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1.
J Cell Mol Med ; 25(7): 3524-3536, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33683826

RESUMO

It has been becoming increasingly evident that long non-coding RNAs (lncRNAs) play important roles in various human cancers. However, the biological processes and clinical significance of most lncRNAs in hepatoblastoma (HB) remain unclear. In our previous study, genome-wide analysis with a lncRNA microarray found that lncRNA HOXA-AS2 was up-regulated in HB. Stable transfected cell lines with HOXA-AS2 knockdown or overexpression were constructed in HepG2 and Huh6 cells, respectively. Our data revealed knockdown of HOXA-AS2 increased cell apoptosis and inhibited cell proliferation, migration and invasion in HB. Up-regulation of HOXA-AS2 promoted HB malignant biological behaviours. Mechanistic investigations indicated that HOXA-AS2 was modulated by chromatin remodelling factor ARID1B and transcription co-activator SUB1, thereby protecting HOXA3 from degradation. Therefore, HOXA-AS2 positively regulates HOXA3, which might partly demonstrate the involvement of HOXA3 in HOXA-AS2-mediated HB carcinogenesis. In conclusion, HOXA-AS2 is significantly overexpressed in HB and the ARID1B/HOXA-AS2/HOXA3 axis plays a critical role in HB tumorigenesis and development. These results might provide a potential new target for HB diagnosis and therapy.

2.
Cancer Biomark ; 2021 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-33780361

RESUMO

BACKGROUND: Hepatoblastoma (HB) is an embryonic solid tumor and the most common primary malignant liver tumor in children. HB usually occurs in infants and children. Although treatment diversity is increasing, some patients still have very poor prognosis. Many studies have investigated USP7 inhibitors for tumors. Using database information, we found that USP7 is highly expressed in HB. METHODS: Lentivirus-mediated USP7 knockdown and overexpression was performed in HB cell lines HepG2 and Huh6. CCK8 and transwell assays were used to determine cell viability and metastasis. Flow cytometry was used to study cell cycle and apoptosis. Levels of proteins were detected using western blots. RESULTS: Downregulation of USP7 resulted in significant decrease in cell proliferation, clonal formation, and cell migration and invasion. With overexpression of USP7, cellular malignant behavior increased. Cell cycle assays showed that USP7 knockdown inhibited G1 to S phase transition in the cell cycle. Upregulation of USP7 promoted the transition. Animal experiments showed USP7 facilitated tumor growth in vivo. Western blots indicated that USP7 may affect HB tumorigenesis through the PI3K/AKT signaling pathway. Furthermore, USP7 inhibitor P5091 inhibited HB development and PI3K/AKT pathway. CONCLUSION: USP7 upregulation contributed to HB genesis and development through the PI3K/AKT signaling pathway. USP7 could be a potential target for future HB treatment.

3.
Discov Med ; 30(160): 63-70, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33382962

RESUMO

Neuroblastoma (NB) is the only pediatric tumor that is screened for nationwide by detecting the urinary levels of homovanillic acid and/or vanillylmandelic acid; however, whether NB screening reduces the mortality rate has not been established. This review compared the incidence and mortality rates among data from international mass screening for NB, as well as an analysis of differences in age of screening, detection methods, and diagnostic biomarkers. A well-designed trial exploring possible benefits and hazards is warranted prior to resuming mass screening for NB.

4.
Discov Med ; 30(160): 71-82, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33382963

RESUMO

Neuroblastoma (NB) is the most common extracranial solid tumor in children. Despite a variety of treatments, patients with advanced stage disease still have a poor prognosis. The molecular mechanisms underlying NB pathogenesis are not fully known. Increasing evidence shows that long noncoding RNA (lncRNA) is important in multiple ways in NB progression. LncRNA could act as competitive endogenous RNA, serve as a scaffold for proteins, or participate in histone modification, thus affecting proliferation, migration, invasion, and differentiation of NB. Numerous lncRNAs polymorphisms are significantly associated with NB susceptibility. Differently expressed lncRNAs can be used to construct risk scores to evaluate patient outcomes. In conclusion, these lncRNAs have the potential to be diagnostic markers as well as promising therapeutic targets in the future.

