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The clinical data of one child with metanephric stromal tumor (MST) and BRAF V600E gene mutation admitted to the First Affiliated Hospital of Zhengzhou University in June 2022 was analyzed retrospectively.Literature was reviewed.The patient, a 2-year-old girl, was diagnosed with a tumor in the left abdomen.The maximum diameter of the tumor was 10.5 cm.A radical nephrectomy was performed on the left kidney, and postoperative pathology revealed MST.Microscopically, the tumor had no envelope and exhibited expansive growth.The tumor cells were fusiform or stellate, and nuclear division was visible in the cell-rich region.Dysplastic blood vessels were seen inside the tumor.The tumor cells around the blood vessels and invaginated renal tubules were arranged like onion skin.CD34 was detected positive by immunohistochemical staining, and BRAF V600E mutation was also detected positive by fluorescent polymerase chain reaction.A total of 21 relevant case reports were retrieved, including 16 in English and 5 in Chinese.Fifty-eight MST patients, including the one in this report were analyzed.These patients were aged 2 days to 15 years, with a median age of 2 years.Except for 2 patients with unknown sex, the ratio of male to female was about 1.4∶1.0.Most MST patients were asymptomatic, with an average tumor size of 5.3 cm.The tumor cell CD34 showed positive expression in different degrees.Eight patients received the BRAF V600E mutation detection, and the results were all positive.Fifty-eight patients underwent nephrectomy and were followed up for 0-156 months, of which 7 patients were assisted with radiotherapy and chemotherapy.During the follow-up, 1 patient died, and 1 patient had a relapse.MST is a rare benign renal stromal tumor. BRAF V600E mutations are detected in a variety of malignancies.This paper is the first to report MST with BRAF V600E mutation in China and points out the importance of molecular detection of BRAF mutation for accurate diagnosis of MST.
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Objective:To summarize the clinical features, treatments and prognoses of aggressive natural killer cell leukemia (ANKL) in children.Methods:Clinical data and follow-up results were retrospectively reviewed for one hospitalized case of ANKL in June 2019.Through a literature search, the relevant items were retrieved from the databases of China National Knowledge Infrastructure, WanFang and PubMed using the Chinese and English keywords of "aggressive natural killer cell leukemia" and "children" up to December 2021.Results:This 8-year-old girl was diagnosed with ANKL by flow cytometric immunophenotype and immunohistochemical stain.Fever was the initial manifestation accompanied by sallow complexion, fatigue, enlargement of liver, spleen and lymph node and hematopenia of three lines.Allogeneic hematopoietic stem cell transplantation (allo-HSCT) was performed after chemotherapy.As of April 2022, the child stayed in a disease-free survival state after follow-ups for over 2 years.The literature search finally yielded 7 eligible Chinese and 10 English reports with a total of 17 pediatric ANKLs.In this group, there were fever (n=15), rash (n=1), perineal mass (n=1) and diarrhea, vomiting and other digestive tract symptoms (n=1). Six cases were misdiagnosed during an early stage of disease.4 cases received chemotherapy alone, 3 cases received chemotherapy regimen for acute lymphoblastic leukemia, 1 child died and one death occurred after received chemotherapy regimen of "cisplatin + vincristine + doxorubicin + ifosfamide". Allo-HSCT was performed in 5 patients after remission with chemotherapy and one child died from multiple organ failure at 9 months after allo-HSCT.Nine cases gave up treatment.Conclusions:ANKL has a rapid disease progression, diverse clinical manifestations, easy misdiagnosis and poor prognosis.For suspected ANKL cases, clinicians perform multiple bone perforations at multiple sites and immunophenotype by flow cytometry as soon as possible to confirm the diagnosis.Currently allo-HSCT offers a long-term survival of ANKL patients.
