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A rare double trisomy 47,XXX/48,XXX,+8 combined Behcet disease:A case report and review of literature

Xin LI ; Qing CHENG ; Yu DING ; Juan LI ; Lei YING ; Yunfang ZHOU ; Xiuming WANG ; Jian WANG

Journal of Clinical Pediatrics.2017;35(5):355-358. doi:10.3969/j.issn.1000-3606.2017.05.008

Objective To investigate the characteristics and essential points of diagnosis and treatment of double trisomy 47,XXX/48,XXX,+8 combined Behcet disease, a rare inherited immunodeficiency disorder. Methods The clinical manifestations, karyotype analysis and gene test results of the patients were analyzed, and relevant literatures were reviewed. Results A 11-year-old girl presented repeated fever for more than 6 years, accompanied with recurrent genital herpes infection and oral apthosis, was clinically diagnosed with Behcet disease. Cytogentic and molecular karyotyping on peripheral lymphocytes demonstrated 47,XXX[12]/48,XXX,+8[18]. Conclusions Conventional karyotype analysis and chromosomal microarray analysis have a complementary role in the diagnosis of the disease. We conclude that patients with constitutional trisomy 8 and those with trisomy 8 confined to the bone marrow are both at increased risk of developing features of Behcet disease. The mechanism may relate to increased gene dosage of candidate genes for Behcet's disease on chromosome 8.

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Characteristics and risk factors of blood transfusion in 180 very low birth weight infants

Qinglian JIANG ; Zhihong ZHANG

Journal of Clinical Pediatrics.2017;35(5):350-354. doi:10.3969/j.issn.1000-3606.2017.05.007

Objective We aimed to study the characteristics and risk factors for blood transfusion in very low birth weight infants (VLBWI). Methods Clinical data of 180 VLBWI, hospitalized from January, 2012 to June, 2016, were studied retrospectively. The infants were divided into two groups according to whether blood transfusion is administered or not. Two groups were compared with general information, diseases in hospital and treatment taken. Results Of the 180 VLBWI, 118 cases (65.6%) were diagnosed with anemia when hospitalized. 57 cases (31.7%) needed blood transfusion with a mean gestational age of 30.3±1.9 weeks and a mean birth weight of 1233.3±123.7 g, The first time to blood transfusion ranged from 2 to 5 weeks after birth, transfusion volume 20ml/Kg once. Fourty-eight (48) cases (84.2%) only took blood transfusion once. Birth weight, gestational age, basal hemoglobin, hematocrit, volume of blood taking before transfusion, duration of hospitalization, duration of continuous positive airway pressure, duration of paraenteral nutrition, duration of vasoactive drugs used, need for intubation、delivery mode, neonatal respiratory distress syndrome, apnea, neonatal hypothyroidism and patent ductus arteriosus showed significant difference between the two groups. Logistic regression analysis revealed that gestational age, birth weight, basal hemoglobin, long duration of hospital stay and blood loss from laboratory testing were risk factors for blood transfusion in VLBWI (P<0.05). Conclusions The rate of blood transfusion in VLBWI was relatively high. Complex complications, critical condition, blood loss from laboratory testing and basal hemoglobin were main risk factors for blood transfusion.

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Clinical analysis of 81 children with urinary system injury after hematopoietic stem cell transplantation

Ruyue CHEN ; Hanyun TANG ; Qing CHEN ; Yajun FENG ; Yunyan SHEN ; Qinying XU ; Yun ZHU ; Xueming ZHU ; Xiaozhong LI

Journal of Clinical Pediatrics.2017;35(5):345-349. doi:10.3969/j.issn.1000-3606.2017.05.006

Objectives To investigate the etiology, renal pathology, treatment, and prognosis of children's urinary system injury after hematopoietic stem cell transplantation (HSCT). Methods Clinical data of 81 children with urinary dysfunction after HSCT admitted to the Hematology Department in Children's Hospital of Soochow University were analyzed, and relevant literatures were reviewed. Results In 81 cases (50 males and 31 females), the age ranges from 8 months to 17 years old. Thirty cases (37%) with prerenal injury were recovered after active rehydration and other symptom specific treatment. There were 9 (11.1%) children with renal injury, four cases were given up therapy or transferred to other hospitals, thus lead to an unknown prognosis. Kidney biopsy was performed in the remaining five cases for pathological investigation. After active symptom-speific and etiology-based treatment, serum creatinine and glomerular filtration rate of four cases return to normal. But in the long-term follow-up,one case died of recurrence of primary disease, reinfusion of hematopoietic stem cell combined with renal failure. The remaining 3 patients were with chronic kidney disease (CKD). One case with renal thrombotic microangiopathy was in the chronic dialysis. Postrenal renal injuries were mainly hemorrhagic cystitis (28.4%) and urinary tract infection (16%). After a large dose of rehydration, urine alkalization and anti-infection therapy, they were recovered in the short term with a good prognosis. Conclusions Urinary injury after HSCT is mainly divided into three categories: prerenal, renal and postrenal, in which renal injury is prone to frequent recurrence.

