Correlation between 25-hydroxyvitamin D and nephroblastoma in children and its value in assessing disease prognosis / 中国当代儿科杂志

OBJECTIVES@#To study the correlation between 25-hydroxyvitamin D [25-(OH)D] and nephroblastoma in children and its value in assessing the prognosis of the disease.@*METHODS@#A total of 50 children with nephroblastoma who were admitted from January 2018 to December 2022 were included as the nephroblastoma group, and according to the postoperative pathological type, they were divided into a good prognosis group with 38 children and a poor prognosis group with 12 children. A total of 50 healthy children who underwent physical examination during the same period of time served as the healthy control group. The above groups were compared in terms of serum creatinine and 25-(OH)D level. A Spearman correlation analysis was used to investigate the correlation between serum 25-(OH)D level and therapeutic effect reaction. A multivariate logistic regression analysis was used to identify the risk factors affecting the prognosis of nephroblastoma in children.@*RESULTS@#The nephroblastoma group had significantly lower levels of serum creatinine and 25-(OH)D than the healthy control group (P<0.05). Compared with the good prognosis group, the poor prognosis group had a significantly larger tumor diameter, a significantly higher proportion of children with stage III-IV tumors, a significantly higher rate of tumor metastasis, and significantly lower serum levels of creatinine and 25-(OH)D (P<0.05). The Spearman correlation analysis showed that serum 25-(OH)D level was negatively correlated with therapeutic effect reaction (rs=-0.685, P<0.001). The multivariate logistic regression analysis showed that tumor diameter ≥10 cm, stage III-IV tumors, presence of tumor metastasis, and 25-(OH)D <19 ng/mL were closely associated with the poor prognosis of nephroblastoma in children (P<0.05). Serum 25-(OH)D level had an area under the curve of 0.805 (95%CI: 0.706-0.903, P<0.001) in evaluating the prognosis of nephroblastoma in children, with a Youden index of 0.512, a sensitivity of 0.938, and a specificity of 0.575 at the optimal cut-off value of 1.764 ng/mL.@*CONCLUSIONS@#There is a significant correlation between 25-(OH)D level and the prognosis of nephroblastoma in children, and 25-(OH)D can be used for prognosis prediction.

Differentiating pediatric cystic nephroma from common renal multicystic lesions: A case report

Pediatric cystic nephroma is a rare, clinically benign, renal tumor. Pediatric renal cystic lesions are complex. Imaging findings and tumor appearance are often nonspecific, and careful pathological examination is necessary. We discuss diagnosis of pediatric cystic nephroma and how to differentiate it from multicystic dysplastic kidney and cystic partially differentiated nephroblastoma.

Hallazgo necrópsico de nefroblastoma asociado a síndrome metabólico experimental / Necropsic finding of nephroblastoma associated to experimental metabolic syndrome

Introducción: El síndrome metabólico presenta entre sus manifestaciones la obesidad, la cual se encuentra relacionada con el desarrollo de cáncer. Sin embargo, no habíamos encontrado en cuatro años ningún caso de neoplasias malignas en animales con síndrome metabólico. Objetivo: Describir el desarrollo de un tumor maligno a nivel renal en modelo experimental de síndrome metabólico. Métodos: El biomodelo experimental se logró por la aplicación de una solución de sacarosa al 35 por ciento, durante 20 semanas a 50 ratas machos Wistar destetados. El diagnóstico de nefroblastoma se realizó mediante necropsia con observación morfológica de la lesión renal. Resultados: Confirmado el síndrome metabólico se detectó en un caso, una masa palpable en abdomen. En la inspección macroscópica se observó un tumor en el polo inferior del riñón derecho, color pardo grisáceo, con hemorragia y cambios quísticos. Histológicamente se observaron alteraciones propias de un nefroblastoma mixto con componentes del blastema, mesenquimal y epitelial. Conclusiones: Se describe por vez primera, en estudio anatomopatológico, la presencia de un caso de nefroblastoma en rata con síndrome metabólico experimental(AU)

Introduction: One of the manifestations of metabolic syndrome is obesity, which is in turn related to the development of cancer. However, in four years we had not found any case of malignant neoplasms in animals with metabolic syndrome. Objective: Describe the development of a malignant renal tumor in an experimental metabolic syndrome model. Methods: The experimental biomodel was made applying a 35 percent saccharose solution to 50 male weaned Wistar rats for 20 weeks. The diagnosis of nephroblastoma was achieved by necropsy with morphological observation of the renal lesion. Results: Upon metabolic syndrome confirmation, a palpable mass was detected in the abdomen of one of the cases. Macroscopic observation revealed a grayish brown tumor in the lower pole of the right kidney with hemorrhaging and cystic changes. Histological examination found alterations typical of mixed nephroblastoma with blastema, mesenchymal and epithelial components. Conclusions: This is the first time a description is provided in an anatomopathological study of a case of nephroblastoma in a rat with experimental metabolic syndrome(AU)

