Venous thromboembolism (VTE) is a complication in children with acute lymphoblastic leukemia (ALL). The Chinese Children's Cancer Group-ALL-2015 protocol was carried out in China, and epidemiology, clinical characteristics, and risk factors associated with VTE were analyzed. We collected data on VTE in a multi-institutional clinical study of 7640 patients with ALL diagnosed in 20 hospitals from January 2015 to December 2019. First, VTE occurred in 159 (2.08%) patients, including 90 (56.6%) during induction therapy and 108 (67.92%) in the upper extremities. T-ALL had a 1.74-fold increased risk of VTE (95% CI 1.08-2.8, P = 0.022). Septicemia, as an adverse event of ALL treatment, can significantly promote the occurrence of VTE (P < 0.001). Catheter-related thrombosis (CRT) accounted for 75.47% (n = 120); and, symptomatic VTE, 58.49% (n = 93), which was more common in patients aged 12-18 years (P = 0.023), non-CRT patients (P < 0.001), or patients with cerebral thrombosis (P < 0.001). Of the patients with VTE treated with anticoagulation therapy (n = 147), 4.08% (n = 6) had bleeding. The VTE recurrence rate was 5.03% (n = 8). Patients with VTE treated by non-ultrasound-guided venous cannulation (P = 0.02), with residual thrombus (P = 0.006), or with short anticoagulation period (P = 0.026) had high recurrence rates. Thus, preventing repeated venous puncture and appropriately prolonged anticoagulation time can reduce the risk of VTE recurrence.
Humans ; Child ; Venous Thromboembolism/etiology* ; East Asian People ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/epidemiology* ; Risk Factors ; Thrombosis/chemically induced* ; China/epidemiology* ; Anticoagulants/adverse effects* ; Recurrence

Objective: To analyze the clinical characteristics of children with Burkitt lymphoma (BL) and to summarize the mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen. Methods: Clinical features of 436 BL patients who were ≤18 years old and treated with the CNCL-B-NHL-2017 regimen from May 2017 to April 2021 were analyzed retrospectively. Clinical characteristics of patients at disease onset were analyzed and the therapeutic effects of patients with different clinical stages and risk groups were compared. Survival analysis was performed by Kaplan-Meier method, and Cox regression was used to identify the prognostic factors. Results: Among 436 patients, there were 368 (84.4%) males and 68 (15.6%) females, the age of disease onset was 6.0 (4.0, 9.0) years old. According to the St. Jude staging system, there were 4 patients (0.9%) with stage Ⅰ, 30 patients (6.9%) with stage Ⅱ, 217 patients (49.8%) with stage Ⅲ, and 185 patients (42.4%) with stage Ⅳ. All patients were stratified into following risk groups: group A (n=1, 0.2%), group B1 (n=46, 10.6%), group B2 (n=19, 4.4%), group C1 (n=285, 65.4%), group C2 (n=85, 19.5%). Sixty-three patients (14.4%) were treated with chemotherapy only and 373 patients (85.6%) were treated with chemotherapy combined with rituximab. Twenty-one patients (4.8%) suffered from progressive disease, 3 patients (0.7%) relapsed, and 13 patients (3.0%) died of treatment-related complications. The follow-up time of all patients was 24.0 (13.0, 35.0) months, the 2-year event free survival (EFS) rate of all patients was (90.9±1.4) %. The 2-year EFS rates of group A, B1, B2, C1 and C2 were 100.0%, 100.0%, (94.7±5.1) %, (90.7±1.7) % and (85.9±4.0) %, respectively. The 2-year EFS rates was higher in group A, B1, and B2 than those in group C1 (χ²=4.16, P=0.041) and group C2 (χ²=7.21, P=0.007). The 2-year EFS rates of the patients treated with chemotherapy alone and those treated with chemotherapy combined with rituximab were (79.3±5.1)% and (92.9±1.4)% (χ²=14.23, P<0.001) respectively. Multivariate analysis showed that stage Ⅳ (including leukemia stage), serum lactate dehydrogenase (LDH)>4-fold normal value, and with residual tumor in the mid-term evaluation were risk factors for poor prognosis (HR=1.38,1.23,8.52,95%CI 1.05-1.82,1.05-1.43,3.96-18.30). Conclusions: The CNCL-B-NHL-2017 regimen show significant effect in the treatment of pediatric BL. The combination of rituximab improve the efficacy further.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Burkitt Lymphoma/drug therapy* ; Child ; Disease-Free Survival ; Female ; Humans ; Lactate Dehydrogenases ; Lymphoma, B-Cell/drug therapy* ; Male ; Prognosis ; Retrospective Studies ; Rituximab/therapeutic use* ; Treatment Outcome