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Characteristics and prognosis of parvovirus B19 infection in Pediatric leukemia patients in Suzhou

Ye LU ; Shaoyan HU ; Hailong HE ; Suxiang LIU ; Lingjun KONG ; Junjie FAN ; Peifang XIAO

Journal of Clinical Pediatrics.2017;35(5):336-339. doi:10.3969/j.issn.1000-3606.2017.05.004

Objective To explore the effect of parvovirus B19 (VB19) infection on pediatric leukemia patients. Methods The pediatric leukemia patients were enrolled in the study in the Children's Hospital of Soochow University. Expression levels of VB19-DNA-PCR were detected using the polymerase chain reaction. Positive patients would be monitored and treated by conventional treatment as well until VB19 gene became negative. The data was compared according to the VB19 clearance time, clinical symptoms and blood counts to evaluate the effect. Results In the 3009 samples from 824 pediatric leukemia patients, there were 36 samples (1.2%) from 12 cases (1.5%) of pediatric leukemia paients with positive VB19 infection. Among the positive patients, 11 cases (1.9%) were from 582 with acute lymphoblastic leukemia (ALL) patients and 1 (0.45%) was from 212 with acute myeloid leukemia (AML). According to the treatment stage, 3 cases were in initially diagnosed period, 2 cases in early stage of consolidation chemotherapy, 4 cases in delayed enhanced chemotherapy period, and 3 cases in maintenance chemotherapy period. According to the treatment response, 4 cases were in continuous treatment, 2 cases were sensitive to treatment, and 3 cases were drug resistant. In the drug resistance group, 2 cases developed into the pure red cell aplastic anemia (PRCA). After treatment, one was recovered from PRCA with VB19 cleared, the other one remained PRCA with continuously positive VB19. Conclusions More VB19 virus infection in pediatric ALL happened in delayed enhanced chemotherapy period. The persistent presence of VB19 infection on pediatric leukemia patients is closely related with PRCA.

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Expression of EVI1 gene and its clinical significance in pediatric acute myeloid leukemia

Chenyu ZHENG ; Xianhao WEN ; Yuxia GUO ; Xianmin GUAN ; Jie YU ; Jianwen XIAO

Journal of Clinical Pediatrics.2017;35(5):331-335. doi:10.3969/j.issn.1000-3606.2017.05.003

Objective To investigate the relationship between EVI1 gene expression and clinical features and prognosis of children with acute myeloid leukemia (AML). Methods EVI1 gene was detected in AML children, correlation of clinical and lab features, prognosis of AML children with EVI1 gene were analyzed. Results EVI1 expression is positive in 38 of 145 children with AML. There were no significant differences in age, gender, hemoglobin concentration, leukocytes and platelet count, subtype of morphology, ratio of chromosomal anomaly and complex karyotypes between EVI1 positive and EVI1 negative group (P>0.05); coexist genes were detected in 9 cases (23.68%) of EVI1 positive group. Rate of complete remission (CR) was 91.67% in 24 cases of EVI1 positive patients received chemotherapy. Relapse rate was 64.29% and 14.29% in EVI1 positive patients who received chemotherapy and allo-hematopoietic stem cell transplantation (allo-HSCT), retrospectively and significant differences were found (P<0.05). There was no significant difference in CR but significant difference was found in event free survival (P<0.05) for EVI1 positive and EVI1 negative patients who received chemotherapy. EVI1 gene kept negative when bone marrow relapse occurred in two patients with EVI1 positive at diagnosis. Conclusion EVI1 gene may play adverse role in pediatric AML; prognosis of EVI1 positive AML patients can be improved by allo-HSCT; follow-up of EVI1 transcript levels is insufficient to monitoring of minimal residual disease.

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Effect of chemotherapy regimen CCLG-ALL-2008 on children with TEL/AML1 fusion gene positive of acute lymphoblastic leukemia

Jing GAO ; Shaoyan HU ; Jun LU ; Hailong HE ; Yi WANG ; Wenli ZHAO ; Jianqin LI ; Jie LI ; Peifang XIAO ; Junjie FAN ; Yihuan CHAI

Journal of Clinical Pediatrics.2017;35(5):325-330. doi:10.3969/j.issn.1000-3606.2017.05.002

