Table of Content

15 May 2018 Volume 36 Issue 5

  

Comparison of two kinds of induction therapy in childhood acute myeloid leukemia

　ZHAI Qin, WANG Yi, HE Hailong, LU Jun, XIAO Peifang, DING Fei, LYU Hui, SUN Yina, FAN Junjie, HU Shaoyan

. 2018, 36(5):  321.  doi:10.3969/j.issn.1000-3606.2018.05.001

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Objective　To compare the efficacy and safety of induction therapy in 3+7 protocol and 3+10 protocol in children with acute myeloid leukemia (AML). Methods　Two protocols were carried out in our hospital during January 2010 to January 2015, namely 3+7 protocol（AML-06，A group） and 3+10 protocol (modified AML protocol, B group). A total of 56 cases aged from 1 year-old to 13 year-old were enrolled in A group with male to female ratio at 31:25. Five of them were classified as FAB M1, 25 as M2, 11 as M4, 10 as M5, 2 as M6 and 3 as M7. Another 44 cases aged from 1 year to 12 years were enrolled in B group with a male to female ratio at 26:18, and 17 cases were classified as FAB M2, 14 as M4, 9 as M5, 2 as M6, and 2 as M7. Efficacy and adverse events were compared between the two groups. Results　The complete remission rate (CR) of B group was 70.4%, while CR in A group was 48.2%. Considering the CR, 3+10 protocol showed higher efficacy than 3+7 protocol (P< 0.05). The major adverse event was bone marrow suppression. Treatment-related mortality (TRD) in A group was 1.8%, which was lower than that in B group (2.3%). The overall survival rate in A group was 75.0%, which was lower than that in B group (86.4%, P< 0.05). Conclusions　The induction therapy of 3+10 protocol and 3+7 protocol showed effectiveness for AML treatment. The 3+10 protocol showed a higher CR than 3+7 protocol with no TRD increase, indicating that the 3+10 protocol should be recommended for AML treatment in children.

Clinical analysis of 60 cases of childhood malignant lymphoma

　WANG Yu，ZHANG Leping，ZUO Yingxi

. 2018, 36(5):  326.  doi:10.3969/j.issn.1000-3606.2018.05.002

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　Objective　To summarize the clinical and biological characteristics and to analyze the prognosis of childhood lymphoma. Methods　Clinical data of 60 patients diagnosed as lymphoma during the past ten years were retrospectively studied. All cases were classified according to the WHO classification criteria of lymphoma and the St. Jude staging system. Results　Of all the 60 patients, the ratio of male to female was 2.5: 1. The median age was 8 years old (range from 6 months to 15 years). Localized mass was the most common chief complaint for first consultation accounting for 35.0%。 The proportion of the bone marrow and central nervous system involvement was 63.3% and 6.7%, respectively. Hodgkin lymphoma (HL) and non- Hodgkin lymphoma (NHL) counted for 8.3% and 91.7%, respectively. All HL were classified as CHL. As to NHL, LBL, BL/ B-ALL, ALCL, and DLBCL were the main types. According to St. Jude staging, the advanced stage accounted for 95.0%. There were 8 cases with progressive disease/relapse with median time of 14 months (ranged from 6 months to 84 months). 6. In all patients, the 5-year anticipated overall survival (OS) rate was (78%±6%); the 5-year OS for HL and NHL were 100% vs (76%±8%). The survival rate of the two groups showed no significant difference (P=0.270). Cox multivariate analysis indicated that sex (P=0.038) was independent prognostic factors. Conclusions　Pediatric lymphoma occurred more in male than in female, peaked at school age. The main histologic type was NHL. Most patients were diagnosed as advanced stage. By gender, female is a poor factor that affects prognosis.

