Pediatric pathology & molecular medicine最新文献

{"title":"Clinico-pathologic conference: newborn with hydrops fetalis caused by CMV infection case report.","authors":"Jorge Lujan-Zilbermann, Atilano Lacson, Enid Gilbert-Barness, Herbert H Pomerance","doi":"10.1080/pdp.22.6.481.494","DOIUrl":"https://doi.org/10.1080/pdp.22.6.481.494","url":null,"abstract":"Jorge Lujan-Zilbermann u Assistant Professor of Pediatrics, University of South Florida College ofMedicine,Tampa, Florida, USA Atilano Lacson u Clinical Associate Professor of Pediatrics, University of South Florida College ofMedicine and Pediatric Pathologist, All Children’s Hospital, St. Petersburg, Florida, USA Enid Gilbert-Barness u Professor of Pathology and LaboratoryMedicine, Pediatrics, and Obstetrics and Gynecology, University of South Florida College ofMedicine,Tampa, Florida, USA Herbert H. Pomerance u Professor of Pediatrics, University of South Florida College of Medicine,Tampa, Florida, USA","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 6","pages":"481-94"},"PeriodicalIF":0.0,"publicationDate":"2003-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.6.481.494","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24044380","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}

{"title":"Pathologic quiz case: missed abortion at 6 weeks gestation.","authors":"Terry Fox, Enid Gilbert-Barness","doi":"","DOIUrl":"","url":null,"abstract":"","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 6","pages":"495-6"},"PeriodicalIF":0.0,"publicationDate":"2003-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24043739","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}

{"title":"Case report: lethal multiple pterygium syndrome.","authors":"David S Brink,&nbsp;Atchawee Luisiri,&nbsp;Dorothy K Grange","doi":"10.1080/pdp.22.6.461.470","DOIUrl":"https://doi.org/10.1080/pdp.22.6.461.470","url":null,"abstract":"<p><p>We report a case of lethal multiple pterygium syndrome (LMPS). Intrauterine fetal demise was diagnosed at 31 weeks' estimated gestational age. Subsequent fetopsy established the diagnosis of LMPS. Macroscopic, microscopic, and radiographic findings in the case are discussed, and LMPS is reviewed.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 6","pages":"461-70"},"PeriodicalIF":0.0,"publicationDate":"2003-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.6.461.470","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24044377","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}

{"title":"Characterization of cellular phenotypes and cytokine expression in balt from children with congenital heart diseases.","authors":"T M Moussallem,&nbsp;F Guedes,&nbsp;E R Fernandes,&nbsp;C Pagliari,&nbsp;H F Andrade,&nbsp;M I S Duarte,&nbsp;C L P Lancellotti","doi":"10.1080/pdp.22.6.449.459","DOIUrl":"https://doi.org/10.1080/pdp.22.6.449.459","url":null,"abstract":"<p><strong>Unlabelled: </strong>The present study was performed to target and call attention to the bronchial associated lymphoid tissue (BALT), part of our immune system, from which, we believe, several forms of prophylactic and therapeutic approaches can be developed. The characterization of its immune components, cells, and cytokines, in absence of antigenic stimuli, is pioneer in literature. Eighteen cases of necropsies were chosen and selected the paraffin-embedded lungs. The ages of 11 females and 7 males varied from 5 to 31 months. Cause of death: congenital heart diseases.</p><p><strong>Exclusion criteria: </strong>lung infection at necropsy and/or arterial hypertrophy greater than Heath-Edwards' 1st degree. Immunohistochemical technique was applied to identify the cell phenotypes and the cytokines in situ. BALT was identified in all cases in this study. The main cellular phenotypes in BALT were T helper (TH) and B lymphocytes surrounded by T cytotoxic lymphocytes, natural killer cells, and dendritic cells in less quantities. Interleukin 10 and Tumor Necrosis Factor alpha were the predominant cytokines in BALT without antigenic stimuli. BALT is an important structure of the lung immune system in infants, with a tendency to maintain an environment favorable to the Th2 arm of immune response. It needs more exploration to define its behavior in front of infections, especially those with pulmonary tropism.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 6","pages":"449-59"},"PeriodicalIF":0.0,"publicationDate":"2003-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.6.449.459","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24044378","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}