Objective To evaluate the predictive role of TEL/AML1 fusion gene in protocol CCLG-ALL-2008 and to identify relevant factors influencing the outcome of ALL with TEL/AML1 fusion gene. Methods Ninety-nine patients with ALL harboring TEL/AML1 fusion gene (positive) and 329 cases without any specific fusion genes (negative) at diagnosis of B-lineage ALL from June 2008 to December 2014 were enrolled and their clinical and biological features were analyzed. Following-up ended in October 2015, the survival status was calculated by K-M curve and prognostic factors were analyzed by COX model. Results There were no differences between the two groups in age, white blood cell at the diagnostic stage, and treatment responses at 4 time points, namely, prednisone good response on day 8, M3 status of BM on D15, and the minimal residual disease (MRD) more than 1.0×10-3 on day 33 and 12th week. During the follow-up period, the relapse rate was lower in the positive group than that in the negative group (14/99 vs 69/329), the mortality rate of the negative group was twice of that in the positive group (55/329 vs 8/99). The five-year overall survival (OS) rate, relapse-free survival (RFS) rate and event-free survival (EFS) rate of the positive group were (86.1 ± 4.9)%, (80.7 ± 5.1)% and (78.9 ± 5.1)%, respectively, and (79 ±2.8)%, (72± 3.1)%, and (69.6+ 3.1)% for the negative group as well. COX regression analysis indicated that relapse and MRD level at the 12th week were independent prognostic factors on OS, RFS, and EFS (P<0.05) for the two groups. Conclusions TEL/AML1 fusion gene could be regarded as a relatively good indicator of risks in ALL children treated by CCLG-ALL-2008 protocol. ALL patients with TEL/AML1 are recommended to receive more intensive therapy including hematopoietic stem cell transplantation when the patients were high level of MRD on the 12th week after treatment.

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Role and mechanism of eosinophils in the pathogenesis of atopic dermatitis

Qianyue XU

Journal of Clinical Pediatrics.2017;35(5):389-393. doi:10.3969/j.issn.1000-3606.2017.05.016

Atopic dermatitis (AD) is a chronic and recurrent inflammatory skin disease. Multiple studies have demonstrated a significant correlation between the eosinophil (EOS) and AD. Th2 cells secret cytokines and chemokines mediating the activation, differentiation and growth of EOS. EOS is recruited from bone marrow and blood into the dermis, and releases granule proteins, cytokines etc., resulting in the deposition of collagen and fibrosis thickening. This article reviews the recent studies of the main role and mechanism of EOS in the pathogenesis of AD, aiming to a further understanding of this disease and provides insights for the development of new drugs.

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Toxicity and management in chimeric antigen receptor T-cell therapy

Guanhua HU

Journal of Clinical Pediatrics.2017;35(5):384-388,393. doi:10.3969/j.issn.1000-3606.2017.05.015

T cells can be genetically modified to target tumors through the expression of a chimeric antigen receptor (CAR). Most notably, CAR T cells have demonstrated its clinical efficacy in hematologic malignancies with evident responses when targeting solid tumors. However, CAR T cells therapy also has the capacity to elicit expected and unexpected toxicities including cytokine release syndrome, neurologic toxicity, on target/off tumor recognition, and anaphylaxis. Theoretical toxicities include clonal expansion secondary to insertional oncogenesis, graft versus host disease, and off-target antigen recognition. Abrogating toxicity has become a critical step in the successful application of this emerging technology. To this end, we review the reported and theoretical toxicities of CAR T cells therapy and strategies to cope with it.

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Study on the clinical outcomes of children with stage Ⅳ malignant extracranial germ cell tumors

Qianghua YAN ; Jingyan TANG ; Ci PAN ; Qidong YE ; Min ZHOU ; Yijin GAO ; Wenting HU

Journal of Clinical Pediatrics.2017;35(5):321-324. doi:10.3969/j.issn.1000-3606.2017.05.001

Objective To evaluate the outcomes of children with stage Ⅳ malignant extracranial germ cell tumors. Methods Twenty-five patients were enrolled in the retrospective analysis. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier method with SPSS 13.0. Results Of the 25 children, there were 13 males and 12 females. The mean age at diagnosis was 2 years old (ranged 1 to 11). Five patients receiving chemotherapy in another hospital before (n=1), or giving up treatment after confirmed diagnosis (n=1), or giving up effective treatment after received less than 2 cycles (n=3) were excluded from this analysis. Of the 20 patients, 90.0% (18/20) achieved complete remission and 5.0% (1/20) achieved partial remission after treatment. The 5-year EFS rate and 5-year OS rate were 70.0%±10.2% and 82.4%±9.2% respectively. There was no death occurred due to complications. Conclusions The effect of this treatment program is positive. The cumulative dose of the drugs is not high, compared with other schemes such as PEB, but there are more drugs involved. Whether these drugs may cause long-term adverse reactions needs further research.

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Progress on saliva tests in diagnosis and treatment of pediatric diseases

Xinqiang NI ; Xiaoli ZHANG ; Limin LI

Journal of Clinical Pediatrics.2017;35(5):394-399. doi:10.3969/j.issn.1000-3606.2017.05.017

Currently, saliva tests have been regarded as a potential promising diagnotic tool because of its noninvasiveness, quickness and simplicity, high accuracy, fullness of biomarkers, and other advantages. It has been applied in clinical diagnosis, treatment evaluation, epidemiological survey and other clinical works, while its potential applications have not yet been fully recognized by Chinese pediatrician. This review presents an overview on the application of salivary diagnostics in pediatrics ophthalmology and otorhinolaryngology, immune system diseases, nervous-mental system diseases and other system diseases, and offers a new potential research strategy for accurate diagnosis and treatment of pediatric diseases.

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