MicroRNA-200c-3p inhibits proliferation of nephroblastoma cells by targeting CCNE2 / 南方医科大学学报

OBJECTIVE@#To predict and verify the target gene of miR-200c-3p and evaluate the inhibitory effect of miR-200c-3p on the proliferation of nephroblastoma cells.@*METHODS@#The putative target genes of miR-200c-3p were predicted by bioinformatics approach. Nephroblastoma cell models with miR-200c-3p overexpression or knockdown were established in SK-NEP-1 and G401 cells with corresponding control groups. The expressions of CCNE2 in SK-NEP-1 and G401 cells in different groups were detected by RT-PCR and Western blotting. A luciferase reporter assay was used to determine the targeting relationship between miR-200c-3p and CCNE2. The effects of miR-200c-3p overexpression or knockdown on cell proliferation was detected by cell counting kit-8 (CCK-8) assay and soft agarose assay.@*RESULTS@#CCNE2 was one of the target genes of miR-200c-3p as predicted by bioinformatics methods. Transfection of the two nephroblastoma cell lines with miR-200c-3p mimic resulted in significantly lowered CCNE2 mRNA and protein expressions ( < 0.05). The results of dual-luciferase assay confirmed that miR-200c-3p bound to the 3'UTR of CCNE2. CCK-8 assay and soft agarose assay demonstrated that overexpression of miR-200c-3p significantly inhibited the proliferation of the nephroblastoma cells ( < 0.01), and knocking down miR-200c-3p in the cells produced the opposite effects.@*CONCLUSIONS@#miR-200c-3p overexpression inhibits the proliferation of nephroblastoma cells by down-regulating its target gene CCNE2.

Factores asociados a la sobrevida en pacientes con tumor de Wilms / Factors associated with survival in patient with Wilms tumor

Introducción. El tumor de Wilms es el segundo tumor abdominal más frecuente en la edad pediátrica y responde por más del 90 % de los tumores renales en pediatría. A pesar de que la sobrevida descrita es mayor del 90 %, en nuestro medio encontramos que solo alcanza al 70 %, por lo que deseamos evaluar cuáles son los factores asociados con dichos resultados desfavorables, con el fin de implementar medidas para mejorar la sobrevida de nuestros pacientes. Métodos. Se realizó un estudio observacional, transversal, en dos centros de alto nivel de atención, que incluyó una muestra de 84 pacientes menores de 15 años, con diagnóstico de tumor de Wilms. Resultados. Los factores que se asociaron significativamente con un aumento en la probabilidad de morir fueron: no completar el protocolo de quimioterapia, (OR 34; IC95% 3,7-312; p 0,000) y presentar recidiva tumoral (OR 35,7; IC95% 6,9-184; p 0,000). Otros factores que aumentaron esta probabilidad sin alcanzar a ser significativos, pero mostrando una evidente tendencia fueron: presentación bilateral (OR 4,1; IC95% 0,6-5,5; p 0,147), complicaciones quirúrgicas (OR 3,2; IC95% 0,7-14,6; p 0,136), compromiso de ganglios linfáticos en tomografía (OR 2,4; IC95% 0,7-8,4; p 0,139) y las metástasis a distancia (OR 2,5; IC95% 0,7-9; p 0,143). Discusión. La sobrevida de nuestros niños con tumor de Wilms es menor que la reportada en la literatura mundial, siendo la falla en terminar la quimioterapia, la recidiva y la necesidad de cirugía bilateral, los factores asociados con este desenlace

Introduction. Wilms tumor is the second most frequent abdominal tumor in pediatric age, and it accounts for more than 90% of kidney tumors in pediatrics. Although the described survival is greater than 90%, in our set-ting we find that it only reaches 70%. Our objective was to evaluate the factors associated with these unfavorable results, in order to implement measures to improve the survival of our patients.Methods. An observational, cross-sectional study was conducted in two tertiary medical centers, which included a sample of 84 patients under 15 years of age with a diagnosis of Wilms tumor.Results. The factors that were significantly associated with an increase in the probability of dying were not com-pleting the chemotherapy protocol (OR 34; 95%CI 3.7-312; p 0.000) and presenting tumor recurrence (OR 35.7; 95%CI 6.9-184; p 0.000). Other factors that increased this probability without being significant, but showing an evident trend were: bilateral presentation (OR 4.1; 95%CI 0.6-5.5; p 0.147), surgical complications (OR 3.2; 95%CI 0.7-14.6; p 0.136), lymph node involvement in tomography (OR 2.4; 95%CI 0.7-8.4; p 0.139) and distant metastases (OR 2.5; 95%CI 0.7-9; p 0.143).Discussion. The survival of the children with Wilms tumor in our study was lower than that reported in the world literature, with failure to complete chemotherapy, recurrence and the need for bilateral surgery being the factors associated with this outcome

