mutations of SRD5A2 gene including 5 compound heterozygous and 4 homozygous mutations. One of mutations was novel based on this study. Four homozygous mutations of AR gene were detected， in which a novel missense mutation was identified. There were two patients with polymorphisms of MAMLD1 gene. Three patients carried mutations in INSL3 or MID1 or NR5A1 gene， respectively. Conclusion    46，XY ambiguous genitalia patients present a variety of clinical phenotypes and hypospadias is the most common congenital anomaly. Gene mutations that involved in sex development play an important role in the occurence of hypospadias（14/64，21.8% in the study）. SRD5A2 and AR mutations are most frequently but other genes like MAMLD1，INSL3，MID1 and NR5A1 should not be ignored.

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Case report of 3 children with disseminated varicella zoster virus infection after allogeneic hematopoietic stem cell transplantation for aplastic anemia and the review of literature CHEN Qi-hui，XU Hong-gui，XUE Hong-man，LIN Shao-fen，CHEN Chun 2016, 31(11): 861-864.  DOI: 10.7504/ek2016110615 Abstract ( )   Objective    To discuss the diagnosis, high risk factors, prevention and treatment of disseminated varicella zoster virus（VZV） infection after allogeneic hematopoietic stem cell transplantation（allo-HSCT） in children with aplastic anemia（AA）. Methods    A retrospective analysis was made about the clinical data of 3 children with disseminated VZV infection after allo-HSCT for AA. Results    Three children, aged 10～13 years old，who didn’t present graft-versus host disease（GVHD） after transplantation, were given single drug as anti-viral prophylaxis of VZV. During the period of prophylaxis, none of them developed VZV reactivation but they showed symptoms of disseminated VZV infection after discontinuation of anti-viral prophylaxis. All of them were treated with intravenous ganciclovir, oral valaciclovir combined with external application of penciclovir and with gamma globulin until the rush crusted, and eventually all of them recovered well. Conclusion    Patients with allo-HSCT for aplastic anemia are at high risk of developing disseminated VZV infection and they have high incidence of mortality and poor prognosis. We recommend anti-viral prophylaxis after allo-HSCT for prevention， and reduced dose of immunosuppressive drugs and combination use of ganciclovir, valaciclovir and gamma globulin when patients present symptoms of VZV infection.

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Clinical characteristics，pathogen distribution and drug resistance status in 268 cases of neonatal sepsis LI Hong-ying，QI Yue-ying，JIN Xiu-mei，WU Hong 2016, 31(11): 865-869.  DOI: 10.7504/ek2016110616 Abstract ( )   Objective    To analyse the pathogen distribution and drug resistance status in 268 cases of neonatal sepsis. Methods    Totally 268 cases of neonatal sepsis were chosen who had been hospitalized between 2010 and 2015 with blood culture being positive. They were divided into early-onset and late-onset sepsis groups and their clinical characteristics，pathogen distribution and drug resistance were explored. Results    The gram-negative bacteria were the major pathogens early-onset sepsis group（58.3%），in which escherichia coli was 31.2% and klebsiella pneumoniae was 23.9%. Gram-positive bacteria were the major pathogen in late-onset sepsis group（65.1%），in which coagulase negative staphylococcus was 46.5% and excrement enterococcus was 11.6%. Gram-positive bacteria were sensitive to vancomycin with durg resistance rate ＞90% to penicillin，＞80% to erythromycin，＞60% to clindamycin，ampicillin and cefazolin . Gram-negative bacteria were sensitive to meropenem. Gram-negative bacteria showed high resistance to ampicillin，piperacillin and cefotaxime，but low resistance to ceftazidime and cefoperazone. Conclusion    The common pathogens and clinical characteristics are different in the early-onset and late-onset sepsis groups. It is important to choose reasonable antibiotic drugs and strengthen drug resistance surveillance.

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Relationship of congenital anomalies of the kidney and urinary tract with environment SUN Qi，KANG Guo-gui，RUAN Pei-sen 2016, 31(11): 870-873.  DOI: 10.7504/ek2016110617 Abstract ( )

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Research progress in the ranibizumab treatment for retinopathy of prematurity DONG Li-juan，CHEN Chao 2016, 31(11): 874-879.  DOI: 10.7504/ek2016110618 Abstract ( )

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Neonatal fungal conjunctivitis：A report of 1 case LIU Xi-juan，YU Jian-ming，XIONG Zhi-yong 2016, 31(11): 880-880.  DOI: 10.7504/ek2016110619 Abstract ( )