A case report of childhood extra-adrenal pheochromocytoma with malignant cell and literature review

WANG Yongren，SHI Yi, HUANG Jie, FANG Yongjun

. 2018, 36(5):  331.  doi:10.3969/j.issn.1000-3606.2018.05.003

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Objectives　To investigate the diagnosis, treatment and genetic characteristics of pheochromocytoma (PGL) in children. Methods　Clinical characters and gene mutation analysis of a child with PGL were retrospectively analyzed and discussed；similar cases in the literature were reviewed. Results　The patient was a 10-year old-boy without obvious complaint but transient headache. Urine 3 methyl-4 hydroxy mandelic acid and homovanillic acid were in normal range, and the pathological reports revealed some malignant cells located at the margin of the tumor, while CT scan of the neck, chest and abdomen were negative, and the cranial plus spine revealed no significant abnormalities by MRI. Gene test of the patient and his parents found mutations in SBHB gene. Thus the diagnosis was confirmed as benign, nonfunctional, extra adrenal pheochromocytoma. The patient was treated with surgery, without chemotherapy, and followed up regularly. Conclusions 　The diagnosis of PGL relies on pathological analysis, and the classification was made by catecholamine metabolites, imaging results, and gene sequencing. Implementation of chemotherapy was dependent upon if there is any distant or invasive metastasis, or a relapse is present.

Imaging and prognostic analysis in different molecular subtypes medulloblastoma

　ZHANG Yuting, LI Lusheng, LIANG Ping, ZHAI Xuan,HE Ling, CAI Jinhua

. 2018, 36(5):  334.  doi:10.3969/j.issn.1000-3606.2018.05.004

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Objective　To detect molecular subtypes of medulloblastoma, and its correlation with prognosis. Methods　 Surgically treated 32 cases of primary medulloblastoma from 2010-2013 were collected, the molecular subtypes were determined by immunohistochemical detection of GAB1 and YAP1 protein in the sample. Clinical characteristics, imaging features and survival condition of different molecular subtypes were analyzed. Results　Molecular typing of the 32 cases (21 males and 11 females) shows 4 (12.5%) cases of SHH, 7 (21.9%) cases of WNT and 21 (65.6%) cases of non-SHH. There was no significant correlation of molecular subtypes with age, gender and pathological classification. Three-year progression free survival rate in SHH, WNT and non-SHH/WNT subtypes were 75%, 57.1% and 38.1%, respectively. Three-year progression free survival rate was significantly higher in patients under 3 years old group than that in patients over 3 years old group in non SHH/WNT (P=0.047). Conclusions　The prognosis of SHH was better than WNT, WNT was better than non-SHH/WNT type, prognosis in patients under 3 years old group of non-SHH/WNT was better than that in patients over 3 years old group.

Analysis of fungal infection complicated with hemophagocytic lymphohistiocytosis in three children

XU Jinyun, ZHOU Xiaoheng, MENG Huihua, DU Lian, LI Ming, LUO Jianming

. 2018, 36(5):  339.  doi:10.3969/j.issn.1000-3606.2018.05.005

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Objective　To improve clinically the recognition of fungal infection associated with hemophagocytic lymphohistiocytosis (HLH) in children. Methods　Clinical data of 3 children with fungal infection complicated with HLH in our hospital was retrospectively analyzed. Results　All the 3 cases complained of recurrent fever, 2 cases with cough and one case with vomiting. Hepatosplenomagaly and lymphadenectasis were found in the medical examination. The time of diagnosis of fungal infection through etiological examination was 5 days after admission. It was further diagnosed as hemophagocytic lymphohistiocytosis after failure of effective antifungal therapy. Routine blood test showed the counts of leukocytes were increased in early stage, while the number of platelets and hemoglobin decreased in different degrees. The recovery is not satisfactory using antifungal therapy alone, and 2 of them are gradually aggravated and treated with mechanical ventilation. On the basis of antifungal therapy, 2 cases were treated under HLH-2004 regimen, 1 received dexamethasone treatment. All the 3 cases received intravenous immune globulin, and showed improvement. Conclusions　Fungal infection complicated with HLH in childhood is rare. The effect of simple antifungal therapy on the progression is limited. However, increasing immunosuppressive therapy based on effective antifungal therapy can improve the prognosis.

A case of childhood systemic lupus erythematosus complicated with macrophage activation syndrome and literature review

WANG Linli, GUAN Fengjun

. 2018, 36(5):  344.  doi:10.3969/j.issn.1000-3606.2018.05.006

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　Objective　To study the clinical features and treatments of childhood systemic lupus erythematosus (SLE) complicated with macrophage activation syndrome (MAS). Methods　Clinical data of a patient with SLE complicated with MAS were retrospectively analyzed, and relevant literatures were reviewed. Results　An eight-year-old girl diagnosed as SLE two years ago was admitted to the hospital with complaint of high fever for over one week. Peripheral blood cytopenia, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia and hepatolienomegaly occurred after admission gradually. Thus the child was diagnosed definitely as MAS. Conclusions　SLE complicated with MAS is not rare. The disease progresses very rapidly and can be life-threatening, while early diagnosis is very difficult. The key to improve prognosis is early diagnosis and treatment.

