Pediatric pathology & molecular medicine最新文献

{"title":"Clinically integrated studies in pathology: their contribution to atherosclerosis research.","authors":"P. Scott","doi":"10.1080/02770930290056497","DOIUrl":"https://doi.org/10.1080/02770930290056497","url":null,"abstract":"This article, prepared in honor of Daria Haust, reviews some features of research into atherosclerosis and coronary heart disease over the past 150 years, and beyond. Attention is drawn to problems arising when people of strong personality dominate a particular field of research to the exclusion of pertinent observations that do not fit neatly into their particular paradigm. As the world becomes more complex and, in theory at least, communications between scientists become easier, these dangers are not necessarily diminished. Clinical medicine in its broadest sense must continue to relate in the traditional way to the specialties grouped within pathology. Undergraduates must receive instruction concerning the importance of the historical record and an understanding of the nature of science, its strengths, limitations, and boundaries.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 3 1","pages":"239-57"},"PeriodicalIF":0.0,"publicationDate":"2002-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/02770930290056497","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59430414","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":"Multifocal myocardial necrosis: a distinctive cardiac lesion in cystic fibrosis, lipomatous pancreatic atrophy, and Keshan disease.","authors":"C. Nezelof, R. Bouvier, F. Dijoud","doi":"10.1080/02770930290056550","DOIUrl":"https://doi.org/10.1080/02770930290056550","url":null,"abstract":"Multifocal myocardial necrosis (MMN) is an unusual cardiomyopathy of childhood, characterized by multiple patchy areas of myocardial fiber necrosis/fibrosis involving mainly the middle part of the left ventricle, but also, to a lesser extent, the right ventricle and the atria. These necrotic lesions are isolated and are not accompanied by an inflammatory reaction or vascular alterations. They are responsible for acute cardiac failure. MMN lesions are observed in various pathologic conditions including cystic fibrosis of the pancreas, pancreatic lipomatous hypoplasia/atrophy, malnutrition due to extensive intestinal resection with subsequent total parenteral feeding, and in Keshan disease. MMN is the main and the most characteristic feature of Keshan disease, an endemic and idiopathic condition affecting Chinese rural children. The causes and mechanisms of MMN presently are unknown. However, the presence of similar cardiac lesions in such different pathological conditions suggests the role of a selective deficiency of a hypothetical extrinsic factor (selenium, molybdenum iodide, other), probably crucial for the metabolism of the myocardial fiber.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 3 1","pages":"343-52"},"PeriodicalIF":0.0,"publicationDate":"2002-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/02770930290056550","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59430350","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":"Evolution of cholesterol concept of atherogenesis from Anitchkov to our days.","authors":"A. Klimov, V. Nagornev","doi":"10.1080/02770930290056532","DOIUrl":"https://doi.org/10.1080/02770930290056532","url":null,"abstract":"Our review addresses the development of the cholesterol concept of atherogenesis from the classical investigations of Anitchkov and Chalatov (1913), who induced experimental cholesterol atherosclerosis in rabbits, to the present time. We conclude that based on data obtained to date relating to the presence of different classes of lipoproteins in blood, on the role of peroxidatively modified low density lipoproteins in atherogenesis, and on the involvement of various arterial and blood cells and other factors, the cholesterol concept of atherogenesis has not lost its significance. Moreover, cholesterol-lowering therapy has a leading role in the primary and secondary prevention of coronary heart disease events and other clinical manifestations of atherosclerosis.