Objective: To study the clinicopathological and genetic features of natural killer (NK)-cell enteropathy for better understanding of this rare disease and prevention of its misdiagnosis. Methods: Two cases of NK-cell enteropathy were diagnosed in the First Affiliated Hospital of Zhengzhou University, China from October 2017 to February 2021. The clinical characteristics, morphology, immunohistochemistry, Epstein-Barr virus-encoded RNA (EBER) in situ hybridization and T cell receptor gene rearrangement were analyzed. The patients were followed up by a telephone interview. Results: The patients were both male, aged 40 and 28 years, respectively. Both patients were admitted to the hospital for an annual checkup without obvious gastrointestinal symptoms. The endoscopy showed that the gastric body of case 1 had a mucosal bulge, small area of congestion and erosion, while the rectum of case 2 had congestion and erosion. Microscopically, the lesions of the 2 cases were relatively limited. Many lymphoid cells infiltrated within the lamina propria of the mucosa and into the muscularis mucosa in case 2. In case 1, the glands were reduced in the lesion, and the glandular cavity was slightly compressed and deformed. There was no infiltration or destruction of the glands in either case. Lymphoid cells were atypical, with medium-to-large cell sizes. Their cytoplasm was medium-to-slightly abundant and appeared eosinophilic or translucent. In case 2, characteristic eosinophilic granules were seen in the cytoplasm of a few cells. The nuclei in both cases were round, oval and irregular, with fine chromatin, inconspicuous nucleoli, and no mitotic figures were noted. Necrosis was seen in case 1 while both cases had no central growth or destruction of blood vessels. Immunophenotyping showed that CD56, granzyme B and TIA-1 were positive in both cases, part of the cells was CD3-positive, and some cells were weakly CD4-positive in case 2. The CD5, CD8, CD30, ALK and B-lineage markers (CD20, CD79α) were all negative. The Ki-67 proliferation index was about 60% and 30%, respectively. Both cases were EBER negative. TCR gene rearrangement was polyclonal. Follow-up showed that none of the 2 patients had any special treatments and stayed well. Conclusions: NK-cell enteropathy is rare, with biological behaviors similar to benign tumors, and occasional recurrence. Its histology and immunophenotype are easily confused with NK/T cell-derived lymphomas. Combination of its unique endoscopic features, EBER negativity, polyclonal TCR gene rearrangement and good prognosis can confirm the diagnosis and avoid misdiagnosis and overtreatment.
Epstein-Barr Virus Infections ; Herpesvirus 4, Human/genetics* ; Humans ; Immunophenotyping ; Killer Cells, Natural ; Lymphoproliferative Disorders ; Male

