Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034067 (emphysema)
11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The study included 40 patients aged 33-71 years with chronic pulmonary obstruction (CPO) and diabetes mellitus (DM). They were examined by multilayer CT with detailed analysis of parenchymal tissues. The main microvascular changes in lung parenchyma included dilation, focal opacity (2-5 mm), and beaded vessels. In patients with isolated CPO, these changes were less pronounced than in those with combined pathology. All patients presented with bronchial deformities, bronchoectasia, emphysema, and cysts. Microvascular pathology in lung parenchyma appears to be a manifestation of universal diabetic microangiopathy responsible for the unfavourable development of CHD in DM patients.
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PMID:[Computed tomographic findings in patients with chronic pulmonary obstruction and diabetes mellitus]. 2157 41

Primary or isolated pulmonary interstitial glycogenosis (PIG) is a rare disease presenting as tachypnea and hypoxemia during the perinatal period. A diffuse interstitial infiltrate with focal hyperinflation is visible on chest imaging. The biopsy findings include diffuse expansion of the interstitium by spindle-shaped cells with pale cytoplasm that, on electron microscopy (EM), are poorly differentiated mesenchymal cells containing abundant monoparticulate glycogen. This glycogenosis appears to be a transient abnormality, usually with a favorable prognosis. Recently, cases of PIG, some associated with other pulmonary or systemic abnormalities, have been described. The clinical significance and potential role of PIG changes remain unknown. We report 28 cases of PIG associated with a spectrum of pediatric pulmonary and cardiovascular disorders, including arterial hypertensive changes with and without abnormal alveolar development (n=9), congenital heart disease (CHD; n=4), hyperplasia of pulmonary neuroendocrine cells resembling neuroendocrine hyperplasia of infancy (NEHI, n=5), congenital pulmonary airway malformation (n=5), congenital lobar emphysema (n=4), and Noonan syndrome (n=1). In all cases, PIG was confirmed by positive periodic acid-Schiff (PAS) staining, immunopositivity for vimentin, and EM. Although some patients improved with age, 7 died of respiratory failure or complications of CHD, suggesting that PIG may be clinically significant when associated with other severe disorders. The association of PIG with a spectrum of mostly congenital lung disorders supports its origin as a developmental abnormality of interstitial fibroblast differentiation rather than a nonspecific reactive process.
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PMID:Pulmonary interstitial glycogenosis associated with a spectrum of neonatal pulmonary disorders. 2887 55