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 3 1","pages":"307-20"},"PeriodicalIF":0.0,"publicationDate":"2002-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/02770930290056532","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59430759","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":"Bradykinin as a major endogenous regulator of endothelial function.","authors":"R. Gryglewski, W. Uracz, S. Chłopicki, E. Marcinkiewicz","doi":"10.1080/02770930290056514","DOIUrl":"https://doi.org/10.1080/02770930290056514","url":null,"abstract":"Healthy vascular endothelium is a powerful generator of nitric oxide (NO), prostacyclin (PGI2), prostaglandin E2 (PGE2), and plasminogen activator (t-PA). These endothelial products protect vascular wall against aggression from activated blood platelets and leukocytes. In particular they protect against thrombosis, promote thrombolysis, maintain tissue perfusion, and inhibit remodeling of vascular and cardiac walls. Endothelial dysfunction appears on one hand as suppression in the release of the above mediators, and on the other as deleterious discharge of prostaglandin endoperoxides (PGH2, PGG2), superoxide anion O2-, peroxynitrite (ONOO-), and plasminogen activator inhibitor (PAI-1). Our data point to endothelial bradykinin (Bk) as a trigger for protective endothelial mechanisms. In cultured endothelial cells (CEC) Bk through kinin B2 receptors raised in a concentration-dependent manner (1pM-10 nM) free cytoplasmic calcium ions [Ca2+]i. This rise was accompanied by the release of NO as quantified by a porphyrinic sensor. Other endothelial agonists were weaker-stimulators of [Ca2+]i than Bk. In vivo we analyed the effects of exogenous Bk and of amplifiers of endogenous Bk, such as perindopril and quinapril (\"tissue type\" angiotensin converting enzyme inhibitors, ACE-I) on endothelial function using our original thrombolytic bioassay and EIA assays for 6-keto-PGF1alpha and t-PA antigen. A major difference found between exogenuous Bk and endogenous Bk (that rendered by \"tissue ACE-I\") was a) prolonged thrombolytic action (> 4h) of quinapril or perindopril. Moreover, only exogenous Bk evoked an immediate and profound hypotensive action. In vivo, Bk-induced thrombolysis was B2 kinin receptor-dependent, PGI2-mediated. The unexpected action of Bk came to light in CEC. Then appeared incubated for 4 h increased expression of mRNAs for haemoxygenase (HO-1), cyclooxygenase 2 (COX-2), prostaglandin E synthase (PGE-S), but hardly for nitric oxide synthase 2(NOS-2). We hypothesize that a network of interactions of Bk-induced enzymes may constitute a delayed phase of Bk effects in the endothelium, whereas the primary phase would be activation by BK of [Ca2+]i-dependent constitutive endothelial enzymes. In blood-perfused rat endotoxemic lungs, NO is the most eminent cytoprotective mediator. Summing up, in peripheral circulation endogenous Bk is the most efficient activator of protective endothelial function. Thrombolytic action of \"tissue-type\" ACE-Is relies on receptor B-2-mediated, [Ca2+]i-dependent release of PGI2. Bk also may act as a \"microcytokine\" by inducing mRNAs for HO-1, COX-2, or PGE-S. Activation of HO-1 may lead to a deficiency in intracellular heme required as a cofactor for both COX and NOS. This network of interactions triggered by Bk call for further studies.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 3 1","pages":"279-90"},"PeriodicalIF":0.0,"publicationDate":"2002-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/02770930290056514","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59430692","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":"Cholesterol and development: the RSH (\"Smith-Lemli-Opitz\") syndrome and related conditions.","authors":"J. Opitz, E. Gilbert-Barness, J. Ackerman, A. Lowichik","doi":"10.1080/15227950252852078","DOIUrl":"https://doi.org/10.1080/15227950252852078","url":null,"abstract":"The half-century of lipophobia in the United States may be abating with some return of sanity on the discussion of health and dietary fat [Taubes, 2001]. The youngest victims of this collective, decades long madness are those infants deprived for one reason or another of breast milk. They are unable to speak for themselves at a time of greatest need for cholesterol during growth, the most critical period of myelination of central and peripheral nervous system, formation of bone and bile, and of every steroid hormone. Some of the commercial formulas they are fed contain only 1 or 2 mg of cholesterol per 100 g edible portion contrasted with almost 14 mg in breast milk. One can only hope that the confidence in their endogenous ability to synthesize sufficient amounts of cholesterol is not misplaced. Pediatric pathology has learned that when this endogenous ability fails during embryogenesis on the basis of mutations in the postsqualene biosynthesis of cholesterol, a startling variety of developmental pathology may present itself ranging from lethal forms of \"idiopathic\" hydrops, microcephaly with cerebral dysgenesis and dysmyelinization, agenesis of corpus callosum, cerebellar vermis dysgenesis, cataracts, cleft palate, many