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)

Exogenic allergic alveolitides are caused by organic dusts which contain bacteria, moulds or vegetable and animal antigens. The farmer's lung as a form of the exogenic allergic alveolitis is a rare disease. The uncharacteristic symptomatology in the initial phase and in particular the retarded beginning of the symptom after several hours handicap the timely recognition in an early phase of the disease so that curative therapeutic measures are rarely possible. The cases of the disease are found only at the chronic stage, at the stage of the pulmonary fibrosis. Then the prognosis is unfavourable. In the Central Clinic for Heart and Lung Diseases Bad Berka 1,110 patients with alveolitides and lung fibroses were diagnosed in the period from 1975 to 1988. 306 of them could be clarified as exogenic allergic alveolitis, 61 of them (19.8%) were farmer's lungs.
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PMID:[Farmer's lung--a form of exogenous allergic alveolitis]. 209 25

Non-germinal cell tumor of the testis is a rare disease, and vascular tumor of the testis is a still rarer disease. Herein, a case of cavernous hemangioma of the testis is reported. A 75-year-old man consulted our department with the complaint of a painless left intrascrotal tumor. Laboratory findings revealed slight leukocytopenia and thrombocytopenia of unknown origin. In chest X-ray, a diffuse reticular shadow was shown and it was considered due to pulmonary fibrosis, but, alpha-fetoprotein and CEA were normal. Left radical orchiectomy was performed under spinal anesthesia. The tumor existed under the tunica albuginea, and the cutting surface of the tumor was brown and irregular. There were hemorrhagic portions in some places. The left epididymis and the left spermatic cord were normal. Histologically, the tumor was diagnosed as cavernous hemangioma. The vascular tumor of the testis is a very rare disease, and only 17 cases have been reported including this case. This case was the 7th case of cavernous hemangioma of the testis, and the first case in Japan. This patient died of respiratory failure due to pulmonary fibrosis and pneumonia. In the autopsy, there was no abnormal finding that was considered to be related to the cavernous hemangioma of the testis.
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PMID:[A case of cavernous hemangioma of the testis]. 409 Nov 43

Idiopathic pulmonary haemosiderosis (IPH) is a rare disease characterized by recurrent episodes of intrapulmonary bleeding, chronic iron deficiency anaemia and pulmonary fibrosis. IPH is a diagnosis made by exclusion of other causes. It occurs in both adults and children. Other conditions than IPH can cause pulmonary haemosiderosis. The etiology is unknown, but might be an immunological mechanism causing a defect in the basement membrane of the pulmonary capillary. IPH should be suspected in patients with recurrent episodes of coughing, haemoptysis, dyspnoea and anaemia. Chest X-ray shows pulmonary infiltrates during an acute attack. Examination of sputum or lung biopsy discloses large numbers of haemosiderin-laden pulmonary macrophages. The mortality-rate is high, but the prognosis is difficult to evaluate because many patients survive for a long time either with a course of recurrent attacks or with chronic symptoms, such as dyspnoea and persistent anaemia. Steroids may improve the condition of the patient during a bleeding episode.
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PMID:[Idiopathic pulmonary hemosiderosis]. 863 26

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by widespread localization of calcispherites in the alveolar spaces. The patients are symptomless for a long time. Nevertheless, this disease slowly develops into pulmonary fibrosis and cardiac failure. The chest X-rays and high-resolution computed tomography strongly point towards a diagnosis of PAM. As for therapeutic approaches, repeated broncho-alveolar lavages (BAL) have been performed with improvement of symptoms but without recovery, and a new oral drug treatment is still under way. We report 2 familial cases of PAM. Both patients underwent chest X-ray examination showing diffuse bilateral micronodular opacities of calcific density. After 5 years, in May 1993, one of them developed exertional dyspnoea, cyanosis, dry cough and was admitted to our Division. Cardiokinetic and diuretic drugs as well as oxygen were administered with satisfactory results. Then repeated BAL were performed. The chest X-ray after 6 months of sodium etidronate (300 mg t.i.d.) administration was unchanged.
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PMID:Pulmonary microlithiasis. Report of two cases. 909 54

Pulmonary fibrosis (PF) is a rare disease that develops as the final stage of scarring after a series of episodes of lung injury. PF is idiopathic in most cases (60%), though the exact rate varies from series to series. Childhood PF, which is even rarer than the adult form, is usually diagnosed when the patient is less than five years old. Some cases are familial. We report the case of a 14-year-old girl admitted with a three-year history of dry cough and increasing dyspnea that eventually appeared even with moderate effort. Pulmonary biopsy by mini-thoracotomy showed diffuse fibrosis of unknown cause. She died four years after diagnosis. The patient's age, the rapid course of disease, and the final outcome are all unusual features of this case.
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PMID:[Idiopathic pulmonary fibrosis in a 14-year-old girl]. 1054 87

