Glomerular deposition of alpha 2-macroglobulin in a child with steroid refractory nephrotic syndrome.
A four-year-old male with steroid refractory nephrotic syndrome was found to have diffuse deposition of alpha 2-macroglobulin (alpha 2M) in the glomeruli which showed diffuse mesangial proliferation and partial hyalinization by light microscopy. Camostat mesylate, a commercially available synthetic proteinase inhibitor, led to biochemical and clinical improvement. Twenty-two patients with 7 biopsy-proven renal diseases did not have any deposition of alpha 2M in their kidney tissue. Though the pathogenetic mechanism is unknown, this is probably the first report of the deposition of alpha 2M in renal tissue.
Asami
T
T
Department of Pediatrics, School of Medicine, Niigata University, Japan.
Ohsawa
S
S
Tomisawa
S
S
Hashimoto
K
K
Toyabe
S
S
Sakai
K
K
D002675:Child, Preschool; D004952:Esters; D016670:Gabexate / Q000031:analogs & derivatives*; D006146:Guanidines / Q000627:therapeutic use; D006801:Humans; D007678:Kidney Glomerulus / Q000378:metabolism*; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology; D011480:Protease Inhibitors / Q000627:therapeutic use; D013256:Steroids / Q000627:therapeutic use; D000511:alpha-Macroglobulins / Q000378:metabolism*
D004952:Esters; D006146:Guanidines; D011480:Protease Inhibitors; D013256:Steroids; D000511:alpha-Macroglobulins; C034532:camostat; D016670:Gabexate