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Background/Aims@#To investigate the autoantibody against fumarate hydratase (FH), which is a specific liver failure-associated antigen (LFAA) and determine whether it can be used as a biomarker to evaluate the prognosis of acute-on-chronic liver failure (ACLF). @*Methods@#An immunoproteomic approach was applied to screen specific LFAAs related to differential prognosis of ACLF (n=60). Enzyme-linked immunosorbent assay (ELISA) technology was employed for the validation of the frequency and titer of autoantibodies against FH in ACLF patients with different prognoses (n=82). Moreover, we clarified the expression of autoantibodies against FH in patients with chronic hepatitis B (n=60) and hepatitis B virus-related liver cirrhosis (n=60). The dynamic changes in the titers of autoantibodies against FH were analyzed by sample collection at multiple time points during the clinical course of eight ACLF patients with different prognoses. @*Results@#Ultimately, 15 LFAAs were screened and identified by the immunoproteomic approach.Based on ELISA-based verification, anti-FH/Fumarate hydratase protein autoantibody was chosen to verify its expression in ACLF patients. ACLF patients had a much higher anti-FH autoantibody frequency (76.8%) than patients with liver cirrhosis (10%, p=0.000), patients with chronic hepatitis B (6.7%, p=0.022), and normal humans (0%, p=0.000). More importantly, the frequency and titer of anti-FH protein autoantibodies in the serum of ACLF patients with a good prognosis were much higher than that of patients with a poor prognosis (83.9% vs 61.5%, p=0.019; 1.41±0.85 vs 0.94±0.56, p=0.017, respectively). The titer of anti-FH autoantibodies showed dynamic changes in the clinical course of ACLF. @*Conclusions@#The anti-FH autoantibody in serum may be a potential biomarker for predicting the prognosis of ACLF.
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Objective:To investigate the clinical features and long-term prognosis of pediatric acute lymphoblastic leukemia (ALL) with renal involvement as the initial manifestation, thus enhancing the diagnostic and therapeutic efficacy.Methods:Twenty-four cases of pediatric ALL with renal involvement as the initial manifestation treated in the First Affiliated Hospital of Zhengzhou University from March 2013 to March 2019 were analyzed retrospectively, and their clinical characteristics were analyzed.According to renal imaging examination findings, they were divided into abnormal group and normal group.The differences in clinical features between the two groups were compared, and the cumulative survival rate was evaluated by Kaplan-Meier method.Results:Among 1 030 newly treated cases of pediatric ALL, 24 cases(2.33%) had renal involvement as the initial manifestation, involving 20 males and 4 females, with a male/female ratio of 5∶1 and the median age of 4.3 years (1.3-14.0 years). There were 16 cases of superficial lymph node enlargement and 21 cases of hepatosplenomegaly.Immature cells in peripheral blood were found in 15 cases.Nine cases were examined with abnormal renal imaging, involving 8 cases returned normal after chemotherapy, and 1 died of renal failure.At the end of follow-up on August 1, 2020, there were 9 cases of bone marrow relapse, 11 survival cases, 10 death cases and 3 cases of loss to follow-up.There were no significant differences in the sex, age, immunophenotype, organ infiltration and urinary protein between the two groups (all P>0.05). The proportion of high creatinine level and intramedullary recurrence rate in the abnormal group were significantly higher than those in the normal group [55.6%(5/9 cases) vs.0(0/15 cases), P=0.003; 66.7%(6/9 cases) vs.20.0%(3/15 cases), P=0.036]. The survival analysis indicated that the 3-year cumulative survival in the abnormal group was significantly lower than that of normal group (17.3% vs.72.7%, χ2=4.047, P< 0.05). Conclusions:For children with unexplained renal involvement as the initial manifestation, clinicians should consider the possibility of leukemic renal infiltration or nephrogenic lymphoma.Physical examinations of the liver, spleen and lymph nodes, morphological analysis of peripheral blood cells, bone marrow examination and renal biopsy are important to make a definite diagnosis in time.Children with imaging abnormalities caused by leukemic renal infiltration are more likely to relapse and have a lower survival rate, which may be a poor prognostic factor for ALL.
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【Objective】 To investigate the dynamic changes of local and systemic inflammatory responses after high-fat diet feeding of ApoE-/- mice so as to establish the method to assess the recruitment of PKH26-labeled monocytes into atherosclerotic plaque. 【Methods】 ApoE-/- mice that had received a high-fat diet (HFD) for 8 and 16 weeks were assessed for: ① atherosclerotic plaque burden (oil red O staining), ② aortic tissue inflammation (by RT-qPCR to detect aortic inflammatory factors and cells), ③ systemic inflammatory responses (by ELISA to measure plasma proinflammatory cytokine levels and flow cytometry to assess inflammatory cellular profile in the peripheral blood), and ④ recruitment of PKH26 labeled monocytes into atherosclerotic lesions (by confocal microscope). 【Results】 Compared to those in high-fat diet 8 weeks group, ApoE-/- mice in high-fat diet 16 weeks group had more serious atherosclerosis in the whole aorta and aortic root (P<0.01); significantly up-regulated aortic mRNA levels of inflammatory factors CCL2, TNF-α and IL-1β (P<0.01); an increased number of aortic local macrophages (P<0.001); significantly increased protein expression of plasma CCL2 and TNF-α (P<0.01); and the increased percentage of peripheral blood monocytes (P<0.05). Moreover, the number of PKH26 labeled monocytes that migrated to aortic lesions were significantly higher in high-fat diet 16 weeks group (P<0.01). 【Conclusion】 High-fat diet promotes the severity of systemic and local inflammatory responses in ApoE knockout mice, which contributes to the migration of peripheral blood monocytes to atherosclerotic plaque.
