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Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association最新文献

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Hyaline cartilage at porta hepatis in extrahepatic biliary atresia. 肝外胆道闭锁时肝门处透明软骨。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01
L D Mirkin, A S Knisely
{"title":"Hyaline cartilage at porta hepatis in extrahepatic biliary atresia.","authors":"L D Mirkin,&nbsp;A S Knisely","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Hyaline cartilage was found on microscopy of sections of the extrahepatic biliary tree in two infants with extrahepatic biliary atresia (EHBA). Respiratory epithelium was not present, and the cartilage did not seem to block the bile duct lumen. Hyperbilirubinemia was manifest in one infant on the second postnatal day, but clinical courses were otherwise unremarkable. In neither infant was the ductal plate malformation found on light microscopy of liver biopsy specimens, and in neither infant was visceral topography abnormal. Hyaline cartilage at the porta hepatis appears to be a novel finding in EHBA. Its significance remains to be defined.</p>","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"17 4","pages":"587-91"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20155129","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
Major histocompatibility complex class II deficiency needs an early diagnosis: report of a case. 主要组织相容性复合体II类缺乏需要早期诊断:1例报告。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01 DOI: 10.1080/15513819709168743
D. Canioni, N. Patey, B. Cuenod, M. Benkerrou, N. Brousse
{"title":"Major histocompatibility complex class II deficiency needs an early diagnosis: report of a case.","authors":"D. Canioni, N. Patey, B. Cuenod, M. Benkerrou, N. Brousse","doi":"10.1080/15513819709168743","DOIUrl":"https://doi.org/10.1080/15513819709168743","url":null,"abstract":"Major histocompatibility complex (MHC) class II deficiency is a rare primary immunodeficiency disorder characterized by defects in human leukocyte antigen class II expression, inconsistent expression of human leukocyte class I molecules, and a lack of cellular and humoral immune responses to foreign antigens. Clinical onset occurs early in life with recurrent infections and chronic diarrhea. The prognosis is poor, and mean age at the time of death is 4 years. The only curative treatment is bone marrow transplantation (BMT), which allows the immune system's reconstitution. BMT should be done early in life, because long-term survival seems to depend on the number of previous viral infections. We report the case of an MHC class II deficiency discovered late in a 4-year-old girl by means of immunohistochemistry of small bowel biopsy revealing the absence of MHC class II expression. The child received a BMT twice but died because of a overwhelming viral infection. This case underlines the necessity to explore children presenting with infections and chronic diarrhea in order to find MHC class II deficiency. Usually, diagnosis is performed on cytospins, but when it has been missed clinically, it can be performed by using immunohistochemistry on small bowel biopsies.","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"2015 1","pages":"645-51"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86230338","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: 7
Skeletal abnormalities in Meckel syndrome. Meckel综合征的骨骼异常。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01 DOI: 10.1080/15513819709168740
J. Shanks, B. Kerr, S. Russell, H. Kingston, L. Moore
{"title":"Skeletal abnormalities in Meckel syndrome.","authors":"J. Shanks, B. Kerr, S. Russell, H. Kingston, L. Moore","doi":"10.1080/15513819709168740","DOIUrl":"https://doi.org/10.1080/15513819709168740","url":null,"abstract":"Meckel syndrome is an autosomal recessive condition with a wide phenotypic variation. The most consistent features are cystic kidneys and intrahepatic bile duct anomalies, frequently accompanied by central nervous system (CNS) malformations and polydactyly. Approximately one sixth of all cases also show skeletal anomalies. We present two cases, siblings born to a consanguineous couple, in whom there was a striking curvature and shortening of the long bones in addition to cystic kidneys, CNS abnormalities, and polydactyly. Histological examination of the long bones in the second affected sibling showed mid-diaphysial ectopic cartilaginous growth plates differentiating the long bone changes from other skeletal dysplasias with similar radiological features.","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"33 1","pages":"625-30"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76933120","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
Lymphangiomatosis of the body wall: a report of two cases associated with chylothorax and fatal outcome. 体壁淋巴管瘤病:两例乳糜胸合并死亡报告。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01 DOI: 10.1080/15513819709168739
P. Moerman, C. van Geet, H. Devlieger
