Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the case of a 61-year-old male patient, in which the search for the cause of chronic respiratory failure, severe pulmonary hypertension and secondary erythrocytosis resulted in a diagnosis of combined pulmonary fibrosis and emphysema (CPFE). This is a unique, recently characterised syndrome with upper-lobe emphysema and pulmonary fibrosis of the lower lungs. The cause is unknown, but one of the main risk factor remains smoking. The patient was a heavy smoker (over 40 pack-years). He complained of dyspnoea on exertion and cough. Physical examination revealed basal crackles and cyanosis. The patient had severe reduction in diffusing capacity, out of proportion to his lung volumes (DLCO 27% of predicted value, FEV1 2.95 l (100%), FVC 4.41 l (118%), FEV1/FVC (66%). The blood gas showed hypoxemia (pO2 37 mm Hg), hypocapnia and respiratory alkalosis. Diagnosis was based on chest computer tomography, which revealed upper lobe emphysema and lower lobe ground glass changes and honeycombing. Severe pulmonary hypertension (SPAP 80 mm Hg) was confirmed by echocardiography and right cardiac catherisation. The patient received long-term oxygen therapy, inhaled corticosteroid and Ca-blocker.
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PMID:[Combined pulmonary fibrosis and emphysema - case report and literature review]. 1946 58

A young man aged 22 years presented with shortness of breath, left sided chest pain, mild dry cough, peripheral cyanosis, fever and generalized weakness for three years. He was diagnosed as having a large infected cystic mediastinal mass with tricuspid regurgitation and severe pulmonary hypertension. On thoracotomy, one litre of pus was aspirated and tumour was excised and sent for histopathology. Biopsy report revealed benign cystic teratoma. This case is reported to highlight the management of a huge infected benign cystic teratoma which is rarely found.
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PMID:Infected benign cystic teratoma of the mediastinum. 1948 84

A 67-year-old woman was admitted to our hospital because of progressive dyspnea, cough, bloody sputum, and backache. Chest radiography and CT scans showed bilateral diffuse interstitial shadows, bilateral pleural effusion and dilatation of the pulmonary artery. Echocardiography indicated pulmonary hypertension, and the serum tumor marker levels were elevated. We performed right cardiac catheterization, and withdrew some blood from a pulmonary artery catheter in the wedge position. We confirmed moderate pulmonary hypertension, and adenocarcinoma-like malignant cells were seen in the aspirated blood. The patient died of progressive respiratory failure despite supportive care. In addition to PTTM and lymphangiosis carcinomatosa, autopsy of the right lung revealed interstitial pneumonia and lipoid pneumonia, both of which were not reported before to be associated with PTTM.
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PMID:[An autopsy case of pulmonary tumor thrombotic microangiopathy complicated with interstitial pneumonia and lipoid pneumonia]. 1960 30

Pulmonary hypertension, a common manifestation of advanced sarcoidosis, is thought to result from fibrosis with chronic hypoxia and destruction of small vessels, extrinsic compression of pulmonary arteries, or granulomatous vasculitis. We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on exertion and was found to have pulmonary artery enlargement, pulmonary venous compression, and mediastinal soft tissue enhancement on magnetic resonance imaging. Pulmonary hypertension was confirmed by right heart catheterization and sarcoidosis was diagnosed by histologic examination of tissue obtained at mediastinoscopy. Treatment with steroids resulted in decreased pulmonary artery pressures as well as symptomatic improvement. While pulmonary hypertension is a common complication of sarcoidosis, fibrosing mediastinitis is an unusual etiology that should be considered by clinicians.
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PMID:Sarcoidosis-associated fibrosing mediastinitis with resultant pulmonary hypertension: a case report and review of the literature. 1979 87

A nonsmoking, 52-year-old woman presented with a 4-month history of persistent dry cough. Chest X-ray film on admission showed small granular shadow in bilateral lung fields. Bronchoscopic biopsy did not yield a diagnosis. Two months later, she complained of dyspnea. Physical examination showed signs of pulmonary hypertension. Five days after the onset of dyspnea, she died of respiratory failure. An autopsy showed pulmonary embolism and swollen abdominal lymph nodes consisting of metastatic signet-ring cell carcinoma and poorly differentiated adenocarcinoma. There was also marked fibrocellular intimal proliferation and thrombus formation causing luminal stenosis in small pulmonary arterioles. Thrombi were organized by recanalization and included atypical cells. We diagnosed pulmonary tumor thrombotic microangiopathy (PTTM). In spite of various immunohistological staining procedure, we could not find out the primary lesion of this cancer. This case suggests that we should aggressively biopsy a large specimen of the lung to make a differential diagnosis of PTTM, because bronchoscopic biopsy is not enough to diagnose PTTM.
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PMID:[Pulmonary tumor thrombotic microangiopathy associated with cancer of unknown origin]. 1999

