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

From 1977 to 1988, 215 patients with a diagnosis of testicular seminoma were referred to the University Hospital, Hamburg, West Germany, for radiation therapy (RT). In 15 patients a careful review of the histologic condition showed signs of embryonal cell carcinoma. Three patients refused completion of therapy. No patient was lost to follow-up. On this basis, a retrospective review of 197 patients was carried out. One hundred thirty-three patients were classified as Stage I (67%), 39 as Stage II (20%), 8 as Stage III (4%), and 17 as Stage IV (9%). One hundred eighty patients had classic seminoma and 17 had anaplastic seminoma. All patients underwent high inguinal orchiectomy before treatment. Seven patients with Stages III and IV received chemotherapy before RT. Patients with Stages I and II were treated with 40-Gy photons to paraaortic and parailiac fields. Ten patients with Stage III and IV seminoma received 30-Gy photons to mediastinal and supraclavicular fields as well. Sixty patients received additional inguinal RT. The overall 5-year survival rate (corrected for intercurrent death, except for treatment toxicity) was 100% for Stage I, 100% for Stage II, 87% for Stage III, and 87% for Stage IV. The mean follow-up time was 6.3 years (range, 0.6 to 11.9 years). An evaluation of all patients showed no difference according to histologic condition or prior chemotherapy. Mediastinal and supraclavicular irradiation showed no improvement in treatment results. Acute toxicity consisted of mild to moderate emesis, increased bowel frequency, erythema, and, in four cases leucopenia and thrombopenia (all World Health Organization [WHO] Grades I to II). However, one patient died of a pulmonary fibrosis 1 month after mediastinal irradiation and 2 months after polychemotherapy, and a gastroduodenal ulcer developed in another patient 1.5 months after paraaortic RT and prior polychemotherapy. Overall, the data suggest that to avoid overtreatment and consecutive treatment morbidity reduced doses of 30 Gy and a restrictive treatment planning adapted to the individual risk are sufficient for RT for testicular seminoma. An alternative to postoperative RT in Stage I (and possibly Stage II) seminoma could be no RT, but close follow-up instead.
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PMID:Treatment results and acute and late toxicity of radiation therapy for testicular seminoma. 211 39

Clinical symptoms and signs and the frequency of abnormalities in the results of enzymatic, electromyographic, histological and serological tests were analysed in 50 patients, including 17 with polymyositis (PM) and 33 with dermatomyositis (DM), groups I and II according to Bohan's classification and followed-up for 25 years. Weakness of the proximal muscles of the extremities was present in both groups in all patients and in a high proportion of cases weakness involved also the distal muscles. Dysphagia was more frequent in DM patients (54.5%) than in PM (17.6%). In DM erythema and facial oedema as well as Gottron's sign were observed more frequently. In 11.8% of PM patients and 15.1% of DM patients deposition of calcium salts in subcutaneous tissue developed. Signs of vasculitis were found in 39.4% of DM cases and 17.6% of PM cases. In one-third of PM and in only 3 DM cases pitting oedema of the distal parts of the extremities was noted. Cardiovascular changes were disclosed in 82.3% of PM and 69.7% of DM patients. On the other hand, radiological signs of interstitial pulmonary fibrosis were noted more frequently in DM (36%) than in PM (23%). Increased serum activity of CPK, AspAT and ALAT was present with similar frequency in both groups from 71% to 81% of cases. EMG showed evidence of primary muscular changes in all PM patients and 69.6% of DM patients. Histological examination confirmed the diagnosis in the studied patients. Antinuclear antibodies were found relatively rarely, from 2% to 24% of PM/DM patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Polymyositis-dermatomyositis--a 25-year follow-up of 50 patients (analysis of clinical symptoms and signs and results of laboratory tests). 213 28

The presence of serum autoantibodies to nuclear antigens (ANA) and other antigens is a hallmark of connective tissue diseases. Antibodies to double-strand DNA were present in high titers in SLE patients. Antibodies to U1RNP correlated with Raynaud's phenomenon and finger erythema in SLE patients. Anticardiolipin antibody was present in 29% of 41 SLE patients. Antibody to Scl-70 was present in 52% of 21 PSS patients. Antibody to Jo-1 was present in patients with PM associated with pulmonary fibrosis. Two of our patients with PM had an autoantibody that precipitated 7SL RNA.
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PMID:[Progress in laboratory tests of connective tissue diseases]. 261 63

