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06 January 2017, Volume 32 Issue 1 Previous Issue    Next Issue

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Expert guidance of pediatric hemophilia in China（2017） WANG Tian-you，FANG Yong-jun，FENG Xiao-qin，et al 2017, 32(1): 1-5.  DOI: 10.19538/j.ek2017010601 Abstract ( )

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Recommended scheme and consensus of ultrasound diagnosis for children with hemophilia arthropathy（2017） YU Guo-hua，WANG Tiao-you，WANG Yan，et al 2017, 32(1): 6-10.  DOI: 10.19538/j.ek2017010602 Abstract ( )

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Interpretation of Expert Guidance of Pediatric Hemophilia in China （2017） LI Xiao-jing，CHEN Zhen-ping，WU Run-hui 2017, 32(1): 11-15.  DOI: 10.19538/j.ek2017010603 Abstract ( )   As a congenital genetic disease，the diagnosis and treatment of hemophilia has made a rapid progress，and new treatment methods and new drugs are emerging in recent years. The interpretation mainly introduces the current progress in several aspects，such as laboratory diagnosis，individual prophylaxis，prevention and control of coagulation factors inhibitor and comprehensive hemophilia care，to help understand well about Expert Guidance of Pediatric Hemophilia in China（2017）.

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Interpretation of Recommended Scheme and Consensus of Ultrasound Diagnosis for Children with Hemophilia Arthropathy （2017） PENG Yun, ZHANG Ning-ning 2017, 32(1): 16-17.  DOI: 10.19538/j.ek2017010604 Abstract ( )   With the application of clinical practice guideline of X-ray and MR imaging，it is essential to set up the comprehensive assessment system of hemophilia arthropathy in children . With the advantages of easier access，lower costs and no need for sedation in younger patients，ultrasound has recently been proposed and joined in the clinical diagnosis for hemophilia arthropathy. In this Recommended Scheme and Consensus of Ultrasonic Diagnosis for Children with Hemophilia Arthropathy（2017），the characteristics，advantage，indication and equipment requirements，standard protocol and scanning level，and scoring system are introduced in detail，respectively. Ultrasound guidelines have a great significance and are meaningful in multiple imaging assessment system of hemophilia arthropathy in Chinese children.

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Entry into the new era of care for children with hemophilia WANG Tian-you, WU Run-hui 2017, 32(1): 18-22.  DOI: 10.19538/j.ek2017010605 Abstract ( )   Thanks to the development of science，hemophilia care is improved gradually，and hemophilia is turning from one of inherited bleeding diseases with death and disability into one of lifelong diseases and it’s likely to deal with successfully in clinics. At present，the main problems of children hemophilia care are how to cost-effectively execute the prophylaxis and how to prevent and control the inhibitor. With the creation of novel technologies and products in hemophilia care，the conceptions and strategies of care are facing updating and challenges. Although China is a developing country and is slow in starting the care，the development of Children Hemophilia Care  has improved rapidly. With the more individualized treatment and novel technologies and products，the Children Hemophilia Care will enter a new era.

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New insights in diagnosis of children hemophilia CHEN Zhen-ping，FANG Yong-jun 2017, 32(1): 23-28.  DOI: 10.19538/j.ek2017010606 Abstract ( )   Hemophilia A（HA） and HB is a X-link recessive hereditary hemorrhagic disorder with genetic characteristics of male suffering and female carrying. In this paper，we will focus on three aspects of hemophilia，including the genetic diagnosis，inhibitor of factors and pharmacokinetics. The method of gene diagnosis for hemophilia included DNA sequencing，PCR，DGGE，SSCP，etc. At present，the FⅧ intron 22 inversion analysis can be used as a clinical screening test for severe HA. Prenatal diagnosis is helpful to reduce the birth rate of hemophilia. The factor inhibitor causes invalid replacement therapy for hemophilia. Individual hemophilia patients have different pharmacokinetics（PK）. In addition to considering the bleeding event，the PK parameters should be considered as an important indicator for adjustment of individual preventive treatment. The relevant PK parameters include the half-life，clearance rate，in vivo recovery rate（IVR），the area under the curve（AUC），peak concentration，etc. The influencing factors for PK include age，body weight，blood type and von Willebrand factor（vWF） level. The above progress contributes to individual adjustment of treatment strategy for patients with hemophilia.