Tumor de Wilms no adulto: neoplasia rara e um desafio diagnóstico e terapêutico / Wilms tumor in adult: a rare neoplasia and a diagnostic and therapeutic challenge

O tumor de Wilms é o tumor renal maligno mais comum na criança. Sua apresentação em adultos é rara. Atualmente, há relato de aproximadamente 250 casos de tumor de Wilms em adultos. Relata-se neste trabalho, um novo caso em paciente do sexo masculino, 18 anos, com histórico de dor lombar direita e hematúria. A ressonância nuclear magnética de pelve demonstrou volumosa lesão expansiva com epicentro no terço médio do rim direito. Tomografia computadorizada de tórax com múltiplos nódulos esparsos pelo parênquima pulmonar, sugestivos de implantes secundários. Foi realizada nefrectomia radical direita e o estudo anatomo-patológico mais imuno-histoquimica confirmaram o diagnóstico de nefroblastoma (Tumor de Wilms) em estágio IV. Embora se tenha conseguido uma boa resposta com os esquemas de tratamento quimioterápicos atuais, estudos mostram que o prognóstico do tumor de Wilms em adultos é inferior quando comparado ao pediátrico. O qual pode estar relacionado ao fato de se tratar de uma doença rara com diagnóstico tardio.

Wilms tumor is the most common malignant renal tumor in children. His presentation in adults is rare. Currently, there are approximately 250 cases of Wilms tumor in adults. This paper reports a new case in a male patient, 18 years old, with a history of right lower back pain and hematuria. Pelvic Nuclear magnetic resonance demonstrated a massive expansive lesion with epicenter in the middle third of the right kidney. Computed tomography of the chest with multiple nodules scattered by the pulmonary parenchyma, suggestive of secondary implants. Right radical nephrectomy was performed and the anatomopathological and immunohistochemical study confirmed the diagnosis of nephroblastoma (Wilms' tumor) in stage IV. Although a good response has been achieved with current chemotherapy regimens, studies have shown that Wilms tumor prognosis in adults is lower when compared to pediatric. This may be related to the fact that it is a rare disease with a late diagnosis.

Mechanism of tumor suppressor gene DKK2 inhibiting proliferation of nephroblastoma cells in children / 实用肿瘤学杂志

Objective The aims of this study were to investigate the expression of DKK2,a WNT signaling pathway regula-tor,in nephroblastoma cells and tissues of children,the effect on the proliferation of nephroblastoma SK-NEP-1 cells,and to explore its mechanism. Methods The relative expression of DKK2 in nephroblastoma cells and tissues was analyzed by qRT-PCR and im-munoblotting assays. Overexpressing DKK2 SK-NEP-1 cells were set as the experimental group( DKK2 group);the blank control plasmid group was set as a control group( Vector group),transfected with pcDNA3. 1 ( +) -Flag-DKK2 plasmid( Experimental group)and pcDNA3. 1( +) -Flag-Vector plasmid(Control group). The over-expression of DKK2 was confirmed in SK-NEP-1 cells by RT-PCR and immunoblotting. CCK-8 and cell cloning assays were used to determine the effect of DKK2 on cell prolifera-tion;flow cell cycle and apoptosis assays were used to confirm the effect on cell proliferation in overexpressed DKK2 cells. The xen-graft formation assay in nude mice was to verify the effect of DKK2 on proliferation in overexpressed DKK2 cells;the mechanism of DKK2 in inhibitory proliferation was analyzed by qRT-PCR,Western blotting and immunohistochemistry. Results Compared with normal renal epithelial tissues,DKK2 mRNA was down-regulated in children with nephroblastoma,and the difference was statistically significant(P<0. 001). Compared with the control group,transfected DKK2 cell viability was significantly inhibited after treatment for 24,48 and 72 h( P<0. 05),cell clone formation in the experimental group was significantly inhibited(31. 11% ± 2. 14% ) ( P<0. 05),the cell cycle in the experimental group was significantly arrested at the G1 phase(P<0. 001),and the apoptosis rate in the experimental group was significantly increased(P<0. 001). Compared with the control group,the tumor weight and volume in nude mice were significantly low in the experimental mice which were injected DKK2 overexpression cells(P<0. 001). Active-β-cate-nin and downstream genes were significantly inhibited in over-expressed DKK2 SK-NEP-1 cells. Conclusion DKK2 is down-regulated in human cutaneous nephroblastoma and participates in the mechanism of nephroblastoma by antagonizing Wnt/β-catenin signaling pathway.