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Objective:To discuss whether platelet distribution width (PDW) can effectively predict the prognosis of neuroblastoma (NB).Methods:The clinical data of 67 NB patients in the First Affiliated Hospital of Zhengzhou University between January 2014 and January 2018 were retrospectively analyzed.They were divided into low PDW group and high PDW group according to the PDW level, and the differences in clinical indicators between the 2 groups were compared.The prognostic effects of PDW were assessed by using the Kaplan- Meier method and Cox regression model. Results:Among the 67 patients, 41 cases were male, 26 cases were female, with the ratio of male to female being 1.58∶1.00, and the average age was 44 months (2-156 months). Five cases were in stage Ⅰ, 1 case in stage Ⅱ, 15 cases in stage Ⅲ and 46 cases in stage Ⅳ.At the first time of diagnosis, there were 14 cases with age ≤ 18 months, 53 cases with age > 18 months, 47 cases with neuron specific enolase (NSE) level ≥ 100 μg/L, 20 cases with NSE level<100 μg/L.The median follow-up time was 20.4 months.At the end of follow-up, 35 cases died and 32 cases survived.There was no statistical difference in age, gender, primary site of tumor, tumor stage and mean platelet volume between the low PDW group and the high PDW group (all P>0.05). The proportion of high-risk patients, the level of NSE, bone marrow metastasis rate, MYCN gene amplification rate and the red blood cell distribution width in the high PDW group were significantly higher than those in the low PDW group, but the high PDW group had a lower level of thrombocytocrit than the low PDW group, and the differences were statistically significant(all P<0.05). Survival analysis revealed that the 2-year overall survival of the low PDW group was significantly higher than that of the high PDW group (69.8% vs.25.3%, χ2=15.761, P<0.05). Univariate analysis showed that NSE ( HR=6.606, 95% CI: 2.018-21.620), MYCN gene ( HR=1.977, 95% CI: 0.794-4.919), tumor risk stratification ( HR=5.926, 95% CI: 1.416-24.794), PDW ( HR=4.036, 95% CI: 1.957-8.322), and red blood cell distribution width ( HR=1.120, 95% CI: 1.005-1.249) were the adverse factors affecting the overall survival, and thrombocytocrit was a protective factor for the prognosis of NB.Multivariate analysis indicated that PDW was an independent risk factor of NB ( HR=2.524, 95% CI: 1.017-6.264, P=0.046). Conclusions:There is a good consistency between the increase of PDW and the known prognostic risk factors, elevated tumor markers and bone marrow metastasis.Increased PDW is associated with poor prognosis in NB patients, and PDW is an independent risk factor for the poor prognosis of NB.
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Objective To analezk thk charactkristics of drug-induckd livkr injure( DIFI)in childrkn with acutk lemphoblastic lkuckmia(LFF),so as to improvk thk phesician's undkrstanding of chkmothkrape DIFI,and to guidk clinical rational drug usk. Methods Onk hundrkd and forte-thrkk casks with LFF diagnoskd in thk Dkpartmknt of Hk-matologe and Oncologe in thk Pirst Lffiliatkd Hospital of Yhkngzhou Rnivkrsite from Januare 2012 to Dkckmbkr 2016 wkrk analezkd rktrospkctivkle. Baskd on DIFI diagnostic critkria and thk ARCLM scalk,thk casks with a scork of ≥3 points wkrk considkrkd to havk chkmothkrape DIFI. Groupkd be gkndkr,agk,immunoteping,risc and stagk of chkmo-thkrape,thk incidknck of DIFI was comparkd. Thk situation aftkr DIFI prkvkntion was comparkd bktwkkn two groups which was groupkd according to whkthkr thk application of hkpatoprotkctivk drugs. ResuIts Onk hundrkd and kight ca-sks(75. 52﹪)had DIFI,66 casks(61. 11﹪)showkd clinical manifkstations of livkr injure,and 42 casks(38. 89﹪) had no clinical semptoms. Lmong all thk casks 57. 41﹪(62 casks)wkrk mild livkr damagk,25﹪(27 casks)wkrk modkratk livkr injure and 17. 59﹪(19 casks)wkrk skvkrk livkr damagk. Thk clinical tepks which wkrk hkpatockllular accounting for 79. 