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Rehabilitation assessment and treatment in children with hemophilia DU Qing，LI Xin，YUAN Xiao-jun，et al. 2017, 32(1): 29-31.  DOI: 10.19538/j.ek2017010607 Abstract ( )   Hemophilia is a hereditary hemorrhagic disease caused by deficiency of coagulation factors. With the development of science and technology，improving the quality of life of hemophilia children has attracted more and more attention on the basis of conventional therapy. By conducting rehabilitation evaluation and treatment during different phases，the function of children should be improved and disability occurrence should be decreased. Appropriate rehabilitation treatment can increase the recovery of injury，prevent or reduce the incidence of complication and deformity in children with hemophilia，and maintain the range of motion，thus improve the quality of life.

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Management  of inhibitors in hemophilic  children FENG Xiao-qin 2017, 32(1): 31-34.  DOI: 10.19538/j.ek2017010608 Abstract ( )   Hemophilic children have high incidence of inhibitor. In addition to genetic risk factors，non-genetic risk factors not only play an important role in the development of inhibitor，but also directly affect the treatment strategy of hemophilia. Surgical procedures and intensive treatment for severe bleeding increased inhibitor risk. Regular prophylaxis，especially after 20 exposure day，was associated with lower risk. Immune tolerance induction（ITI） to eradicate high-titre and high-responding inhibitors is the mainstay of management. The high-dose and low-dose ITI were equally effective in inducing tolerance in good-risk patients，but high-dose ITI could reduce intercurrent bleeding and achieve tolerance more rapidly than low-dose ITI. Immunomodulation is not recommended as a first-line component of ITI. Immunomodulation may be considered in subsequent ITI attempts in patients with high-responding inhibitors who do not respond to initial treatment. The addition of rituximab to ITI may be useful for patients with inhibitors that are resistant to standard treatment.

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Individualized prophylaxis in children with hemophilia　 HU Qun，LIU Yu-feng 2017, 32(1): 34-37.  DOI: 10.19538/j.ek2017010609 Abstract ( )   The prophylaxis can prevent disability  in hemophilia. The ideal regimen  has not been determined yet. Individualized prophylaxis in children with hemophilia will be the direction of future development. Designing a prophylactic project for severe hemophilia must take into account each patient’s bleeding phenotype，pharmacokinetic profiles and joint assessment，even thrombin time. Optimal outcome，such as reducing hemorrhage，preventing joint damage，and improving quality of life， can be achieved through an individualized prophylaxis.

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Emergency treatment of critically ill childhood hemophilia LI Chang-gang*，WANG Xi-ge 2017, 32(1): 37-40.  DOI: 10.19538/j.ek2017010610 Abstract ( )   Haemophilia is a group of rare hereditary hemorrhagic diseases. Medical staff in general outpatient department and emergency room usually lack the experiences in  diagnosis and treatment of hemophilia. So patients with haemophilia may often be missed or delayed diagnosis，and some of them might lost the best chance to receive treatment on time，which may lead to various complications，and even life-threatening events occur. Therefore，it is very necessary to establish the process of diagnosis， differential diagnosis and emergency treatment for the disease. It is especially necessary to identify patients who haven’t been diagnosed with the hemorrhagic disease before， make the assessment of the degree of bleeding for these patients，and quickly make out the diagnosis and reasonable treatment to prevent the occurrence of related complications.

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Multidisciplinary treatment for children with hemophilia ZHOU Min，LI Xiao-jing 2017, 32(1): 40-43.  DOI: 10.19538/j.ek2017010611 Abstract ( )   Hemophilia is an X-linked congenital bleeding disorder. Comprehensive care by a multidisciplinary team is the optimal treatment model for hemophilia. Multidisciplinary team members include pediatricians and/or adult hematologists，nurses，the experts in surgery，stomatology and laboratory，etc. Comprehensive care includes not only factor replacement therapy but also multidisciplinary management for surgery， oral cavity problem， vaccine， genetic counseling，etc. Coordinated by a multidisciplinary team， comprehensive care can improve the quality of life of patients with hemophilia，   decrease the cost of treatment and the burden of family or society.