{"title":"Clinico-pathologic conference: 12-year-old girl with fatigue, anemia, weight loss, abdominal distention, and occasional nausea and vomiting.","authors":"L Julio Reinstein,&nbsp;Afif Ahmed,&nbsp;Enid Gilbert-Barness,&nbsp;Herbert H Pomerance","doi":"10.1080/pdp.22.6.471.480","DOIUrl":"https://doi.org/10.1080/pdp.22.6.471.480","url":null,"abstract":"<p><p>A clinicopathologic case is presented. The patient was a 12-year-old white girl with a history of fatigue and dizziness, occasional nausea and vomiting, and anemia, who showed abdominal distention, especially in the epigastrium. An epigastric mass was palpable. Gastroscopy was done, and surgery followed. Pertinent laboratory findings are presented, and clinical discussion follows. The pathology is then presented and discussed.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 6","pages":"471-80"},"PeriodicalIF":0.0,"publicationDate":"2003-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.6.471.480","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24044379","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}

{"title":"An unusual renal malignancy of childhood: unilateral teratoid Wilms's tumor.","authors":"Nasir Bakshi,&nbsp;Ibrahim Mansoor,&nbsp;N K Venkataramu,&nbsp;S Katariya","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Teratoid Wilms's tumor is a rare histologic variant of the classical Wilms's tumor, containing predominantly heterolougus tissues (adipose, glial, muscle, cartilage, or bone). We report an unusual variant of such tumor, which simulated renal teratoma because of abundant fat within the tumor. The child underwent radical excision; postoperatively, he was treated with chemotherapy, and now the child is well at 36 months after surgery. The behavior of this kind of tumor usually is not aggressive, and the outcome is good. Surgery should be the treatment of choice, because the efficacy of chemotherapy and radiotherapy is probably reduced by the high amount of differentiated and mature tissue that characterizes this neoplasm. Radiologic imaging and pathological features of this rare entity are described.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 5","pages":"435-41"},"PeriodicalIF":0.0,"publicationDate":"2003-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24138904","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}

{"title":"Peutz-Jeghers polyposis with enteritis cystica profunda--a diagnostic pitfall.","authors":"Nandita Kakkar,&nbsp;R K Vasishta,&nbsp;Ujjal Poddar,&nbsp;B R Thapa","doi":"10.1080/pdp.22.5.369.373","DOIUrl":"https://doi.org/10.1080/pdp.22.5.369.373","url":null,"abstract":"A10-year-old female child presentedwith history of o¡-and-on abdominal pain present for 2 years. The pain had become more frequent and colicky in the last 15 days. It was present in the hypogastrium and periumbilical area.There was history of constipation and an occasional episode of fresh blood with stools. She was the secondof twins and the other twin sister was asymptomatic. On examination,melanosis was seen on the lips, oral mucosa, and tips of ¢ngers and toes since 1 year of age. Her mother had similar symptoms at 15 years of age for which she underwent an abdominal surgery. A part of the small bowel was resected and it was said to be studded with a few polyps. No written document or histopathology report is available. The mother has been asymptomatic ever since.The mother and the younger brotheralsohadoralmelanosis.Thebrother, however, hasbeenasymptomatic so far. On examination of the index case there was amass present in the periumbilical area measuring 5 3 cm. Reducible intussusception was present. Resection of the small intestine at two places was done with end-to-end anastomosis.Two segments (18 cm each) of the small intestine were resected, 9 inches from the duodenojejunal junction and at the jejunoilial junction. Both segments of the small intestine contained two polyps each.The largest polyp (5 cm in diameter) was seen 9 cm distal to the duodenojejunal junction, was pedunculated, and there were small cysts with mucoid material present at all levels of the wall including the serosa (Figure 1). The other three polyps were sessile and ranged from 2 to 4 cm in diameter. They too showed small cystic spaces with mucoid material. Microscopically the polyps revealed a classic morphology. Normal smallintestinal glands rested on a branching smooth muscle framework (Figure 2). There was no evidence of dysplasia. Transmural herniation of the mucosal glands admixed with smooth muscle ¢bers and lamina propria was seen until the serosa (Figure 3). Apart from this there were cystically dilated spaces (0.2^1 cm diameter)","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 5","pages":"369-73"},"PeriodicalIF":0.0,"publicationDate":"2003-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.5.369.373","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24138959","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}