different forms of congenital heart defect, pyloric stenosis and/or Hirschsprung dysganglionosis, adrenal (cortical) insufficiency, cholestatic liver disease, limb malformations, and genital ambiguity in genetic males. Population genetic considerations suggest a hypothetical birth prevalence of the RSH (so-called Smith-Lemli-Opitz) syndrome, the commonest of these Garrodian errors of cholesterol biosynthesis, of 1/2500; since only about 1/15,000 to 1/20,000 homozygotes are liveborn and biochemically confirmed, over 80% prenatal or perinatal mortality must occur and deserves the most discerning of services from birth attendants, perinatologists, neonatologists, and fetal/pediatric pathologists. An easy, reliable, economical biochemical test for the presence of 7-dehydrocholesterol is available and the commonest mutation, the IVS8-1G-->C mutation, is quickly and reliably tested for molecularly. Thus, the successful diagnosis, even after death, will contribute substantially to correct genetic counseling, carrier detection, prenatal diagnosis, and treatment in those known to be affected prenatally andplanned to be liveborn. Thus, developmental pathology plays an integral, vital role in preventive medicine.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"15 1","pages":"153-81"},"PeriodicalIF":0.0,"publicationDate":"2002-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950252852078","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59824714","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":"Cardiovascular causes for sudden infant death.","authors":"M. Valdeś-Dapena, E. Gilbert-Barness","doi":"10.1080/15227950252852096","DOIUrl":"https://doi.org/10.1080/15227950252852096","url":null,"abstract":"Although sudden infant death syndrome (SIDS) is a cause for sudden infant death, other causes should be ruled out before diagnosing SIDS. Cardiac causes for sudden infant death include viral myocarditis, congential heart disease particularly congential aortic stenosis, endocardial fibroelastosis, and anomalous origin of the left coronary artery from the pulmonary artery. Other cardiac conditions that may result in sudden death include rhabdomyomas of the heart in tuberous sclerosis and conduction system disorders. The most frequent conduction system disorders resulting in sudden death include histiocytoid cardiomyopathy, congential heart block that may be associated with maternal lupus erythematosus, arrhythmogenic right ventricular dysplasia, noncompaction of the left ventricle, and long QT syndromes.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 2 1","pages":"195-211"},"PeriodicalIF":0.0,"publicationDate":"2002-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950252852096","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59824313","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":"Pathology of lethal fetal growth retardation syndrome with aminoaciduria, iron overload, and lactic acidosis (GRACILE).","authors":"J. Rapola, P. Heikkilä, V. Fellman","doi":"10.1080/15227950252852087","DOIUrl":"https://doi.org/10.1080/15227950252852087","url":null,"abstract":"Autopsy study of 17 newborn infants with lethal autosomal recessive disease presenting as growth retardation with lactic acidosis, Fanconi aminoaciduria, and hepatic hemosiderosis is reported. The patients succumbed between day 1 and 4 months of life; 9 patients died within the first month. All patients showed severe pathologic changes of liver with cholestasis in all livers. Extensive accumulation of stainable iron of the hepatocytes was present in 9/17 autopsy tissues and in two biopsy specimens. Moderate to abundant iron storage in the Kupffer cells was seen in all liver specimens. The amount of hepatocytic iron was high in livers up to 1 month of age and decreased thereafter. The general features and liver findings of this disorder suggest the name Growth Retardation Aminoaciduria Cholestasis Iron Overload, Lactacidosis and Early Death (GRACILE, OMIM 603358). Calcified concrements were seen in the medulla of 13/16 kidney specimens. Pancreas of 13/14 patients showed interstitial fibrosis and exocrine atrophy. Various pathologic findings such as renal tubular dysgenesis, paucity of hepatic bile ducts and iron storage in the macrophages of spleen and pulmonary alveoli were observed in some cases. Previous extensive clinical genetic and laboratory investigations have revealed that the patients had a previously unrecognized genetic disease. It is inherited as an autosomal recessive trait. The gene locus is 2q33-37. The basic defect of the disease remains unknown.