5.
Mol Ther Oncolytics ; 19: 149-162, 2020 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-33209975

RESUMO

A previous study on hepatoblastoma revealed novel mutations and cancer genes in the Wnt pathway and ubiquitin ligase complex, including the tumor suppressor speckle-type BTB/POZ (SPOP). Moreover, the SPOP gene affected cell growth, and its S119N mutation was identified as a loss-of-function mutation in hepatoblastoma. This study aimed to explore more functions and the potential mechanism of SPOP and its S119N mutation. The in vitro effects of SPOP on cell proliferation, invasion, apoptosis, and in vivo tumor growth were investigated by western blot analysis, Cell Counting Kit-8, colony formation assay, flow cytometry, and xenograft animal experiments. The substrate of SPOP was discovered by a protein quantification assay and quantitative ubiquitination modification assay. The present study further proved that SPOP functioned as an anti-oncogene through the phosphatidylinositol 3-kinase/Akt signaling pathway to affect various malignant biological behaviors of hepatoblastoma both in vitro and in vivo. Furthermore, experimental results also suggested that solute carrier family 7 member 1 (SLC7A1) might be a substrate of SPOP and influence cell phenotype by regulating arginine metabolism. In conclusion, these findings demonstrated the function of SPOP and revealed a potential substrate related to hepatoblastoma tumorigenesis, which might thus provide a novel therapeutic target for hepatoblastoma.

6.
Int J Biol Sci ; 16(15): 3050-3061, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33061817

RESUMO

Our previous studies demonstrated that MEG3 was significantly downregulated in neuroblastoma (NB) and its expression was negatively associated with the INSS stage. Overexpression of MEG3 promoted apoptosis and inhibited proliferation in NB cells. In this study, we discovered more potential functions and molecular mechanisms of MEG3 in NB. According to the database, MEG3 positively correlated with the NB survival rate and was negatively associated with malignant clinical features. Moreover, we determined that MEG3 was mainly located in the nucleus by nuclear-cytoplasmic separation and RNA fish assays. Upregulation of MEG3 in stably transfected cell lines was accomplished, and CCK8, colony formation, and EDU assays were performed, which indicated that MEG3 significantly suppressed cell proliferation. Both wound healing and transwell experiments demonstrated that MEG3 decreased cell migration and invasion. CHIRP enrichments showed the anticancer effects of MEG3 were probably linked to autophagy and the mTOR signaling pathway. LC3 fluorescence dots and western blots showed that MEG3 attenuated autophagy by inhibiting FOXO1, but not the mTOR signaling pathway. Furthermore, MEG3 inhibited metastasis through epithelial-mesenchymal transition via the mTOR signaling pathway. Consistent with the above results, downregulation of MEG3 facilitated NB malignant phenotypes. Mechanistically, MEG3 and EZH2 regulated each other via a negative feedback loop and promoted NB progression together. In conclusion, our findings suggested that MEG3 was a tumor suppressor in NB and could be a potential target for NB treatment in the future.

7.
Cancer Cell Int ; 20: 506, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33088214

RESUMO

Increasing evidence has indicated that long noncoding RNAs (lncRNAs) play various important roles in the development of cancers. The widespread applications of ribosome profiling and ribosome nascent chain complex sequencing revealed that some short open reading frames of lncRNAs have micropeptide-coding potential. The resulting micropeptides have been shown to participate in N6-methyladenosine modification, tumor angiogenesis, cancer metabolism, and signal transduction. This review summarizes current information regarding the reported roles of lncRNA-encoded micropeptides in cancer, and explores the potential clinical value of these micropeptides in the development of anti-cancer drugs and prognostic tumor biomarkers.