{"title":"Lymphangiomatosis of the body wall: a report of two cases associated with chylothorax and fatal outcome.","authors":"P. Moerman, C. van Geet, H. Devlieger","doi":"10.1080/15513819709168739","DOIUrl":"https://doi.org/10.1080/15513819709168739","url":null,"abstract":"We report on two cases of an unusual but distinctive variant of lymphangiomatosis, presenting at birth with predominantly cutaneous involvement of the body wall and complicated by chylothorax. The lesion manifested clinically as a slowly progressive, diffuse, and fluctuant skin swelling. Eventually, almost the entire trunk became affected. There were no bone lesions. Histologically, the soft tissues were diffusely infiltrated by interconnecting mazelike lymphatic vessels. The two infants died from infection at the age of 23 days and 10 months, respectively. Our experience confirms that premortem histologic diagnosis of lymphangiomatosis is difficult to establish. Awareness of the condition and knowledge of its various clinical presentation forms are essential for proper recognition, assessment of the outcome, and evaluation of new therapeutic measures such as interferon.","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"48 1","pages":"617-24"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86794681","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: 19
Inflammatory cytokine mRNAs in surgical specimens of necrotizing enterocolitis and normal newborn intestine. 坏死性小肠结肠炎和正常新生儿肠道手术标本中的炎性细胞因子mrna。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01
R M Viscardi, N H Lyon, C C Sun, J R Hebel, J D Hasday
{"title":"Inflammatory cytokine mRNAs in surgical specimens of necrotizing enterocolitis and normal newborn intestine.","authors":"R M Viscardi,&nbsp;N H Lyon,&nbsp;C C Sun,&nbsp;J R Hebel,&nbsp;J D Hasday","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Coagulation necrosis, inflammation, and hemorrhage are pathologic hallmarks of necrotizing enterocolitis (NEC). Because cytokines are peptides that mediate inflammatory cell recruitment and amplify the immune response, several of the inflammatory cytokines have been implicated in NEC. We hypothesized that mRNA levels for the interrelated cytokines interleukin-1 beta (IL-1 beta), tumor necrosis factor-alpha (TNF-alpha), IL-6, and the neutrophil chemotactic factor IL-8 would be increased in NEC and would be associated with the presence of inflammation. In this study, we determined the relative levels and localization of mRNA for these cytokines in surgical pathology archival intestinal tissue from 29 premature infants with acute NEC and 15 control infants with congenital intestinal malformations using a novel quantitative in situ hybridization technique. Compared with controls, there were higher IL-1 beta mRNA levels in full-thickness sections and higher TNF-alpha mRNA levels in full-thickness and mucosa sections of acute NEC samples, suggesting a potential role for these cytokines in the pathogenesis of local inflammation in NEC. IL-6 and IL-8 mRNA levels were similar in samples of control and acute NEC cases. Analysis of covariance including all subjects showed that the presence of acute inflammation was associated with increased IL-1 beta mRNA levels in mucosa (P = .035) and increased IL-8 in full-thickness sections (P = .005) and mucosa (P = .01). In four of five NEC cases in which intestinal specimens were available from reanastomosis surgery, cytokine mRNA levels decreased to low or undetectable levels. These data suggest that the inflammatory cytokines are involved in neutrophil recruitment and augmentation of the inflammatory response in neonatal intestine.</p>","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"17 4","pages":"547-59"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20155818","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
Cell populations in the bone marrow of guinea pig fetuses with intrauterine growth retardation. 宫内发育迟缓豚鼠胎儿骨髓细胞群的研究。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01
L M Ernst, M M Sanders, C M Salafia, A M Carter
{"title":"Cell populations in the bone marrow of guinea pig fetuses with intrauterine growth retardation.","authors":"L M Ernst,&nbsp;M M Sanders,&nbsp;C M Salafia,&nbsp;A M Carter","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Bone marrow smears and blood samples were examined in guinea pig fetuses in which intrauterine growth retardation (IUGR) had been induced by uterine artery ligation and compared with those of control (well-grown) fetuses from uterine horns with intact circulation. Differential bone marrow cell counts were obtained from a count of 300 cells per smear and blood samples were assayed for hemoglobin concentration and 2,3-diphosphoglycerate (DPG). Results of blood assays showed no difference in hemoglobin concentration. DPG levels were reduced in the IUGR guinea pigs (P < .05), which could be a consequence of decreased glucose availability or represent an adaptation to reduced oxygen availability. Comparisons of bone marrow counts revealed an increase in total erythrocyte precursors (P < .05) and a decrease in total granulocytic precursors (P < .05) in IUGR fetuses. Within the erythroid lineage there was a significant increase in late (orthochromatic) erythroblasts (P < .005) in the IUGR animals compared with control animals. The granulocytic lineage of the IUGR fetuses showed a significant decrease in mature neutrophils (P < .05) and eosinophilic precursors (P < .05) compared with controls. These data suggest that the hypoxic stress of uterine artery ligation leads to an increase in medullary erythropoiesis. In concert with a previous study that showed a reduction in hepatic erythropoiesis, these data suggest a precocious shift of the anatomic site of erythropoiesis from the liver to the bone marrow under conditions of hypoxia.</p>","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"17 4","pages":"561-8"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20155820","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
Mucosal calcified nodule of the hard palate in an infant: case report and review of the literature. 1例婴儿硬腭粘膜钙化结节:病例报告及文献复习。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01 DOI: 10.1080/15513819709168738