Comparative analysis of MRI and clinical characteristics of juvenile dermatomyositis

DAI Chunyang1,YUAN Dingfen1, ZHEN Hui2, YAO Zhirong3

. 2018, 36(5):  348.  doi:10.3969/j.issn.1000-3606.2018.05.007

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Objective　To explore the relationship of magnetic resonance imaging (MRI) performance in juvenile dermatomyositis (JDM) with clinical characteristics, especially serum muscle enzyme abnormalities. Methods　The images of 50 cases of JDM were reviewed, and the affected skin, subcutaneous connective tissue, muscle fascia and muscle were evaluated. The range of muscle edema under MRI images was categorized into 1-3 grades from mild to complete involvement. By comparing the MRI classification with muscle enzyme, we aim to reveal the linkage between the degree of muscle damage and the levels of muscle enzymes. The sequence of MRI examination of the thigh included rapid spin echo sequence FSE T1W, and the fat suppression sequence STIR in both coronary and axial position. Results　In the 50 cases of dermatomyositis, there were 49 cases of muscle edema, 28 cases of myofasciitis, 9 cases of subcutaneous connective tissue inflammation, and 4 cases of thickening skin. Of 49 cases of muscle edema, there are 16 cases in grade 1, 24 in grade 2 and 9 in grade 3. The median value of all muscle enzyme in the grade 2 group was higher than that in the other two groups, and the value of AST was statistically significant, P<0.05. Conclusions　The detection rate of JDM using MRI is high, which can help judge the severity and range of involvement. The MRI findings of JDM mainly demonstrated muscle edema, mostly moderate degree, followed by muscular fasciitis.

Clinical evaluation of risk factors for ventilator-associated pneumonia in pediatric intensive care unit

ZHOU Xiong, XIAO Zhenghui, HE Jie, YANG Meiyu, CAO Jianshe, CAI Zili, XU Zhiyue, LU Xiulan, FANG Jianghua, ZHANG Xinping

. 2018, 36(5):  352.  doi:10.3969/j.issn.1000-3606.2018.05.008

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Objectives　To determine the risk factors of ventilator-associated pneumonia (VAP) in the pediatric intensive care unit and to explore effective strategies to reduce the morbidity of VAP. Methods　A retrospective analysis was conducted on 455 children admitted into the PICU of Hunan Children's Hospital from June 2014 to June 2017. The 455 children were divided into VAP group (n=43) and non-VAP group (n=412). The incidence of VAP was identified and risk factors were compared using the logistic regression analysis via SPSS 19.0 software. Results　There were 311 males and 144 females with a median age of 11 months old (29 days to 9 years and 4 months). The incidence of VAP was 9.45% (43/455). Congenital laryngeal and trachea malformation with pulmonary infection was the first reason for the occurrence of VAP (23.3%), followed by congenital heart diseases with pulmonary infection (18.6%). Via univariate analysis, types of endotracheal intubation (χ2=45.33, P＜0.001), duration of mechanical ventilation (Z=1.21, P=0.034), re-intubation (χ2=20.22, P=0.004), early usage of antibiotics (χ2=4.98, P=0.026), and methods of nutritional support (χ2=10.15, P=0.006) were identified as risk factors of VAP in the pediatric intensive care unit patients (P＜0.05). Based on the multivariate logistic regression analysis, the followings were all independent predictor for VAP: types of endotracheal intubation (OR=1.87, 95%CI:1.48~9.75), duration of mechanical ventilation (OR=1.14, 95%CI:1.08~2.35), re-intubation (OR=3.42, 95%CI:1.26~5.57), early usage of antibiotics (OR=4.55, 95%CI:2.21~8.77). Conclusions　Many risk factors were found related with the occurrence of VAP. A comprehensive analysis of the host factors and iatrogenic factors should be conducted. Rational use of antibiotics and daily assessment of extubation might help reduce the incidence of VAP.

