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Biomedical subjects

B E Favara

Publications and source records attributed to B E Favara.

At least 73 records · Page 4Linked to original sources
PubMed1968

Renal tumors in the neonatal period.

Eight infants with renal tumors diagnosed in the first month of life are presented. Three well-differentiated mesenchymal tumors were studied using angiographic and electron-microscopic techniques in addition to conventional histologic methods. The morphology of these unusual tumors is presented in detail and classification as benign fibromyoma or fibromyomatous hamartoma is defended. An association between renal tumors in the newborn and maternal polyhydramnios is also documented for the first time.

Female↗
PubMedDate not supplied

Histopathology of the liver in histiocytosis syndromes.

Liver biopsies were studied in 47 cases representing various histiocytosis syndromes. These included 32 cases of hemophagocytic syndrome, 11 cases of Langerhans cell histiocysis (LCH), and 4 cases of other histiocytic disorders. All cases of hemophagocytic syndrome, except one with cytomegalovirus infection, displayed portal lymphohistiocytic infiltrates dominated by T lymphocytes. Activation of the hepatic mononuclear phagocytic system (MPS), evidenced by enlarged von Kupffer cells, some of which were hemophagocytic, was seen in 28 cases of hemophagocytic syndrome. Endothelial enlargement, minor degrees of hepatocellular degeneration, and steatosis were also noted. Ten of the 11 cases of LCH also showed activation of the MPS. It was the only lesion in two biopsies. Seven cases demonstrated nonspecific "triaditis." In three this was associated with cholangiocentric and random acinar histiocytic lesions. Evidence of activation of the MPS was also observed in both cases of Rosai-Dorfman disease and was accompanied by acinar histiocytic lesions in one and triaditis in the other. Likewise, both cases of juvenile xanthogranuloma showed activation of the MPS and focal granulomatous lesions. It is concluded that activation of the MPS is a common feature of liver disease in histiocytosis syndromes and that hepatic enlargement may be the result of this process instead of, or in addition to, the liver lesions known to be featured in these disorders. Hepatic lesions of the various histiocytosis syndromes resemble typical lesions in other sites and, in some instances, are accompanied by nonspecific changes. These nonspecific changes may occur in the absence of lesions that are diagnostic or typical of the particular histiocytosis syndrome. The location and character of hepatic lesions are important factors in the significance of liver involvement.

Adolescent↗
PubMedDate not supplied

Criteria for the discontinuation of antibiotic therapy during presumptive treatment of suspected neonatal infection.

Unstable newborns are often subjected to multiple diagnostic tests and then treated presumptively for bacterial infection. Inconclusive test results may perpetuate unnecessary therapy. One hundred twenty-three neonates were prospectively evaluated for infection. Complete physical examinations and chest radiographs were performed. Of ten screening tests only the white blood cell count, absolute band count, absolute band/neutrophil ratio and C-reactive protein showed statistical differences (P less than 0.05) among 32 patients with positive "nonpermissive" (blood, cerebrospinal fluid, suprapubic or catheter urine, needle aspirate, tracheal aspirate) cultures and 50 with negative cultures who had antibiotic therapy discontinued within 72 hours. Forty-one additional patients were continued on therapy despite negative cultures. Incomplete bacteriologic evaluation resulted in unconfirmed "pneumonia" in 16 of these children. No statistical differences between the culture negative groups existed regardless of treatment status, suggesting that patients in the latter group may not have required continued treatment. A definitive, bacteriologic evaluation emphasizing "nonpermissive" cultures should be completed in newborns suspected of infection which, when negative, should allow discontinuation of antibiotics. In equivocal cases, a negative C-reactive protein best supports cessation of therapy. This approach can reduce the total duration of newborn exposure to antibiotics in a high risk nursery by 20%. A prospective, definitive, diagnostic evaluation (emphasizing non-permissive cultures) avoids uninterpretable but frequently used tests and reduces cost while adding confidence in the termination of therapy.

Anti-Bacterial Agents↗
PubMedDate not supplied

An unusual histiocytic disorder responding to cyclosporine therapy: a case report.

