Nesidioblastosis versus islet cell hyperplasia.
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Adult↗
Biomedical subjects
E Kahn
Publications and source records attributed to E Kahn.
Squamous cell carcinoma in the sacrococcygeal area: a case report and review of the literature.
Squamous cell carcinoma is a rare malignancy in the pediatric age group. We describe a 12-year-old boy with squamous cell carcinoma of the sacrococcygeal region. This is the first reported case of that pathology in this region. The patient was treated with surgery, chemotherapy and radiation therapy and is now free of disease 3 years later. The etiology of sacrococcygeal squamous cell carcinoma is unclear, and its possible pathophysiology in relation to its location is discussed.
Carcinoma, Squamous Cell↗
Does rapidly progressive iron overload in a young girl with sideroblastic anemia also signify the presence of hereditary hemochromatosis?
A severely anemic 3-year-old girl with refractory sideroblastic anemia and fulminant, fatal hemochromatosis is described. The patient had transfusion-dependent anemia with clinical cardiac, liver, and endocrine dysfunction that resulted from iron loading. The patient was minimally transfused, and deferoxamine chelation was started at age 34 months. Despite treatment, the patient died at age 46 months as a result of severe iron overload. Sideroblastic anemia and iron overload in childhood are reviewed, and a pathophysiologic mechanism for the patient's clinical course is postulated.
Anemia, Sideroblastic↗
Pathologic characterization of a renin-secreting juxtaglomerular cell tumor in a child and review of the pediatric literature.
We describe a rare case of renal hypertension in a 15-year-old caused by juxtaglomerular cell tumor and compare our findings with those of 20 children reported in the literature. These tumors are usually encapsulated and composed microscopically of polyhedral cells with bland nuclei separated by fibrovascular septa. Characteristic renin granules can be demonstrated by Bowie's stain or electron microscopy. These tumors are benign. Pathologists should recognize the morphologic characteristics of these tumors when dealing with renin-producing neoplasia.
Adolescent↗
Intracardiac teratoma in a child simulating an atrioventricular nodal tumor.
Intracardiac teratoma is an extremely rare pediatric neoplasm. We studied the case of a 6-year-old girl with a right intraventricular cardiac mass. The tumor consisted of clusters of monotonous round epithelial cells scattered in a dense fibrotic stroma and was thought to represent an atrioventricular nodal tumor. Three years later the tumor recurred, with multiple mature elements derived from all three germ layers, and was diagnosed as mature cystic teratoma. Still present, however, were multiple areas that were histologically similar to the earlier lesion. Immunostaining revealed strong positivity for insulin, glucagon, somatostatin, and chromogranin consistent with overgrown pancreatic islets of Langerhans within a mature teratoma.
Atrioventricular Node↗
Rapid molecular genetic subtyping of serotype M1 group A Streptococcus strains.
Serotype M1 group A Streptococcus, the most common cause of invasive disease in many case series, generally have resisted extensive molecular subtyping by standard techniques (e.g., multilocus enzyme electrophoresis, pulsed-field gel electrophoresis). We used automated sequencing of the sic gene encoding streptococcal inhibitor of complement and of a region of the chromosome with direct repeat sequences to unambiguously differentiate 30 M1 isolates recovered from 28 patients in Texas with invasive disease episodes temporally clustered and thought to represent an outbreak. Sequencing of the emm gene was less useful for M1 strain differentiation, and restriction fragment length polymorphism analysis with IS1548 or IS1562 as Southern hybridization probes did not provide epidemiologically useful subtyping information. Sequence polymorphism in the direct repeat region of the chromosome and IS1548 profiling data support the hypothesis that M1 organisms have two main evolutionary lineages marked by the presence or absence of the speA2 allele encoding streptococcal pyrogenic exotoxin A2.
Amino Acid Sequence↗
Ontogeny of renal dysplasia in Ivemark syndrome: light and immunohistochemical characterization.
Ivemark syndrome is a rare sporadic or autosomal recessive disorder characterized by pancreatic fibrosis, renal dysplasia and hepatic dysgenesis. There have been no data describing the renal changes during embryologic development in this syndrome. In this report, we document the pathological findings of the kidney in three subjects with Ivemark syndrome: 6 months, 21 weeks and 16 weeks, respectively. Kidneys of subjects and age-matched controls were examined by light microscopy and immunohistochemically for cytokeratin, AE1/AE3 and epithelial membrane antigen. Renal dysplasia in Ivemark syndrome becomes apparent at 16 weeks of gestation and progresses thereafter in severity. It is characterized by disturbance in glomerular differentiation, delay in tubular differentiation and abnormal expression of epithelial markers in glomeruli and tubules. Cytokeratin and epithelial membrane antigen expression of cysts is similar to that of the collecting ducts.
Abnormalities, Multiple↗
Liver pathology in children with AIDS: a comparison between the South American and North American population.
Liver tissue from autopsies of twenty-nine cases of children with AIDS were collected from three major South America (S.4) pediatric hospitals. The hepatopathologic findings were classified in the same fashion as in a series of sixty-one children with AIDS from North America (NA): inflammation, non-specific, lymphoproliferative disorders, and giant cell transformation. By comparing both groups. we noted that the SA children were Younger at time of death consistent with a more rapid progression of the disease. Opportunistic infections varied with a higher prevalence of Cytomegalovirus (CMV) infection in SA children. The histopathologic features of CMV in the liter of SA children were associated with a conspicuous inflammation absent in the NA group. Finally, different non-specific hepatic changes were found in SA children, including one case of peliosis hepatis.
