Worsening fluid retention in a patient with hereditary angioedema and end-stage renal disease.

A 60-year-old woman who was diagnosed with hereditary angioedema (HAE) developed nephrotic syndrome, with end-stage renal disease (ESRD) occurring about 2.5 years later. During her slide toward ESRD, she experienced three severe episodes of angioedema that each resulted in significant fluid retention. Though the therapeutic administration of C1-inhibitor concentrate was effective in controlling her angioedema, seemed ineffective in preventing her from developing ESRD requiring hemodialysis treatment. We theorized that the patient's low colloid osmolality and glomerular perfusion were important facilitators of her attacks of angioedema.

[1]  A. Davis,et al.  Increased vascular permeability in C1 inhibitor-deficient mice mediated by the bradykinin type 2 receptor. , 2002, The Journal of clinical investigation.

[2]  R. Colman Role of the Light Chain of High Molecular Weight Kininogen in Adhesion, Cell-Associated Proteolysis and Angiogenesis , 2001, Biological chemistry.

[3]  S. Choquet,et al.  Frequent de novo mutations and exon deletions in the C1inhibitor gene of patients with angioedema. , 2000, The Journal of allergy and clinical immunology.

[4]  M. Cicardi,et al.  Local bradykinin generation in hereditary angioedema. , 1999, The Journal of allergy and clinical immunology.

[5]  K. Bhoola,et al.  Tissue kallikrein and kinins in renal disease. , 1999, Immunopharmacology.

[6]  M. Kojima,et al.  Activities of the MBL-associated serine proteases (MASPs) and their regulation by natural inhibitors. , 1999, Molecular immunology.

[7]  M. Cicardi,et al.  Pathogenetic and clinical aspects of C1 inhibitor deficiency. , 1998, Immunobiology.

[8]  P. Beck,et al.  IgA nephropathy in hereditary angioedema. , 1993, Postgraduate medical journal.

[9]  A. J. Mcadams,et al.  Long-term follow-up of non-systemic lupus erythematosus glomerulonephritis in patients with hereditary angioedema: report of four cases. , 1992, American journal of kidney diseases : the official journal of the National Kidney Foundation.

[10]  H. Mabuchi,et al.  Hereditary angioedema complicated with chronic renal failure: report of sibling cases. , 1992, Internal medicine.

[11]  Z. Oltvai,et al.  C1 inhibitor deficiency: molecular and immunologic basis of hereditary and acquired angioedema. , 1991, Laboratory investigation; a journal of technical methods and pathology.

[12]  B. Hory,et al.  Glomerulonephritis and hereditary angioedema: report of 2 cases. , 1989, Clinical nephrology.

[13]  P. Matricardi,et al.  Immune complexes in hereditary angioneurotic edema (HANE) , 1986, The Journal of allergy and clinical immunology.

[14]  J. Urmson,et al.  Hereditary angioneurotic edema: immunochemical 'activity' without clinical expression. , 1980, International archives of allergy and applied immunology.

[15]  J. Plaza,et al.  Hereditary angio-oedema with mesangiocapillary glomerulonephritis. , 1977, Postgraduate medical journal.

[16]  A. Michael,et al.  The complement system in chronic glomerulonephritis: three newly associated aberrations. , 1971, The Journal of pediatrics.