Pediatric pathology & molecular medicine最新文献_第8页

THE ISOPRENOID PATHWAY AND THE PATHOGENESIS OF REYE'S SYNDROME 类异戊二烯途径与雷氏综合征的发病机制

Pediatric pathology & molecular medicine Pub Date : 2003-01-01 DOI: 10.1080/pdp.22.2.159.170

R. Kurup, P. A. Kurup

{"title":"THE ISOPRENOID PATHWAY AND THE PATHOGENESIS OF REYE'S SYNDROME","authors":"R. Kurup, P. A. Kurup","doi":"10.1080/pdp.22.2.159.170","DOIUrl":"https://doi.org/10.1080/pdp.22.2.159.170","url":null,"abstract":"The isoprenoid pathway produces three key metabolites: endogenous digoxin (regulator of neurotransmitter uptake), dolichol, and ubiquinone (free radical scavenger). Because a mitochondrial dysfunction has been described in Reye's syndrome, we thought it pertinent to assess the pathway in this disease. Since endogenous digoxin can regulate neurotransmitter transport, the pathway also was assessed in patients with right hemispheric, left hemispheric, and bihemispheric dominance to find out the role of hemispheric dominance in its pathogenesis. The plasma/serum activity of HMG CoA reductase, magnesium, digoxin, dolichol, ubiquinone, tryptophan/tyrosine catabolic patterns, free radical, and lipid levels aswellas (red blood cell) RBC Na + -K + ATPase activity were measured in the above mentioned groups. Results: In the patient group as well as in individuals with right hemispheric dominance similar patterns were obtained. There was elevated digoxin and dolichol levels with low levels of ubiquinone in patients with Reye's syndrome as well as in those with right hemispheric dominance. The serum magnesium and RBC Na + -K + ATPase activity were reduced. There also was an increase in tryptophan catabolites and a reduction in tyrosine catabolites as well as increased free radical levels. Reye's syndrome is associated with an upregulated isoprenoid pathway, elevated hypothalamic digoxin secretion, and right hemispheric chemical dominance.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"87 1","pages":"159 - 170"},"PeriodicalIF":0.0,"publicationDate":"2003-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.2.159.170","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"60773119","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

THE CONFUSED IDENTITY OF CANTRELL'S PENTAD: ECTOPIA CORDIS IS RELATED EITHER TO THORACOSCHISIS OR TO A DIAPHRAGMATIC HERNIA WITH AN OMPHALOCELE cantrell 's五型心外异位的混淆身份可能与胸裂或膈疝合并脐膨出有关

Pediatric pathology & molecular medicine Pub Date : 2003-01-01 DOI: 10.1080/pdp.22.5.383.390

B. Davies, M. Durán

引用次数: 9

Infant with high arched palate, bell-shaped chest, joint contractures, and intrauterine fractures. 婴儿上颚高弓，胸部钟形，关节挛缩，宫内骨折。

Pediatric pathology & molecular medicine Pub Date : 2002-11-01 DOI: 10.1080/pdp.21.6.569.584

Atilano G Lacson, Gail Donaldson, Enid Gilbert Barness, Judith D Ranells, Herbert H Pomerance

引用次数: 9

Expression and regulation of the transcriptional repressor ZNF43 in Ewing sarcoma cells. 转录抑制因子ZNF43在尤文氏肉瘤细胞中的表达与调控。

Pediatric pathology & molecular medicine Pub Date : 2002-11-01 DOI: 10.1080/15227950290112789

Domingo González-Lamuño, Noureddine Loukili, Miguel García-Fuentes, Timothy M Thomson

