Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0085383 (hypocapnia)
 1,697 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Methotrexate, an antifolate cytotoxic drug, is used in anticancer chemotherapy as well as an immuno suppressive in rheumatoid arthritis. It is responsible for numerous secondary effects, amongst which is a characteristic acute pneumonia known since 1969. This pneumonitis has been described in detail, up to the present time in 78 cases gathered in this review. The prevalence of this complication is estimated at around 7%. This pneumonia may occur whatever the age, indication for which methotrexate is prescribed, the route of administration of the product (including the intra-thecal route) and the dose. It includes dyspnoea, fever, (sometimes quite marked) and frequently an acute reversible respiratory failure. Radiologically the opacities are usually diffuse interstitial and symmetrical with a basal predominance with sometimes some confluence and occasionally a pleural reaction. In a small number of cases a transient mediastinal adenopathy has been described. Respiratory function tests show a rapidly developing restrictive syndrome accompanied by hypoxia and hypocapnia. Broncho-alveolar lavage is characterised by hypercellularity with a frank and apparently transitory lymphocytosis. Histologically the most frequent lesion sighted is an extensive acute granulomatous reaction with or without oedema. Most often the outcome is favourable (75% of cases). However 6 deaths due to respiratory failure have been reported. Even though there has not been any formal test, steroid therapy in high dosage seems to accelerate recovery. Progress to an irreversible pulmonary fibrosis is possible but rare. The mechanism of this drug related acute pneumonia is not known but would seem to resemble that of other granulomatosis. Besides this rapidly progressive pneumonitis, methotrexate is responsible for a very small number of cases of severe pulmonary oedema and of acute painful pleurisies.
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PMID:[Pneumopathy caused by methotrexate]. 225 35

A case is reported of severe interstitial paraquat poisoning in a 23 year old man. Initial treatment consisted of gastric lavage, fuller's earth, haemoperfusion on activated charcoal and peritoneal dialysis. On the third day, appeared both renal and early respiratory failure, with hypocapnia and hypoxia. Peritoneal dialysis was kept up to the thirteenth day. Pulmonary function tests showed a restrictive syndrome. Chemotherapy with 1 mg.kg-1.24 h-1 prednisone and 3 mg.kg-1.24 h-1 cyclophosphamide was started on day 4. Radiotherapy (13 Gy) was also given to both lung fields between days 6 and 10. Despite this treatment, respiratory failure worsened, and the patient died at the 28th day, with resistant severe hypoxaemia and pneumothorax on fibrous lungs. Several reports have shown conflicting results with chemotherapy and radiotherapy in the treatment of pulmonary fibrosis due to paraquat poisoning. The failure of such treatment in the case reported showed the need for large prospective clinical trials of the treatment of paraquat poisoning, as well as for urgent preventive measures.
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PMID:[Failure of chemotherapy and radiotherapy in pulmonary fibrosis caused by paraquat]. 336 14

At least 31 cases of familial fibrocystic pulmonary dysplasia, within 10 families, have been described in the world literature. The mode of genetic transmission of this disease, however, has been uncertain until now. The author observed three unequivocal and five probable cases of familial fibrocystic pulmonary dysplasia among 56 members of one family. Diagnostic criteria included progressive dyspnea and cyanosis, digital clubbing, pulmonary hypertension, negative sweat tests, polycythemia, arterial hypoxia and hypocapnia, chest radiographs showing diffuse bilateral pulmonary fibrosis, and diffuse fibrocystic pulmonary dysplasia at postmortem examination (two cases). Among the three unequivocal cases one father-to-son transmission was observed. Non-sex-linked dominant transmission of familial fibrocystic pulmonary dysplasia is thereby proved for the first time. One patient also developed a bronchial carcinoma in addition to fibrocystic pulmonary dysplasia; this is considered to be a cause-and-effect relationship and not a coincidental complication.
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PMID:FAMILIAL FIBROCYSTIC PULMONARY DYSPLASIA: OBSERVATIONS IN ONE FAMILY. 1427 97

Tracheomalacia is a process characterized by softness of the supporting tracheal cartilages, by the extension of the posterior membranous wall and by reduction of the tracheal antero-posterior diameter. Exceptionally, tracheomalacia can be associated with tracheobronchomegaly or Mounier-Kuhn syndrome. Fibro-bronchoscopy represents the ''gold standard'' for diagnosis. The case of a 79-year-old male observed after hospitalization in a medical ward for chronic pulmonary obstructive disease (COPD) decompensation, and with basal left bronchopulmonary focus, is described. During this period, a progressive worsening of clinical conditions occurred, despite cortisone and antibiotic therapy, and the patient was transferred to the ICU for dyspnea, hypoxia, hypocapnia and with a diagnosis of pulmonary fibrosis. Bronchoscopy, performed during spontaneous breathing, revealed tracheomalacia which was responsible for tracheal dynamic complete stenosis during expiration and dynamic subtotal stenosis of the left primary bronchus in the first tract, together with sputum retention. Moreover, this investigation confirmed the diagnosis of tracheobronchomegaly already seen on CT. It was suggested to place a Freitag stent, since the insertion of another model would not have had enough chance of stability, due to the enormous extension of the tracheal lumen and could not have guaranteed good clearance of the secretions. Seven days after this intervention, performed in an outpatients' setting, the patient was dismissed from the ICU, without the help of O2, with good ventilation, saturation in line with his age and good expectoration.
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PMID:Tracheomalacia associated with Mounier-Kuhn syndrome in the Intensive Care Unit: treatment with Freitag stent. A case report. 1546 97

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