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Membranous nephropathy in a patient with hereditary angioedema: a case report.

Majoni SW, Smith SR - J Med Case Rep (2008)

Bottom Line: We present the challenges involved in the clinical management of the patient.This patient's presentation with severe nephrotic syndrome, renal impairment and hypertension required aggressive treatment of the membranous nephropathy given the high risk for progression to end stage renal failure.The contraindication to angiotensin converting enzyme inhibitors and angiotensin II receptor blockers in this patient, the lack of published evidence on the use of alkylating agents and other immunosuppressive agents in patients with hereditary angioedema and the lack of published data on the management of similar cases presented a clinical challenge in this patient's management.

View Article: PubMed Central - HTML - PubMed

Affiliation: Russells Hall Hospital Renal Unit, Dudley Group of Hospitals NHS Trust, Dudley, West Midlands, UK. sandawanaw@aol.com

ABSTRACT

Introduction: Hereditary angioedema is the commonest inherited disorder of the complement system and has been associated with several immune glomerular diseases. A case of nephrotic syndrome and renal impairment due to idiopathic membranous glomerulonephritis in a patient with hereditary angioedema has not been described before.

Case presentation: We present the first reported case of the association of membranous nephropathy and hereditary angioedema in a 43-year-old male Caucasian patient who presented with acute intestinal angioedema, hypertension, acute pancreatitis, renal impairment and generalised body swelling due to severe nephrotic syndrome. We present the challenges involved in the clinical management of the patient.

Conclusion: This patient's presentation with severe nephrotic syndrome, renal impairment and hypertension required aggressive treatment of the membranous nephropathy given the high risk for progression to end stage renal failure. The contraindication to angiotensin converting enzyme inhibitors and angiotensin II receptor blockers in this patient, the lack of published evidence on the use of alkylating agents and other immunosuppressive agents in patients with hereditary angioedema and the lack of published data on the management of similar cases presented a clinical challenge in this patient's management.

No MeSH data available.


Related in: MedlinePlus

Stage 3 membranous glomerulonephritis with medium-sized subepithelial dense deposits and basement membrane reaction surrounding most of the deposits (arrows) (transmission electron microscopy, original magnification ×11,000).
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Figure 1: Stage 3 membranous glomerulonephritis with medium-sized subepithelial dense deposits and basement membrane reaction surrounding most of the deposits (arrows) (transmission electron microscopy, original magnification ×11,000).

Mentions: Ultrasound scan showed normal sized kidneys and ascites, findings confirmed by computerised tomography (CT) scan. The CT also confirmed acute pancreatitis and bowel oedema. A renal biopsy performed 4 days after diuretic treatment to reduce the oedema showed stage 3 membranous glomerulonephritis (Figure 1).


Membranous nephropathy in a patient with hereditary angioedema: a case report.

Majoni SW, Smith SR - J Med Case Rep (2008)

Stage 3 membranous glomerulonephritis with medium-sized subepithelial dense deposits and basement membrane reaction surrounding most of the deposits (arrows) (transmission electron microscopy, original magnification ×11,000).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2577685&req=5

Figure 1: Stage 3 membranous glomerulonephritis with medium-sized subepithelial dense deposits and basement membrane reaction surrounding most of the deposits (arrows) (transmission electron microscopy, original magnification ×11,000).
Mentions: Ultrasound scan showed normal sized kidneys and ascites, findings confirmed by computerised tomography (CT) scan. The CT also confirmed acute pancreatitis and bowel oedema. A renal biopsy performed 4 days after diuretic treatment to reduce the oedema showed stage 3 membranous glomerulonephritis (Figure 1).

Bottom Line: We present the challenges involved in the clinical management of the patient.This patient's presentation with severe nephrotic syndrome, renal impairment and hypertension required aggressive treatment of the membranous nephropathy given the high risk for progression to end stage renal failure.The contraindication to angiotensin converting enzyme inhibitors and angiotensin II receptor blockers in this patient, the lack of published evidence on the use of alkylating agents and other immunosuppressive agents in patients with hereditary angioedema and the lack of published data on the management of similar cases presented a clinical challenge in this patient's management.

View Article: PubMed Central - HTML - PubMed

Affiliation: Russells Hall Hospital Renal Unit, Dudley Group of Hospitals NHS Trust, Dudley, West Midlands, UK. sandawanaw@aol.com

ABSTRACT

Introduction: Hereditary angioedema is the commonest inherited disorder of the complement system and has been associated with several immune glomerular diseases. A case of nephrotic syndrome and renal impairment due to idiopathic membranous glomerulonephritis in a patient with hereditary angioedema has not been described before.

Case presentation: We present the first reported case of the association of membranous nephropathy and hereditary angioedema in a 43-year-old male Caucasian patient who presented with acute intestinal angioedema, hypertension, acute pancreatitis, renal impairment and generalised body swelling due to severe nephrotic syndrome. We present the challenges involved in the clinical management of the patient.

Conclusion: This patient's presentation with severe nephrotic syndrome, renal impairment and hypertension required aggressive treatment of the membranous nephropathy given the high risk for progression to end stage renal failure. The contraindication to angiotensin converting enzyme inhibitors and angiotensin II receptor blockers in this patient, the lack of published evidence on the use of alkylating agents and other immunosuppressive agents in patients with hereditary angioedema and the lack of published data on the management of similar cases presented a clinical challenge in this patient's management.

No MeSH data available.


Related in: MedlinePlus