{"title":"Expression and regulation of the transcriptional repressor ZNF43 in Ewing sarcoma cells.","authors":"Domingo González-Lamuño, Noureddine Loukili, Miguel García-Fuentes, Timothy M Thomson","doi":"10.1080/15227950290112789","DOIUrl":"https://doi.org/10.1080/15227950290112789","url":null,"abstract":"In vitro, cells derived from Ewing sarcoma (ES) with the characteristic somatic rearrangement between the genes EWS and FLII can be induced to differentiate toward a neuronal phenotype by exposure to agents such as dibutyryl cyclic AMP (db cAMP) or retinoic acid. Therefore, expression of the chimeric Ews-Flil protein does not irreversibly block the capacity of Ewing cells to engage in the neuronal differentiation program initiated by these agents. To identify genes that might be involved in the maintenance of Ewing cells in their undifferentiated state, a PCR-based differential display method was used to compare gene expression patterns in Ewing cell lines with those induced to differentiate toward a neuronal phenotype. A cDNA was expressed at high levels in proliferating Ewing-derived EW-1 cells and downregulated in EW-1 cells induced to differentiate, which corresponds to ZNF43, a multi-zinc finger protein containing the Krüppel-associated box (KRAB) transcriptional repression domain. Treatment of EW-1 cells with antisense oligonucleotides complementary to ZNF43 mRNA induces morphological differentiation and growth arrest. These findings suggest a role for ZNF43 in the maintenance of ES cells in an undifferentiated state, and that ZNF43 could be a primary target for differentiation stimuli in Ewing cells.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 6","pages":"531-40"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950290112789","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22204125","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 11

Chorangiopagus parasiticus twins. 寄生绒毛膜虫双胞胎。

Pediatric pathology & molecular medicine Pub Date : 2002-11-01 DOI: 10.1080/15227950290112806

Nandita Kakkar, Rakesh Kumar Vasishta, Monika Panwar, Anil Narang, Indu Gupta, Ashru Kumar Banerjee

引用次数: 4

Molecular analysis and pathology of a second pediatric HIV-associated Burkitt lymphoma. 第二例儿童hiv相关伯基特淋巴瘤的分子分析和病理。

Pediatric pathology & molecular medicine Pub Date : 2002-11-01 DOI: 10.1080/15227950290112770

Eve Cabalo, Robert Wilkinson, Nu T Lu, Sheri Shimizu, Marian Melish, Kenneth L McClain, Hal B Jenson, Bruce Shiramizu

引用次数: 4

Follicular bronchiolitis (FBB) associated with Legionella pneumophilia infection. 滤泡性细支气管炎(FBB)与嗜肺军团菌感染相关。

Pediatric pathology & molecular medicine Pub Date : 2002-11-01 DOI: 10.1080/15227950290104823

T Masuda, Y Ishikawa, Y Akasaka, T Ishii, K Tateda, Y Ishii, K Yamaguchi, H Kiguchi

引用次数: 4

Reduced acute phase response to differentiate between viral and bacterial infections in children. 减少急性期反应以区分儿童病毒感染和细菌感染。

Pediatric pathology & molecular medicine Pub Date : 2002-11-01 DOI: 10.1080/15227950290112815

Joseph Urbach, Rivka Rotstein, Renato Fusman, David Zeltser, Itzhak Shapira, David Branski, Shlomo Berliner

{"title":"Reduced acute phase response to differentiate between viral and bacterial infections in children.","authors":"Joseph Urbach, Rivka Rotstein, Renato Fusman, David Zeltser, Itzhak Shapira, David Branski, Shlomo Berliner","doi":"10.1080/15227950290112815","DOIUrl":"https://doi.org/10.1080/15227950290112815","url":null,"abstract":"The objective of this study was to document the reduced acute phase response that appears in children with viral as opposed to bacterial infections. The white blood cell count (WBCC), the erythrocyte sedimentation rate (ESR), and leukocyte and erythrocyte adhesiveness/aggregation were determined in 36 children with acute bacterial infection, 29 children with viral infection, and 19 controls. A significant reduced WBCC, ESR, and leukocyte and erythrocyte adhesiveness/aggregation was noted in the children with acute viral infection as opposed to those with bacterial infection: 10,800 +/- 3600 and 20,000 +/- 10,000 cells/cm2, 29 +/- 21 and 53 +/- 35 mm Hg, 23 +/- 9 and 41 +/- 15%, and 3.4 +/- 5.1 and 9.8 +/- 13.6 microns, respectively. The results indicate that a reduced acute phase response can be observed in children with an acute viral infection. This can have diagnostic implications and pathophysiological consequences in terms of less flow impairment in the microcirculation due to less red and white blood cell aggregation.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 6","pages":"557-67"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950290112815","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22204128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 6