Objective: To investigate the relationship between body mass index (BMI) and sexual development in Chinese children. Methods: A nationwide multicenter and population-based large cross-sectional study was conducted in 13 provinces, autonomous regions and municipalities of China from January 2017 to December 2018. Data on sex, age, height, weight were collected, BMI was calculated and sexual characteristics were analyzed. The subjects were divided into four groups based on age, including ages 3-<6 years, 6-<10 years, 10-<15 years and 15-<18 years. Multiple Logistic regression models were used for evaluating the associations of BMI with sexual development in children. Dichotomous Logistic regression was used to compare the differences in the distribution of early and non-early puberty among normal weight, overweight and obese groups. Curves were drawn to analyze the relationship between the percentage of early puberty and BMI distribution in girls and boys at different Tanner stages. Results: A total of 208 179 healthy children (96 471 girls and 111 708 boys) were enrolled in this study. The OR values of B2, B3 and B4+ in overweight girls were 1.72 (95%CI: 1.56-1.89), 3.19 (95%CI: 2.86-3.57), 7.14 (95%CI: 6.33-8.05) and in obese girls were 2.05 (95%CI: 1.88-2.24), 4.98 (95%CI: 4.49-5.53), 11.21 (95%CI: 9.98-12.59), respectively; while the OR values of G2, G3, G4+ in overweight boys were 1.27 (95%CI: 1.17-1.38), 1.52 (95%CI: 1.36-1.70), 1.88 (95%CI: 1.66-2.14) and in obese boys were 1.27 (95%CI: 1.17-1.37), 1.59 (95%CI: 1.43-1.78), and 1.93 (95%CI: 1.70-2.18) (compared with normal weight Tanner 1 group,all P<0.01). Analysis in different age groups found that OR values of obese girls at B2 stage and boys at G2 stage were 2.02 (95%CI: 1.06-3.86) and 2.32 (95%CI:1.05-5.12) in preschool children aged 3-<6 years, respectively (both P<0.05). And in the age group of 6-10 years, overweight girls had a 5.45-fold risk and obese girls had a 12.54-fold risk of B3 stage compared to girls with normal BMI. Compared with normal weight children, the risk of early puberty was 2.67 times higher in overweight girls, 3.63 times higher in obese girls, and 1.22 times higher in overweight boys, 1.35 times higher in obese boys (all P<0.01). Among the children at each Tanner stages, the percentage of early puberty increased with the increase of BMI, from 5.7% (80/1 397), 16.1% (48/299), 13.8% (27/195) to 25.7% (198/769), 65.1% (209/321), 65.4% (157/240) in girls aged 8-<9, 10-<11 and 11-<12 years, and 6.6% (34/513), 18.7% (51/273), 21.6% (57/264) to 13.3% (96/722), 46.4% (140/302), 47.5% (105/221) in boys aged 9-<10, 12-<13 and 13-<14 years, respectively. Conclusions: BMI is positively correlated with sexual development in both Chinese boys and girls, and the correlation is stronger in girls. Obesity is a risk factor for precocious puberty in preschool children aged 3-<6 years, and 6-<10 years of age is a high risk period for early development in obese girls.
Adolescent ; Body Mass Index ; Child ; Child, Preschool ; China/epidemiology* ; Cross-Sectional Studies ; Female ; Humans ; Male ; Obesity/epidemiology* ; Overweight/epidemiology* ; Puberty ; Puberty, Precocious ; Sexual Development

Objective: To explore the heterogeneity and correlation of clinical phenotypes and genotypes in children with disorders of sex development (DSD). Methods: A retrospective study of 1 235 patients with clinically proposed DSD in 36 pediatric medical institutions across the country from January 2017 to May 2021. After capturing 277 DSD-related candidate genes, second-generation sequencing was performed to analyzed the heterogeneity and correlation combined with clinical phenotypes. Results: Among 1 235 children with clinically proposed DSD, 980 were males and 255 were females of social gender at the time of initial diagnosis with the age ranged from 1 day of age to 17.92 years. A total of 443 children with pathogenic variants were detected through molecular genetic studies, with a positive detection rate of 35.9%. The most common clinical phenotypes were micropenis (455 cases), hypospadias (321 cases), and cryptorchidism (172 cases) and common mutations detected were in SRD5A2 gene (80 cases), AR gene (53 cases) and CYP21A2 gene (44 cases). Among them, the SRD5A2 mutation is the most common in children with simple micropenis and simple hypospadias, while the AMH mutation is the most common in children with simple cryptorchidism. Conclusions: The SRD5A2 mutation is the most common genetic variant in Chinese children with DSD, and micropenis, cryptorchidism, and hypospadias are the most common clinical phenotypes. Molecular diagnosis can provide clues about the biological basis of DSD, and can also guide clinicians to perform specific clinical examinations. Target sequence capture probes and next-generation sequencing technology can provide effective and economical genetic diagnosis for children with DSD.
3-Oxo-5-alpha-Steroid 4-Dehydrogenase/genetics* ; Child ; China/epidemiology* ; Cryptorchidism/genetics* ; Disorders of Sex Development/genetics* ; Female ; Genital Diseases, Male ; Genotype ; Humans ; Hypospadias/genetics* ; Male ; Membrane Proteins/genetics* ; Penis/abnormalities* ; Phenotype ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics*