Nephroblastoma in a black-tufted marmoset (Callithrix penicillata) / Nefroblastoma em um sagui-de-tufos-pretos (Callithrix penicillata)

A renal nephroblastoma is described in a free-living black-tufted marmoset (Callithrix penicillata) in Central Brazil. The monkey was found dead and subjected to necropsy. Gross anatomic changes consisted of a ruptured left kidney, which was almost completely effaced by a white to yellow, partially encapsulated friable mass. The left ureter was distended due to obstruction by a red, spherical, 2mm in diameter friable mass. The urinary bladder was also distended. Histologically the renal and ureteral masses consisted of a triphasic embryonal neoplasm composed of embryonic epithelium forming glomeruli and tubules, polygonal blastemal cells, and a mesenchymal stroma. The embryonic epithelium exhibited rare nuclear immunoreactivity for WT-1, whereas blastemal cells exhibited robust cytoplasmic and rare nuclear immunoreactivity for WT-1; blastemal cells were also immunoreactive for vimentin. No immunoreactivity was detected for pan-cytokeratin (AE1/AE3), actin, and desmin. Morphological and immunohistochemical features of the present neoplasm are consistent with those described for renal nephroblastoma.(AU)

Descreve-se um caso de nefroblastoma maligno em um sagui de vida livre no Brasil Central. O macaco foi encontrado morto e encaminhado para necropsia. Na macroscopia, o rim esquerdo apresentava-se rompido e o parênquima estava substituído por um tecido neoplásico friável, parcialmente encapsulado e de superfície natural branca e de corte amarela. O ureter esquerdo apresentava-se distendido devido à obstrução por uma massa friável, vermelha, esférica, de 2mm de diâmetro. Histologicamente, as massas renal e ureteral consistiam de uma neoplasia embrionária composta por três populaçõies de células neoplásicas, composta por epitélio embrionário formando glomérulos e túbulos, células blastemais poligonais e um estroma mesenquimal. O epitélio embrionário exibiu imunorreactividade nuclear rara para WT-1, enquanto que as células blastemais exibiram imunorreactividade nuclear citoplasmática e rara para WT-1; As células blastemais também foram imunorreativas à vimentina. Nenhuma imunorreatividade foi detectada para pan-citoqueratina (AE1/AE3), actina e desmina. As características morfológicas e imuno-histoquímicas da presente neoplasia são consistentes com as descritas para o nefroblastoma renal.(AU)

CCN3 inhibits deposition of extracellular matrix of glomerular mesangial cells via promoting the expression of microRNA-29a / 中华肾脏病杂志

Objective To investigate the effects of nephroblastoma over-expressed protein (CCN3) on the formation of extracellular matrix (ECM) induced by transforming growth factor-β1 (TGF-β1) in human mesangial cells (HMCs) and its underlying signal transduction mechanism related with microRNA-29(miRNA-29).Methods HMCs were pretreated with different doses of exogenous CCN3 (5 μg/L,50 μg/L and 500 μg/L) or transfected with pcDNA3.1(+)-CCN3 before exposed to TGF-β1(2 μg/L),to observe the expression of fibronectin (FN),type I collagen (COL I) and miRNA-29a,b and c.The mimics or inhibitor of the miRNA-29a were transfected into HMCs to analyze whether miRNA-29a affect CCN3.The expressions of FN mRNA,COL I mRNA and miRNA-29 family were detected by real time PCR.The protein expressions of FN and COL I were detected by Western blotting and cell immunofluorescence.Results (1) Compared with the normal control group,the expressions of FN and COL I were up-regulated in TGF-β1 group,while the expressions of miRNA-29a,b,c were down-regulated in TGF-β1 group (all P ＜ 0.05).(2) Compared with the TGF-β1 group,the expressions of FN and COL I were decreased when pretreated with the different doses of exogenous of CCN3 or transfected with pcDNA3.1(+)-CCN3 (all P ＜ 0.05).Meanwhile,the expression of miRNA-29a was significantly increased when pretreated with 50 μg/L and 500 μg/L CCN3 or transfected with pcDNA3.1(+)-CCN3 (all P ＜ 0.05);whereas miRNA-29b and c had no statistical difference (all P ＞ 0.05).(3) Compared with TGF-β1+CCN3 group,the expressions of FN and COL I were decreased in CCN3+TGF-β1+miRNA-29a mimics group (all P ＜ 0.05),whereas the expressions of FN and COL I in CCN3+TGF-β1+miRNA-29a inhibitors group were increased (all P ＜ 0.05).Conclusions CCN3 reduces the TGF-β1-induced production of ECM by the up-regulation of miRNA-29a.