63﹪(86 casks),cholkstatic 7. 41﹪(8 casks)and mixkd 12. 96﹪(14 casks). Malk wkrk 80 casks (79. 21﹪)and fkmalk 28 casks(66. 67﹪),but thk incidknck of DIFI bktwkkn diffkrknt gkndkr group had no statistical diffkrknck(χ2 ﹦2. 524,P﹦0. 112). Skvknte-fivk casks(77. 32﹪)wkrk <7 ekars agk and 33 casks(71. 74﹪)≥7 ekars agk,and thk incidknck of DIFI bktwkkn 2 groups was not statisticalle diffkrknt(χ2 ﹦0. 526,P﹦0. 468). Thkrk was no significant diffkrknck in T-LFF(8 casks,61. 54﹪)and B-LFF(100 casks,76. 92﹪)( χ2 ﹦0. 795,P﹦0. 372). Thk incidknck had significant diffkrknck in diffkrknt risc(P﹦0. 002). Thk incidknck of DIFI bktwkkn thk middlk risc group(60 casks,88. 24﹪)and standard risc(21 casks,58. 33﹪)had statistical diffkrknck( P <0. 05 ). Thk incidknck of DIFI bktwkkn thk middlk risc group and skvkrk risc(27 casks,69. 23﹪)had statistical diffkrknck( P﹦0. 015). Thk incidknck was diffkrknt in diffkrknt stagks of chkmothkrape(P<0. 05). Thk incidknck of DIFI in induckd stagk was diffkrknt comparkd to othkr stagks(P<0. 05). ARCLM scork >8 points accountkd for 21 casks(19. 45﹪), 6-8 points accountkd for 59 casks(54. 63﹪)and 3 -5 points accountkd for 28 casks(25. 92﹪). Eighte -nink patiknts(92. 71﹪)wkrk kffkctivk in thk hkpatoprotkctivk group and 8 patiknts(66. 67﹪)in thk no hkpatoprotkctivk thkrape group. Thk diffkrknck bktwkkn thk 2 groups was statisticalle significant(χ2 ﹦5. 317,P﹦0. 021). ConcIusions Thk clinical semptoms of drug-induckd livkr injure in childrkn with LFF chkmothkrape ark lacc of spkcificite. Thke ark mainle charactkrizkd be mild livkr injure. Thk clinical tepk of hkpatic injure is common in hkpatockllular. Thk ARCLM scork was mostle 6 to 8. Thkrk is no rklationship bktwkkn thk incidknck in LFF and gkndkr,agk,tepk of lkuck-mia. Thk incidknck with modkratk risc tepk is highkr than that of thk standard and high-risc tepk. Thk incidknck in induction rkmission stagk is highkst. Lpplication of hkpatoprotkctivk drugs is bknkficial to DIFI prognosis.
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Objective: To explore the diagnostic value and efficiency of using whole body bone scintigraphy (WBS) combined with the levels of tumor markers to evaluate non-small cell lung cancer (NSCLC) patients with bone metastases. Methods: One-hundred and eighty-five cases of NSCLC, confirmed by pathology or cytological examination from January 2014 to June 2016, were emrolled from the Affiliated Tumor Hospital of Guangxi Medical University. WBS and test results of tumor markers, such as serum carcinoembryonic antigen (CEA), serum carbohydrate antigen (CA125), and cytokeratin CK19 (CYFRA21-1), were analyzed. WBS results were assessed by the Soloway classification criteria and divided into four grades: Correlations between WBS classification and the levels of tumor mark-ers were determined with Spearman correlation analyses. Results: Seventy-eight of the 185 NSCLC patients had bone metastases (a rate of 42.16%). The sensitivity and specificity of WBS were 91.02%(71/78) and 85.98%(92/107), respectively. The CEA, CA125, and CYFRA21-1 levels in bone metastases patients were higher than those in NSCLC patients without bone metastases (P<0.05). In the 78 patients with bone metastases, there were seven cases of EOD0 (8.98%), 39 cases of EOD1 (50%), 17 cases of EOD2 (21.8%), and 15 cases of EOD3 (19.2%). The correlations between WBS grade and CEA, CA125, and CYFRA21-1 levels were: rs=0.579, 0.274, and 0.327, respectively (P<0.05). The combined WBS and tumor marker diagnostic performance was significantly better than either alone (AUC=0.922), and their sensitivity and specificity increased (92.3%and 86.0%, respectively). Conclusions: WBS shows high clinical efficacy in the diagnosis of NSCLC with bone metastases. Furthermore, it can be used as a screening test for bone metastases of NSCLC, which has important clinical implications. WBS combined with CEA, CA125, and CYFRA21-1 examination improves the detection rate of NSCLC bone metastases, thereby enhancing its clinical utility.