PURPOSE: To describe the clinical and pathologic presentation and course of a 7-week-old girl with anemia, thrombocytopenia, and organomegaly who was found to have a histiocytic disorder distinct from previously reported cases. METHODS: Bone marrow specimens were studied with conventional methods. A liver biopsy specimen was evaluated by routine and immunohistochemical methods and electron microscopy. RESULTS: The patient was found to have a unique histiocytic disorder in which lesional cells displayed an atypical phenotype. Cyclosporine therapy was associated with a prompt, complete, and apparently permanent resolution of disease. CONCLUSION: This case appears to represent an atypical histiocytic disorder with unique clinical and pathologic features. The disorder resolved after the initiation of cyclosporine therapy.

Anemia↗
PubMedDate not supplied

Electron microscopy in histiocytosis X.

In an ultrastructural study of 24 cases of histiocytosis X, we were able to demonstrate Langerhans cell granules in all of 18 cases from which tissues had been submitted primarily for electron microscopy and in 4 of 6 cases from which only tissue retrieved from paraffin blocks was available for examination. In a subsequent correlative study we were able to demonstrate Langerhans cell granules in deparaffinized material from 11 of 14 cases (79%) in which they were known to exist. The incidence of histiocytes displaying Langerhans cell granules (in a single plane of section) fell from a median of 48% in samples processed primarily for electron microscopy to 14% in those retrieved from paraffin blocks. Our data suggest that electron microscopy, even when applied to the study of suboptimally preserved material, is a highly sensitive technique for confirming a diagnosis of histiocytosis X.

Adolescent↗
PubMedDate not supplied

Foreign-body pulmonary embolism.

The minimal incidence of pulmonary foreign-body embolism in a general pediatric pathology experience was ascertained by reviewing autopsy records, and the true incidence of pulmonary foreign-body embolism was determined by studying lung sections from 64 autopsies of patients who had undergone cardiac procedures. Seventeen cases of embolism were reported from 370 autopsies, an incidence of 4.6%. The true incidence of pulmonary foreign-body embolism was found to be 21.9% in the cardiac surgical autopsies and the minimal incidence was 5.1% in patients who had other types of surgery. No patients other than those having surgical procedures had embolism. Hair was found to be the embolic material in 35% of cases. Embolic lesions were characterized, staged, and correlated with clinical data. The pathogenesis of this condition is unclear, but it probably involves contamination of surgical materials with particulate matter.

Cardiac Surgical Procedures↗
PubMedDate not supplied

Adrenal cytomegaly: quantitative assessment by image analysis.

Adrenal cytomegaly was found in 23 or 0.8% of 2711 pediatric autopsies, including a 13-year-old girl with leukemia and a 20-year-old woman with cystic fibrosis. Cytomegaly of pancreatic islet and acinar cells and pituitary cells was also noted. DNA quantitation by image analysis indicates that cytomegalic cells of the adrenal cortex, pancreas, and pituitary contain increased amounts of DNA (hyperdiploid aneuploid), with adenocytes containing over 25 times the normal amount of DNA in some instances. These data and morphological features suggest that cytomegaly is a reflection of polyploidy due to either partial DNA replication or partial DNA replication plus other modes of polyploidization. Although the mechanism(s) by which these changes evolve remains unknown, regressive processes do not seem to be directly involved. We speculate that polyploidization is, in this setting, a response to physiological demand, a concept that also has relevance to the interpretation of morphology and DNA ploidy profiles of endocrine tumors.

Adolescent↗
PubMedDate not supplied

Langerhans cell histiocytosis of lymph nodes: a morphological assessment of 43 biopsies.

The morphology of Langerhans cell histiocytosis (LCH) involving lymph nodes was analyzed in 43 biopsies from 39 patients and findings were correlated with clinical data. Five histological motifs were recognized: sinusoidal, limited sinusoidal, epithelioid granulomatous, partial effacement, and total effacement. Lesions were composed of histiocytes of the Langerhans cell (LC) family, macrophages, multinucleated giant histiocytes, T lymphocytes, and eosinophils in varying proportions. Proliferative fractions ranged from 2.6 to 48% and 2 of 25 specimens showed a hyperdiploid aneuploid DNA ploidy profile. Epithelioid granulomas composed of histiocytes with the LC phenotype dominated three abdominal specimens, reflecting a picture of LCH not previously reported. Total effacement seen in three patients, was associated with unmarked histiocytoid cells, high proliferative fraction, aneuploid DNA ploidy profile, and, in two, a fatal outcome. Different histologic appearances in lesions from separate sites of the same patient were seen in the cases with epithelioid granulomas and in those with total effacement. The diverse histopathology in lymph nodes involved by LCH is considered in the context of current knowledge of this enigmatic disease.

Adolescent↗