Monogenic lupus due to DNASE1L3 deficiency in a pediatric patient with urticarial rash, hypocomplementemia, pulmonary hemorrhage, and immune-complex glomerulonephritis

Author(s):  
Rabia Miray Kisla Ekinci ◽  
Sibel Balci ◽  
Dilek Ozcan ◽  
Bahriye Atmis ◽  
Atil Bisgin
Nephron ◽  
1992 ◽  
Vol 60 (1) ◽  
pp. 49-55 ◽  
Author(s):  
T. Ideura ◽  
M. Ogasawara ◽  
S. Tomura ◽  
T. Ida ◽  
Y. Chida ◽  
...  

PEDIATRICS ◽  
1985 ◽  
Vol 75 (6) ◽  
pp. 1127-1131
Author(s):  
Ching-Yuang Lin ◽  
Hey-Chi Hsu ◽  
Han-Yang Hung

A 4-year-old boy developed nephrotic syndrome following varicella infection. Serologic studies during the early phase of the disease demonstrated a decrease in serum C3, C4, and properdin factor B. Renal biopsy revealed an acute proliferative glomerulonephritis with deposition of immunoglobulins A (IgA) and M, C3, Clq, and varicella virus antigen in the glomerulus, suggesting an immune complex deposition. Ultrastructurally, this suggested a postinfectious immune complex glomerulonephritis. These phenomena suggested that varicella virus antigen antibody complexes were deposited in the glomerulus and activated the classic and alternative pathway of complements, leading to an immune complex glomerulonephritis. During the nephrotic phase, an increase in OKT8 cells and decrease of the OKT4 cells were demonstrated. Two months later, this alteration returned to normal as the renal disease was in remission. This change of lymphocyte subsets during varicella infection may play a role in the pathogenesis of nephrotic syndrome.


2018 ◽  
Vol 102 ◽  
pp. 130-131
Author(s):  
Alexander Jacob ◽  
Jessica Reynolds ◽  
Anthony Chang ◽  
Richard Quigg ◽  
Jessy Alexander

2006 ◽  
Vol 10 (1) ◽  
pp. 68-73 ◽  
Author(s):  
Hiroshi Kanamori ◽  
Maiko Ota ◽  
Hiroya Takeoka ◽  
Kenji Osafune ◽  
Seibun Yonezu ◽  
...  

Author(s):  
Christine K. Abrass ◽  
Wayne A. Border ◽  
Richard J. Glassock

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