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Objective To investigate the expression of human platelet antibodies(HPA)in children with immune thrombocytopenia(ITP)at different ages and their clinical significance in the treatment of ITP.Methods Two hundred eighty-eight cases of children who were newly diagnosed as primary ITP and detected with HPA from January 2013 to December 2015 in the First Affiliated Hospital of Zhengzhou University were selected.According to the HPA values,they were divided into <1:10 group,1:10 group and >1:10 group.According to their age,they were di-vided into <3-year-old group and ≥3-year-old group.Data were organized and the relationship among platelet antibody,age,gender and short-term efficiency of treatment was analyzed by using SPSS 21.0 statistical analysis soft-ware.Results In this study,there were 288 cases of children,with male to female ratio of about 1.3:1.0.As to the<3-year-old group and ≥ 3-year-old group,this difference of male to female ratio was statistically significant (respectively 1.93:1.00 and 1.02:1.00,χ2=6.629,P<0.05),and the difference of the HPA positive rate was statistically significant(respectively 72.5% and 59.5%,χ2=5.716,P<0.05);the HPA positive rate of boys and girls respectively was 65.9% and 63.7%,so their diffe-rence of the HPA positive rate was not statistically significant (χ2=0.143,P>0.05).Regarding the short-term efficiency,HPA in <1: 10 group,1: 10 group and >1:10 group was respectively 89.1%,89.1%,100.0%.Statistical analysis suggests:the short-term efficiency of <1:10 group and 1:10 group was basically the same(χ2=0.000,P>0.05);In comparison of <1:10 group with >1:10 group(χ2=4.268,P<0.05),and in comparison of 1:10 group with >1:10 group(χ2=4.411,P<0.05),their differences were statistically significant.Conclusions This study suggests that boys are more susceptible to ITP,espe-cially in the <3 age group.The total positive rate of HPA is higher in <3 years old group.The HPA has a certain guiding significance for the diagnosis of ITP.Compared with the other 2 groups,HPA>1:10 group may have higher short-term efficiency in clinical practice.
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Objective To explore the clinical and pathological features, diagnosis and treatment of eosinophilic cystitis in children. Method The clinical data of 7 patients with eosinophilic cystitis admitted from 2012 to 2016 were retrospectively analyzed, and the related literature were reviewed. Results The median age of the 7 patients was 9 years, and clinical manifestations were urgent urination, frequent micturition, odynuria, hematuria, abdominal pain and nocturnal enuresis. Ultrasonography and CT examination showed thickened bladder wall and space occupying lesions.All the 7 children received bladder biopsy, and pathology was consistent with eosinophilic cystitis. Six of them were cured after 2 months of drug therapy, and the other one was cured by repeated drug treatment for 1 year.All patients were followed up for 3 months to 4 years until the abnormal symptoms of voiding disappeared and the abnormal changes of bladder disappeared by imaging examination. Conclusion Eosinophilic cystitis in children is a benign lesion, having extremely similar clinical manifestations to bladder tumor. Without biopsy, the diagnosis of eosinophilic cystitis can also be made according to the clinical manifestation, laboratory examination and treatment effect. The treatment for this disease mainly includes hormone, antihistamine and anti-inflammatory drugs.
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Objective To investigate the effect of bedside comprehensive ability training and critical thinking on the improvement of the personality maturity of pediatric nurses. Methods A total of 102 nurses with pediatric working years within one year were selected,and then they were arranged to receive critical thinking in combination with bedside comprehensive ability training for 6 months. After the training, pediatric nurses′ critical thinking ability, bedside comprehensive ability and personality maturity were evaluated. Results Before training, the total score of Critical Thinking Disposition Inventory-Chinese Version (CTDI-CV) , total score of bedside comprehensive ability, personality maturity score were 288.21±25.44, 75.24±2.51, 84.01±32.26, after training, they were respectively 324.18±25.93, 92.65±3.17, 125.57±35.47. Comparison of the scores before training and after training showed statistically significant difference(t=7.071, 30.750, 6.190,P < 0.05 or 0.01). Setting personality maturity as the dependent variable, and and CTDI-CV and bedside comprehensive ability as the independent variables, regression analysis showed that CTDI-CV and bedside comprehensive ability had a positive impact on the personality maturity. Conclusions The ability of pediatric nurses' critical thinking ability and bedside comprehensive ability training can promote the improvement of personality maturity of pediatric nurses.