Table of Content

15 August 2018 Volume 36 Issue 8

  

Analysis of clinical features of children with Mycoplasma pneumoniae pneumonia with A2063G gene mutation in 23S rRNA V domain

　LI Yan, ZHONG Lili, ZHANG Bing, HUANG Han, DING Xiaofang, LIN Lin, CHEN Haofeng, HUANG Zhen, PENG Li, LIN Xiaojuan

. 2018, 36(8):  569.  doi:10.3969/j.issn.1000-3606.2018.08.001

Abstract ( 421 )   PDF (1066KB) ( 581 )  

Related Articles | Metrics

　Objective　To investigate the clinical characteristics of children with Mycoplasma pneumoniae pneumonia (MPP) who had detected A2063G mutation (A-to-G transition at position 2063 in domain V on the 23S rRNA domain) in bronchoalveolar lavage fluid (BALF). Methods　A foral of  297 hospitalized children diagnosed with MPP in the pediatric ward of the First Affiliated Hospital of Hunan Normal University from April 2016 to October 2017, were enrolled. All children were underwent fiberoptic bronchoscopy treatment and A2063G mutation test in 23S rRNA V domain in BALF specimen. The patients were divided into mutant group and non-mutant group according to genetic test, and clinical features between the two groups were compared. Results　Among the 297 cases, 155 cases were detected with A2063G mutation in the 23S rRNA V domain in the BALF, with a positive rate of 52.1%. Clinical features, such as gender, age, C-reactive protein  level, white blood cell count, pleural effusion and thoracic thickening percentage, the ratio of severe lesions found under fiberoptic bronchoscopy (erosion, plastic or yellowish-white phlegm plug), co-infection rate, the percentage of severe pneumonia and refractory MPP in the two groups of patients showed no significant difference （P>0.05）. The admission duration and fever duration in the mutant group were longer than those in the non-mutant group. The MP-DNA load in the BALF was higher than that in the non-mutant group. The MP-DNA load decreased slower and duration of fever was longer after the use of macrolide antibiotics in the mutant group, without statistical significance between the two groups (P<0.05). Logistic regression analysis revealed that the MP-DNA load and febrile days after the use of macrolide antibiotics were correlated with A2063G mutation in the 23S rRNA V domain. Conclusions　The MP-DNA load in BALF and the febrile days after using macrolide antibiotics may have certain reference value for identifying the A2063G mutation in 23S rRNA V domain in children with MPP.

Clinical significance of glomerular hyperfiltration in assessment of early renal damage in childhood type 1 diabetes

　SUN Hui, XUE Ying, HUANG Feng, WU Haiying, XIE Rongrong, WANG Fengyun, CHEN Xiuli, CHEN Ting, CHEN Linqi

. 2018, 36(8):  575.  doi:10.3969/j.issn.1000-3606.2018.08.002

Abstract ( 443 )   PDF (1041KB) ( 454 )  

Related Articles | Metrics

Objective　To investigate the screening value of glomerular hyperfiltration (GHF) in early renal damage of type 1 diabetes. Methods　The estimated glomerular filtration (eGFR) was calculated using the Macisaac’s equation in 100 cases of type 1 diabetes. According to eGFR’s level, the diabetes were divide into two groups: Normo-GFR group, 57 cases; and GHF group , 43 cases. The age, course of disease, glycosylated hemoglobin (HbA1c)、triglyceride (TG), renal glomerular damage indexes (UACR), and renal tubular damage indexes (urinary NAG/Cr, urinary α1-MG/Cr and serum NGAL) of each group were observed. Results　The HbA1c and TG values in the GHF group was significantly higher than those in the Normo-GFR group(P＜0.05). Serum NGAL values in the GHF group was significantly lower than those in the Normo-GFR group (P＞0.05). The UACR, urine NAG/Cr and urine α1-MG/Cr values between the GHF group and Normo-GFR group were not significantly different. Logistic regression analysis showed shorter diabetes duration and higher HbA1c levels were important influence factors of whether diabetes patients had hyperfiltration. Conclusions　GHF may reflect abnormal glucose and lipid metabolism that could be detected at early stage of the type 1 diabetes, but its independent correlation with diabetic kidney damage needs further research.

