1983
DOI: 10.1001/archderm.1983.01650270016009
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Familial Occurrence of a1-Antitrypsin Deficiency and Weber-Christian Disease

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Cited by 30 publications
(8 citation statements)
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“…There have been reports of associations between W-CD and severe alpha-1 antitrypsin deficiency (phenotype PiZZ) in family members [12][13][14][15]. Familial cases of W-CD are the exception rather than the rule, and some have speculated that cases of W-CD associated with alpha-1 antitrypsin deficiency might constitute a separate disease.…”
Section: Discussionmentioning
confidence: 99%
“…There have been reports of associations between W-CD and severe alpha-1 antitrypsin deficiency (phenotype PiZZ) in family members [12][13][14][15]. Familial cases of W-CD are the exception rather than the rule, and some have speculated that cases of W-CD associated with alpha-1 antitrypsin deficiency might constitute a separate disease.…”
Section: Discussionmentioning
confidence: 99%
“…The decrease of its action may lead to the development of a local exacerbated inflammatory response. α 1 ‐AT has been identified in the cytoplasm of cells, such as neutrophils, monocytes, macrophages, pancreatic islet cells, and mast cells, all of which have a high serine protease content. Thus, enzymatic deficiency may allow the proteolytic action of these cells in the surrounding connective tissue. The involvement of subcutaneous tissue is favored by high concentrations of fatty acids, which alter the conformation of elastin, rendering it more susceptible to degradation by neutrophil elastase, the main enzyme inhibited by α 1 ‐AT 9 …”
Section: Discussionmentioning
confidence: 99%
“…The involvement of subcutaneous tissue is favored by high concentrations of fatty acids, which alter the conformation of elastin, rendering it more susceptible to degradation by neutrophil elastase, the main enzyme inhibited by α 1 ‐AT 9 …”
Section: Discussionmentioning
confidence: 99%
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