Removal of bronchial foreign body in children by bronchoscopic CO2 Cryotherapy

　ZHANG Lei, YIN Yong, YUAN Shuhua, ZHANG Jing, PAN Chunhong, ZHANG Hao, CHEN Jie

. 2018, 36(5):  356.  doi:10.3969/j.issn.1000-3606.2018.05.009

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　Objective　To assess the efficiency and safety of the bronchoscopic CO2 cryotherapy to remove bronchial foreign body in children. Methods　From Oct 2012 to Dec 2016, 8 cases diagnosed with the presence of bronchial foreign body were undergone the treatment with the bronchoscopic CO2 cryotherapy. The clinical data about the efficiency and complication were collected and retrospectively analyzed.  Results　In the 8 cases, we successfully removed the bronchial foreign bodies without complications. In 2 cases, the foreign bodies were removed successfully, but cryotherapy partially damaged the airway mucosa, causing topical airway obstruction because of the newly developed granulation tissue. No serious adverse reactions or complications were observed after the treatment. Conclusions　Removal of bronchial foreign bodies using bronchoscopic CO2 cryotherapy is an easy and effective method which can be used as a supplementary procedure for the bronchoscopic removal of foreign bodies in children’s airways.

Value of serum albumin combined with red blood cell distribution width on evaluation of severity of heart failure in children

　JIN Ling, LONG Xingjiang, YANG Tong, HUANG Xianwen

. 2018, 36(5):  360.  doi:10.3969/j.issn.1000-3606.2018.05.010

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Objective　To explore the relationship between serum albumin (ALB) combined with red blood cell distribution width (RDW) in progression and prognosis of children with heart failure. Method　A total of 144 children with heart failure were divided into light, medium, and heavy group according to the modified ROSS score; another aspect, they also were divided into survival group and death group. ALB, RDW, and modified ROSS score were measured to assess the relationship between the factors and the death by logistic regression analysis. Results　There are 73 boys and 71 girls. The cases of mild, moderate, and severe group were 78, 39, and 27, respectively. Age and gender difference in three groups were not statistically significant (P>0.05). The ratio of high RDW was 54.86% (79/144), the ratio of low serum ALB was 33.33% (48/144). Chi-square analysis indicated that a severer status of the disease correlated with a lower ALB level (P <0.05) and a higher RDW. The analysis of rank correlation indicating the degree of heart failure was negatively correlated with the related trends of ALB and RDW (P<0.05). Logistic regression analysis indicating a higher risk of death of the disease correlated with a lower ALB level (P<0.05， OR=0.839) and a higher modified ROSS score (P<0.05，OR =1.324). Conclusions　The ALB, RDW, and the modified ROSS score are closely related to the severity in heart failure children. The ALB and the modified ROSS score have important clinical prognostic significance in evaluating the prognosis.

J wave syndromes in children: a case report and literature review

　ZHAO Jian, LI Yun, XIAO Tingting, XIE Lijian，WANG Jianyi， JIANG Xunwei

. 2018, 36(5):  364.  doi:10.3969/j.issn.1000-3606.2018.05.011

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　Objective　To explore clinical characteristics of J wave syndromes. Methods　Clinical data was retrospectively analyzed in a child with fainting and showing big J wave in ECG. Meanwhile, related literatures were reviewed. Results　A 10-year-old boy was admitted to hospital with fainting. ECG showed J point up, reflecting Osbron wave. There was no abnormality in EEG and cranial CT. Ultrasound cardiograph showed approximately normal heart structure and left ventricular systolic function. Fainting was frequently occurred during follow-up and accompanied by malignant arrhythmia. Conclusions　Patients with J wave syndromes has high risks of malignant arrhythmia and sudden cardiac death.