8.
Eur J Med Genet ; 63(11): 104047, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32891756

RESUMO

BACKGROUND: WT1 mutations cause a wide spectrum of renal and extrarenal manifestations concerning urogenital development and the development of tumors. METHODS: We retrospectively collected the information on the genotype and phenotype of WT1 nephropathy from the multicenter registry since 2014 to 2019. All patients were stratified by renal function decline status or by sequence timing. Rapid progressive group was defined as rapidly developing into ERSD within 12 months since disease onset. Early sequencing group was defined as gene mutation identified before ERSD. RESULTS: Thirty-three (3.5%) cases were identified with a WT1 mutation in patients with steroid resistant nephrotic syndrome (SRNS), proteinuria and chronic kidney disease (CKD) 3-5 stage of unknown origin. ESRD developed in twenty patients at a median age of 4.3 years old. Comparing study between the rapid progressive group (n = 8) and non-rapid progressive group (n = 25) showed no significant difference in age of onset, gender, syndrome phenotype, genotype and proteinuria except for initial estimated glomerular filtration rate (eGFR) (p = 0.021) or sequencing timing (p = 0.003). In multivariable logistic regression analysis, the delayed sequencing was associated with rapid renal function decline, even after adjusting for established clinical factors including syndromic phenotype, genotype, age onset and eGFR at initial stage (p = 0.019). The renal survival analysis did not show a significantly better outcome in early sequencing group than in delayed sequencing group (p > 0.05). CONCLUSION: Screening for WT1 mutations should be performed in children with Wilms' tumor, proteinuria/SRNS or CKD. Early diagnosis of WT1 nephropathy through clinical and genetic findings is warranted.

9.
Cancer Cell ; 38(5): 716-733.e6, 2020 Nov 09.
Artigo em Inglês | MEDLINE | ID: mdl-32946775

RESUMO

Neuroblastoma (NB), which is a subtype of neural-crest-derived malignancy, is the most common extracranial solid tumor occurring in childhood. Despite extensive research, the underlying developmental origin of NB remains unclear. Using single-cell RNA sequencing, we generate transcriptomes of adrenal NB from 160,910 cells of 16 patients and transcriptomes of putative developmental cells of origin of NB from 12,103 cells of early human embryos and fetal adrenal glands at relatively late development stages. We find that most adrenal NB tumor cells transcriptionally mirror noradrenergic chromaffin cells. Malignant states also recapitulate the proliferation/differentiation status of chromaffin cells in the process of normal development. Our findings provide insight into developmental trajectories and cellular states underlying human initiation and progression of NB.

10.
Oncol Lett ; 20(4): 1, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32774475

RESUMO

Neuroblastoma (NB) is the most common type of extracranial solid tumor found in children. Despite several treatment options, patients with advanced stage disease have a poor prognosis. Previous studies have reported that enhancer of zeste homolog 2 (EZH2) and long non-coding RNAs (lncRNAs) have abnormal expression levels in NB and participate in tumorigenesis and NB development. However, the association between EZH2 and lncRNAs remain unclear. In the present study, RNA immunoprecipitation-sequencing (RIP-seq) was used to analyze the lncRNAs binding to EZH2. Following EZH2 knockdown via short hairpin RNA, RNA-seq was performed in shEZH2 and control groups in SH-SY5Y cells. Chromatin IP (ChIP)-seq was used to determine the genes that may be regulated by EZH2. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes analyses were performed to identify the signaling pathways involved in NB. The results from RIP-seq identified 94 lncRNAs, including SNHG7, SNHG22, KTN-AS1 and Linc00843. Furthermore, results from RNA-seq demonstrated that, following EZH2 knockdown, 448 genes were up- and 571 genes were downregulated, with 32 lncRNAs up- and 35 downregulated and differentially expressed compared with control groups. Certain lncRNAs, including MALAT1, H19, Linc01021 and SNHG5, were differentially expressed in EZH2-knockdown group compared with the control group. ChIP-seq identified EZH2 located in the promoter region of 138 lncRNAs including CASC16, CASC15, LINC00694 and TBX5-AS1. In summary, the present study demonstrated that certain lncRNAs directly bound EZH2 and regulated EZH2 expression levels. A number of these lncRNAs that are associated with EZH2 may participate in NB tumorigenesis.

11.
Pediatr Blood Cancer ; 67(10): e28311, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32729220

RESUMO

As a sympathetic nervous system-derived tumor, aggressive neuroblastoma (NB) is currently attracting interest from researchers seeking diagnostic and prognostic markers via less invasive procedures. The analysis of circulating tumor DNA (ctDNA) in peripheral blood can provide genetic information from multiple tumor lesions and is not dependent on a surgical procedure. The identification of genetic alterations, chromosomal variations, and hypermethylation contained within plasma DNA yields clinical value in the diagnosis, risk stratification, monitoring of treatment effects, and survival prediction for patients. With the widespread application of genome sequencing, droplet digital polymerase chain reaction, and other advanced technologies, the detection of ctDNA may guide therapeutic schedules, enhance the quality of life, and improve the prognosis for patients with NB.