Differential analysis of clinicopathological features in children with hepatoblastoma

　NI Jing, CAI Rong, REN Gang, TANG Wen, WANG Xinyun, HE Wenguang

. 2018, 36(8):  580.  doi:10.3969/j.issn.1000-3606.2018.08.003

Abstract ( 541 )   PDF (1055KB) ( 276 )  

Related Articles | Metrics

Objective　To explore  the differences of the clinicopathological characteristics of children with hepatoblastoma (HB). Methods　Clinical data of 68 patients with HB confirmed by surgery and pathology in Xinhua Hospital were retrospectively analyzed. Results　In the 68 children (44 males and 24 females) with HB, 55 cases were under 3 years of age, and 13 cases older than 3 years. The patients presented with elevated AFP level and unifocal lesions when diagnosed. 27 patients were classified into high risk group according to SIOPEL, and 13 patients were in PRETEXT Ⅳ stage according to imaging results before operation. There are 22 children with metastases and/or extrahepatic abdominal diseases and/or involvement of the main portal vein or of all three hepatic veins and/or the IVC. There was an obvious relationship between PRETEXT staging and the age, the level of AFP and the pathological classification. (P<0.05). There was an obvious relationship between the pathological classification and the level of AFP (P=0.002).  The pathological classification showed association with AFP level (P=0.002). There was also an obvious relationship between the pathological classification and the risk classification (P=0.043). Conclusions　The difference of clinicopathological features in children with hepatoblastoma may help to guide clinical treatment and prognostic evaluation.

An evaluation of clinical treatment in children with rhabdomyosarcoma

　ZHOU Li, FANG Yongjun, RUI Yaoyao, WU Peng，HE Lulu

. 2018, 36(8):  584.  doi:10.3969/j.issn.1000-3606.2018.08.004

Abstract ( 325 )   PDF (1021KB) ( 240 )  

Related Articles | Metrics

　Objectives　To investigate and evaluate clinical treatment and prognosis of children with rhabdomyosarcoma (RMS). Methods　A total of 31 cases of children (19 males and 12 females) diagnosed as RMS in Children's Hospital of Nanjing Medical University from December 2009 to December 2016 were followed up, with a median age of onset at 42.2 months (7.2～109.9 months). Prognosis of patients with RMS was clinically investigated. Result　Among the 31 cases, there were 2 cases with stage Ⅰ, 6 cases with stage Ⅱ, 16 cases with stage III and 7 cases with stage IV. Protocol RS-2009 of Shanghai Children’s Medical Center was used to treat these patients until May 1, 2017. Two cases were lost to follow up during the treatment course, one case lost after replase. Median of the follow-up time is 39.3 months. After high intensive treatment, two cases with stageⅠachieved complete remission and long-term survival, two cases with stage Ⅱ and four cases with stage Ⅲ recurred, three cases with stage Ⅳ recurred and died soon, one with stage Ⅳ did not response to treatment and progressed to death. The 3-year event free survival (EFS) of the cases in stage Ⅰ, Ⅱ and Ⅲ was (61.8%±12.9%), the 3-year overall survival (OS) of them was 100%, while the 3-year EFS and OS both were (42.9±18.7%) for the cases with stage Ⅳ. The 3-year EFS of the cases with no radiotherapy was 37.7%±14.2%, while it was 82.1%±11.7% for those with regular radiotherapy. Conclusions　The protocol RS-2009 of SCMC in the treatment of RMS in stage Ⅰ, Ⅱ and Ⅲ can get a curative effect, but it need to be improved and optimized to the cases with stage Ⅳ. Radiotherapy can highly increase EFS.