Objective:To explore the method and effect of free thining anterolateral thigh perforator flap (ALTP) in repairing soft tissue defect of scar contracture deformity after hand trauma.Methods:From March, 2015 to August, 2019, 12 patients who suffered scar contracture after hand trauma were repaired with thin ALTP. First, completely resected the scar contracture tissue from the hands and restored the normal bone structure and force line of the hand. The area of hand wound defects were 5.0 cm×6.0 cm-8.0 cm×10.0 cm. The wound was repaired by free ALTP, and the flap was micro-thinned for the first time. The flaps did not carry broad fascia, and the donor sites were directly sutured. The wound healing, the flap appearance, texture, sensation, scarring of the donor area, and functional recovery of the affected hand were observed regularly after surgery. The patients were followed-up by outpatient review and WeChat.Results:All the flaps survived well after the operation. Two cases suffered crisis because hematoma entraps vein cause by bleeding from perforator branch. After surgical exploration, the flaps survived successfully. All 12 flaps were followed-up successfully, including 6 cases reviewed in outpatient clinic, 4 cases followed by WeChat video and 2 cases by telephone consultation. The follow-up time was 3-20 months, with an average of 11 months. The flaps were not bloated, soft, non-pigmented, and beautiful in appearance. Only linear scars remained in the donor sites. The gripp function, palm function, thumb opposition function and finger function of the affected hand were largely restored. According to the TAM method of Upper Limb Function Evaluation of the Chinese Medical Association: 7 cases were excellent, 4 cases were good, and 1 case was fair.Conclusion:The thinning ALTP can be used to repair the scar contracture deformity after hand trauma. It can carry different tissues for 3-dimensional repair. After operation, the flap has a beautiful appearance, the donor site can be closed directly, and the damage of donor site can be reduced. It is an effective method to repair the hand contracture deformity.

Objective To explore the composition and drug resistance characteristics of pathogenic isolates from cerebrospinal fluid (CSF) cultures to facilitate empirical therapy of pediatric patients with meningitis in Guangzhou district.Methods During 2011 Jan 1st to 2015 Dec 31st,cerebrospinal fluids were collected from pediatric patients with suspected meningitis for regular culture,identification and drug susceptibility test of pathogenic isolates according to the national clinical laborato ry standard operation procedure,followed by analysis of their composition and drug resistance characteristics.Results There were 132 pathogenic isolates from CSF cultures,including Gram-positive strains (39.40％,52/132),Gram-negative strains (757.58％,6/132),fungi (3.03 ％,4/132),respectively.The main isolates were Escherichia coli (E.coli) (23.48％,31/132),Streptococcus pneumonia (S.pneumonia) (22.73％,30/132),Staphylococcus aureus (S.aureus) (12.12％,16/132)and Streptococcus agalactiae (S.agalactiae) (9.85％,13/132),respectively.E.coli had no resistance to piperacillin/tazobactam,furantoin,cefepime,amikacin,tobramycin,imipenem,ertapenem and meropenem.3.22 ％ resistance rate to cefotetan,9.38％ to ceftazidime,12.90 ％ to aztreonam,approximately 30 ％ to ampicillin/sulbactam,ofloxacin,ciprofloxacin,ceftriaxone,gentamycin,cefazolin,over 60 ％ to both sulfamethoxazole and ampicillin,31.25 ％ strains were ESBL positive.S.pneumonia had no re sis tance to ertapenem,5.88 ％ resistance rate to telithromycin,14.71％ to chloromycetin,17.76 ％ to ceftriaxone,23.53 ％ to amoxicillin,32.30 ％ to meropenem,35.29 ％ to cefotaxime,over 70 ％ to tetracycline,erythromycin,penicillin,and sulfamethoxazole.S.a ureus had no resistance to rifampicin,tigecycline,quinuprin/dupletin,linezolid and vancomycin,6.25％ resistance to ciprofloxacin,6.25％ to gentamicin,12.50％ to sulfamethoxazole,18.75％ resistance to clindamycin,31.25 ％ to tetracycline,62.50 ％ to erythromycin,over 90 ％ to cephalosporins and nearly 100 ％ to penicillin,the rate of MRSA strain was 56.25 ％.S.agalactiae had no resistance to penicillin,23.08 ％ resistance rate to ofloxacin,7.69 ％ to ciprofloxacin,over 60％ to tetracycline,erythromycin and clindamycin.Conclusion The main pathogenic isolates from CSF cultures were E.coli,S.pneumonia,S.aureus,S.agalactiae,different species of isolates have different drug resistance characteristics.This will provide instructions for the prevention,pathogenic diagnosis and treatment of meningitis in pediatric patients.

