Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reported 29 kidneys with angiomyolipoma in 23 patients (14 women and 9 men). One of these cases was associated with tuberous sclerosis. Their age ranged from 20 to 82 years (mean 48.9). Six patients had bilateral renal involvement and 7 had multiple lesions in one kidney. Synchronous renal tumors were noted in three patients. One patient was associated with renal cell carcinoma, and the other two patients with transitional cell carcinoma. Nineteen tumors in 16 patients were larger than 4 cm. Among them 93.8% (15/16 patients) were symptomatic and tumor hemorrhage was found in 57.9% (11/19 kidneys). Of these patients, 17 renal units underwent surgical intervention (including 8 total nephrectomy, 7 partial nephrectomy and 2 enucleation of tumor). Preoperative diagnosis was made in 82.4% (14/17) with combined imaging of sonogram and CT scan. One patient received embolization only. There were two mortalities: one patient with tumor rupture who received emergent nephrectomy but died of irreversible hypovolemic shock, and the other with tuberous sclerosis and pulmonary fibrosis who received embolization for spontaneous rupture of tumor but expired due to respiratory failure. Among nine patients receiving renal preserving operation, the post-operative effective renal plasma flow of the affected kidneys ranged from 96 to 184 ml/min (mean 131.7 ml/min). There was no evidence of recurrence during a mean followup of 2.6 years. Eight asymptomatic patients with 9 renal masses had incidental angiomyolipoma and did not undergo exploration. We did not note any progression of the lesions in the cases under observation for a mean followup of 2.6 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal angiomyolipoma: experience of 23 patients. 165 40

Bleomycin is an agent with significant antitumor efficacy whose major dose limiting toxicity is pulmonary fibrosis. Attempts have thus been made to identify congeners with reduced toxicity and with comparable or greater antitumor activity. Tallysomycin S10b is a bleomycin analogue possessing significantly greater potency, equal or reduced lung toxicity, and slightly greater antineoplastic activity when compared to the parent compound in preclinical studies. This report describes our experience with tallysomycin S10b in 30 patients with a variety of non-hematologic neoplasms. Pulmonary toxicity, occurring in 4 patients, was the major toxicity. The recommended cumulative dose of tallysomycin S10b was difficult to establish from the results of this study, as pulmonary toxicity appeared to be more idiosyncratic than dose- or schedule-dependent. The employment of more sensitive methods for detecting pulmonary toxicity in this study suggest that tallysomycin S10b may have reduced pulmonary toxicity compared to the parent compound. Both bleomycin and tallysomycin S10b have similar t1/2 beta half-lives of 2-4 h. Six patients had prolonged terminal elimination half-lives of tallysomycin S10b, but no clear relationship between this phenomenon and efficacy or toxicity was evident. No complete or partial responses occurred. Disease stabilization occurred in 4 of 15 patients with diagnoses of renal cell carcinoma, rectal cancer and lung cancer. Five of eight patients with non-measurable disease had stable disease, including one with mesothelioma, one with carcinoma of the head and neck, two with renal cell cancer and one with colon carcinoma.
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PMID:Phase I trial of tallysomycin S10b, a bleomycin analogue. 169 67

The members of the NOX/DUOX family of NADPH oxidases mediate such physiologic functions as host defense, cell signaling, and thyroid hormone biosynthesis through the generation of reactive oxygen species (ROS), including superoxide anion and hydrogen peroxide. Moreover, ROS are involved in a broad range of fundamental biochemical and cellular processes, and data accumulated in recent years indicate that the NOX enzymes comprise one of the most important biological sources of ROS. Given the high biochemical reactivity of ROS, it is not surprising that they have been implicated in a wide variety of pathologies and diseases. Prominent among the settings that feature ROS-mediated tissue injury are disorders associated with inflammation, aging, and progressive degenerative changes in cells and organ systems, and it appears that essentially no organ system is exempt. Among the disorders currently believed to be mediated at least in part by NOX-derived ROS are hypertension, aortic aneurysm, myocardial infarction (and other ischemia-reperfusion disorders), pulmonary fibrosis and hypertension, amyotropic lateral sclerosis, Alzheimer's disease, Parkinson's disease, ischemic stroke, diabetic nephropathy, and renal cell carcinoma. Several small-molecule and peptide inhibitors of the NOX enzymes have been useful in experimental studies, but issues of specificity, potency, and toxicity militate against any of the existing published compounds as candidates for drug development. Given the broad array of disease targets documented in recent work, the time is here for vigorous efforts to develop clinically useful inhibitors of the NOX enzymes. As most (though not all) NOX-related diseases appear to be mediated by a single member of the NOX family, agents with isoform specificity will be preferred, although broadly active NOX inhibitors may prove to be useful in some settings.
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PMID:NOX enzymes as novel targets for drug development. 1850 46

A 46-year-old woman known with relapsing Hodgkin's lymphoma diagnosed at age 5, treated with repeated cycles of radiotherapy and chemotherapy, presented with severe symptomatic radiation-induced aortic stenosis. She also had other late sequelae of radiotherapy including thyroid cancer, mediastinal fribrosis and left pulmonary fibrosis with severe restrictive lung disease and a newly diagnosed renal carcinoma. Due to the prohibitively high surgical risk and need for urgent treatment, she underwent successful transcatheter aortic valve replacement with transfemoral implantation of a 23 mm Edwards SAPIEN-XT prosthesis, which was performed without valvuloplasty of the noncalcified fibrotic valve. The final result was excellent with reduction of the transaortic gradient and no residual aortic regurgitation.
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PMID:Percutaneous valve replacement in a young adult for radiation-induced aortic stenosis. 2245 Aug 61