Effects of rAd-p53 Injection on the Proliferation,Apoptosis and Autophagy of Nephroblastoma Cells / 中国药房

OBJECTIVE:To study the effects of rAd-p53 injection on the proliferation,apoptosis and autophagy of nephroblas-toma cells. METHODS:Nephroblastoma cells were respectively cultured 20 h with high,medium,low concentration(1×109,1× 108,1×107 VP/mL)of Recombinant human rAd-p53 injection,cells with no injection were the blank control. Cell proliferation,cy-cle,apoptosis and related gene p21,Bax protein expressions were detected,and autophagy gene LC-3,Atg7,Atg12 expressions and number of autophagosomes were also detected. RESULTS:The proliferation inhibition rates of high,medium,low concentra-tion of Recombinant human rAd-p53 injection to nephroblastoma cells were(42.86±3.18)%,(33.64±7.25)%,(16.26±9.07)%;apoptotic rates were (53.85 ± 9.36)%,(37.35 ± 9.64)%,(23.64 ± 10.65)%,respectively. Compared with blank control,cells at period G0/G1 were increased under high,medium,low concentration Recombinant human rAd-p53 injection,effects were relatively obvious under high,medium concentration (P0.05). CONCLUSIONS:Recom-binant human rAd-p53 injection can inhibit the cell proliferation of nephroblastoma,and induce its apoptosis and autophagy.

Clinical significance of expression of RGS4 in pediatric nephroblastoma tissue / 天津医药

Objective To investigate the expression levels of regulator of G-protein signaling 4 (RGS4) in pediatric nephroblastoma and pericancerous tissues, and explore the relationship between RGS4 and the occurrence and development of pediatric nephroblastoma. Methods Thirty-seven samples of pediatric nephroblastoma tissues and 8 samples of pericancerous tissues were collected after surgery to detect the expression of RGS4 protein by immunohistochemistry. Another 8 samples of fresh cancer tissues and corresponding pericancerous tissues were collected to detect the mRNA and protein levels of RGS4 by qRT-PCR and Western blot assay, respectively. Results Immunohistochemistry results showed that RGS4 protein was positively expressed both in pediatric nephroblastoma and pericancerous tissues, and its high expression rate was lower in pediatric nephroblastoma than that in pericancerous tissues [(37.83%(14/37) vs. 87.5%(7/8),χ2=4.675, P<0.05]. The expression level of RGS4 mRNA was significantly lower in pediatric nephroblastoma than that in pericancerous tissues (1.064 ± 0.549 vs. 5.374 ± 0.735, t=13.290, n=8, P < 0.01). Western blot results showed that the expression level of RGS4 protein was lower in pediatric nephroblastoma than that of pericancerous tissues (0.301±0.092 vs. 0.779 ± 0.041, t=13.424, n=8, P < 0.01). Conclusion The expression level of RGS4 is down-regulated in pediatric nephroblastoma, which may be related to the occurrence and development of pediatric nephroblastoma.

Molecular typing markers of wilms tumor in children: A research progress / 第二军医大学学报

Wilms tumor (nephroblastoma) is the most common malignant renal tumors of childhood. With the development of various medical skills, the survival rate has been greatly improved for Wilms tumor children. Researches have shown that patients with Wilms tumor have different outcomes due to diverse histological classification. Deepening the understanding of tumor molecular typing may help to understand the biological characteristics of tumor better and distinguish different tumors, contributing to treatment and prognosis prediction of children with Wilms tumor. In this review, we summarized the progress of molecular typing markers in pediatric Wilms tumor.

Wilms tumor in 3 years old female patient – A case report

Nephroblastoma or Wilms' tumor is an embryonic tumor derived from primitive renal epithelial and mesenchymal components. It is the most common abdominal malignant tumour of young children. Overall, Wilms' tumor incidence is 7.8 cases per million children. Peak age of incidence is 2 to 3 years of age, or 99% occurring less than six years of age. Here, we are presenting a case of 3 years old female patient with diagnosis of Nephroblastoma.