Serum IL-17A, IL-18, and SCD25 (IL-2R) in early diagnosis of Kawasaki disease

　SONG Sirui, ZHU Danying, CHEN Liqin, XIE Lijian, XIAO Tingting, HUANG Min

. 2018, 36(5):  367.  doi:10.3969/j.issn.1000-3606.2018.05.012

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　Objective　To investigate the acute phase expression of serum cytokines in children with Kawasaki disease (KD) and its association with coronary artery lesions (CALs). Methods　Expression of 13 cytokines in serum of 104 KD patients including 11 cases with CALs and 74 febrile control cases admitted to the hospital from October 2016 to March 2017 were retrospectively analyzed. Results　Interleukin (IL)-8, IL-1, IL-2, IL-6, IL-10, IL-17A, IL-18, tumor necrotic factor-α and SCD25 (IL-2R) were declined significantly after intravenous immunoglobulin administration (IVIG) in KD patients (all P <0.05）. Compared with the fever control group, Pre-IVIG, an IL-17A level ≥ 0.155 pg/mL had a sensitivity of 50% and 93.2% specificity for predicting KD; IL-18 level ≤ 15.43 pg/mL had a sensitivity of 71.2%, and the specificity was 54.1%; SCD25 (IL2R) ≥ 29475.29 pg/mL had a 65.4% sensitivity and 81.1% specificity for predicting KD. Before IVIG treatment, the level of IL-10 was significantly lower in KD patients with CALs than in those without CALs. Conclusions　Determination of various cytokines profile may be helpful for predicting the disease prognosis and targeting treatment strategies in patients with KD.

Association of ring-finger protein 3 (MKRN3) gene rs2239669 polymorphism with central precocious puberty susceptibility

　CHEN Zhanfeng, ZHAO Peiwei, CAI Xiaonan, YAO Hui, CHEN Xiaohong, HE Xuelian, WAN Chunhui

. 2018, 36(5):  372.  doi:10.3969/j.issn.1000-3606.2018.05.013

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Objectives　To explore the association between the single nucleotide polymorphism (SNP) rs2239669 in makorin ring-finger protein 3 (MKRN3) gene and the susceptibility to central precocious puberty (CPP). Methods　A case-control study including 246 children with CPP and 269 healthy children was performed. The genotype and MKRN3 expression levels of patients were analyzed by PCR-HRM and RT-PCR，respectively. Results　SNP rs2239669 genotype (TT，TC，CC) and allele frequencies (T and C) were different between cases and controls，with higher CC genotype in CPP patients. Under recessive model (CC/TT+TC)，CC genotype was higher in CPP group and associated with higher risk of CPP (95%CI：1.062-2.143， P=0.021). MKRN3 expression levels were different among patients with different genotypes，of which TT genotype had the highest level followed by TC and CC (0.376±0.094, 0.330±0.068, 0.250±0.072， P=0.041). Conclusions　MKRN3 SNP rs2239669 was associated with increased risk of CPP, and patients with TT genotype had higher MKRN3 levels.

Electrophysiological and clinical characteristics of children with startle epilepsy

　LUO Xiaoqing, LI Cheng, KUANG Guangtao, LIANG Jufang, YU Xiaoli, WANG Xiaolu, JIANG Jun

. 2018, 36(5):  376.  doi:10.3969/j.issn.1000-3606.2018.05.014

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Objective　To investigate the characteristics of ictal and interictal electroencephalogram (EEG) and clinical manifestation in children with startle epilepsy. Methods　The age of onset, inducing factors, the types of attacks, EEG features, cognitive function, treatment and prognosis were retrospectively analyzed in 8 cases of children with startle epilepsy from June 6, 2012 to March 2016. Results　In 8 cases, 3 cases were male and 5 were female. The onset age was from 2.3 to 8.1 years old. The forms of onset were varied from generalized (tonic, myoclonic, atonic) to partial seizures (the asymmetry of posture rigidity, spasm). The most common ictal EEG finding was a diffuse electrodecremental pattern (5 cases), and the interictal EEG showed a large number of multifocal, generalized spines, slow waves and multiple spinous waves. There was one case with no history of brain injury while the other 7 cases had a history of brain injury. There were 7 cases with imaging abnormality, and the lesions of the frontal, parietal and temporal regions were indicated with a partial cerebral softening and brain atrophy. In 7 cases, all children had abnormal mental and motor development, and 1 case had normal cognitive function. The 7 cases with shock epilepsy showed no obvious response to the treatment of multiple antiepileptic drugs, and 1 case had no clinical onset after 2 months of treatment with VPA. Conclusions　Startle epilepsy is mostly symptomatic, and few are non-symptomatic. The former had history of brain structure abnormalities, certain degree of motor retardation and mental disability, and no clinical response to antiepileptic drug therapy. The latter had basically normal cognitive function, and antiepileptic drug VPA treatment is effective. The degree of interictal epileptic was not an indicator of cognitive impairment and prognosis in children with startle epilepsy.

