1/39. Hemolytic uremic syndrome associated with immunoglobulin a nephropathy: a case report and review of cases of hemolytic uremic syndrome with glomerular disease.A 35-year-old man with immunoglobulin a (IgA) nephropathy who developed hemolytic uremic syndrome (HUS) presented with transient elevation of serum creatinine, thrombocytopenia, and hemolytic anemia with fragmented red cells with nephrotic syndrome. Hemolytic anemia and the temporarily deteriorated renal function were improved after hemodialysis and plasma exchange. Histological findings were consistent with HUS and IgA nephropathy. Including this case, we reviewed the cases of HUS accompanied by glomerular diseases reported from 1969 to 1996. Surprisingly, most cases showed nephrotic syndrome at the onset of HUS. Several possible relationships between HUS and nephrotic syndrome are discussed.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
2/39. nail-patella syndrome and IgA nephropathy in a Chinese woman.nail-patella syndrome (NPS), also known as hereditary onycho-osteodysplasia, is an autosomal dominant pleiotropic disorder characterized by nailbed dysplasia or hypoplasia, absent or hypoplastic patellae, iliac horns and deformation or luxation of the radial head. Nephropathy is a known serious complication associated with NPS. In this report, we describe an adult Chinese woman with the clinical and radiological features of NPS who presented with the nephrotic syndrome. Renal biopsy disclosed focal segmental glomerulosclerosis on light microscopy, while immunofluorescence revealed predominant staining for IgA in the glomerular mesangium and along some capillary walls. Ultrastructural study confirmed the presence of paramesangial deposits as well as subendothelial collagen fibrils in the glomeruli. The histological findings were those of combined NPS and IgA disease, an association which has rarely been described.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
3/39. immunoglobulin a nephropathy associated with hepatitis a virus infection.Renal involvement in association with nonfulminant hepatitis a virus (HAV) infections have been reported infrequently and when present have been characterized by mild proteinuria, microscopic hematuria and slight urinary sediment abnormalities. Acute renal failure and nephrotic syndrome are extremely rare complications. This report describes a case of acute renal failure and nephrotic syndrome following a nonfulminant course of HAV infection with biopsy proven immunoglobulin a nephropathy. The cause of acute renal failure in this case is primarily due to tubulointerstitial nephritis secondary to pigment toxicity.- - - - - - - - - - ranking = 0.66666666666667keywords = nephrotic syndrome (Clic here for more details about this article) |
4/39. Superimposition of post-streptococcal acute glomerulonephritis on the course of IgA nephropathy: predominance of Th1 type immune response.A 23-year-old man was admitted with macrohematuria and systemic edema appearing after an acute upper respiratory tract infection. He had been diagnosed 6 years earlier with IgA nephropathy (IgA-N). On admission, hypertension, nephrotic syndrome and hypocomplementemia were evident together with a high titer of anti-streptokinase (ASK). Renal biopsy showed severe glomerular mesangial proliferation, segmental endocapillary proliferation and crescent formation. Immunofluorescence microscopy (IF) showed strong deposition of C3 and reduced deposition of IgA. Electron microscopy showed a so-called "hump" on the epithelial side of the glomerular basement membrane.These features were consistent with post-streptococcal acute glomerulonephritis (PSAGN) superimposed on IgA-N. Following 2 weeks of observation, blood pressure, C3 level and ASK titer returned to normal ranges, although nephrotic syndrome was still evident, which necessitated oral prednisolone (30 mg/day) therapy. Another biopsy taken 2 months later demonstrated regression of endocapillary proliferation and IF showed decreased deposition of C3. Immunohistochemical staining of the specimen taken on admission revealed the presence of numerous T cells and macrophages in the interstitium. macrophages were also seen in the glomerular tuft. Many interstitial infiltrating cells were positive for interferon-gamma, but their number diminished after treatment. Our findings suggest that PSAGN complicating pre-existing IgA-N activates cellular immunity and augments renal tissue injury.- - - - - - - - - - ranking = 0.66666666666667keywords = nephrotic syndrome (Clic here for more details about this article) |
5/39. IgA nephropathy (IgAN) presenting with the nephrotic syndrome.A 20 year old Nigerian male with the nephrotic syndrome was found to have IgA nephropathy (IgAN) on renal biopsy. The renal function at presentation was normal and his nephrotic syndrome initially responded to steroid therapy. However, significant deterioration in renal function was noticed 2 years later when his nephrotic syndrome relapsed. This is the first documented case of IgAN in a Nigerian. Considering the rather low renal biopsy rate in this environment, a number of cases with this nephropathy may be missed.- - - - - - - - - - ranking = 2.3333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