12.
Pediatr Dermatol ; 37(4): 677-680, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32212177

RESUMO

We present a retrospective case series of 3 patients with retroperitoneal kaposiform hemangioendothelioma (KHE) complicated by Kasabach-Merritt phenomenon (KMP) and biliary obstruction. We found sirolimus to be a safe and effective treatment for these patients who were refractory to other treatment modalities. However, our patients were slow to respond in comparison to published reports of sirolimus use for KHE without biliary obstruction. We postulate that therapeutic serum levels of sirolimus may be affected by biliary obstruction and improved with surgical alleviation of the obstruction.

13.
BMC Gastroenterol ; 20(1): 8, 2020 Jan 13.
Artigo em Inglês | MEDLINE | ID: mdl-31931724

RESUMO

BACKGROUND: Interleukin-10 (IL10) signalling pathway deficiency results in severe very early onset inflammatory bowel disease (VEOIBD), and enterostomy is often inevitable. However, studies in these surgical populations are lacking. This study aims to determine the enterostomy characteristics, postoperative complications and related risk factors in enterostomy patients. METHODS: From March 1, 2015, to December 31, 2018, patients with IL10R-mutation who underwent enterostomy were recruited for analysis. We collected data on the patients' clinical characteristics, enterostomy characteristics, postoperative complications and related risk factors. RESULTS: Twelve patients required emergency enterostomy, and 10 patients underwent elective enterostomy. Twelve patients experienced postoperative complications, including wound infection (27.3%), wound dehiscence (18.2%), reoperation (18.2%), etc. Compared with the pre-enterostomy values, there was a decrease in C-reactive protein (CRP) (P = 0.001), an increase in albumin (P = 0.001) and an improvement in the weight-for-age (P = 0.029) and body mass index (BMI) Z-scores (P = 0.004) after enterostomy. There was a significant difference between the pre-operation and postoperation medicine expenses (P = 0.002). Univariate binary logistic regression analysis revealed a statistically significant influence of CRP (OR: 1.43, 95% CI: 1.07-1.91, P = 0.016) and a tendency towards a significant influence of intestinal perforation, albumin level, BMI Z-score and weighted paediatric Crohn's disease activity index (wPCDAI). Multivariate logistic regression analysis showed that CRP (OR: 1.40), wPCDAI (OR: 2.88) and perforation (OR: 1.72) showed a tendency to behave as independent risk factors for postoperative complications, but the results were not significant (all P > 0.05). CONCLUSIONS: Surgery and enterostomy showed benefits for VEOIBD with IL-10 signalling deficiency. The timing of intervention, potential postoperative complications, economic burden and other related problems should be considered.


Assuntos
Enterostomia/efeitos adversos , Doenças Inflamatórias Intestinais/cirurgia , Complicações Pós-Operatórias/genética , Receptores de Interleucina-10/deficiência , Transdução de Sinais/genética , Adolescente , Adulto , Idade de Início , Criança , Pré-Escolar , Efeitos Psicossociais da Doença , Enterostomia/economia , Feminino , Humanos , Doenças Inflamatórias Intestinais/economia , Doenças Inflamatórias Intestinais/genética , Interleucina-10/genética , Modelos Logísticos , Masculino , Mutação , Complicações Pós-Operatórias/economia , Período Pós-Operatório , Período Pré-Operatório , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
14.
J Pediatr Surg ; 54(12): 2559-2564, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31668401

RESUMO

PURPOSE: Cholangitis after Kasai procedure has been previously shown to be related to poor prognosis in Biliary Atresia (BA). To investigate the risk factors and clinical outcomes of cholangitis, we did a retrospective study in post-Kasai BA patients. METHODS: Two-year follow-up data of 180 type-III BA patients after Kasai procedure in 2016 in our hospital were analyzed, including 119 cholangitis patients (66.11%). Among the cholangitis group, patients were further divided into early vs late cholangitis and single vs recurrent cholangitis groups. Liver pathology, liver function, cholangitis occurrence and frequency, jaundice clearance, and survival rates were examined. RESULTS: Higher gamma-glutamyl transferase level before Kasai is a risk factor for cholangitis (p = 0.0393). Older age and higher liver fibrosis score at Kasai are risk factors for recurrent cholangitis (p < 0.05). Shorter prophylactic intravenous antibiotics usage may contribute to early cholangitis, which may lead to higher cholangitis frequency (p < 0.0001). Recurrent cholangitis is associated with earlier cholangitis onsets (p < 0.0001). Cholangitis patients have a relatively delayed jaundice clearance, while early and recurrent cholangitis may contribute to lower overall survival. CONCLUSIONS: Personalized treatment considering risk factors in individual BA patients is needed to prevent cholangitis, especially early onsets, and to improve postoperative outcomes. LEVEL OF EVIDENCE: III.