Mineral Chinese medicine is the distinctive part of the Chinese traditional medicine. The mineral Chinese medicines containing mercury elements such as cinnabaris, calomelas and hydrargyri oxydum rubrum are widely applied in the clinical conditions because of their efficacy of sedative, sterilization, removing necrotic tissue and promoting granulation. However, the rationality and security of clinical application are questioned because of the toxic effect caused by mercury compounds. This paper would summarize the efficacy of the mineral Chinese medicines containing mercury element, as well as their hepatotoxicity, nephrotoxicity, embryotoxicity, and neurotoxicity effect and mechanisms. Improper usage or high dose of the mineral Chinese medicines containing mercury element would cause acute hepatotoxicity. Cinnabaris, calomelas and hydrargyri oxydum rubrum may lead to chronic hepatotoxicity, nephrotoxicity, embryotoxicity and neurotoxicity when they were applied externally to the skin for long-term use. In addition to the accumulation of mercury elements in the tissues and organs, the species and forms of mercury compounds absorbed into the body in different ways, should be also studied in order to understand the toxicity of the mineral Chinese medicines containing mercury element. Meanwhile the dose and period of treatment shall be also considered in order to provide the references for rational and safe clinical application of the mineral Chinese medicines containing mercury element.

OBJECTIVETo explore the effects of obesity on the peak level of luteinizing hormone (LH) in the gonadotropin-releasing hormone (GnRH) agonist test and obesity-related hormones in girls with central precocious puberty (CPP).

METHODSThree hundred and thirty-three girls with CPP who underwent the GnRH agonist test between 2012 and 2014 were classified into three groups: normal weight (n=123), overweight (n=108), and obesity (n=102), according to body mass index (BMI). The sexual development indices were compared between the three groups. Twenty girls were randomly selected from each group for evaluation of the serum levels of leptin, sex hormone binding globulin (SHBG), neurokinin B, and kisspeptin. The correlation of BMI with the levels of various hormones was assessed using Pearson correlation analysis.

RESULTSThere was no significant difference in mean age at diagnosis between the three groups; however, the bone age was significantly higher in the overweight and obesity groups than in the normal weight group (P<0.05). The peak level of LH in the GnRH agonist test and SHBG level in the normal weight group were significantly higher than those in the overweight and the obesity groups, while the serum levels of leptin and neurokinin B were significantly lower in the normal weight group than in the overweight and the obesity groups (P<0.05). BMI was negatively correlated with the peak level of LH in the GnRH agonist test and SHBG level (P<0.05), and positively correlated with the levels of leptin and neurokinin B (P<0.05).

CONCLUSIONSThe effects of BMI on the result of the GnRH agonist test and levels of obesity-related hormones should be taken into account in girls with precocious puberty.

Body Mass Index ; Child ; Female ; Gonadotropin-Releasing Hormone ; agonists ; Humans ; Leptin ; blood ; Luteinizing Hormone ; blood ; Neurokinin B ; blood ; Obesity ; blood ; Puberty, Precocious ; blood ; Sex Hormone-Binding Globulin ; analysis

OBJECTIVETo study the expression of transforming growth factor-β1 (TGF-β1) and plasminogen activator inhibitor-1 (PAI-1) and its significance in premature infants with bronchopulmonary dysplasia (BPD).

METHODSA retrospective analysis was performed on the clinical data of 96 very low birth weight infants (gestational age of ≤ 32 weeks) who survived for more than 28 days and were admitted to the Neonatal Intensive Care Unit between January 2010 and December 2012. These subjects were divided into BPD group (n=21) and non-BPD group (n=75). The expression of TGF-β1 and PAI-1 in blood was measured by ELISA.

RESULTSThe levels of TGF-β1 and PAI-1 in the BPD group increased gradually from the 7th day to the 14th day and then to the 21st day after birth, and were significantly higher than in the non-BPD group at all time points (P<0.01). The TGF-β1 and PAI-1 levels in the non-BPD group on the 7th, 14th, and 21st days after birth were not significantly different from each other (P>0.05).

CONCLUSIONSThe expression of TGF-β1 and PAI-1 in blood is elevated in premature infants with BPD, which may be associated with the development of BPD.

Bronchopulmonary Dysplasia ; etiology ; metabolism ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Infant, Newborn ; Infant, Premature ; Male ; Plasminogen Activator Inhibitor 1 ; blood ; Retrospective Studies ; Transforming Growth Factor beta1 ; blood