anderson-fabry disease is a rare inherited x-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase a. hereby we report a 39 year old male that presented with proteinuria and edema. histopathologic, immunofluorescence and ultrastractural examination of renal tissue were in favor  of  fabry disease in associate with iga nephropathy. fabry's disease associated wit...

Methods: One hundred and thirty two consecutive patients of nephrotic and nephritic syndrome, both children and adult, were included in the study. After baseline investigations and serum IgA level, 33 patients having clinical suspicion of IgA nephropathy and 1 patient of Henoch Schonlein purpura nephritis were admitted and renal biopsies were taken by well trained nephrologists after consent fr...

BACKGROUND There are many reports of incompletely glycosylated O-linked oligosaccharides on the IgA1 hinge region in certain IgA nephropathy patients. In addition, other reports have noted a relationship between tonsillectomy and IgA nephropathy. METHODS Immunoglobulins from extracts of tonsillectomized tissue and other sources were analysed by isoelectric focusing (IEF) and by enzyme-linked ...

  Anderson-Fabry disease is a rare inherited X-linked lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A. Hereby we report a 39 year old male that presented with proteinuria and edema. Histopathologic, immunofluorescence and ultrastractural examination of renal tissue were in favor  of  Fabry disease in associate with IgA nephropathy. Fabry's disease associated ...

IgA nephropathy is the most common form of primary glomerulonephritis worldwide. Mucosal infections and food antigens, including wheat gluten, have been proposed as potential contributing environmental factors. Increased immune reactivity to gluten and/or association with celiac disease, an autoimmune disorder triggered by ingestion of gluten, have been reported in IgA nephropathy. However, stu...

lar pathological finding in renal diseases, and it is observed in most proliferative nephritises (including immunoglobulin A (IgA) nephropathy). The mechanism of action of the expansion of the glomerular mesangial region, a particularly notable feature of IgA nephropathy, is not well elucidated, but it is presumed that the inflammatory reactions after deposition of IgA play a critical role. Spo...

Implication for health policy/practice/research/medical education: Thrombotic microangiopathy (TMA) occurs in IgA nephropathy, but is uncommon in the setting of IgA nephropathy. However, the reports of overlapping morphologic lesions of TMA and IgAN are poorly understood and debatable and needs more attention in larger series of IgAN. Copyright © 2013, Iranian Red Crescent Medical Journal; Publ...

Of the proposed causes and associations of nonuremic calciphylaxis, IgA is not included. We encountered a 60-year-old Hispanic female with stage III chronic kidney disease secondary to IgA nephropathy, who presented with a painful abdominal eschar. The diagnosis of nonuremic calciphylaxis was made, with IgA nephropathy being the most interesting potential cause. Our case represents the first re...

The diagnosis of IgA-dominant post-infectious glomerulonephritis (PIGN) may be challenging, as it must be differentiated from that of active IgA nephropathy. Predominant clinicopathologic features of IgA-dominant PIGN substantially overlap with those of active IgA nephropathy. Here, we present a case of a 67-year-old woman with rapidly rising serum creatinine, proteinuria and severe hypertensio...

abstract pseudoxanthoma elasticum is a rare, hereditary, multisystemic disease affecting the skin, eye, and cardiovascular system. renal involvement is uncommon. we describe two cases of pseudoxanthoma elasticum (pxe) in two women with distinctive skin lesions and nephrocalcinosis that renal ultrasonography showed a characteristic pattern of dotted increased echogenicity in the corticomedullary...