Assuntos
Atresia Biliar/cirurgia , Colangite/etiologia , Portoenterostomia Hepática/efeitos adversos , Complicações Pós-Operatórias/etiologia , Fatores Etários , Antibacterianos/administração & dosagem , Antibioticoprofilaxia , Atresia Biliar/sangue , Atresia Biliar/complicações , Estudos de Casos e Controles , Seguimentos , Humanos , Lactente , Icterícia/etiologia , Cirrose Hepática/complicações , Período Pré-Operatório , Prognóstico , Recidiva , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Fatores de Tempo , gama-Glutamiltransferase/sangue
15.
Oncol Rep ; 2019 Aug 02.
Artigo em Inglês | MEDLINE | ID: mdl-31524279

RESUMO

Previous research from our group revealed that the long coding RNA (lncRNA) linc01105 is associated with neuroblastoma proliferation and apoptosis, and that its expression is correlated with the International Neuroblastoma Staging System stage. The purpose of the present study was to investigate the functions of Linc01105 in neuroblastoma. Lentivirus­mediated linc01105 knockdown was performed in the neuroblastoma cell line SH­SY5Y. The expression levels of linc01105 and of other associated genes were measured by reverse transcription­quantitative PCR. Cell Counting Kit­8 assay and flow cytometry were used to determine cell viability and apoptosis. The levels of proteins were detected using western blot analysis. Bioinformatics analysis and luciferase reporter assays were used to examine the relationship between linc01105, miR­6769b­5p and vascular endothelial growth factor A (VEGFA). Angiogenesis ability was measured using a tube formation assay. The results demonstrated that HIF­1α overexpression promoted the transcription of linc01105 by acting as a transcription factor. Knockdown of linc01105 inhibited neuroblastoma cell proliferation, migration and invasion, and it induced apoptosis. In addition, linc01105 affected the expression of p53 and Bcl­2 family proteins and activated the caspase signaling pathway. Further functional experiments revealed that linc01105 promoted the expression of the miR­6769b­5p target gene VEGFA by acting as a sponge of miR­6769b­5p. In conclusion, linc01105 may contribute to neuroblastoma tumorigenesis and development. The present findings indicated that the interplay between the p53/caspase pathway and the linc01105/miR­6769b­5p/VEGFA axis may have important roles in the development of neuroblastoma.

16.
J Dermatol ; 46(11): 956-961, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31489702

RESUMO

Mammalian target of rapamycin inhibitors have shown promising results in the management of kaposiform hemangioendothelioma (KHE). The purpose of this study was to present our experience involving sirolimus therapy for KHE. A retrospective study was conducted to review the medical documents of 26 patients with KHE who were treated with sirolimus at our hospital between March 2012 and December 2016. Fifteen males and 11 females manifested KHE in infancy with an average age of 2.9 ± 1.8 months. Multiple anatomical sites were involved. Four patients had multifocal lesions, while 22 patients had solitary lesions. Twenty-five patients had Kasabach-Merritt phenomenon (KMP). Twenty patients completed sirolimus therapy in 28.3 ± 12.5 months. Nineteen KHE lesions reduced to small residuals with platelet counts reaching normal levels 3.7 ± 2.8 weeks after treatment; one KHE lesion had no response to therapy. One patient with multifocal lesions died due to a severe infection, although the patient had previously responded to sirolimus. Five patients remained in treatment and had good responses with normal platelet counts. Nineteen patients with anemia had normal hemoglobin levels after 3.5 ± 1.9 weeks of treatment. Mild side effects were observed. The median follow-up time was 32 months (26-60 months), with no evidence of recurrences. Sirolimus was shown to be efficacious in the management of KHE with an average course of 28 months. The time-to-response was variable, with an average of 1 week. After 4 weeks of treatment, the platelet count and hemoglobin level had normalized. Multifocal KHE with KMP is more severe than solitary KHE.