Clinical findings of 16 cases of neonatal influenza

　LEI Min, LI Chi, TIAN Shufeng, ZHOU Gaofeng, DENG Jikui

. 2018, 36(5):  381.  doi:10.3969/j.issn.1000-3606.2018.05.015

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　Objective　To investigate the clinical features, laboratory diagnostics, treatments, and prognosis of neonates infected with influenza. Methods　The clinical data of 16 neonates diagnosed as influenza admitted to the neonatal ward from January 2015 to May 2017 were retrospectively analyzed. Results　Of the 16 neonates, 11 were male and 5 were female. Mean age was 18.5 days. 75% (12/16) of them were reported to be exposed to family members with common cold- like symptoms before hospitalization. Clinical manifestations included nasal obstruction (11/16), fever (10/16), cough (10/16), and rhinorrhea (8/16). Influenza antigen rapid detection (colloidal gold method) was positive in all cases. Influenza immunofluorescence assays were performed in 15 cases, only 6.67% (1/15) was positive. Sputum culture was performed in 13 cases, 8 of which were positive. Of them, 75% (12/16) neonates were diagnosed with pneumonia. Only 12.5% (2/16) neonates were treated with neuraminidase inhibitor. All cases recovered well and were discharged after antibiotic treatment. Conclusions　Neonates contacted with family members displaying common cold-like symptoms should be examined for influenza in time. The common clinical manifestations include catarrhal symptoms, fever and cough. The sensitivity of the influenza immunofluorescence assay is lower as compared with the colloidal gold method. Pneumonia may often be developed in neonatal influenza. The prognosis of neonatal influenza is satisfactory if treated.

The value of genetic polymorphisms of mycophenolate mofetil in individualized therapy

ZHANG Zhiqing

. 2018, 36(5):  384.  doi:10.3969/j.issn.1000-3606.2018.05.016

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　Mycophenolate mofetil (MMF) is a cornerstone immunosuppressant in clinical therapy, such as organ transplantation. Because of the huge interindividual difference in genetic polymorphisms and some other, fixed-dose MMFtreated strategy can neither attain the targeted effective and safe MPA blood concentration nor satisfy the needs of individualized therapy, especially in children. The article reviews the value of genetic polymorphism of UGT, IMPDH, ABCC2, SLCO in MMF therapy in children to highlight its role in the precision treatment.

Advances in neuroblastoma immunotherapy based on chimeric antigen receptor

ZHAO Jie，LI Benshang

. 2018, 36(5):  389.  doi:10.3969/j.issn.1000-3606.2018.05.017

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Neuroblastoma is the most common extra-cranial malignant tumor in childhood. About 50% of the children have widespread metastatic diseases at initial diagnosis and the outcomes are poor. Chimeric antigen receptor (CAR) is a kind of genetically engineered receptors usually generated by extracellular antigen recognition region, spacer, transmembrane domain and intracellular endodomain. The antigen recognition region joining heavy and light chain variable regions of a monoclonal antibody with a linker to form a single-chain variable fragment (scFv) which can bind to tumor-associated antigens and lead to anti-tumor activity in an MHC-unrestricted manner. In this review, the recent advances and applications of CAR-T cell in neuroblostoma are reviewed and the application-related problems and possible solving strategies are discussed.

Research progress on childhood anaplastic large cell lymphoma

ZHANG Zhixiao

. 2018, 36(5):  394.  doi:10.3969/j.issn.1000-3606.2018.05.018

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Anaplastic large cell lymphoma (ALCL) is a clinically rare non-Hodgkin’s lymphoma which is characterized by malignant “hallmark cells” and strong expression of CD30 in membrane and golgi patterns in almost all cases. The most frequent chromosomal translocation is t (2;5)(p23;q35). ALCL in children is nearly universally anaplastic lymphoma kinase (ALK) positive and commonly present with advanced systemic disease. Many different treatment strategies have been utilized with similar event free survival rates of 65%~75%. High-risk and recurrent/refractory ALCL can be treated by vinblastine weekly or hematopoietic stem cell transplantation. Novel therapies such as CD30 targeted treatment and ALK inhibitors may soon radically change the treatment paradigm for this disease. This review will provide an overview of the biological characteristics, clinical features, treatment and prognostic factors for children with ALCL.