{"title":"Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis. 2: A case report and review of the literature.","authors":"Yu Chen,&nbsp;Alan S Diamond,&nbsp;Kirubahara R Vaheesan,&nbsp;Steven Schneider,&nbsp;Elsa Valderrama","doi":"10.1080/pdp.22.5.375.381","DOIUrl":"https://doi.org/10.1080/pdp.22.5.375.381","url":null,"abstract":"<p><p>Neurofibromatosis (NF) type I (NF1) is the most common familial cancer-predisposing syndrome in humans, while type 2 (NF2) accounts for an extremely small percentage of the total cases of NF. Tumors occurring in patients with NF1 are primarily peripheral neurofibromas, while NF2 patients present with central schwannomas. Malignant transformation has been described in NF1 patients; however, in NF2 the risk of malignant transformation is extremely rare. In this case report, the authors document a retroperitoneal neurogenic sarcoma occurring in a 20-year-old woman with NF2 (bilateral acoustic schwannomas, meningioma, and multiple intraspinal tumors).</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 5","pages":"375-81"},"PeriodicalIF":0.0,"publicationDate":"2003-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/pdp.22.5.375.381","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24138960","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}

{"title":"The isoprenoid pathway and the pathogenesis of Reye's syndrome.","authors":"Ravi Kumar Kurup,&nbsp;Parameswara Achutha Kurup","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Unlabelled: </strong>The isoprenoid pathway produces three key metabolites: endogenous digoxin (regulator of neurotransmitter uptake), dolichol, and ubiquinone (free radical scavenger). Since a mitochondrial dysfunction has been described in Reye's syndrome, it was considered pertinent to assess the pathway in this disease. Since endogenous digoxin can regulate neurotransmitter transport, the pathway was also 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 hydroxy methyl glutaryl (HMG) coenzyme A (CoA) reductase, magnesium, digoxin, dolichol, ubiquinone, tryptophan/tyrosine catabolic patterns, and free radical and lipid levels, as well as RBC Na+, K(+)-ATPase activity, were measured in the groups mentioned.</p><p><strong>Results: </strong>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 was also 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.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 5","pages":"423-34"},"PeriodicalIF":0.0,"publicationDate":"2003-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24138905","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}

{"title":"Rectal primitive neuroectodermal tumor.","authors":"Ricardo Drut,&nbsp;Mónica Drut,&nbsp;Carlos Müller,&nbsp;Adriana Marrón","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>We report the clinical, histologic, immunohistochemical, and molecular findings of a Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) localized to the rectum in a 17-year-old boy. Notably the 4.5 x 4 x 4-cm sessile mass was spontaneously eliminated through the anus, producing an episode of hemorrhagic shock. Histologically the tumor presented as a proliferation of membrane CD99-positive, small round cells. EWS/FLI1 chimeric mRNA was demonstrated by nested reverse-transcription polymerase chain reaction (RT-PCR) analysis in the tumor tissue. The patient is alive and well 2 years after initial symptoms. We also review reported cases of ES/PNET of the digestive system.</p>","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"22 5","pages":"391-8"},"PeriodicalIF":0.0,"publicationDate":"2003-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24138963","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}