A case of neurofibromatosis type I with hypertension as the main manifestation and literature review

　WEI Liya, GONG Chunxiu, CAO Binyan, WU Di

. 2018, 36(8):  587.  doi:10.3969/j.issn.1000-3606.2018.08.005

Abstract ( 495 )   PDF (1049KB) ( 292 )  

Related Articles | Metrics

Objective　To investigate the clinical characteristics of neurofibromatosis type I (NF1) in children with hypertension. Methods　Clinical data of a pediatric patient with NF1 presented mainly with hypertension was retrospectively analyzed, and related literature was reviewed. Results　we report a case of uncontrolled persistent hypertension in a 4-yearold child with more than 30 café-au-lait spots. The enhanced MRI of the renal artery showed the right renal artery stenosis and aneurysm. Gene test found a de novo heterozygous c.3623delT mutation in NF1. At last, he was diagnosed with NF1, but he had no neurofibroma, Lisch nodules, axillary freckles, and other performances of neurofibromatosis. His blood pressure was well controlled after percutaneous transluminal renal angioplasty.  Conclusions　For children of persistent hypertension with skin lesions only, NF1 should be considered and blood pressure be monitored. Once the vascular lesions were found, intervention should be done timely.

Study of clinical characteristics and prognosis in pediatric inv(16)/CBFβ-MYH11-positive acute myeloid leukemia　

WU Jun, YU Shui, LU Aidong, ZUO Yingxi, JIA Yueping, ZHANG Leping

. 2018, 36(8):  590.  doi:10.3969/j.issn.1000-3606.2018.08.006

Abstract ( 897 )   PDF (1120KB) ( 523 )  

Related Articles | Metrics

Objective　To investigate clinical and biological characteristics and prognosis of pediatric inv(16)/CBFβMYH11-positive acute myeloid leukemia. Methods　From December 2008 to September 2017, 30 patients diagnosed as inv(16)/ CBFβ-MYH11-positive acute myeloid leukemia were enrolled in this study and their clinical and biological features were retrospectively analyzed. Following-up of 30 patients were ended in November 2017, and the survival status was calculated by K-M curve and prognostic factors were analyzed by COX model. Results　At preliminary diagnosis of the total 30 patients, 19 patients had hepatomegaly (63.3%), 18 cases had splenomegaly (60.0%), and 19 cases had lymphoadenopathy (63.3%). Patients at diagnosis with high leukocyte counts （≥50×109/L） accounted for 63.3%. In all patients, the 5-year anticipated EFS and OS were （84.2±7.6）% and（89.8±5.6）%, respectively. Cox multivariate regression analysis showed that gender, age at onset, leukocyte count, lactate dehydrogenase level, extramedullary leukemia, c-kit gene mutation, chromosome inv (16) alone, initial CBFβ-MYH11 quantification and the response to the first course of treatment did not statistically affect EFS and OS (P>0.05).  The level of CBFβ-MYH11 gene at diagnosis was used as the baseline to determine whether the level of gene after treatment had a more than 2 logarithmic (2-log) reduction. When patients with a more than 2-log reduction of CBFβ-MYH11 transcript levels (≥2-log) compared with other patients after a course of induction therapy, the differences of 5-year EFS and OS were not statistically significant (P>0.05). When periodically monitoring the CBFβ-MYH11 gene, three cases with progressively increasing CBFβ-MYH11 fusion gene during the consolidation therapy were identified, and no relapse was found after adjustment of treatment intensity. All of them survived so far. Conclusions　In patients with inv(16)/CBFβ-MYH11-positive AML, hepatosplenomegaly, lymphadenopathy and high initial leukocyte counts are most common features, and the therapeutic effect is good. It is important to regularly monitor CBFβ-MYH11 quantitation in the consolidation therapy phase.