AIDS-Related Opportunistic Infections↗
[Microbeads and nanobeads in microscopy: a tool for spatial, spectral and dynamic settings. Application to molecular co-localization].
This article illustrates a methodology which can be used on cellular and tissular specimens prepared with fluorochromes and analyzed by laser scanning confocal microscopy. Fluorescent beads are used to simulate the fluorochromes and they determine their detectability inside the preparations. They can also be used to verify that the microscopes are set properly so that the resulting images can be analyzed and are reliable to evaluate possible co- localizations. Methods of factor analysis are applied to optical section series obtained on the microscope to characterize the fluorochromes that are used to stain different locations of the specimen. Photophysical properties (emission spectra, decay rates) of the fluorochromes are used to obtain the differentiation. Section series are obtained either by spectral selection via a sequence of filters or by successive scans of the same specimen. Concerning the three- dimensional analysis, differentiation of series that are obtained by z displacement leads to the restoration of focal planes and improves the legibility of specimens. These planes which are not at the same depth when the excitation sources are not aligned can the be superimposed to achieve the co-localization.
Fluorescent Dyes↗
The impact of relationships.
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Attitude of Health Personnel↗
Arteriohepatic dysplasia (Alagille's syndrome): a common cause of conjugated hyperbilirubinemia.
Syndromatic paucity of interlobular bile ducts is a common cause of conjugated hyperbilirubinemia in children. The clinical presentation is not always obvious. Therefore, the liver biopsy may be a useful diagnostic tool in the definition of this entity. The hepatic and biliary morphology of five children with arteriohepatic dysplasia (Allagille' syndrome) is described. Prior to diagnosis, four underwent Kasai procedures after intraoperative cholangiograms failed to demonstrate patency of the extrahepatic bile ducts. In three patients, a focal proximal hypoplasia of the common hepatic duct was demonstrated. Hypoplasia of the gallbladder occurred in two patients. Hepatic features of sequential liver biopsies obtained on the five patients, were divided into early and late changes. From birth to four months of age, the pathology consistent of cholestasis, paucity of interlobular bile ducts and portal fibrosis. The etiology of arteriohepatic dysplasia is unclear. The main pathogenic mechanisms are discussed. It is felt that the syndromatic duct paucity represents an acquired primary ductal defect resulting from a genetically determined immune response to as yet undefined agent or agents.
Bile Ducts↗
Primary multicystic undifferentiated embryonal sarcoma of the liver in an adult presenting with peripheral eosinophilia.
Primary undifferentiated embryonal sarcoma of liver is an extremely rare pathologic entity in the adult population. An unusual case is reported of a 44-year-old female who presented with multiple hepatic cysts and peripheral eosinophilia, initially thought to be hydatid disease of the liver. Angiogram revealed neovascularization. Surgical biopsy showed an anaplastic mesenchymal neoplasm. Pathologic findings are presented and the relevant literature reviewed. Hepatic undifferentiated embryonal sarcoma should be included in the differential diagnosis of eosinophilia accompanying hepatic cysts.
Adult↗
Intrathoracic pleomorphic liposarcoma in a child: study of an unusual case.
Primary intrathoracic liposarcoma is an extremely rare neoplasm in the pediatric age group. We report an unusual case of a 3-year-old boy who presented with a large intrathoracic tumor arising from the right chest wall. Biopsy showed a pleomorphic sarcoma with histological, immunohistochemical, and ultrastructural features of pleomorphic liposarcoma. The tumor displayed an aggressive clinical behavior, and the patient continued to have recurrences despite rigorous chemotherapy and radiation treatment. He subsequently died 2 years after the initial diagnosis.
Child, Preschool↗
Nonteratoid medulloepithelioma of the retina with electron microscopic and immunohistochemical characterization.
Medulloepitheliomas are rare intraocular tumors, predominant in children, and originate mainly from undifferentiated nonpigmented epithelium of the ciliary body. These tumors rarely involve the optic nerve or the retina. They are classified as nonteratoid and teratoid types; the latter contains heterologous tissues. The teratoid variant of medulloepitheliomas involving the optic nerve or the retina is reported in four patients only. We describe the first case of a benign nonteratoid medulloepithelioma of the retina in a 3 1/2-year-old girl with immunohistochemical and electron microscopic characterization.
Child, Preschool↗
Gastrointestinal manifestations in pediatric AIDS.
The pathologic changes in the gastrointestinal tract of children with AIDS are variable, clinically significant, and reflect multisystemic disease processes. Inflammation, changes in the lymphoid tissue, miscellaneous lesions, and tumors are documented in 58 patients in addition to cases reported in the literature. Cytomegalovirus infection of the gastrointestinal tract, associated with ulcerations, hemorrhage, perforations, and intestinal obstruction, carries a high morbidity and mortality, whereas the remaining infections are not life threatening. Special stains and electron micrographic examination are important to identify correctly certain microorganisms such as mycobacterium avium intracellulare, cryptosporidia, and microsporidia. Lymphoproliferative changes of the gastrointestinal tract, a component of the generalized lymphoproliferative process, need to be characterized by tumor markers and cytogenetic studies. Within the miscellaneous lesions, AIDS associated arteriopathy can be complicated by intestinal ulceration and perforation. Both lymphomas and smooth muscle tumor in children with AIDS are related to Epstein-Barr virus infection. The smooth muscle tumors are frequently malignant and multiple.
AIDS-Related Opportunistic Infections↗