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2017 ; 7
(2
): 91-101
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Tubuloreticular Inclusions in the Absence of Systemic Lupus Erythematosus and HIV
Infection: A Report of Three Pediatric Cases
#MMPMID28868299
Elmaghrabi A
; Brown E
; Khin E
; Hassler J
; Hendricks AR
Case Rep Nephrol Dial
2017[May]; 7
(2
): 91-101
PMID28868299
show ga
Tubuloreticular inclusions (TRIs) are subcellular structures located within the
cisternae of endoplasmic reticulum. Formation of TRIs has been linked to the
exposure of excess interferon (IFN), either from endogenous or exogenous sources.
In renal disease, TRIs have been most commonly associated with systemic lupus
erythematosus (SLE), and human immunodeficiency virus-associated nephropathy
(HIVAN). Case reports of patients with renal biopsies showing TRIs without
underlying SLE or HIV are infrequent in adults, and to our knowledge none have
been reported in children. We report 3 pediatric cases in which the renal biopsy
showed TRIs on electron microscopy without underlying SLE or HIV infection. The
first patient presented at 2 years of age with nephrotic syndrome and renal
failure. His renal biopsy revealed focal segmental glomerulosclerosis and TRIs.
The second patient presented at 6 months of age with infantile nephrotic
syndrome, and his renal biopsy revealed membranous glomerulopathy and TRIs. The
last patient presented at 4 years of age with acute kidney injury of unclear
etiology leading to chronic kidney disease. Her biopsy revealed acute and chronic
tubulointerstitial nephritis with TRIs. Despite extensive evaluation in all 3
patients, including testing for HIV infection and SLE, we could not identify an
underlying etiology to explain the presence of TRIs. In conclusion, renal biopsy
with TRIs in the absence of underling SLE and HIV remains obscure. We propose a
possible role for excess IFN triggered by an abnormal immune response to common
viral infections in the formation of TRIs and renal injury.