Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of cancer, each associated with a different collagen-vascular disease, are reported. The first patient, a 71-year-old white man, had a history of acute dermatomyositis and malignancy for a few weeks only. Death was associated with adenocarcinoma of the lesser curvature of the stomach with metastases to the liver and beyond. The second patient, a 69-year-old white man, had had symptoms associated with Raynaud's phenomenon for more than a decade and difficulty in swallowing, attributed to progressive systemic sclerosis for more than two years. He died with an epidermoid carcinoma of the esophagus with extension to the lung and metastases to the liver and lungs.
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PMID:Cancer associated with collagen-vascular disease. 42 89

From 1969-1974 1000 unselected enucleated globes have been examined histopathologically. 277 derive from the University Eye Hospital in Hamburg, 723 from various Eye Hospitals in northern and southern Germany. They originate from 589 men and 408 women, three times the sex was unknown. 86 globes had to be removed from children less than 15 years old. 6 groups of etiologies have been distinguished: trauma (308), histologically confirmed neoplastic disease (281), ocular manifestations of systemic diseases (diabetes mellitus, occlusions of central retinal vessels presumably following generalized vascular disease etc.: 128), "operative ocular disease" (164), primary inflammatory disease (71), miscellaneous (malformations, high myopia, pseudo-glioma and pseudo-melanoma: 48). The etiology "operative ocular disease" consists of 67 primary glaucomas (57 adults, 10 buphthalmus), 41 idiopathic cataracts (7 of these congenital) and 3 primary corneal dystrophies, as well as 53 cases of primary retinal detachment. Among the 281 neoplastic diseases, there are 238 primary intraocular malignant melanomas of the uvea, 18 retinoblastomas, 4 primary reticulumcellsarcomas of the retina, 2 choroidal nevi, 10 intraocular metastases and 9 orbital tumors. 16 enucleations among the 1000 enucleations have been performed for pseudo-gliomas (5 x Coats disease, 5 x persistent primary hyperplastic vitreous, 2 x retrolental fibroplasia, others 4 x). The manifestations of systemic disease are consisting of 68 central retinal vein-occlusions, 30 complications of diabetes mellitus and 10 central retinal artery occlusions as well as 20 other generalized diseases. A primary inflammatory disease led to enucleation 50 times due to an intraocular process, 5 times due to scleritis and 18 times as a consequence of keratitis (including 13 times herpes simplex). As the final clinical cause for enucleation the following categories have been elaborated: secondary glaucomas (416), clinical diagnosis of "tumor" (275), atrophy and phthisis bulbi (118), inflammation (112), acute trauma to 4 weeks after the accident (72), others (7). In conclusion the central role of rubeosis iridis leading to secondary angle closure glaucoma is emphasized. This process presents a challenge to ophthalmologic research. Finally the significance of early surgery for primary angle closure glaucomas and for complete restoration of the anterior chamber after trauma and any intraocular procedure is stressed.
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PMID:[Etiology and final clinical cause for 1000 enucleations. (A clinico-pathologic study) (author's transl)]. 95 59

The authors describe a juxtaglomerular cell tumor (JGCT) which caused severe hypertension in a 58-year-old man. Light microscopy showed a circumscribed tumor composed of interlacing cords and occasional nodules of relatively uniform cells with no mitotic activity. Rhomboid crystals characteristic of "prerenin" were present within the cytoplasm of tumor cells, and there was a close relationship between the tumor and unmyelinated nerve axons. Intracytoplasmic renin was demonstrated by immunofluorescence, and tumor granules were shown to contain zinc by electron-beam microanalysis. Review of 14 prior cases, with additional follow-up of 9, showed that no patient had developed recurrence, metastasis, or another tumor. Four patients, however, are hypertensive but probably because of secondary tumor effects rather than recurrent hyperreninism. The distinction of JGCT from hemangiopericytoma with renal involvement is important because of the high mortality associated with the latter. The authors conclude that JGCT is benign, but patients with JGCT may remain hypertensive postnephrectomy because of hypertensive angiopathy.
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PMID:Juxtaglomerular cell tumor of the kidney. 636 18

