Cl inhibitor deficiency

Cl inhibitor deficiency causes angioedema as a result of excessive bradykinin production. Thus the pathways and control mechanisms for bradykinin formation and... 

Caldwell J, Ruddy S, Schur P, Austen K Acquired Cl inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol 1972 1 39-52. 

Hauptmann G, Lang J, North M, Oberling F. Mayer G, Lachmann P Acquired Cl-inhibitor deficiencies in lymphoproliferafive diseases with serum immu-noglobuhn abnormalities. A study of three cases. Blut 1976 32 195-206. 

Schreiber A, Zweiman B, Atkins P. Goldwein F, Pietra G, Atkinson B, et al Acquired angioedema with lymphoproliferafive disorder association of Cl inhibitor deficiency with cellular abnormality. Blood 1976 48 567-580. 

Zuraw B, Curd J Demonstration of modified inactive first component of complement (Cl) inhibitor in the plasmas of Cl inhibitor-deficient patients. J Chn Invest 1986 78 567-575. 

Zuraw B, Sugimoto S, Curd J The value of rocket Immunoelectrophoresis for C4 activation in the evaluation of patients with angioedema or Cl-inhibitor deficiency. J Allergy Clin Immunol 1986 78 1115-1120. 

Activation of factor XII and cleavage of high molecular weight kininogen during acute attacks in hereditary and acquired Cl-inhibitor deficiencies. Immunopharmacology 1996 33 361-364. 

Han E, MacFarlane R, Mulhgan A, Scafidi J, Davis AR Increased vascular permeabihty in Cl inhibitor-deficient mice mediated by the bradykinin type 2 receptor. J Clin Invest 2002 8 1057-1063. 

Geha R, Quinti I, Austen K, Cicardi M, Sheffer A, Rosen F Acquired Cl-inhibitor deficiency associated with anti-idiotypic antibody to monoclonal immunoglobuhns. N Engl J Med 1985 312 534-540. Donaldson V, Hess E, McAdams A Lupus-erythe-matosus-like disease in three umelated women with hereditary angioneurotic edema. Arm Intern Med 1977 86 312-313. 

Bork K, Witzke G. Long-term prophylaxis with Cl-inhibitor (Cl INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired Cl-inhibitor deficiency. J Allergy Clin Immunol 1989 83(3) 677-82. 

Age. Synthesis levels in infants and children are up to three times adult levels, perhaps as a protective mechanism against increased exposure to intestinal proteases in infancy and bacterial or leukocytic proteases during childhood. The high levels may also be responsible in part for the delay in development of clinical signs and symptoms until after puberty in most individuals with either antithrombin III or Cl inhibitor deficiency. 

Acquired Depciency. Levels of C4 are more commonly depressed because of consumption > 60% of individuals with SLE and low C4 levels do not have genetic deficiency. Other disorders associated with consumption and low levels include hereditary angioedema (Cl inhibitor deficiency), autoimmune hemolytic anemia, and autoimmune nephritides. 

Milk-derived recombinant human C1 inhibitor Pharming is conducting human cHni-cal trials for recombinant human Cl inhibitor (rhCll) in patients suffering hereditary angioedema (HAE), a condition caused by human Cl inhibitor deficiency [58]. Human Cl inhibitor is a 105 kDa protein which has a critical role in the inhibition of proteases involved in fibrinolytic and complement pathways. Deficiency of this inhibitor is manifested in acute swelling of soft tissues in the hands, feet, limbs, face, intestinal tract, mouth, or airway (larynx or trachea). If swelling closes the air-... 

Lumry W, Manning ME, Hurewitz DS, Davis-Lorton M, Fitts D, Kalfus IN, et al. Nanofiltered Cl-esterase inhibitor for the acute management and prevention of hereditary angioedema attacks due to Cl-inhibitor deficiency in children. J Pediatr May 2013 162(5) 1017-22. el-2. 

---