Although infectiological stimuli, environmental factors and genotypic features are known to contribute to the initiation and perpetuation of systemic sclerosis (SSc), its etiology still remains to be enigmatic, and less elusive insights are to be achieved by ongoing and future investigations. Being characterized, however, as chronic autoimmune disease with excessive collagen accumulation in skin, synovia and visceral organs such as lung, heart, and digestive tract along with obliterating angiopathy, the pathophysiology of SSc can be summarized as being based on imbalances of the cellular and humoral immune system, vascular dysfunction and activation of resident connective tissue cells. A complex interplay between these major components manages to establish and maintain the inability of the vasculature to adequately react to the need for dilatation, constriction and growth of new vessels, to cause the increased deposition of extracellular matrix constituents as well as to facilitate immunological disarrangement. Despite parallels to the chicken and egg causality dilemma, all of these account for what later clinicians observe in patients suffering from Raynaud's phenomenon, digital ulcers, sclerodactyly, rigidity of the face, microstomia, sicca syndrome, dyspnea, dry cough, pulmonary hypertension, palpitations, syncopes, renal insufficiency, dysphagia, gastroesophageal reflux, dyspepsia, generalized arthralgias, but also dyspareunia, or erectile dysfunction.
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PMID:The pathogenesis of systemic sclerosis revisited. 2008 91

Pertussis, or whooping cough, is a highly infectious respiratory disease, endemic all over the world, caused by bacteria Bordetella pertussis. The authors describe the case of a 36 day old infant, brought to the emergency room due to respiratory distress and cyanosing cough, admitted with suspected pertussis infection complicated by bacterial pneumonia, having begun large spectrum antibiotics. Labs showed hyperleukocytosis and thrombocytosis. She was transferred to an Intensive Care Unit because of a worsened state, dying 20 hours after admission with pulmonary hypertension and haemorrhage. Pertussis was diagnosed by PCR. Over the last few years, there has been an increase in the number of cases of pertussis. Adolescents and adults have become an under-recognized but significant source of infection, particularly to small unvaccinated infants. The authors underline the importance of recognizing pertussis, so that even in its atypical presentation, one can suspect, treat, report and prevent a disease that is not, and is not expected to be, eradicated any time soon. New vaccination strategies are necessary, to avoid this disease, which can be fatal to the most vulnerable.
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PMID:[Pertussis keeps on killing]. 2043 7

A 58-year-old woman presented with cough and dyspnea on exertion. A chest CT scan showed infiltrative cuneiform shadows in the peripheral lung fields. Pulmonary perfusion scintigraphy showed multiple nonsegmental defects. Histological analysis of the transbronchial lung biopsy specimens obtained from the right lower lobe showed tumor cell embolism and fibrocellular intimal proliferation, but no thrombus formation or recanalization in the small arteries. On the basis of these findings, we diagnosed pulmonary tumor embolism, not pulmonary tumor thrombotic microangiopathy (PTTM), because the pathological findings did not reveal either thrombus formation or recanalization, and the patient did not show hemodynamic effects such as hemolytic anemia, severe pulmonary hypertension, or disseminated intravascular coagulation. Systemic examinations revealed uterine cervical cancer. Her symptoms improved after the administration of chemotherapy and radiation therapy. Furthermore, the multiple nonsegmental defects observed on pulmonary perfusion scintigraphy disappeared. She was discharged, and her uterine cervical cancer has not recurred to date. Generally, a diagnosis of pulmonary tumor embolism and PTTM is difficult to establish in living patients. It is important that therapy is started before the disease progresses to PTTM, if pulmonary tumor embolism is diagnosed.
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PMID:[A pulmonary tumor embolism which mimicked pulmonary tumor thrombotic microangiopathy caused by uterine cervical cancer]. 2080 77

Selective right pulmonary arteriography and 3-dimensional computed tomography revealed multiple severe stenoses of the peripheral pulmonary artery associated with poststenotic aneurysms in a 65-year-old woman. She was referred to the hospital for evaluation of dry cough, gradually increasing dyspnea and multiple nodular shadows on a chest radiograph. Echocardiography and cardiac catheterization showed severe pulmonary hypertension, though other structural heart diseases or well-characterized congenital syndromes were ruled out. She was diagnosed as isolated peripheral pulmonary artery branch stenosis. Recent advances in CT technology enable a less-invasive assessment of pulmonary artery, and can be useful in the management of pulmonary arterial hypertension.
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PMID:A case of isolated peripheral pulmonary artery branch stenosis associated with multiple pulmonary artery aneurysms. 2082 53

Sleep hypoventilation is seen in patients with neuromuscular disease, as well as in those with obesity hypoventilation syndrome (OHS), which is defined as the combination of obesity, chronic hypercapnia, and hypoxemia during wakefulness that is aggravated during sleep. In 90% of cases, OHS is accompanied by obstructive sleep apnea. The diagnosis of OHS is based on hypoventilation and pulmonary hypertension that cannot be explained by alterations in pulmonary function. The mortality of patients with OHS is greater than is that of obese patients without hypoventilation. The principal neuromuscular diseases associated with OHS are the muscular dystrophies. The progression to chronic respiratory failure results from respiratory muscle weakness and impaired airway secretion clearance, causing atelectasis and pneumonia. With a decrease of greater than 50% in respiratory muscle strength, there is a reduction in VC. Cough peak flow < 160 L/min is associated with impaired airway secretion clearance, and values near 270 L/min indicate the need for assisted cough techniques. Obstructive sleep apnea usually worsens sleep hypoventilation. Noninvasive ventilation during sleep can improve survival, symptoms, and hypoventilation during wakefulness, as well as being able to improve pulmonary function in patients with neuromuscular disease. Patients with OHS can require oxygen therapy.
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PMID:[Sleep hypoventilation]. 2094 82


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