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 2 1","pages":"183-93"},"PeriodicalIF":0.0,"publicationDate":"2002-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950252852087","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59824721","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":"Chronic disease from early malnutrition.","authors":"L. Barness","doi":"10.1080/15227950252852041","DOIUrl":"https://doi.org/10.1080/15227950252852041","url":null,"abstract":"Chronic diseases as a consequence of early malnutrition have been reported for over a century. First reports concerned chiefly undernutrition. As overnutrition has become more common, attention has been directed to its equally devastating effects.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 2 1","pages":"87-92"},"PeriodicalIF":0.0,"publicationDate":"2002-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950252852041","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59824604","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":"Natural history and risk factors of atherosclerosis in children and youth: the PDAY study.","authors":"A. Zieske, G. Malcom, J. Strong","doi":"10.1080/15227950252852104","DOIUrl":"https://doi.org/10.1080/15227950252852104","url":null,"abstract":"The Pathobiological Determinants of Atherosclerosis in Youth (PDAY) study was organized to document the natural history of athersclerosis and to determine the relation of cardiovascular risk factors to atherosclerosis in young subjects. Pathology laboratories in 15 centers collected coronary arteries, aortas, and other tissues from over 3,000 subjects age 15 to 34 who died of external causes between 1987 and 1994. The extent, prevalence, and topography of arterial lesions were evaluated and risk factors were analyzed in a central laboratory. Postmortem risk factors included serum lipoproteins, serum thiocyanate (smoking), glycohemoglobin (diabetes), thickness of panniculus adiposus and body mass index (obesity), changes in small renal arteries (hypertension), and apoprotein isoforms. The PDAY study confirmed the origin of atherosclerosis in childhood, showed that progression toward clinically significant lesions may occur in young adulthood and demonstrated that the progression of atherosclerosis is strongly influenced by coronary heart disease (CHD) risk factors. Recent PDAY studies have shown that a significant number of advanced coronary artery lesions have microscopic qualities associated with susceptibility to rupture and that CHD risk factors are associated with the development of these characteristic microscopic qualities. The PDAY archive continues to provide an important resource for new investigators throughout the world that contribute to the understanding of atherosclerosis, the underlying cause of most cardiovascular disease and the leading cause of debilitating illness and death in this country. The PDAY findings emphasize the need to modify risk factors in young people to retard the development of atherosclerotic lesions, particularly clinically significant lesions. Thus, true primary prevention of atherosclerosis must being in childhood or early adolescence.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"452 1","pages":"213-37"},"PeriodicalIF":0.0,"publicationDate":"2002-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950252852104","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59824323","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":"Cardiovascular involvement in metabolic diseases.","authors":"E. Gilbert-Barness","doi":"10.1080/15227950252852050","DOIUrl":"https://doi.org/10.1080/15227950252852050","url":null,"abstract":"Cardiomyopathies are a group of disorders that result in dysfunction of the contractility of the myocardium anda decrease in cardiac function. Cardiomyopathies may be primary or idiopathic or secondary due to an underlying definable systemic disorder. Cardiomyopathies may be further defined as according to the World Health Organization (WHO) as: 1) congestive or dilated; 2) obstructive as in hypertrophic cardiomyopathy; 3) restrictive as in infiltrative disorders such as storage diseases. Two additional categories have been added that include arrhythmogenic right ventricular cardiomyopathy and unclassified cardiomyopathies. A number of metabolic disorders result in cardiomyopathy and are frequently the cause of death in these disorders.","PeriodicalId":82276,"journal":{"name":"Pediatric pathology & molecular medicine","volume":"21 2 1","pages":"93-136"},"PeriodicalIF":0.0,"publicationDate":"2002-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/15227950252852050","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"59824640","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}