Long-term penicillamine therapy for Wilson's disease.
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Biomedical subjects
Publications and source records attributed to A Flint.
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A case of inflammatory fibrous histiocytoma arising in soft tissue near the sacrum is presented. The patient's mode of presentation, clinical course, and tumor histology were typical of this disease. The tumor was inoperable, and radiotherapy combined with doxorubicin, cyclophosphamide, and intermediate-dose oral methotrexate produced a dramatic complete response lasting over 27 months. Favorable results with nonsurgical therapy have not previously been reported for this disease. The gratifying result obtained, although of relatively short duration to date, indicates that combined modality therapy may provide significant back-up to aggressive surgery. The use of these agents in an adjuvant setting merits study.
We studied 680 patients with Hodgkin's disease, treated at Stanford University Medical Center from July 1, 1968, through December 31, 1975, to determine the risk of development of hematologic neoplasia. Six cases of leukemia occurred in patients in clinical remission, one 7 1/2 years after diagnosis. Two additional cases occurred in patients with active Hodgkin's disease. No cases were seen in 320 patients treated with radiotherapy alone or in 30 treated with chemotherapy alone. A single case of subacute leukemia occurred in a patient treated initially with radiation therapy and colloidal gold. The actuarial probability of development of leukemia at five and seven years is 1.5 and 2.0 per cent for the entire group and 2.9 and 3.9 per cent for the 330 patients treated with combined radiation and chemotherapy. The medium survival after diagnosis is four months, with no patient living beyond six months.
The characteristic oily skin in individuals with parkinsonism has long been observed by clinicians. The oiliness seems to be associated with periods when the disease is most active. This seborrhea has been observed particularly in post-encephalitic parkinsonism, as well as in idiopathic paralysis agitans. It also occurs in phenothiazine-induced parkinsonism.
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OBJECTIVES: We employed a neuroendocrine challenge paradigm to study serotonergic abnormalities associated with poststroke depression. METHOD: Twelve depressed stroke patients (major depression N= 5, minor depression N = 7), 8 nondepressed stroke patients and 12 healthy volunteers completed a single-blind, placebo-controlled, challenge tests. Baseline cortisol (CORT) and prolactin (PRL) values, and these hormonal responses to 30 mg of oral d-FEN and placebo over a 4 hour period were measured in the three groups. RESULTS: There were intergroup differences for baseline adjusted PRL responses (change scores from baseline) to d-FEN (group effect F = 4.38, df = 2,29, p = 0.02) while these responses to placebo were comparable between groups (group effect F = 1.82, df = 2,29, p = 0.18). Peak PRL responses (post d-FEN maximal PRL change from baseline scores) in depressed stroke patients were significantly greater than in nondepressed patients (p = 0.005) but comparable to healthy normals (p = 0.47). However, these responses between major and minor depression were not significant (p = 0.34). There was a trend suggesting a negative correlation between peak PRL response and severity of depression (p = 0.056). Depressed patients were younger than the controls (p = 0.054). Also, the depressed group was more functionally impaired (p = 0.04) and more likely to have right-sided lesions (p = 0.009) compared with the nondepressed group. Differences in baseline adjusted PRL changes between depressed and nondepressed groups became non significant when the influence of laterality of lesions was covaried, whereas covariation of functional scores and age did not alter the significance. CORT responses did not show intergroup differences. LIMITATIONS: The study group was small and was heterogenous in lesion characteristics, time since stroke and type of depression. A fixed-order design was used in the challenge test paradigm. CONCLUSIONS: When laterality of stroke lesion was taken into account, depressed and nondepressed stroke patients did not differ in PRL responses to d-FEN.
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Based upon a review of cytologic specimens obtained from 46 patients, the morphologic characteristics of metastatic colon carcinoma and primary pulmonary adenocarcinoma were compared and contrasted. A monoclonal antibody (D-14) reported to be helpful in identifying colorectal carcinoma was also evaluated. Colon brushings and washings from 10 patients with colonic carcinoma and a variety of respiratory tract cytology samples from 16 and 20 patients with metastatic colonic and primary pulmonary adenocarcinomas, respectively, formed the basis of this review. The presence of well-formed glands and dirty necrosis was significantly more characteristic of colon carcinoma. Other cytologic features and D-14 staining were not sufficiently distinctive to allow a separation from primary pulmonary adenocarcinoma.
The cytologic appearances of two examples of adenoma of the nipple are described. The cellular samples from both lesions contained large numbers of epithelial cells, which were present both singly and in numerous clusters. The uniform nuclei contained finely distributed chromatin and inconspicuous nucleoli. Some of the cells varied in size and shape; a few had hyperchromatic nuclei. Small amounts of cellular debris, inflammatory cells and hemosiderophages were also present. Adenoma of the nipple can usually be distinguished cytologically from other lesions that may involve the nipple, such as Paget's disease, adenocarcinoma, fibrocystic disease, mammary duct ectasia, intraductal papilloma and chronic subareolar abscess; the cellular features of fibroadenoma, however, overlap those of adenoma and may cause diagnostic confusion. The increasing use of cellular samples obtained from breast lesions underscores the importance of recognizing this uncommon neoplasm and its cytologic manifestations. Adenoma of the nipple can be treated successfully by limited resection, and preoperative recognition will obviate unnecessary extensive surgery.
The cytologic manifestations of pulmonary Hodgkin's disease in transthoracic fine needle aspirates from 13 patients with pulmonary radiologic abnormalities and a previous diagnosis of Hodgkin's disease are described. Classic Reed-Sternberg cells and lacunar cells were present in most cases. The so-called "mononuclear" Reed-Sternberg cells were identified in all cases. A cellular background consisting of variable numbers of histiocytes, eosinophilic and neutrophilic leukocytes and lymphocytes was frequently present. Such a background should stimulate a search for cells diagnostic of Hodgkin's disease. We conclude that the cytologic features of Hodgkin's disease are not only characteristic, but are also diagnostic, in patients with a prior history of Hodgkin's disease in whom pulmonary recurrence is suspected.
The papillary variant of renal-cell carcinoma is characterized by distinctive histologic, clinical and angiographic features. A study was undertaken to delineate the cytologic features of this tumor as it is encountered in cellular samples. Cytologic specimens containing tumor cells from eight patients who underwent resection for papillary renal-cell carcinoma were examined and compared to corresponding cytologic samples obtained from ten other patients who had nonpapillary renal-cell carcinoma. The cytologic appearance of papillary renal-cell carcinoma, which is deceptively benign, is marked by distinctive papillary structures that often resemble branched chains. The cells are usually small and contain uniform nuclei; numerous macrophages with foamy cytoplasm are often found in the background. These cytologic features were not observed in the cellular specimens from the nonpapillary renal carcinomas. We conclude that papillary renal-cell carcinoma can be confidently recognized in cytologic specimens.
The cytologic changes of pregnancy and the postpartum period are presented. Atypical cells were identified in 12% of smears; the majority of these atypical cells were related to degenerating decidual cells. Cells thought to represent changes due to folic acid deficiency are also described, and the features that help separate decidual cells from other cell types in the genital tract are illustrated. True neoplasia, when present, tended to persist after delivery, and these cells should not be confused with pregnancy-associated atypia.
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