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Biomedical subjects

B E Favara

Publications and source records attributed to B E Favara.

At least 37 records · Page 2Linked to original sources

Pediatric pathology and the autopsy.

The damaging effects of low and declining autopsy rates on the discipline of pediatric pathology are discussed. A survey of autopsy rates in 25 children's hospitals indicates that current rates are about 51%. Strategies for improving autopsy rates are presented.

Adolescent↗

Anitschkow nuclear structure: a study of pediatric hearts.

Anitschkow nuclear structures are commonly found in hearts of children who have died of various disorders. The peculiar chromatin pattern, as shown by immunohistochemical methods, occurs in striated muscle cells, histiocytes, Schwann cells, nondescript mesenchymal cells of the heart, and, rarely, cells outside of the heart. They are most abundant in the atrioventricular valves and in valve rings of premature infants. The histogenesis and significance of these unique nuclei are unknown, but their association with hypoxia and their similarity to forms seen in cell division suggest that the presence of increased numbers of Anitschkow nuclear structures may be a response to cardiac injury.

Cardiomyopathies↗

Pathology of Langerhans cell histiocytosis.

The spectrum of the histopathology of Langerhans cell histiocytosis was presented with special attention paid to the pathologic Langerhans cell as the diagnostic lesional cell. Features that distinguish this histiocyte from others are defined by electron microscopic demonstration of Birbeck granules and by immunohistochemical characteristics such as possession of antigenic determinant(s) that react with the monoclonal antibody T6. The relationship between Langerhans cells and the other histiocytes from which they are derived seems to be determined by events initiated by surface antigens reacting with specific antibodies. Immunohistochemical tests that are of value in diagnosing and classifying the various histiocytosis syndromes include those used to identify the S-100 antigen, T6 antigenic determinant(s), and peanut agglutinin binding sites. The use of a standard nomenclature and diagnostic criteria for the histiocytosis syndromes is encouraged as a step forward in progress to better understand and communicate about these puzzling disorders.

Cell Differentiation↗

Advances in the care of the child with cancer. The importance of histologic subclassification of tumors.

The importance of histologic subclassification of tumors lies principally in the correlation between nosology and tumor biology, including response to therapy. Subclassifications that are practically achieved, reproducible and uniquely predictive are of great value. As new methods are engaged and new clinical data are reported, classifications may be changed. Subclassifications of solid tumors with proven therapeutic application are exemplified by renal tumors and lymphomas that are commonly referred to as "favorable" or "unfavorable histology." Subclassification of soft tissue sarcomas, neuroblastoma and tumors of the central nervous system are being investigated, but are presently of undetermined relevance. Classifications and subclassifications of solid tumors of children are presented in the context of prognostic relevance.

Adrenal Cortex Neoplasms↗

Diagnostic histopathologic criteria in pediatric tumors: an eclectic review.

Malignant tumors of infants and children, unlike their adult counterparts, are commonly undifferentiated or show minimal evidence of differentiation. Because of this, many of these tumors pose a formidable dilemma to the general pathologist in distinguishing one from another. A broad sampling of the more common malignant neoplasms and of newly recognized histologic subtypes which are restricted more or less to the pediatric age group is presented. The minimal microscopic criteria required to establish a diagnosis are given using widely accepted pathologic classifications and seminal journal references as a guide. Ancillary microscopic features, helpful histochemical stains, immunohistochemistry, and ultrastructural morphology are described for most tumors. Histologic distinction from diagnostic mimickers is discussed. Where appropriate, histologic grading and tumor subclassification are presented and their prognostic relevance is reviewed.

Biopsy↗

S-100 protein immunohistochemistry and electron microscopy in the diagnosis of Langerhans cell proliferative disorders: a comparative assessment.