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Family therapy and nursing care for children with hemophilia JIN Jiao 2017, 32(1): 44-47.  DOI: 10.19538/j.ek2017010612 Abstract ( )   We reviewed some relevant literatures at home and abroad in recent years， and summarized the developmental status of family therapy and nursing care of children with hemophilia.During more than 40 years of practice， their life expectancy and their quality of life have significantly been improved after the hemophilia patients received treatment from the Hemophilia Treatment Center in Europe and the United States. Comprehensive hemophilia care and family therapy and care started late in China， and large domestic Hemophilia Centers played greater roles in improvement of the life quality of the patients，but there were still many problems. Family therapy and nursing care can improve the quality of life of children with hemophilia， and save the cost of health care. Family therapy and nursing care for children with hemophilia should be strengthened in Hemophilia Treatment Centers.

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Multimodal imaging evaluation for hemophilic arthropathy in children ZHANG Ning-ning，WU Run-hui，PENG Yun 2017, 32(1): 47-50.  DOI: 10.19538/j.ek2017010613 Abstract ( )   The multimodal imaging method can be used to asseses hemopiliac arthropathy in different periods， different degree and even different areas of the joints.In order to comprehend the understanding on pathological changes of hemophilic osteoarthropathy and clinical treatment strategy needs， and to provide accurate imaging diagnosis and prognosis evaluation，we need to know the characteristics and evaluation range in different imaging methods and the selection and put this into the clinical application and scientific research qualitatively and quantitatively. The imaging examination and evaluation system can evaluate early changes objectively and comprehensively and provide individual assessment for hemophilic arthropathy，which plays an important role in the diagnosis，staging， treatment and follow-up of hemophilic arthropathy.

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Value of combination of high frequency ultrasound and MRI in assessment of changes of hemophiliac arthropathy in children ZHANG Ning-ning，HUO Ai-hua，Lü Yan-qiu，et al 2017, 32(1): 51-54.  DOI: 10.19538/j.ek2017010614 Abstract ( )   Objective    To discuss the value of combination of high frequency ultrasound and MRI in assessment of changes of joints involvement in children with haemophiliac arthropathy. To compare the consistency of the two methods in detecting the disease. Methods    Totally 17 cases of children with hemophilia were included，and 6 knee joints，6 ankle joints and 5 elbow joints were performed by ultrasonic and MRI examination. According to IPSG scores，assesement of effusion/hemothrosis，hemosidaring，cartilage loss and bone cyst was performed to get the comparison of two kinds of examination method in the detection consistency of soft tissue and osteochondral lesions. Results    The two kinds of examination methods for detection of joint disease has a high consistency in synovial thickening， hemosiderin deposition，cartilage loss and bone destruction；Kappa＝1.000，0.850，0.761 and 0.876（P＜0.05）；in joint effusion they showed medium consistency，and kappa value was 0.605（P＜0.01）. Detection consistency in joint cyst was poor，and Kappa value was 0.261（P＝0.110）. Conclusion　On the detection of soft tissues and osteochondral joint changes of hemophilia in children， ultrasound and magnetic resonance imaging have poor consistency in the bone cyst，while in the detection of other pathological changes，two kinds of examination have high consistency in detection rate.