Macrophage activation syndrome preceded by necrotizing lymphadenitis in two children

　HUANG Ying，HE TingYan， YANG Jun

. 2018, 36(8):  595.  doi:10.3969/j.issn.1000-3606.2018.08.007

Abstract ( 505 )   PDF (1329KB) ( 437 )  

Related Articles | Metrics

　Objective　To explore the clinical phenotype, treatment and prognosis of macrophage activation syndrome (MAS) preceded by Kikuchi–Fujimoto disease (KFD). Methods　Clinical data of two children with MAS preceded by KFD were retrospectively analyzed, and related literatures were reviewed. Result　Two cases, aged fourteen year two month and ten year seven month, respectively, were admitted due to fever. Palpation of the two boys showed enlargement of cervical lymph nodes and mild hepatosplenomegaly. Experimental examination revealed a progressively elevated levels of lactate dehydrogenase, aspartate aminotransferase and ferritin. Lymph node pathology showed destruction of lymph node structure and lymphocyte necrosis with positive histiocytic marker CD123 and CD68. Bone marrow smear of the first case showed a lot of tissue cells and hemophagocytic cells.  Symptoms were soon quickly relieved with treatment of dexamethasone and cyclosporine. Bone marrow smear of the second case revealed a small amount of tissue cells and sparse hemophagocytic cells. Symptoms were soon quickly relieved with treatment of oral prednisone. Conclusions　When KFD manifests as sustained high fever for more than two weeks, accompanied by progressively elevated levels of ferritin and lactate dehydrogenase, and gradually decreased level of fibrinogen, macrophage activation syndrome should be considered. Timely treatment using glucocorticoid or its combination with cyclosporine is effective and leads to good prognosis.

Hemophagocytic syndrome induced by Streptococcus pneumoniae: a case report and literature review

SHAN Mingfeng, ZHOU Kai, TIAN Ye

. 2018, 36(8):  599.  doi:10.3969/j.issn.1000-3606.2018.08.008

Abstract ( 451 )   PDF (1156KB) ( 387 )  

Related Articles | Metrics

Objective　To explore the clinical characteristics of hemophagocytic syndrome (HPS) associated with Streptococcus pneumoniae. Methods　The clinical data of one child with HPS associated with Streptococcus pneumoniae was retrospectively analyzed. The relevant literatures were reviewed. Results　A 12-months boy presented with fever, cough, shortness of breath, hepatomegaly as the main clinical manifestations, and accompanied by cytopenia, hyperferroteinemia, high level of soluble IL-2 receptor and C-reactive protein, and phagocytes were observed in bone marrow. Lung imaging examination indicated lung infection, and multidrug-resistant Streptococcus pneumoniae was detected. After the treatment of antibiotics, immunoglobulin, dexamethasone, etc, the condition was improved. Four cases with relatively complete clinical data were retrieved from literature. There were three males and one female aged 3, 8, 25 and 37 years old respectively. All of the cases were accompanied by fever, and other common features include hepatomegaly, cytopenia, abnormally elevated C-reactive protein and hemophagosis. The reported infection includes two in the lung, one in the ear and one as sepsis. Two patients with severe underlying diseases had poor prognosis and the other two had better prognosis. Conclusions　When patients diagnosed with Streptococcus pneumoniae infection should be highly suspected of HPS, particularly with multi-resistant bacteria after effective antibiotic therapy, high fever, hepatosplenomegaly, cytopenia, abnormally elevated C-reactive protein. Immediate laboratory investigation is highly recommended.

