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)

Five cases of von Recklinghausen's disease were studied with regard to their association with chest diseases, especially pulmonary emphysematous and fibrotic changes. CT analysis revealed that four patients out of five had multiple pulmonary emphysematous lesions and that those lesions were seen in all segments. In two cases out of the four, it was difficult to detect their abnormalities by an ordinary chest roentgenogram, suggesting the possibility of higher incidence of emphysematous changes in von Recklinghausen's disease than has been expected. On the other hand, pulmonary fibrosis was observed in two cases. There were two patients who had no fibrosis but emphysematous lesions. TBLB was performed in one case, in whom no fibrotic changes were observed. Usually pulmonary fibrosis is associated with emphysematous bullae in patients with von Recklinghausen's disease. However, fibrotic and emphysematous changes may not always occur in combination in von Recklinghausen's disease.
 ...
PMID:[CT analysis of pulmonary emphysematous and fibrotic changes in patients with von Recklinghausen's disease]. 251 35

The neurofibromatoses are autosomal dominant diseases that have widespread effects on ectodermal and mesodermal tissue. The commonest member of the group is neurofibromatosis type 1 (NF1) which varies in severity but which can affect all physiological systems. Neurofibromas are the characteristic lesions of the condition and not only occur in the neuraxis but may also be found in the oropharnyx and larynx; these may produce difficulties with laryngoscopy and tracheal intubation. Pulmonary pathology includes pulmonary fibrosis and cystic lung disease. The cardiovascular manifestations of NF1 include hypertension, which may be associated with phaeochromocytoma or renal artery stenosis. Neurofibromas may also affect the gastrointestinal tract and carcinoid tumours may be found in the duodenum. This review documents the aetiology and clinical manifestations of the neurofibromatoses and discusses their relevance to the anaesthetist.
 ...
PMID:Neurofibromatosis: clinical presentations and anaesthetic implications. 1157 32

Gene expression changes in the lungs induced by paraquat (PQ) administration were studied in rats using DNA microarrays that were detectable for 1,090 genes per DNA microarray. The rats were subjected to subacute PQ exposure (7 mg/kg, s.c., daily for eight administrations). Two days after the final administration, the rats were divided into two groups. Group 1 experienced significant body weight loss and displayed signs of subacute PQ toxicity, but Group 2 showed no significant effects due to the PQ treatment. A control group, Group 3, was also included. In the comparison of the gene expression levels in the animals from Group 1 or Group 2 to the control animals treated by vehicle, 48 genes in Group 1 and 29 genes from Group 2 were differentially expressed. The twenty-eight genes were common to these two groups. These differentially expressed genes following paraquat treatment were classified as follows: 5 neurotransmitter receptor genes; 4 transporter genes; 4 voltage-gated ion channel genes; 2 lipid metabolism enzyme genes; 2 G-proteins involved in endocytosis and exocytosis genes; 7 cytokine genes; 4 ADP ribosylation genes involved in cell death and regeneration; CFTR gene, which is the causal gene for cystic fibrosis; neurofibromatosis type 1 gene, which is the causal gene for the neurofibromatosis type 1 that is known to accompany pulmonary fibrosis; and the causal gene for spinocerebellar ataxia. These genes may prove to be the keys for the elucidation of the mechanism of PQ toxicity, e.g. PQ-induced pulmonary fibrosis.
 ...
PMID:Gene expression analysis of the lung following paraquat administration in rats using DNA microarray. 1520 77

The neurofibromatosis type I (NF I, von Recklinghausen's disease) is an autosomal dominant neurocutaneous disorder with systemic involvement. The respiratory manifestations include: chest wall deformities, upper airway obstruction by neurofibromas, parenchymal neurogenic tumours, pulmonary fibrosis, cystic lung disease, primary pulmonary hypertension, central hypoventilation, diaphragm paralysis. We present 4 cases of NF I associated with chronic respiratory diseases. The common feature is the presence of severe chest deformities. The bullous emphysema was present in 2 of them. All 4 patients suffered from depressive syndrome with neuroleptic use.
 ...
PMID:[Respiratory manifestations in von Recklinghausen's disease]. 1706 22