Despite the lack of precision of asbestos exposure assessments and the limitations of the main diagnostic epidemiological tool for asbestos-related diseases (i.e. standard X ray films), several issues concerning the risk of development of asbestos-related diseases are well established. For asbestosis, now a rare disease, the existence of a positive dose-response relationship, with a threshold or no-effect level, has been clearly demonstrated. The slope of the relationship curve is steeper for amphiboles than for chysotile, as it is for increased fiber length. Asbestosis is associated with an increased risk of bronchial carcinoma, however it is now known that exposure to asbestos of itself increases the risk of cancer even in the absence of any radiographic signs of pulmonary fibrosis. Pleural plaques occur even when the level of asbestos exposure is low. They are not only dose-dependent but are also latency-related. They have no prognostic significance in asbestos-exposed workers, but are associated with an increased risk for the subsequent development of mesothelioma and bronchial carcinoma when compared to the risk of the general population. Diffuse pleural thickening is associated with higher levels of asbestos exposure than those associated with pleural plaques. It usually follows a benign pleural effusion, which is a less frequent but earlier consequence of asbestos exposure than the other asbestos-related diseases documented above.
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PMID:[Risk assessment of benign asbestosis (dose-effect relationship, time-effect relationship, co-factors)]. 1089 48

Relapsing Polychondritis is a rare disease which causes the repetitive inflammation of cartilage and connective tissues. Although the large airway is sometimes involved and the stenosis of them often influences the prognosis of the patients, there have been few reports concerning the manifestation of the peripheral lung. A 60-year-old man with pulmonary fibrosis was admitted to a regional hospital due to sudden deafness, and then he suffered from relapsing polychondritis. During the steroid therapy, he also suffered from bilateral pneumothoraces. His computed tomogram revealed many bilateral bullae, emphysematous changes, and fibrotic changes in bilateral lungs. The mechanism of generating peripheral pulmonary manifestations is also discussed.
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PMID:Pulmonary fibrosis with intractable pneumothorax: new pulmonary manifestation of relapsing polychondritis. 1169 68

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive inherited disease consisting of (1) partial oculocutaneous albinism (with nystagmus, strabism, and visual acuity loss), (2) platelet storage pool deficiency (with bleeding diathesis), and (3) disorder of "ceroid" metabolism with a multisystem tissue lysosomal ceroid deposition. HPS is less uncommon in Puerto Rico, where the most important studies have been performed, but is a very rare disease in Europe. HPS basic defect remains unknown, even if an HPS-causing gene was identified in chromosome segment 10q23-q23.3, and several mutations have been reported. The aim of this article is to discuss, on the basis of a review of relevant literature, a new familial HPS clinical variant observed in 2 young sisters (aged 16 and 23 years old, respectively), characterized by the typical symptoms of this syndrome. Our patients also suffered from diffuse interstitial pulmonary disease and an unexpectedly increased platelet aggregation and were prone to bacterial infections. Interestingly, we observed urinary tract abnormality in the younger HPS sister and a porencephalic cyst in the older HPS sister; both of these developmental defects have been reported in the Cross syndrome (or oculocerebral hypopigmentation syndrome). It seems that in our patients, an overlapping of the phenotypic manifestations of different rare syndromes may be present. The presence of ceroid-like autofluorescent material in urinary sediment together with the histologic aspects and the autofluorescence of oral mucosa biopsy are consistent with a ceroid-like lipofuscin storage. HPS should be carefully tested for in suspected cases to prevent the severe visual impairment, rapidly progressive pulmonary fibrosis, and other complications associated with this disorder.
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PMID:A clinical variant of familial Hermansky-Pudlak syndrome. 1282 64

Combined interstitial lung disease and the presence of 'mechanic's hands' could be a clinical clue in the early diagnosis of the rare disease, amyopathic antisynthetase syndrome for which anti-Jo-1 antibody is a useful diagnostic tool. The case is reported of a patient who suffered from shortness of breath and dry cough, with pulmonary fibrosis on CXR and CT scan, and interstitial pneumonitis on trans-bronchial biopsy. She was also positive for anti-Jo-1 and anti-Ro antibodies. 'Mechanic's hands' were noted bilaterally but with no evidence of myopathy in either the electromyogram or on muscle biopsy. The patient was treated with prednisolone and her clinical picture, including the 'mechanic's hands' and lung fibrosis, subsided gradually, suggesting that the sign may be a useful follow up tool in this disease. Early diagnosis and corticosteroid therapy could be beneficial for these patients.
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PMID:Combined lung fibrosis and 'mechanic's hand': a clinical diagnostic clue to amyopathic antisynthetase syndrome. 1849 52

We report a case of Langerhans cell histiocytosis (LCH), a rare disease caused by the proliferation of abnormal Langerhans cells, coincident with severe external ear canal inflammation. A 27-year-old man with a 1-year history of external ear canal discharge was found in computed tomography (CT) to have left temporal bone erosion with tissue granulation. Chest X-ray showed pulmonary fibrosis, necessitating transbronchial lung biopsy that yielded a definitive diagnosis of multisystem, multisite LCH involving the bone, skin, lung, pituitary, thyroid, and lymph node systems. He underwent combination chemotherapy directed by the Japan LCH Study Group. LCH should therefore be considered in the case of a patient with severe external ear canal inflammation coincident with temporal bone erosion.
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PMID:[A case of Langerhans cell histiocytosis coincident with severe external ear canal inflammation]. 1999 53


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