Immunotactoid glomerulopathy in sickle cell anemia |
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Authors: | Diego H Aviles Randall Craver R P Warrier |
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Institution: | (1) Department of Pediatrics, Louisiana State University Health Sciences Center and Children’s Hospital, New Orleans, Louisiana, USA, US;(2) Louisiana State University School of Medicine, 1542 Tulane Avenue, T8-1, New Orleans, LA 70112, USA e-mail: davile@mail.lsumc.edu Tel.: +1-504-568-2875, Fax: +1-504-568-5953, US |
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Abstract: | A 12-year-old African American male with homozygous sickle cell disease (SCD) was admitted with insidious onset of periorbital
and scrotal edema. The initial evaluation failed to reveal any underlying monoclonal gammopathy, or cryoglobulinemia, or other
systemic causes for the renal disease. A percutaneous renal biopsy was consistent with immunotactoid glomerulopathy (ITG),
which is rare in children and is characterized histologically by fibrillar deposits in the glomeruli. Children can present
with symptoms of nephrotic syndrome and progress to end stage renal disease. Our patient was treated with an ACE inhibitor
and is currently free of edema and with normal renal function on follow-up at 1 year. Immunotactoid glomerulopathy should
be considered in the differential diagnosis of nephrotic syndrome in children with sickle cell disease. Renal biopsy is indicated
in children with sickle cell disease and nephrotic syndrome and ITG should be considered as potential cause. Although there
is no effective treatment for this condition, ACE inhibitors can decrease the proteinuria and possibly delay the progression
to end stage renal disease. The side effects related to the use of ACE inhibitors should be monitored. These include renal
impairment, hyperkalemia, anemia, neutropenia, and angioedema. Since we have a short follow-up in our patient, the role and
safety of ACE inhibitors in the management of ITG need further evaluation.
Received: 4 February 2000 / Revised: 3 July 2000 / Accepted: 19 July 2000 |
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Keywords: | Immunotactoid nephropathy Nephrotic syndrome Sickle cell anemia |
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