Congenital heart disease and its association with other congenital malformations found at autopsy. 先天性心脏病及其与尸检中发现的其他先天性畸形的关系。

Pediatric pathology & molecular medicine Pub Date : 2002-11-01 DOI: 10.1080/15227950290112798

Belinda R Davies, Thelma Rizo, America Arroyo-Valerio

{"title":"Congenital heart disease and its association with other congenital malformations found at autopsy.","authors":"Belinda R Davies, Thelma Rizo, America Arroyo-Valerio","doi":"10.1080/15227950290112798","DOIUrl":"https://doi.org/10.1080/15227950290112798","url":null,"abstract":"The objective was to determine if a study of other malformations found autopsy in patients with congenital heart disease would contribute to an understanding of the mechanisms involved in the formation of these anomalies. In a large general hospital autopsies in children with congenital heart disease were selected, and the different cardiopathies were divided into 3 groups: those with isolated heart lesions, those with single gene mutants, as well as chromosome malformations, and those with idiopathic malformations not associated with a genetic syndrome. Because the cardiopathies most often associated with genetic syndromes were the septal defects in general (p = .001), it was presumed that these are influenced to a considerable extent by genetic factors. The association of left heart hypoplasia and coarctation of the aorta with multiple idiopathic malformations, particularly in the lower half of the body (p = .002), suggests that the latter may be due to vascular disruptions, because of the interruption of the flow of oxygenated blood in the embryo-fetus produced by these heart defects. Cranial defects were not associated with left heart obstruction, and are therefore unlikely to be produced by vascular disruption.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 6","pages":"541-9"},"PeriodicalIF":0.0,"publicationDate":"2002-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950290112798","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22204746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 3

Association between Breus' mole and partial hydatidiform mole: chance or can hydropic villi precipitate placental massive subchorionic thrombosis? 勃氏痣与部分葡萄胎的关系:绒毛积水是否会导致胎盘绒毛膜下血栓形成?

Pediatric pathology & molecular medicine Pub Date : 2002-09-01 DOI: 10.1080/02770930290097507

Dae Tong Kim, D Christie Riddell, J Philip Welch, Heather Scott, Robert B Fraser, James R Wright

{"title":"Association between Breus' mole and partial hydatidiform mole: chance or can hydropic villi precipitate placental massive subchorionic thrombosis?","authors":"Dae Tong Kim, D Christie Riddell, J Philip Welch, Heather Scott, Robert B Fraser, James R Wright","doi":"10.1080/02770930290097507","DOIUrl":"https://doi.org/10.1080/02770930290097507","url":null,"abstract":"Breus' mole (massive subchorionic hematoma) is a rare entity most often found in the placentae of macerated stillborn fetuses. Previously considered to represent a postmortem event, recent evidence suggests that it occurs prior to fetal demise. A 23-week gestation male neonate was delivered of a 23-year-old gravida 3, para 2 woman and survived for 49 min. An autopsy with chromosomal studies resulted in a diagnosis of triploidy. Placental examination showed the presence of both Breus' mole and also partial hydatidiform mole. DNA samples extracted from portions of the fresh hematoma and from the fetal spleen were compared using molecular techniques. PCR analysis showed the presence of Y chromosome specific DNA in the placental clot, but a semiquantitative Southern blot demonstrated that roughly 85% of the clot DNA was of maternal origin. These findings suggest that Breus' mole represents primarily maternal thrombosis rather than fetal hemorrhage. We hypothesize that the partial mole could have contributed to the formation of the Breus' mole as some of the hydropic villi may have focally obstructed the maternal venous return from the intervillus space causing sluggish flow and promoting thrombosis. A review of the literature on Breus' mole shows that the majority of reported cases have not included cytogenetic findings. However, several authors have reported an association with triploidy and other chromosomal anomalies characterized by scattered placental hydropic villi. Thus, we suggest that obstruction of maternal venous return by hydropic villi may have played a contributory role in some of these other reported cases.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 5","pages":"451-9"},"PeriodicalIF":0.0,"publicationDate":"2002-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/02770930290097507","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22081341","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0