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Value of 3D T1 weighted water selective excitation sequence in cartilage display of pediatric hemophiliac joints   Lü Yan-qiu，CHENG Hua，ZHANG Ning-ning，et al 2017, 32(1): 55-58.  DOI: 10.19538/j.ek2017010615 Abstract ( )   Objective    To detect the value of 3D T1W-WATS sequence on cartilage display of hemophilic arthropathy using quantitative and qualitative assessment. Methods    Totally 16 joints including 8 knees and 8 ankles （13 joints with hemosiderin deposition） from 16 hemophilia patients were scanned on a 3.0 T MR （Achieva TX，Philips Health care，Best，The Netherlands） with sagittal 3D T1W-WATS sequence and 2D T2*W sequence. Signal to noise ratio （SNR） of cartilage and relative contrast between cartilage and surrounding tissues were employed to measure the performance of two sequences quantitatively：SNRcartilage= SIcartilage/standard deviationnoise， relative contrast=（SIcartilage-SIsurrounding tissues）/（SIcartilage+SIsurrounding tissues）. Cartilage of 13 joints were divided into 142 subareas according to the literature. The impact of hemosiderin deposition was evaluated by counting the number of subareas where the display of cartilage was affected qualitatively. Results    SNR of 3D T1W-WATS sequence was（53.93±18.80），and SNR of 2D T2*W sequence was（52.96±24.32）. There was no statistical difference for SNR between two sequences（P＞0.05）；3D T1W-WATS sequence exhibited better relative contrast between cartilage and bone marrow， cartilage and fat， cartilage and fluid than 2D T2*W sequence（P＜0.05）；42 of 142 subareas of cartilage were covered with hemosiderin partially or completely in 2D T2*W sequence，while the number was only 16 in 3D T1W-WATS sequence，there being statistical difference （P＜0.05）. Conclusion    3D T1W-WATS sequence is superior in the evaluation of cartilage display for hemophiliac joints with better relative contrast and immunity to hemosiderin deposition， compared with 2D T2*W sequence.

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Efficacy and safety of third-generation full length recombinant human coagulation factor Ⅷ in the prophylactic treatment of childhood patients with moderate-to-severe hemophilia A: A single center experience ZHOU Rong-fu，LI Qian，OUYANG Jian，et al 2017, 32(1): 59-62.  DOI: 10.19538/j.ek2017010616 Abstract ( )   Objective　To evaluate the number of annual bleeding and annual joint bleeding and safety of third-generation full length recombinant human coagulation factor Ⅷ（ADVATE） in the prophylactic treatment of childhood patients with moderate-to-severe hemophilia A. Methods　Fifteen children aged from 5 to 18 years with moderate-to-severe hemophilia A were enrolled in the one center，prospective，nonrandom，self-control study from Nov.01，2014 to Nov.30，2016. Children were treated with one-year long-course infusion of ADVATE with a dose of 20～40 U/kg twice-weekly or three times weekly. The numbers of annual bleeding and annual joint bleeding after the prophylaxis treatment were compared with that on-demand. Moreover，any adverse effect and serious adverse reaction related to ADVATE usage were documented during the study. The FⅧ inhibitor titer were measured with modified Nijmegen method before and after the prophylaxis every 3 to 6 months. Results　The FⅧ∶C for these 15 boys，median age 15，was less than 2.2%，with 8 patient severe type. The number of annual bleeding was reduced from（27.60±13.67） on-demand treatment to（2.57±1.36） after the prophylaxis treatment（P＜0.001），and the number of annual joint bleeding was also reduced from（11.13±8.81） to（0.93±1.10）（P＜0.001）. Seven patients had zero annual joint bleeding the mean annual bleeding for mild，moderate and severe degree was decreased significantly with zero times for severe degree bleeding after the prophylactic treatment. No adverse effect or serious adverse reaction related to ADVATE was recorded during one-year period treatment and FⅧ inhibitor detection was negative. Conclusion　Prophylactic treatment with ADVATE can effectively decrease the annual bleeding and annual joint bleeding in children with moderate-to-severe hemophilia A. This therapy is safe with FⅧ inhibitor test negative.

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Clinical significance of bone marrow megakaryocyte in childhood immune thrombocytopenia PAN Yan-sha，JIA Cang-song，CHEN Ting-ting 2017, 32(1): 63-65.  DOI: 10.19538/j.ek20170106017 Abstract ( )

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Frontier and prospect of the treatment for hemophilia HU Qun 2017, 32(1): 66-69.  DOI: 10.19538/j.ek2017010618 Abstract ( )

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Effect of hemarthrosis on the joints of patients with hemophilia FANG Yong-jun，HONG Kai 2017, 32(1): 70-75.  DOI: 10.19538/j.ek2017010619 Abstract ( )

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Comprehensive assession of hemophilia XU Wei-qun，TANG Ling，WANG Yan 2017, 32(1): 76-80.  DOI: 10.19538/j.ek2017010620 Abstract ( )