Acquired C1 esterase inhibitor deficiency or serendipity? The chance finding of a paraprotein after an apparently low C1 esterase inhibitor concentration
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Acquired C1 esterase inhibitor deficiency or serendipity? The chance finding of a paraprotein after an apparently low C1 esterase inhibitor concentration.
Acquired C1 esterase inhibitor deficiency is a rare condition, usually presenting after the 2nd decade of life, and is often related to underlying conditions such as autoimmune and lymphoproliferative disorders. This case report describes a man whose initial clinical presentation with acute angioedema and whose initial estimation of a low C1 esterase inhibitor concentration indicated that he ha...
متن کاملCLINICAL REVIEW Acquired C1 Esterase Inhibitor Deficiency
Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. I...
متن کاملangioedema in a patient with c1 esterase inhibitor deficiency
angioedema is characterized by recurrent, circumscribed, solitary or multiple subcutaneous and mucosal swelling, involving the extremities, face, larynx, bowel wall. angioedema is due to hereditary or acquired varieties of c1 esterase inhibitor (c1inh) deficiency. a case of atypical acquired angioedema in a 49-year old man, responding favourably to cinnarizine and alcohol abstinence, is present...
متن کامل[Hereditary angioedema due to C1-esterase inhibitor deficiency].
The paper presents a case of family oedema by shortage of C1-esterase inhibitor, characterized by appearance at an early age (8 months), participation in some attacks of nasal mucous membrane, with nasal obstruction and sinusal manifestations and also the abnormally long period, up to 9 days, of some localized oedemas. The crises were absent during pregnancy. The study of four generations of th...
متن کاملC1 esterase inhibitor deficiency, airway compromise, and anesthesia.
H ereditary angioedema (HAE) is a serious genetic abnormality involving the complement system and characterized by episodic and sometimes life-threatening airway edema. In 1882, von Quincke (1) published the first detailed description, and 3 yr later, Strubing used the term angioedema to describe this disorder. By 1888, Osler (2) had demonstrated the hereditary nature of the clinical presentati...
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ژورنال
عنوان ژورنال: Journal of Clinical Pathology
سال: 2004
ISSN: 0021-9746
DOI: 10.1136/jcp.2003.013524