Utility of blink reflexes in subtyping of inherited polyneuropathy

　SUN Ruidi1，WANG Hengdong2, JIANG Jun1

. 2018, 36(8):  602.  doi:10.3969/j.issn.1000-3606.2018.08.009

Abstract ( 360 )   PDF (1023KB) ( 359 )  

Related Articles | Metrics

　Objective　To investigate the utility of R1 latency of blink reflexes in typing of inherited polyneuropathy: Charcot-Marie-Tooth (CMT). Methods　Patients diagnosed with CMT by a genetic test underwent blink reflex testing and extremity NCS were retrospectively analyzed. Blink reflex R1 latency, extremity NCS, and severity were compared between demyelinating subtypes and axonal subtypes. Results　We included 24 demyelinating (14 males and 10 females) and 12 axonal (6 males and 6 females) CMT patients. R1-latency in demyelinating CMT group (14.41±0.73 ms) were longer than that in axonal CMT group (11.39±0.89 ms) (P<0.01). R1-latency were all >13 ms in demyelinating CMT group and ≤13 ms in axonal CMT group. Conclusions　Blink reflex test is reliable for identification of inherited demyelinating polyneuropathy regardless of severity and can guide genetic testing.

Clinical characteristics and genetic analysis of two children with Andermann syndrome

　CHEN Yin，RUAN Yiyan, WANG Jinqiu

. 2018, 36(8):  605.  doi:10.3969/j.issn.1000-3606.2018.08.010

Abstract ( 722 )   PDF (1699KB) ( 459 )  

Related Articles | Metrics

　Objective　To analyze the clinical features and gene mutations of Andermann syndrome. Methods　Clinical features and gene testing results in two children with Andermann syndrome were retrospectively analyzed. Results　The proband is a 6 years old boy, and his elder brother is 8 years old, both of them had progressive sensory neuropathy and developmental delay. Electromyography of the younger brother indicates nerve damage, and cranial magnetic resonance shows corpus callosum dysplasia; the elder brother has joint contracture and scoliosis. Genetic analysis found the two patients had homozygous missense mutation of c.592 C>T (p.R198C) in SLC12A6, both of their parents were heterozygous mutation carrier of c.592 C>T (p.R198C). SLC12A6 mutations cause clinical symptoms by affecting the normal function of the K+/Cl－ cotransporter 3. Conclusions SLC12A6 is the only known pathogenic gene of Andermann syndrome, and its mutation types are related to the severity of clinical phenotypes.

Clinical and etiological features of hand, foot and mouth disease with facial rash

　XU Shanshan, LI Huajun, QIAO Peng, CHEN Jian, ZHANG Xi, LIN Xiaoyan, HUANG Suli

. 2018, 36(8):  609.  doi:10.3969/j.issn.1000-3606.2018.08.011

Abstract ( 374 )   PDF (1049KB) ( 246 )  

Related Articles | Metrics

Objective　We aimed to compare the clinical and etiological features of hand, foot, and mouth disease (HFMD) with or without facial rash. Methods　Clinical data of children with HFMD reported from January to December 2016 and pathogen test results of some children were retrospectively analyzed. The children were divided into two groups based on whether it is combined with facial rashes or not. Results　A total of 1134 cases of atypical HFMD with facial rash were enrolled, accounting for 9.92% of all HFMD cases (1134/11430), which easily occurring among children under one year of age and over 3 years old. The clinical symptoms of cases with facial rash were milder than those without facial rash, and the incidence of fever and nervous system complications were lower. Coxsackievirus A6 infection was predominant, and the ratio was significantly higher than that without facial rash (P<0.05). Conclusions　The majority of HFMD with facial rashes was caused by Coxsackie virus A6 infection and its clinical symptoms were mild and atypical.

Study of the characteristics of the maturation of gastrointestinal motility and secretion function in preterm infants  of different gestational age

　LU Lu, WU Wei, GU Meng, GUO Huimei, YAO Jiejin, TU Wenjuan

. 2018, 36(8):  613.  doi:10.3969/j.issn.1000-3606.2018.08.012

Abstract ( 358 )   PDF (1089KB) ( 414 )  