This report documents the results of therapy in 23 patients treated for malignant thymoma between 1944 and 1979. Of the group, 22 patients had neoplasms which invaded mediastinal structures; six had distant metastases. Four patients had myasthenia gravis and one had erythroid hypoplasia associated with collagen vascular disease. No deaths were associated with primary therapy, which included an operative procedure in all cases. Follow-up ranged from 4 months to 18 years (mean 5.63 +/- 1.03 years, SEM). Fifteen patients died, with postoperative survival times ranging from 4 months to 18 years (mean 3.8 +/- 1.27 years). Five patients were alive without recurrence from 3 to 11 years postoperatively (mean 6.8 +/- 1.36 years), and three patients were alive with recurrence or distant metastases from 4 to 17 years postoperatively (mean 10.75 +/- 2.66 years). Differences in survival on the basis of tumor cell type were not statistically significant. Therapeutic groups were analyzed for 5 year survivors, tumor deaths within 5 years of therapy, deaths due to other causes, deaths due to tumor after 5 years, those presently alive, and longest known survivor. The data suggest that complete surgical excision offers the best chance of long-term survival when compared to partial resection plus irradiation (p less than 0.05). No statistical significance could be demonstrated between the groups who had complete resection with versus without postoperative irradiation. There also was no statistically significant difference between the group of patients receiving irradiation following partial excision of most of their tumor and the group receiving irradiation following only biopsy of the lesion. This observation suggests there is no value in so-called "debulking procedures" and suggests that irradiation may be of value in local control of thymoma. Perpetual surveillance is necessary since late recurrence is common.
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PMID:Management of patients with malignant thymoma. 669 21

427 radionuclide brain scans, performed in 1981 at Guy's Hospital Nuclear Medicine Department, were reviewed retrospectively to define clinical circumstances in which the study provided useful information. It was concluded that the radionuclide brain scan was the appropriate first line investigation in patients with known non-cerebral malignancy, in whom the possibility of cerebral metastases exists; and in patients with a low to moderate probability of subdural haematoma. The radionuclide brain scan can provide a valuable alternative to computed tomography studies:--(1) in patients without known underlying disease who develop localising signs; (2) in patients with focal fits; (3) in patients with underlying vascular disease and gradual onset of localising signs; and (4) in patients with suspected inflammatory conditions of the central nervous system. Outside these groups, the radionuclide brain scan rarely provided useful information.
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PMID:Clinical indications for optimal use of the radionuclide brain scan. 685 Feb 24

Familiar Amyloid Polyneuropathy (FAP), an autosomal dominant inherited multisystemic disorder was first observed by Corino de Andrade, a Portuguese neurologist, in 1939. This disease of Portuguese origin was probably spread by fishermen, mainly to Sweden and Japan. It is characterized by a progressive peripheral polyneuropathy and autonomic neuropathy (erectile sexual disfunction, gastrointestinal disfunction, bladder dysfunction and cardio vascular disease) and malnutrition. There are neural and systemic amiloid deposits. Type I FAP, of Portuguese origin, is the most common variety. The amyloid protein is the variant transthyretin (TTR) in which methionine (MET) is a substitute for valine in position 30 (TTR MET 30). It is mainly produced by the liver (90%) and, in small amounts, by the choroidal plexus. Symptoms usually start in the 3rd and 4th decade of life and the patients usually die within 10-15 years. From the therapeutic options--plasmapheresis, immunoadsorption and liver transplantation; the latter seems to be the only one, which stops the production of TTR MET 30 in a permanent way, by means of the liver. The lack of any other effective therapy and the success of the first liver transplantation performed in Sweden arouse great hope. So far, around 300 patients have been transplanted all over the world. A hundred and thirty of them were transplanted in Portugal. A Kaplan Meier survival curve of the Portuguese patients shows a survival rate of 78% at 5 years. However, in spite of the progression of the disease being halted, the irreversibility of some neurological lesions seems to persist. This fact raises the problem of the timing of the transplantation. It seems that the patients should be transplanted as soon as the symptoms start, since mortality and severe morbidity seems to mainly involve those in whom symptomatic disease has lasted longer than six years. As the explanted liver is a morphologic normal liver, a sequential (domino) transplant has been carried out in 16 cases so far done--by one of the authors (ALF) on patients with either hepatocellular carcinoma or liver metastatic disease.
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PMID:Liver transplantation for familial amyloid polyneuropathy. 984 68