M. Afzal, S. Dancea, J. de Nanassy
{"title":"Mucosal calcified nodule of the hard palate in an infant: case report and review of the literature.","authors":"M. Afzal, S. Dancea, J. de Nanassy","doi":"10.1080/15513819709168738","DOIUrl":"https://doi.org/10.1080/15513819709168738","url":null,"abstract":"Pathological calcifications of skin manifest as small or large deposits of calcium in the dermis and subcutaneous tissues. One form of these conditions is described as subepidermal calcified nodule seen on the facial skin of young children without any underlying connective tissue disease or any abnormality in calcium or phosphorus metabolism. The oral cavity is rarely affected. Recently, two cases were reported in the oral mucosa and the term \"mucosal calcified nodule\" was coined for such an entity. We report another case of such a process involving the oral mucosa of a 5-month-old infant who presented with an enlarging lesion at the junction of the hard and soft palate.","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"95 1","pages":"611-5"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79241875","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: 8
Hepatic heterotopias in the jejunum: a case study over time showing progressive degenerative changes. 空肠肝脏异位:一个随时间推移显示进行性退行性改变的病例研究。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01
M S Magid, T A Godwin, W Zheng
{"title":"Hepatic heterotopias in the jejunum: a case study over time showing progressive degenerative changes.","authors":"M S Magid,&nbsp;T A Godwin,&nbsp;W Zheng","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Multiple foci of heterotopic liver in the jejunum were sequentially discovered in an infant boy at the ages of 1 day, 2 months, and 4 months. This is the second reported case of jejunal heterotopic liver, a rare entity in any site. Progressive histological changes indicative of biliary duct obstruction were observed in the hepatic heterotopias, which demonstrated no connections to the main body of the liver or biliary tree.</p>","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"17 4","pages":"663-70"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20157116","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
Reduced inferior olivary neuron number in early Down syndrome. 早期唐氏综合征患者下橄榄神经元数量减少。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01
S S Pine, B H Landing, W R Shankle
{"title":"Reduced inferior olivary neuron number in early Down syndrome.","authors":"S S Pine,&nbsp;B H Landing,&nbsp;W R Shankle","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Counts of total neuron number per section and of neurons per microscopic field of inferior olivary principal nuclei were made on sections from 10 patients with Down syndrome (DS) aged 0.36 to 28 months and seven control (C) patients aged 1 to 29 months. After stereologic and appropriate shrinkage corrections of the count data, the ratios of values for DS/C were calculated. For mean principal olivary nucleus neuron number, DS/C = 0.64; for mean number of neurons per field, DS/C = 0.84; for mean volume of olivary neuronal band per section, DS/C = 0.79; and for mean volume of neuronal band per neuron, DS/C = 1.27. The data are in accord with other data suggesting that (1) numbers of cells in various cell populations, including various areas of the cerebrum, in DS approximate two-thirds normal (DS/C approximately 0.67); (2) for the volumes of such cell populations, DS/C = 0.82 normal; and (3) for volumes of individual cells, DS/C = 1.22 normal. The data of the present study suggest that the inferior olivary nuclei in DS are affected in the same way and to a similar degree as other brain areas, with the age distribution and histologic features of the specimens studied suggesting that the reduced olivary principal nucleus number in early Down syndrome results from reduced initial neuron production rather than postnatal neuron loss.</p>","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"17 4","pages":"537-45"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20155817","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
Lymphangioma of the right kidney in an infant boy. 男婴右肾淋巴管瘤。
Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association Pub Date : 1997-07-01 DOI: 10.1080/15513819709168741
R. Caduff, M. Schwöbel, U. Willi, Jakob Briner
{"title":"Lymphangioma of the right kidney in an infant boy.","authors":"R. Caduff, M. Schwöbel, U. Willi, Jakob Briner","doi":"10.1080/15513819709168741","DOIUrl":"https://doi.org/10.1080/15513819709168741","url":null,"abstract":"A 9-month-old boy with a primary cystic lymphangioma of the right kidney is reported. The clinical and radiological features favored a malignant tumor. Histology revealed the lesion to be a lymphangioma; the diagnosis was confirmed by immunohistochemistry and chemical analysis of the intracystic fluid. Lymphangiomas of the kidney are rare in adults; they are even rarer in infants and children. Nevertheless, renal lymphangiomas should be considered in the differential diagnosis of multicystic, unilateral renal masses, independent of the age of the patient.","PeriodicalId":79453,"journal":{"name":"Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association","volume":"27 1","pages":"631-7"},"PeriodicalIF":0.0,"publicationDate":"1997-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74602903","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: 9
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