Related Articles | Metrics

Objective　To investigate the characteristics of maturation of gastrointestinal motility and secretion function in different gestational age preterm infants within 28 days after preterm infant birth. Methods　The preterm infants were divided into young gestational age (GA) group (<32 weeks, n=15) and older GA group (32 - 37 weeks, n=15) by GA, and 15 term infants were selected as control group. On the 7th, 14th, 21th and 28th day after birth of preterm infants, and the 7th day after birth of term infants, gastric emptying rate (GER) and gastric motility index (MI) were detected by ultrasound; pH and pepsinogen (PG) levels of gastric juice extracted by stomach tube were tested. Results In both GA groups, GER and MI gradually increased with age. GER on the 21th and 28th day and MI on the 14th to 28th day of young GA group were lower than that of older GA group (P<0.05). And the time of GER and MI levels up to term infants in young GA group was later than that of older GA group. The pH in two GA groups both increased at first and then decreased (P<0.05). Compared to the older GA group, the pH was higher at 7th, 14th and 21th day in young GA group, and the difference was statistically significant (P<0.05). Only the gastric pH on the 14th and 21st day of young GA group was higher than that of term infants (P<0.05). PG level of young GA group in each point was lower than that of older GA group (P<0.05). Compared to term infants, the PG level of both groups of preterm infants was lower(P<0.05), and the PG level only on the 28th day of older GA group showed no difference with that of term group (P>0.05). Conclusions The gastrointestinal motility and gastric secretion of young GA group was lower than that of older GA group and term group. More cautious feeding strategies should be adopted for preterm infants with younger GA. GER and MI can be used as objective indicators to evaluate gastrointestinal motility of preterm infants.

Congenital tufting enteropathy caused by mutation of EPCAM gene: a case report and review of literature

YUAN Chuanjie, HUANG Zhuo, SUN Xiaomei, LIU Ying, WU Jing

. 2018, 36(8):  618.  doi:10.3969/j.issn.1000-3606.2018.08.013

Abstract ( 811 )   PDF (1105KB) ( 627 )  

Related Articles | Metrics

　Objective　To explore the clinical characteristics of diagnosis and treatment in patients with congenital tufting enteropathy. Methods　A rare case of congenital tufting enteropathy was diagnosed at West China Second University Hospital, Sichuan University in July 2016, Clinical data of congenital tufting enteropathy was retrospectively analyzed, and related literature were reviewed. Original papers on congenital tufting enteropathy published until Oct.2017 were retrieved at PubMed, CNKI databases and Wangfan databases by the use of the key words " EPCAM ", " congenital tufting enteropathy". Results　A 2-year and 2-month old girl began to develop intractable chronic diarrhea soon after birth, accompanied with growth restriction, repeated infections, anemia and so on. Next-generation DNA sequencing revealed a homozygous C>A substitution at exon 3 in EPCAM of the affected patient (c.412C>T, p. R138X), and she was finally diagnosed as congenital tufting enteropathy. A total of 60 cases of congenital tufting enteropathy caused by EPCAM gene mutation were found in 14 papers, all of which were published abroad, and 34 EPCAM gene mutations were reported, 11 of them were located in exon 3. Conclusions　Congenital tufting enteropathy is rare and difficult to diagnosis. Chronic intractable diarrhea associated with growth retardation in infants should be highly alert to the possibility of congenital tufting enteropathy.

The behavior characteristics and executive functions of ADHD children with autistic traits

　JI Yiting, FAN Yun, ZHANG Ting, FENG Wei, SHU Yan, CHEN Liangliang, ZHU Tao, LI Fei, LIU Xin

. 2018, 36(8):  621.  doi:10.3969/j.issn.1000-3606.2018.08.014

Abstract ( 359 )   PDF (1054KB) ( 358 )  