In the middle third of the 20th century, Mount Sinai radiologists were able to describe and establish specific radiologic criteria for the diagnosis of many gastrointestinal diseases. They then delineated specific radiologic patterns to diagnose such diverse conditions as inflammatory bowel disease of the small bowel and colon, protein-losing disorders, vascular disease of the small bowel, benign and malignant tumors, metastases and lymphoma of the gastrointestinal tract. Richard H. Marshak, Bernard S. Wolf, and Mansho T. Khilnani were the leaders in these radiologic investigations which established criteria that enabled generations of subsequent radiologists to arrive at definitive diagnoses.
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PMID:Pioneer gastroenterological radiologic studies. 1082 5

Gene therapy directed specifically to the vascular wall, particularly to angiogenic endothelial cells is a prerequisite in vascular disease treatment. Angiogenesis is a major feature in many pathological conditions including wound healing, solid tumors, developing metastases, ischemic heart diseases and diabetic retinopathy. In the present study we developed a tissue-specific gene therapy to the angiogenic blood vessels of tumor metastasis using an adeno-based vector containing the murine preproendothelin-1 (PPE-1) promoter. Genes activated by the PPE-1 promoter were highly expressed in bovine aortic endothelial cells in vitro. Systemic injection of the adenoviral vectors AdPPE-1-luciferase and AdCMV-luciferase to normal C57BL/6 mice, resulted in higher activity of PPE-1 promoter compared with CMV promoter in the aorta and vascularized tissues such as heart, kidney, lung and pancreas. Systemic administration of the adenoviral vector, in mice bearing Lewis lung carcinoma, resulted in high and specific activity of PPE-1 in the new vasculature of primary tumors and lung metastasis. Cellular distribution of the delivered gene revealed highest expression of GFP in angiogenic endothelial cells of the metastasis. We expect that this approach of 'vascular-directed' gene therapy will be applicable to both vascular diseases and cancer.
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PMID:Tissue-specific gene therapy directed to tumor angiogenesis. 1142 29

As well as the long-T2 relaxation components normally detected with conventional imaging techniques, the brain has short-T2 components. We wished to use ultra-short (0.08 ms) echo time (UTE) pulse sequences to assess the feasibility of imaging these in normal subjects and patients. UTE sequences were employed, with or without fat suppression, 90 degree long-T2 suppression pulses, and selective nulling of long-T2 components using an inversion pulse. Subtraction of later echoes from the first was also used to reduce the signal from long-T2 components. We studied dive normal subjects and 15 patients with various diseases. Short-T2 components were demonstrated in grey and white matter. Increased signal from these components was seen in meningeal disease, probable calcification, presumed cavernomas, melanoma metastases and probable gliosis. Reduced signal was seen in some tumours, infarcts, mild multifocal vascular disease and vasogenic oedema. Further development and evaluation of these pulse sequences is warranted.
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PMID:MRI of the brain with ultra-short echo-time pulse sequences. 1450 20

Sixteen patients with ballism not related to vascular disease are reported. Ballism was caused by subthalamic metastases and cerebral tumours in four patients, lesions after functional stereotaxy in three, presumed neurodegenerative disease in two, and by an ipsilateral intraventricular cyst after resection of a meningioma, cerebral toxoplasmosis with AIDS, severe head trauma and sepsis, late recurrence of rheumatic fever, meningoencephalitis, perinatal hypoxia, and conversion syndrome in one, respectively. Two patients had bilateral ballism, 11 had hemiballism, and three had monoballism. Involvement of the contralateral subthalamic nucleus was found in 10/13 patients with symptomatic unilateral ballism. One patient with presumed neurodegenerative disease had bilateral alterations of caudate and putamen on MR. The effect of different treatment strategies was evaluated. Treatment was directed to the underlying disease and/ or to the movement disorder. Response to pharmacotherapy was poor except in one patient. Five patients underwent various neurosurgical interventions. Functional stereotactic operations were performed in eight patients. Lesions were placed in the contralateral ventrolateral thalamus and the zona incerta, the internal pallidum, and in the zona incerta and the pulvinar thalami. Four out of eight patients had complete sustained relief of hemiballism after the operation. No patient was lost to follow-up, which ranged from 3 months to 27 years. After various therapeutic strategies ballism was no longer present in 10 patients and had improved in three, while another three patients did not benefit from therapy. Review of the literature illustrates the shift of aetiologic factors over decades underlying this rare symptom. A multidisciplinary approach should be considered in these patients to alleviate the severe and disabling movement disorder.
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PMID:Ballism not related to vascular disease: A report of 16 patients and review of the literature. 1859 Oct 15


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