6/39. brucellosis with nephrotic syndrome, nephritis and IgA nephropathy.A patient with systemic brucellosis due to brucella melitensis had severe renal involvement. Clinical features included hypertension, macroscopic haematuria, massive proteinuria of 10 g per 24 hours and azotaemia. Following treatment with antibiotics, the azotaemia resolved and proteinuria decreased to less than 0.5 g per 24 hours, but microscopic haematuria and hypertension persisted. Renal biopsy during recovery revealed IgA nephropathy with minimal mesangial changes, suggesting a causal relation between brucellosis and IgA nephropathy with a reversible nephrotic syndrome.- - - - - - - - - - ranking = 1.6666666666667keywords = nephrotic syndrome (Clic here for more details about this article) |
7/39. IgA-associated glomerulonephritis with membranoproliferative glomerulonephritis-like pattern in two children.In this article, we report two patients with IgA-associated glomerulonephritis with a membranoproliferative glomerulonephritis (MPGN) -like pattern. Both patients had nephrotic syndrome at onset. One patient was treated with high-dose alternate-day prednisolone (PSL), and the other with indomethacin and low-dose PSL. One lost the urinary abnormalities 3 years after starting treatment. The other lost the nephrotic state and hematuria over a 5-year period, but proteinuria persisted until the last follow-up. Both patients had diffuse proliferative changes with mesangial interposition and subendothelial deposits, associated with strongly positive deposits of C3 and IgA along the capillary walls of the glomeruli. These two patients showed histological changes compatible with type-I MPGN, but the pattern of IgA deposits was not typical of idiopathic MPGN or IgA nephropathy. We assume this is a rare form of MPGN, not associated with liver disease or other systemic diseases.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
8/39. Secondary IgA nephropathy presenting as nephrotic syndrome with glomerular crescentic changes and acute renal failure in a patient with autoimmune hepatitis.patients with end-stage liver disease are prone to hemodynamic and immunologic renal injury, the latter at times manifesting as glomerulonephritis. Elevated serum immunoglobulin a (IgA) levels and mesangial IgG-IgA deposits are common in these patients, but are often clinically silent. We report a patient with autoimmune hepatitis and secondary IgA nephropathy (IgAN) who presented with nephrotic syndrome, acute renal failure (ARF), with 30% of the renal glomeruli having undergone crescentic change, and with IgA2 deposits in the glomerular mesangium. This article discusses secondary IgAN pathogenesis and its therapeutic management.- - - - - - - - - - ranking = 1.6666666666667keywords = nephrotic syndrome (Clic here for more details about this article) |
9/39. IgA-dominant glomerulonephritis associated with hepatitis A.Unlike hepatitis b and C, renal involvement has been extremely uncommon in patients with hepatitis Avirus (HAV) infection. nephrotic syndrome has been documented as a rare complication in association with HAV infection. In this report, we describe a patient with serologically documented HAV infection, who presented with nephrotic syndrome. The renal biopsy showed an immunoglobulin a- (IgA) dominant glomerulonephritis (GN) with subendothelial immune deposits. This is the second biopsy-proven case report of a patient with acute HAV associated with IgA-dominant immune complex glomerulonephritis and nephrotic syndrome. This is perhaps the first case in which a patient experienced both IgA-dominant glomerulonephritis and cutaneous cryoglobulinemic vasculitis.- - - - - - - - - - ranking = 0.66666666666667keywords = nephrotic syndrome (Clic here for more details about this article) |
10/39. Unusual presentation of IgA nephropathy in childhood: a case report.immunoglobulin a (IgA) nephropathy is the most common form of primary glomerulonephritis worldwide, and approximately 20% to 30% of adult patients with the disorder develop chronic renal failure within 20 years. This type of nephropathy is also an important risk factor for chronic renal failure in children. The pathogenesis of IgA nephropathy is still unknown, and treatment remains controversial. Microscopic hematuria and recurrent episodes of macroscopic hematuria are the most common clinical manifestations of this condition in children. This article describes the case of a young girl who presented with steroid-resistant nephrotic syndrome unaccompanied by hematuria. Renal biopsy findings were consistent with IgA nephropathy. The patient's condition was a rare clinical manifestation of IgA nephropathy.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
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