Related Articles | Metrics

Objective　To assess behavior characteristics and social functioning of children with attention-deficit/ hyperactivity disorder (ADHD)-autistic traits (ATs) and ADHD control (without ATs), and to explore the associations between the executive functions and the impairments of behavior. Method　A total of 175 ADHD children diagnosed in the Departmen of Behavior Pediatrics in Shanghai Children's Medical Center and Xinhua Hospital were selected and divided into ADHD with autism traits (ADHD-ATs) group (n=80) and ADHD without autism traits (ADHD) group (n=95) by the scores of social response scale. All participants completed IQ test and questionnaires such as SNAP-Ⅳ, Conners Parent Symptom Questionnaire and Behavior Rating Inventory of Executive Function.  Results  There was no significant difference between the two groups in age, gender and IQ (P＞0.05). ADHD-ATs children behaved worse in hyperactivity/impulsive behavior (P＜0.05), worse impaired in theory of mind than ADHD children (P＜0.05). Further regression analysis found that the impact of cold and hot executive functions had a significant relationship with social behavior problems (behavior management index: β =－1.17, P <0.05, 95% CI: －2.00~－0.34; theory of mind (oral): β =0.72, P<0.05, 95% CI:0.51~0.93). Conclusions　Children with ADHD-ATs had more serious symptoms than children with ADHD, and with impaired cold and hot executive functions.
·

Advances in influencing factors on intestinal microbiota establishment and its relationship with diseases in infants

WANG Xiaoming, YANG Jing

. 2018, 36(8):  626.  doi:10.3969/j.issn.1000-3606.2018.08.015

Abstract ( 427 )   PDF (1074KB) ( 419 )  

Related Articles | Metrics

　Human gastrointestinal microbiota plays a major role in the regulation of gastrointestinal immune activity and development of intestinal system. Normal intestinal microflora modulated the physiological functions and affected development of diseases. Infancy is a critical period for the establishment of gastrointestinal microbiota. Intestinal flora in early infancy is dynamic and fragile, and is liable to be influenced by many factors. In this paper, we reviewed the research progress on the influencing factors of infant intestinal microbiota establishment and its relationship with metabolic diseases.

Advances in the study of environment factors on DNA methylation and its association with congenital heart diseases  

ZHOU Shuang, SHI Xin, CHEN Sun, YU Yu

. 2018, 36(8):  630.  doi:10.3969/j.issn.1000-3606.2018.08.016

Abstract ( 369 )   PDF (1054KB) ( 297 )  

Related Articles | Metrics

The current internationally recognized etiology of congenital heart disease is mainly the result of a combination of heredity and the environment factors. Environment-induced changes in epigenetic level may be a key reason for the origin of the disease, which have important clinical significance, and DNA methylation is a major epigenetic mechanism. This article reviews the progress on some environmental factors and DNA methylation and their association with congenital heart disease so that we can better understand the pathogenesis of congenital heart diseases, which is good for early diagnosis and early treatment.

Progress on clinical use of decitabine in hematological malignancies

WAN Yuanyuan

. 2018, 36(8):  634.  doi:10.3969/j.issn.1000-3606.2018.08.017

Abstract ( 246 )   PDF (1085KB) ( 418 )  

Related Articles | Metrics

Development of malignant tumors is related to epigenetic changes caused by abnormal methylation of DNA. Therefore, the demethylation treatment represented by decitabine has been gradually playing an important role in the field of tumor therapy in recent years. This article reviews the clinical use of decitabine in myelodysplastic syndromes, acute myeloid leukemia, juvenile myelomonocytic leukemia and other hematological malignancies, and provides evidences for optimal dosage and safety of this drug.

New therapy of childhood refractory/relapsed acute lymphoblastic leukemia

ZHAO Yang

. 2018, 36(8):  639.  doi:10.3969/j.issn.1000-3606.2018.08.018

Abstract ( 437 )   PDF (1019KB) ( 1007 )  

Related Articles | Metrics

Nowdays, the curability of childhood acute lymphoblastic leukemia(ALL) has improved dramatically. However, the subgroup of children with refractory/relapsed ALL still presents a dismal prognosis. New trials such as targeted therapy and immunotherapy are used in practice based on a better genomic characterization, and good clinical effect has been achieved. In this article, we review the current new treatments of childhood refractory/relapsed ALL.