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Expression of mesangial IgA nephropathy in a family with several HLA-dentical members by Carol A FENTON-LEE; Bruce A PUSSELL; Mark A BROWN; John A CHARLESWORTH is a Medicine article available to read on EtoBox.

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SUMMARY: The familial incidence of mesangial IgA nephropathy (IgAN) is well recognized. However, the genetic implications of this finding remain uncertain. We report a family of East Timorese origin where six out of eight members, including the mother and five first‐degree offspring, have had histologically proven mesangial IgAN. All eight members have undergone HLA A, B, C and DR typing as well as determination of C4 allotype. Among the offspring, there are HLA‐identical male twins (A11,‐; B13,15; Cw3,‐; DR2,3) and three HLA‐identical females (A11,‐; B13,27; Cw3,‐; DR2,3). Their C4 allotype is also identical (i.e. A3; B1,2,96 and A3; B1,96, respectively). There were histological and biochemical differences between the twins: subject ii‐2 showed focal mesangial proliferation on light microscopy and a 24 h urine protein excretion of 1–2 g on presentation while subject ii‐3 was normal at that time. the latter subsequently underwent a renal biopsy 15 years later and this showed changes only on immunofluorescence microscopy. There were major differences in expression of renal disease among the HLA‐identical sisters: two developed end‐stage renal failure (ESRF) by the age of 30 years,

Who reads Expression of mesangial IgA nephropathy in a family with several HLA-dentical members?

Author

Carol A FENTON-LEE; Bruce A PUSSELL; Mark A BROWN; John A CHARLESWORTH

Publisher

John Wiley and Sons; Wiley (Blackwell Publishing); Blackwell Publishing Inc.; Wiley (ISSN 1320-5358)

Published

1998

Language

EN

Field

Medicine (Health Sciences)