Assuntos
Antibióticos Antineoplásicos/uso terapêutico , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/uso terapêutico , Feminino , Seguimentos , Hemangioendotelioma/patologia , Humanos , Lactente , Recém-Nascido , Síndrome de Kasabach-Merritt/patologia , Masculino , Estudos Retrospectivos , Sarcoma de Kaposi/patologia
17.
J Pediatr Surg ; 54(12): 2503-2508, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31522794

RESUMO

PURPOSE: The purpose of this study was to investigate (i) postoperative course of apple-peel atresia (APA), (ii) long-term follow-up of APA children, and (iii) risk factors for poor prognosis. METHODS: We conducted a retrospective review of 39 APA neonates treated at our institution between 2008 and 2017. Patient characteristics, operative details, postoperative course, long-term outcomes, and prognostic factors were analyzed. RESULTS: Of the 39 APA neonates, 30 (76.9%) were born preterm, and 20 (51.3%) were diagnosed prenatally. All patients underwent primary anastomosis within the first week after birth: 10 laparoscopic-assisted (25.6%) and 29 open (74.4%). Postoperative complications occurred in 28 patients (71.8%), of which 20 (71.4%) developed cholestasis. Survival at hospital discharge was 94.9%. Median parenteral nutrition period was 59 days. Reoperation was required in 7 children (17.9%) owing to anastomotic obstruction (n = 3) and adhesive intestinal obstruction (n = 4). 32 children (82.1%) were followed up for an average of 5.7 years, of which 23 children (71.9%) showed normal growth and development. APA patients with low birth weight and associated anomalies had significantly worse outcomes. CONCLUSION: Most of the patients with apple-peel atresia have excellent long-term outcomes, though initial postoperative complications are common. Low birth weight and the presence of associated anomalies are independent prognostic factors in APA. TYPE OF STUDY: Prognosis study (case series). LEVEL OF EVIDENCE: Level IV.


Assuntos
Desenvolvimento Infantil , Atresia Intestinal/cirurgia , Obstrução Intestinal/etiologia , Obstrução Intestinal/cirurgia , Anormalidades Múltiplas/diagnóstico , Anastomose Cirúrgica/efeitos adversos , Criança , Pré-Escolar , Colestase/etiologia , Feminino , Seguimentos , Humanos , Íleo/anormalidades , Lactente , Recém-Nascido , Atresia Intestinal/diagnóstico , Atresia Intestinal/terapia , Jejuno/anormalidades , Masculino , Nutrição Parenteral Total , Complicações Pós-Operatórias/etiologia , Prognóstico , Reoperação , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo
18.
J Pediatr Surg ; 54(12): 2589-2594, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31522796

RESUMO

BACKGROUND: Neuroblastoma is the most common extracranial solid tumor in childhood. In this work, the clinical value of long noncoding RNA LINC01296 was evaluated in patients with neuroblastoma. METHODS: LncRNA microarray was conducted to identify differentially expressed lncRNAs in 5 early stage and 5 advanced stage tumor tissues of neuroblastoma. RT-qPCR was carried out to validate the result of microarray. Clinical information was reviewed to analyze the relationship between lncRNA and clinical characteristics. The public database R2 was used to analyze prognosis. RESULTS: 765 differentially expressed lncRNAs were identified. LINC01296 was the most overexpressed lncRNA in advanced stage patients. RT-qPCR was conducted in 28 tumor tissues, confirming the tendency with microarray. Higher expression of LINC01296 was associated with age > 18 months (p = 0.004) and advanced stage (p = 0.021). Furthermore, LINC01296 was correlated with tumor size (r = 0.4060, p = 0.0321), LDH level (r = 0.6904, p = 0.0002), and NSE level (r = 0.5772, p = 0.0013). The neuroblastoma dataset shows patients with overexpression of LINC01296 obtained a shorter overall survival than low expression (n = 88, log-rank: P < 0.0001). CONCLUSION: LINC01296 is associated with clinical characteristics of neuroblastoma and may function as a prognostic predictor. LEVEL OF EVIDENCE: II.