Aumento renal bilateral como manifestação de leucemia linfocítica aguda / Bilateral renal increase as a manifestation of acute lymphocytic leukemia

As leucemias são o tipo mais frequente de câncer em crianças e adolescentes. A leucemia linfocítica aguda é a forma mais comum de leucemia na infância. A doença surge normalmente entre dois e quatro anos de idade, sendo incomum antes de um ano de vida. As manifestações mais comuns incluem febre, fadiga, letargia, dor óssea e articular. Em 50% dos casos, há hepato e/ou esplenomegalia e linfonodomegalias. Este relato de caso fala sobre uma menina de nove meses que foi levada à emergência do hospital por distensão abdominal, fraqueza em membros inferiores e constipação duas semanas antes. Apresentava massas abdominais endurecidas em flancos, abaulamento em região fronto-temporal direita e linfonodomegalias cervicais. A primeira impressão foi de nefroblastoma bilateral (Tumor de Wilms). A ecografia abdominal e a tTC de abdome mostraram aumento de volume renal bilateral e descartaram nefroblastoma. Em setor de oncologia pediátrica, foi realizada imunofenotipagem compatível com leucemia/linfoma linfoblástico B. No líquor havia 400 células p/uL com 81% de blastos. A avaliação neurológica e a RNM de crânio e neuro-eixo descartaram alteração que justificasse a paresia de membros inferiores. No décimo dia de tratamento, os rins já haviam reduzido até o limite superior da normalidade. O abaulamento da face já havia desaparecido. A paciente apresentou anemia, neutropenia, plaquetopenia e hipoalbuminemia severas. Ao final da indução, apresentou sepse fúngica e bacteriana evoluindo para choque séptico e parada cardiorrespiratória não responsiva às manobras de reanimação. Não foi realizado medulograma no final da indução por piora do quadro clínico e posterior óbito (AU)

Leukemias are the most frequent type of cancer in children and adolescents. Acute lymphocytic leukemia is the most common form of childhood leukemia. The disease usually arises between two and four years of age, being uncommon before one year of life. The most common manifestations include fever, fatigue, lethargy, bone and joint pain. In 50% of cases, there is hepatomegaly and/or splenomegaly and lymph node enlargement. This case report tells of a nine-month old girl who was taken to the hospital emergency room due to abdominal distension, weakness in the lower limbs, and constipation for two weeks. She had abdominal masses that were hardened on the flanks, bulging in the right fronto-temporal region and cervical lymph node enlargements. The first impression was bilateral nephroblastoma (Wilms tumor). Abdominal ultrasound and abdominal CT showed bilateral renal volume increase and ruled out nephroblastoma. In the pediatric oncology sector, immunophenotyping compatible with lymphoblastic leukemia/lymphoma was performed. In the CSF there were 400 cells/L with 81% blasts. Neurological evaluation and MRI of the skull and neuro-axis ruled out alterations justifying lower limb paresis. By the tenth day of treatment, the kidneys had already reduced to the upper limit of normal. The bulging of the face was gone. The patient presented severe anemia, neutropenia, thrombocytopenia and hypoalbuminemia. At the end of the induction, she presented fungal and bacterial sepsis evolving to septic shock and cardiorespiratory arrest unresponsive to resuscitation maneuvers. No myelogram was performed at the end of the induction due to worsening of the clinical picture and subsequent death (AU)

Identification of differential inflammation factors in nephroblastoma tissue and clinical significance / 中华泌尿外科杂志

Objective To identify the differential inflammation factors in nephroblastoma tissue using proteomics technology and analyze its relationship with clinical stage,pathological phenotype,lymph node metastasis,vascular invasion.Methods From Jan 2010 to Dec 2014,nephroblastoma tumor tissues from 40 patients were obtained.Meanwhile,the 35 tissue near proximal kidney and 25 tissues distal kidney were also obtained.The classification of clinical stage included Ⅰ stage in 6 cases,Ⅱ stage in 12 cases,Ⅲ stage in 13 cases and Ⅳ stage in 9 cases.Other characters contained good prognosis type in 37 case,poor prognosis type in 3 cases,lymphatic metastasis in 17 cases,no sign of lymphatic metastasis in 23 cases,vascular invasion in 9 cases and non-vascular invasion in 31 cases.The SELDI-TOF-MS was used for screening differential protein peaks among three groups.Then,SPE and TRICINE-SDS-PAGE were used to separate and purificate the protein,which showed high peaks expression in tumor tissue,respectively.After in-gel digestion,we received the identification of targeted proteins according to sequence information through Nano-LC-MS/MS.Finally we compared differential expression of inflammatory peaks in different groups of clinical stage,pathological type,lymph node metastasis and vascular invasion.Results All the peaks high expression in tumor tissue,m/z12138 and m/z 13462 are identified as MIF and NAP-2.Expression of two protein peaks in tumor tissue(1437.8 + 997.3,1730.4 + 1147.8) is higher than those in proximal tissue (952.6 + 591.2,1031.1 + 1120.8) and in distal tissue(315.4 + 296.5,114.7 + 118.9),which showed the significant difference (P ＜ 0.001).According to the clinic stage classification,the expression of those protein were 678.8 + 189.0,746.2 + 238.7 in stage Ⅰ,664.0 + 202.0,1180.7 + 404.9 in stage Ⅱ,1524.7+407.9,2160.4 + 1252.3 in stage Ⅲ and 2850.2 + 861.2,2498.4 + 1290.5 in stage Ⅳ.Based on the other characters,expression of those protein were the 1271.7 + 809.2,1553.3 + 991.4 in good prognosis type,3487.2 + 166.2,3915.1 +507.3 in poor prognosis type,2207.1 +961.7,2569.5 + 1285.2 in lymph node metastasis,869.2 + 474.6,1110.2 + 433.6 in non-lymph node metastasis,2850.2 + 861.2,2498.4 +1290.5 in vascular invasion and 1027.8 + 521.3,1507.5 + 1019.9 in non-vascular invasion.All the comparison results have significant statistical difference (P ＜ 0.001).Conclusion MIF and NAP-2significantly increase in nephroblastoma tumor tissue.Meanwhile,there was obvious relationship between those protein with clinical stage,pathological type,lymph node metastasis and vascular invasion.