Side effects of bleomycin therapy are most frequent in the skin and as pulmonary fibrosis because of the nearly selective concentration in these organs. The cutaneous manifestations include a variety of clinical symptoms, most frequently erythema and infiltrations with marked hyperpigmentation. A 38-year-old patient is reported who received 615 mg bleomycin for therapy of a malignant testicular teratoma and in the following time developed numerous morphoea-like lesions, acrocyanosis, Raynaud's phenomenon, acrosclerosis, and cutaneous calcinosis. Histologically, the alterations were similar to those of scleroderma. In contrast to progressive systemic sclerosis the symptomatic character of the lesions is evident, for the skin changes resolved several months after the drug was discontinued.
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PMID:[Symptomatic scleroderma caused by bleomycin]. 618 16

Several organs (lung, skin, thyroid, heart, bone marrow) are potential targets of cobalt (Co). Whereas there is no doubt that inhalation of Co alone may cause bronchial asthma, its role in the occurrence of hard metal disease is still controversial because most cases were reported in workers exposed not only to Co but also to other substances such as tungsten carbide, titanium carbide, iron, silica and diamond. To assess whether exposure to pure Co dust (metal, oxides, or salts) may lead to adverse health effects a cross sectional study was carried out among 82 workers in a Co refinery. The results were compared with those in a sex and age matched control group. The Co group had been exposed for 8.0 years on average (range 0.3-39.4). The geometric mean time weighted average exposure assessed with personal samplers (n = 82) was about 125 micrograms/m3 and 25% of the values were higher than 500 micrograms/m3. The concentrations of Co in blood and in urine after the shift were significantly correlated with those in air. Concentration of Co in urine increased during the workweek. A slight interference with thyroid metabolism (decreased T3, T4, and increased TSH), a slight reduction of some erythropoietic variables (red blood cells, haemoglobin, packed cell volume) and increased white cell count were found in the exposed workers. The exposed workers complained more often of dyspnoea and wheezing and had significantly more skin lesions (eczema, erythema) than control workers. Within the exposed group a dose-effect relation was found between the reduction of the forced expiratory volume in one second/vital capacity and the intensity of current exposure to Co assessed by the measurement of Co in air or in urine. The prevalence of dyspnoea was related to the dustiness of the workplace as reflected by statistically significant logistic regression between this symptom and the current levels of Co in air and in urine. No difference between lung volumes, ventilatory performances, carbon monoxide diffusing capacity, and serum myocardial creatine kinase and procollagen III peptide was found between the Co and control groups and no lung abnormalities were detected on the chest radiographs in both groups. The results suggest that exposure to high airborne concentrations of Co alone is not sufficient to cause pulmonary fibrosis. This finding is compatible with experimental studies indicating that interaction of other airborne pollutants with Co particles play a part in the pathogenesis of parenchymal lung lesions.
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PMID:Epidemiological survey of workers exposed to cobalt oxides, cobalt salts, and cobalt metal. 839 78

The papain superfamily member bleomycin hydrolase (Blmh) is a neutral cysteine protease with structural similarity to a 20S proteasome. Bleomycin (BLM), a clinically used glycopeptide anticancer agent, is deaminated in vitro by Blmh. We used gene targeting to generate mice that lack Blmh and demonstrated that Blmh is the sole enzyme required for BLM deamination. Although some Blmh null mice were viable and reproduced, only about 65% of the expected number survived the neonatal period, revealing an important role for Blmh in neonatal survival. Mice lacking Blmh exhibited variably penetrant tail dermatitis that resembled rodent ringtail. The histopathology of the tail dermatitis was similar to skin lesions in humans with pellagra, necrolytic migratory erythema, and acrodermatitis enteropathica. Compared with controls, Blmh null mice were more sensitive to acute BLM lethality and developed pulmonary fibrosis more readily following BLM treatment. Thus, we have established that Blmh is an essential protectant against BLM-induced death and has an important role in neonatal survival and in maintaining epidermal integrity.
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PMID:The neutral cysteine protease bleomycin hydrolase is essential for epidermal integrity and bleomycin resistance. 1020 Mar 22

Postoperative radiotherapy (RT) forms an intrinsic part of breast conservation therapy, substantially reducing the risk of breast relapse. It is given 4-6 weeks postoperatively using medial and lateral beams to achieve a near homogeneous dose to the breast while minimising the dose to adjacent structures. Typically, a dose of 40 Gy in 15 daily fractions over three weeks or 50 Gy in 25 fractions over five weeks is given, with a boost of 10 Gy using low energy electrons or superficial X-rays. RT is delivered to the chest wall after mastectomy in patients with large tumours (> 5 cm), close surgical margins, or significant axillary node involvement and may result in an additional 10% survival benefit. Early complications of RT include tiredness, skin erythema and moist desquamation. Late reactions, breast fibrosis, telangiectases and peau d'orange are reduced if more treatment fractions are given. Lung irradiation is minimised by careful treatment planning and < 1% of patients experience radiation pneumonitis or pulmonary fibrosis. Patients with locally advanced breast cancer may be offered a combination of systemic therapy, endocrine, chemotherapy and radical RT. Preoperative systemic and radiation therapy reduces the mastectomy rate but at present does not improve survival. Those who are unfit for radical treatment and have large ulcerating or fixed cancers are offered either endocrine or cytotoxic therapy, depending upon the oestrogen receptor status, followed by RT, which is unlikely to cure but may achieve local control for a variable duration. RT forms part of the multidisciplinary approach to palliative treatment for metastatic disease. Bone metastases occur in 75% of women with metastatic disease and RT provides effective pain relief and reducing risk of pathological fractures. Patients with brain metastases need high-dose steroids followed by cranial RT.
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PMID:Radiotherapy for early and advanced breast cancer. 1177 Mar 58

