Fabry disease previously diagnosed as Henoch- Schonlein purpura
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Henoch-Schonlein purpura (HSP) is a generalized vasculitis characterized by manifestations of the skin, joints, gastrointestinal tract, and renal involvement. Although HSP can occur at any age and is usually a self-limited disease in children, it is less common and has a poor prognosis in adults. Renal involvement affects approximately one-third of HSP patients, and varies from intermittent hematuria and proteinuria, to severe nephrotic-nephritic syndrome with kidney histology being identical to that of immunoglobin A (IgA) nephropathy.
Abstract
Henoch-Schonlein purpura (HSP) is a generalized vasculitis characterized by manifestations of the skin, joints, gastrointestinal tract, and renal involvement. Although HSP can occur at any age and is usually a self-limited disease in children, it is less common and has a poor prognosis in adults. Renal involvement affects approximately one-third of HSP patients, and varies from intermittent hematuria and proteinuria, to severe nephrotic-nephritic syndrome with kidney histology being identical to that of immunoglobin A (IgA) nephropathy.
참고문헌 (5)
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- Whybra C, "IgA nephropathy in two adolescent sisters heterozygous for Fabry disease", Pediatr Nephrol, 21, pp.1251~1256 (2006)
- Shimohata H, "Hemizygous Fabry disease associated with IgA nephropathy: a case report", J Nephrol, 22, pp.682~684 (2009)