To better define the roles of S-100 protein immunohistochemistry and electron microscopy in the diagnosis of Langerhans cell proliferative disorders, a comparative assessment of the two techniques was performed using material from 39 cases of histiocytosis X and 2 cases of infantile self-healing reticulohistiocytoma. Both techniques proved highly reliable, but neither alone enabled diagnostic confirmation in all instances. The two techniques proved complementary and used together did enable identification of Langerhans-type histiocytes in all cases studied. Neither was judged clearly superior and both offered certain advantages. Electron microscopy was found overall to be a slightly less sensitive technique, but more specific and less subject to misinterpretation. The S-100 stain was found to be particularly useful in situations where sampling problems were likely to be encountered or where the available specimen was otherwise suboptimal for electron microscopic examination. Because the S-100 stain is the more cost-effective to employ, we now recommend it for the purpose of providing routine diagnostic confirmation. In an investigative setting, however, we continue to recommend electron microscopy as the primary technique.

Adolescent↗

Malignant peritoneal mesothelioma in a child.

Malignant mesothelioma of the peritoneum in young children is very rare. We describe a fatal case in a child who presented with groin swellings at age 9 years 9 months. Initial histology simulated an inflammatory mesothelial hyperplasia. After a prolonged response to steroids, masses that had the histology of mixed epithelial and mesenchymal malignant mesothelioma became apparent in the abdomen and pleura. There was no significant response to subsequent chemotherapy. No definite etiologic factors were identified. Pathologists should be aware that this tumor may rarely present in young children and may have a significant inflammatory component. Etiologic considerations are discussed.

Ascites↗

Histiocytosis X.

To clarify salient issues pertaining to histiocytosis X--a syndrome that includes Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma--the authors review the epidemiologic data and the histologic, morphologic, and clinical bases for diagnosis and prognosis. Histiocytes are defined and their possible histogenesis outlined, and Langerhans cells, which may be a leading element in active lesions, are characterized. The authors outline hypothetic pathogenetic schema, which they recommend be tested by recently developed immunologic and genetic means, since histiocytosis X, at least in its disseminated form, remains an unpredictable disease for which there is no proven effective therapy.

Adolescent↗

Rhabdomyosarcoma in children: ultrastructural study of 31 cases.

Thirty-one cases of rhabdomyosarcoma in children were studied by electron microscopy. All of the tumors examined fit into a continuum of progressive cellular differentiation mimicking that of normal myogenesis, with increasingly differentiated examples being decreasingly common. We conclude that all childhood forms of rhabdomyosarcoma are essentially embryonal tumors, with many not being sufficiently differentiated at the cellular level to allow positive ultrastructural diagnosis.

Child↗

Fatal airsacculitis and pneumonia, with abortion, in an orangutan.

A 14-year-old, 76-kg female orangutan (Pongo pygmaeus) aborted a premature fetus and within hours experienced severe respiratory distress and died. Necropsy revealed evidence of chronic airsacculitis, acute bronchopneumonia, and terminal sepsis. Escherichia coli was isolated from cultures of heart blood, tracheal fluid, air-sac tissue and fluid, lung, and bile. Retrospectively, it was established that the animal had had a periodic nasal discharge for many months. The aborted infant had no evidence of infection.

Abortion, Veterinary↗

Successful treatment of lymphohistiocytic reticulosis with phagocytosis with epipodophyllotoxin VP 16-213.

Lymphohistiocytic reticulosis with phagocytosis is a rare, familial disorder affecting infants and children. It is characterized by fever, pancytopenia, hepatosplenomegaly, and a rapidly fatal course. Prior attempts to treat this disease have been unsuccessful. We describe two patients with lymphohistiocytic reticulosis with phagocytosis and hyperlipidemia. A sibling of one patient had died of the same disease. One patient also had abnormal lymphocyte response to mitogens. Both patients who were treated with epipodophyllotoxin VP 16-213 (VP-16) had remission of their disease and resolution of hyperlipidemia. VP-16 appears to be an effective agent for treating lymphohistiocytic reticulosis with phagocytosis.

Blood Cell Count↗

Foamy changes of placental cells in fetal storage disorders.

Vacuolar cytoplasm in placental cells was observed in four cases. In each instance the baby was either stillborn or died soon after birth. One infant was shown to have Inclusion cell disease (Mucolipidosis II), whereas in the other cases biochemical studies were noncontributory or could not be carried out. The histologic changes of the placenta described in this report were restricted to the fetal elements and also included the X cells, providing further evidence that these cells are of fetal rather than maternal origin.

Adult↗