The research progress of gene mutations in children with Wilms tumor / 国际儿科学杂志

Nephroblastoma is the most common pediatric renal tumor.The research progress of gene mutations in Wilms tumor children is reviewed in this paper.The WT1 gene,CTNNB1 gene and WTX gene plays a role in onset of Wilms tumor,and the MYCN gene,P53 gene and SIX1 gene may be associated with the prognosis.

Fetal rhabdomyomatous nephroblastoma: diagnosis and therapy / 中华泌尿外科杂志

Objective To explore the characteristic clinical profiles and treatment modalities of Fetal rhabdomyomatous nephroblastoma(FRN).Methods A retrospective study was conducted for 14 FRN patients from Jan.2000 to Oct.2015.Their clinical data were collected including clinical presentations,pathology and treatment modalities.There were 8 males and 6 females with a mean age of 23 months.There were 3 cases at left side and right side 5 cases,bilateral 6 cases.2 patients were classified as stage Ⅰ,1 stage Ⅱ,5 stageⅢ and 6 stageⅤ.Abdominal mass was the main clinical presentation in 11 patients,and 1 case with hematuria,1 with abdominal pain,and 1 with vomit.Most tumors showed cysts or completely solid from the ultrasonography.Computed tomographic scan revealed a large inhomogeneous enhancement tumor from the kidney pole with necrotic,cystic,bleeding or calcification.Ultrasonography and Computed tomography (CT) had no different performance from Wilms' tumor.9 patients received preoperative chemotherapy,and the response was none in all of them.8 unilateral patients underwent tumor nephrectomy and another 4 had nephron-sparing surgery.Results Pathology showed that FRN contained more than 70％ of fetal rhabdomyomatous tissue.Immunohistochemistry had no specificity,most FRN shows Desmin (+) and Myogenin(+).Bilateral FRN tumors were seen in 2,one side with FRN and another side with nephroblastomatosis were seen in 3,one side with FRN and another side with Wilm's tumor was seen in 1 patient.Postoperative pathology confirmed FRN in all 14 cases.All patients received postoperative chemotheraphy:Act-D and VCR for 6 month(stage Ⅰ),Act-D and VCR for 15 month(stage Ⅱ),Act-D +VCR + ADR and radiotherapy for 15 month(stageⅢ).During follow-up of 6 months to 15 years,10 of them were alive without tumor and no evidence of recurrence.Conclusions FRN is a rare histologic variant of Wilm's tumor with less aggressive behavior.FRN usually has a huge volume and is bilateral with a poor responder to preoperative chemotherapy,but it is associated with a generally favorable outcome.Surgery and chemically treatment appears the effective measure.

Detection and identification of non-stress protein biomarkers for nephroblastoma / 中华实用儿科临床杂志