Sulfur mustard is an alkylating agent that reacts with ocular, respiratory, cutaneous, and bone marrow tissues, resulting in early and late toxic effects. We compare these effects based on the experience in Iranian veterans exposed to the agent during the Iran-Iraq conflict (1983-88). The first clinical manifestations of sulfur mustard poisoning occurred in the eyes with a sensation of grittiness, lacrimation, photophobia, blepharospasm, and corneal ulceration. Respiratory effects appeared as rhinorhea, laryngitis, tracheobronchitis, and dyspnoea. Skin lesions varied from erythema to bullous necrotization. Initial leukocytosis and lymphopenia returned to normal within four weeks in recovered patients, but marked cytopenia with bone marrow failure occurred in fatal cases. Late toxic effects of sulfur mustard were most commonly found in lungs, skin and eyes. Main respiratory complications were chronic obstructive pulmonary disease, bronchiectasis, asthma, large airway narrowing, and pulmonary fibrosis. Late skin lesions were hyperpigmentation, dry skin, atrophy, and hypopigmentation. Fifteen of the severely intoxicated patients were diagnosed with delayed keratitis, having corneal vascularization, thinning, and epithelial defect. Respiratory complications exacerbated over time, while cutaneous and ocular lesions decreased or remained constant. Both the severity and frequency of bronchiectatic lesions increased during long-term follow-up. The only deteriorating cutaneous complication was dry skin. The maximum incidence of delayed kaeratitis was observed 15 to 20 years after initial exposure. Being suggested as the main cause ofassociated with malignancies and recurrent infections, natural killer cells were significantly lower 16 to 20 years after intoxication.
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PMID:Comparison of early and late toxic effects of sulfur mustard in Iranian veterans. 1704 Feb 11

We describe a 70-year-old man with rheumatoid arthritis and pulmonary fibrosis who presented with a month's history of pain in the left lateronasal region and inferior eyelid. On examination there was left exophthalmos, difficulty in coordinating eye movements, inflammation, erythema, and pain. Computed tomography showed a 3 cm mass in the left posterior ethmoid region, a biopsy specimen from which showed a small cell neuroendocrine tumour. He refused operation and was treated unsuccessfully with four cycles of cisplatin and etoposide.
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PMID:Small cell neuroendocrine carcinoma of the sinonasal region. A propose of a case. 1711 45

Dermatomyositis, a connective tissue disease syndrome where antibodies to the endothelium of the microvasculature of the skin, muscle and lung are implicated in lesional propagation, is characterized by photodistributed erythema, heliotrope rash, Gottron's papules, muscle weakness and interstitial pulmonary fibrosis. Endotheliotropic viruses and underlying neoplasia are among the inciting triggers. Uncommon drugs, namely the lipid-lowering agents, have been implicated in dermatomyositis. The patient, a 57-year-old man, developed a photodistributed rash and muscle weakness following treatment with the antifungal medication, terbinafine. A skin biopsy was performed, showing an atrophying interface dermatitis with pandermal mucinosis and striking vasculopathic changes including endothelial cell necrosis with denudement and basement membrane zone reduplication. Ultrastructural studies confirmed the presence of endothelial cell injury. Direct immunofluorescent testing showed prominent staining of C5b-9 along the dermal-epidermal junction and within the vasculature. Western blot studies showed strong seroreactivity of his serum to an endothelial-based protein weighing 45,000, a common target described in other microvascular injury-based syndromes. We have shown a temporal association between use of terbinafine and the development of dermatomyositis. The exact basis remains speculative. One potential hypothesis is based on the fact that terbinafine, the active agent in terbinafine, triggers apoptosis of human endothelial cells in culture. Enhanced endothelial cell apoptosis results in the displacement of various cellular antigens creating a state of neoantigenicity; its attendant sequelae is held to be one of anti-endothelial cell antibody formation, a defining pathogenetic event in the evolution of dermatomyositis. The second may be because of the effects of the drug on the promotion of an interferon-rich T-helper-1-dominant cytokine milieu.
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PMID:Terbinafine-induced dermatomyositis: a case report and literature review of drug-induced dermatomyositis. 1809


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