Assuntos
Neuroblastoma , RNA Longo não Codificante , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Humanos , Neuroblastoma/epidemiologia , Neuroblastoma/genética , Neuroblastoma/metabolismo , Neuroblastoma/mortalidade , Prognóstico , RNA Longo não Codificante/análise , RNA Longo não Codificante/genética , RNA Longo não Codificante/metabolismo
19.
J Pediatr Surg ; 54(12): 2585-2588, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31521373

RESUMO

BACKGROUND: Stage 4S neuroblastoma is a unique category of metastatic disease in infants with a favorable outlook. The purpose of this study was to clarify the prognostic factors of patients with stage 4S neuroblastoma. METHOD: Data were retrospectively collected from infant patients with stage 4S neuroblastoma in our department from May 2000 to May 2018. Patient characteristics, operative variables, perioperative outcomes, overall survival (OS), and recurrence were evaluated. Univariate and multivariate analyses were performed to identify the prognostic factors of stage 4S neuroblastoma. RESULT: A total of 28 infant patients (71. 4% males) with a mean age of 3.7 ±â€¯2.7 months were recruited. The involved metastatic sites included liver (n = 18), skin (n = 9), and bone marrow (n = 5). Nine patients received biopsy, and 14 patients underwent original lesions resection followed by postchemotherapy. Five patients accompanied with abdominal compartment syndrome (ACS) were given experiential chemotherapy. The follow-up time ranged from 12 M to 156 M, with a mean of 32 months. Twenty-two patients completed treatment and survived. Two patients were still under treatment. Four patients died, including three with ACS. No recurrence was observed. According to Kaplan-Meier method, the 5-year overall survival was 84.4%. ACS (p = 0.001) and chemotherapy sensitivity (p < 0.001) were associated with all causes of mortality of stage 4S neuroblastoma, while neuroblastoma liver metastasis (P = 0.107), skin metastasis (P = 0.137), bone marrow metastasis (P = 0.89), tumor radical resection (P = 0.202), prenatal diagnosis (P = 0.314), and younger than 2 months of age (P = 0.683) did not emerge as prognostic factors. CONCLUSION: ACS and chemotherapy sensitivity were highly important factors that had an association with the prognosis of stage 4S neuroblastoma. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level IV.


Assuntos
Neuroblastoma , Feminino , Humanos , Lactente , Masculino , Neuroblastoma/diagnóstico , Neuroblastoma/epidemiologia , Neuroblastoma/patologia , Prognóstico , Estudos Retrospectivos
20.
J Laparoendosc Adv Surg Tech A ; 29(10): 1297-1301, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31393202

RESUMO

Background: Laparoscopic repair has become the preferred option for the treatment of pediatric hernias. Recently, laparoscopic operations have also been conducted in pediatric recurrent hernia cases. Laparoscopic percutaneous extraperitoneal closure (LPEC) is a simple and reliable minimally invasive procedure for pediatric inguinal hernia repair. However, it is still unclear whether LPEC is an ideal procedure for recurrent inguinal hernias in children. The aim of this study is to evaluate the efficacy of LPEC in the treatment of pediatric recurrent inguinal hernias. Patients and Methods: We retrospectively reviewed all children with primary inguinal hernia repairs in our hospital from 2016 to 2017 and analyzed the outcomes of recurrent inguinal hernia repairs with LPEC. Results: There were 1703 children with 1985 indirect inguinal hernias: 1549 were laparoscopic (91.0%) and 154 open (9.0%). Thirty-five (2.1%) of these children had recurrent inguinal hernia with no difference in prevalence between laparoscopic and open (P = .24). One case was bilateral. LPEC was performed successfully in all children with recurrent inguinal hernias. There were no severe intra- or postoperative complications in any of the recurrent cases. No children had inguinal hernia recurrence at follow-up (8 months-2 years). Conclusions: LPEC of the internal ring is a reliable and effective minimally invasive technique for the treatment of recurrent inguinal hernia in children.


Assuntos
Hérnia Inguinal/cirurgia , Herniorrafia/métodos , Laparoscopia/métodos , Reoperação/métodos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Recidiva , Estudos Retrospectivos , Resultado do Tratamento
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