Objective To test the differential proteomics by using proteomic technology of children diagnosed with nephroblastoma and healthy children,thereby for screening non-stress-related protein markers of Wilms' tumor.Methods The serum of children with Wilms' tumor,children with trauma in 1-3 hours and healthy children were collected in the First Affiliated Hospital of Zhengzhou University from May 2010 to May 2014.Then,the differential proteomics were screened and the interference of traumatic stress proteins in the process were eliminated by using proteomic technology of surfaced enhanced laser desorption/ionization time of flight mass spectroscopy (SELDI-TOF-MS),matrix-assisted laser desorption/ionization time-of-flight mass spectroscopy (MALDI-TOF-MS),high performance liquid chromatography(HPLC),and two dimensions-liquid chromatography-linear trap quadrupole-mass spectrometer(2D-LC-LTQ-MS),therefore the non-stress-related protein biomarkers of Wilms' tumor were determined.Results A mass-to-charge ratio 6630.58 Da protein or peptide was selected as tumor-specific marker.And there were no same or similar proteins in mass spectrometry of children with traumatic stress by SELDI-TOF-MS.Finally,by purification through HPLC and identification through MALDI-TOF-MS and 2D-LC-LTQ-MS,apolipoprotein CI(APO CI) was found to be the non-stress-related serum protein of Wilms' tumor.Conclusions It is identified that APO CI is an important serum protein biomarker of Wilms' tumor by using proteomic technology in eliminating the influences of interference factors of stress.Therefore,the results provide possibilities of further studies and investigating the mechanisms of the protein expression changes and early diagnosing the Wilms' tumor.

Study on the influence of curcumin on chemosensitivity of nephroblastoma cells

OBJECTIVE@#To study the influence of curcumin on chemosensitivity of nephroblastoma cells.@*METHODS@#Human nephroblastoma cells line SK-NEP-1 was transplanted to the nude mice subcutaneously to establish the implantation tumor model of human nephroblastoma cells. A total of 30 tumor-bearing mice were divided into three groups of ten randomly. The routine chemotherapy group was given vincristine (0.05 mg/mL·0.2 mL/d) and actinomycin D (15 ng/mL·0.2 mL/d) combined chemotherapy regime. The curcumin chemotherapy group was given the same combined chemotherapy regimens and curcumin (30 mg/kg/d) by intraperitoneal injection. The control group was given normal saline (NS) of the same volume by intraperitoneal injection. Continuous administration would be kept for 4 weeks and 3 days a week. The volumetric changes of every group were recorded. The serum of every group in different time was collected and the VEGF content was detected by ELISA. All mice were cercrificed and the tumor tissues were stripped and weighed after 4 weeks' treatment. The tumor inhibition rate was calculated. The cell proliferation activity and apoptosis rate were detected by MTT and flow cytometry method. All data were statistically analyzed by SPSS 19.0.@*RESULTS@#The tumor volume, serum VEGF content, tumor inhibition rate, cell proliferation activity and apoptosis rate of routine chemotherapy group and curcumin chemotherapy group had significant differences comparing with the control group (P < 0.05) after 4-week's treatment. The cancer growth of curcumin chemotherapy group was obviously decreased and even tended to shrink comparing with routine chemotherapy group (χ(2) = 15.732, P = 0.007). The cell proliferation activity was significantly reduced and the apoptosis rate was significantly higher, (χ(2) = 9.427, P = 0.012) which showing the effect of chemotherapy was enhanced.@*CONCLUSIONS@#The chemosensitivity of nephroblastoma cells could be improved by curcumin, then the effect of preoperative adjuvant chemotherapy scheme would be enhanced, the growth of nephroblastoma cells would be inhibited and the surgical risk of nephroblastoma would be reduced.

Study on the influence of curcumin on chemosensitivity of nephroblastoma cells

Objective To study the influence of curcumin on chemosensitivity of nephroblastoma cells. Methods Human nephroblastoma cells line SK-NEP-1 was transplanted to the nude mice subcutaneously to establish the implantation tumor model of human nephroblastoma cells. A total of 30 tumor-bearing mice were divided into three groups of ten randomly. The routine chemotherapy group was given vincristine (0.05 mg/mL·0.2 mL/d) and actinomycin D (15 ng/mL·0.2 mL/d) combined chemotherapy regime. The curcumin chemotherapy group was given the same combined chemotherapy regimens and curcumin (30 mg/kg/d) by intraperitoneal injection. The control group was given normal saline (NS) of the same volume by intraperitoneal injection. Continuous administration would be kept for 4 weeks and 3 days a week. The volumetric changes of every group were recorded. The serum of every group in different time was collected and the VEGF content was detected by ELISA. All mice were cercrificed and the tumor tissues were stripped and weighed after 4 week's treatment. The tumor inhibition rate was calculated. The cell proliferation activity and apoptosis rate were detected by MTT and flow cytometry method. All data were statistically analyzed by SPSS 19.0. Results The tumor volume, serum VEGF content, tumor inhibition rate, cell proliferation activity and apoptosis rate of routine chemotherapy group and curcumin chemotherapy group had significant differences comparing with the control group (P < 0.05) after 4-week's treatment. The cancer growth of curcumin chemotherapy group was obviously decreased and even tended to shrink comparing with routine chemotherapy group (χ