Case Of Minimal Change Disease Research Articles

Molecular Basis of Proteinuria

Molecular Basis of Proteinuria

Adult-Onset Minimal Change Disease among Taiwanese

There are some racial differences in the prevalence and prognosis of idiopathic nephrotic syndrome; however, reports about minimal change disease (MCD) in Chinese were rare. We retrospectively analyzed 123 Chinese adults with idiopathic nephrotic syndrome, who received percutaneous renal biopsy in our institution within the last 10 years. In total, 46 patients (37.4%) were compatible with the pathological diagnosis of MCD. The male to female ratio was 1.2:1. The mean age of onset was 30.9 years, and 80% of the patients with MCD were less than 40 years. The mean daily proteinuria was 10.2 g, and serum albumin was 1.8 mg/dl. Azotemia occurred in 16 (35%) of 46 cases; hypertension, 13%; and microscopic hematuria, 13%. High selectivity index for proteinuria (SI <0.1) was noted in 12 (39%) of 31 cases; and high IgE level was found in 83.7% of the study subjects, although only one case had allergic history. Complete remission in 36 MCD patients treated with corticosteroid was achieved by 42% (15/36), 80% (29/36), and 94% (34/36) within 4, 8, and 12 weeks, respectively. The time interval to remission was similar between the younger group (<40 years old, 1.7 months) and older group (>40 years old, 1.6 months). Nineteen (56%) of 34 cases with steroid response did not relapse, and the other cases (44%) had a mean relapse rate of 1.5 times per patient within a period of 45 months. The age of onset in MCD cases was not significantly correlated with steroid-responsive rate, and the time interval to remission. However, a tendency existed between the onset in the young age and the sequentially relapsing rate (p = 0.06). Two cases with primary steroid resistance and 5 cases with frequent relapse or steroid dependence responded well to intravenous pulse therapy of cyclophosphamide, except one refractory case. No thrombotic episode was ever noted in our group. Regarding infectious complications, primary peritonitis occurred in one, pneumonia in one, and cellulitis in 6 cases during active nephrotic stage. Two mortality cases, one with E. coli-related necrotizing fasciitis and one from pneumonia, were noted. In brief, compared with children, adult patients with MCD had lesser high selectivity index for proteinuria, the same steroid-responsive rate (94%), but slower response, and significantly lesser relapsing rate. The intravenous pulse therapy of cyclophosphamide may be an alternative regimen for adult patients with steroid resistance or dependency. In addition, the Asian adult-onset MCD had younger age, male predominance, and lesser relapsing rate in comparison to those of the Western population.

Immunoglobulin-E-specific suppressor factors in primary glomerulonephritis with nephrotic syndrome.

Human immunoglobulin (Ig) E synthesis is regulated by T-cell-derived binding factors which include potentiating factors and suppressor factors (IgE-SFs) and B-cell-derived factors. Seventeen cases of primary glomerulonephritis (GN) with nephrotic syndrome and high serum IgE were studied for their serum IgE-SFs. These include 8 cases of minimal-change disease (MCD), 3 cases of IgM nephropathy, 4 cases of focal segmental glomerulosclerosis, and 2 cases of IgA nephropathy, aged between 20 and 60 years (mean: 37.2). A significantly lower activity of these factors was noted in the patient group as shown by a lower overall inhibition rate on in vitro IgE synthesis (median: 22.9 vs. 53.2%, p less than 0.05). Some of them even exhibited an enhancing activity in their serum as shown by a negative inhibition rate. These findings suggest that there is a T cell disorder in some of the primary GNs with high serum IgE, especially in MCD, which causes an abnormal regulation of IgE synthesis.

Anti-α-galactosyl antibodies and immune complexes in children with Henoch–Schönlein purpura or IgA nephropathy

Episodes of hematuria in IgA nephropathy or Henoch-Schönlein purpura are frequently associated with microbial infections. Some of those infectious agents bear alpha-galactosyl residues on their cell surface. These observations prompted us to determine, by passive hemagglutination, the titers of natural anti-galactosyl antibodies in the serum of children presenting with Henoch-Schönlein purpura (10 cases) or IgA nephropathy (7 cases). Antibody titers of normal subjects (103 cases), children with a pharyngitis of unknown etiology (7 cases), and children exhibiting mesangial IgA deposits but no hematuria at the time of testing (6 cases) ranged from 1:20 to 1:80. Elevated titers (greater than 1:80) were observed in nine of 11 patients with mesangial IgA deposits and micro- or macroscopic hematuria, in nine of 19 children with other evolutive glomerular diseases (5 cases of acute glomerulonephritis and 4 cases of minimal change disease), and in most subjects presenting with a M. pneumoniae (4/5 cases) or a E. Coli (4/5 cases) infection. Antibody titers decreased after incubation of normal and pathological sera with D-galactose (10 mM) or with alpha-galactosyl-glucoside (10 mM), but not with D-glucose (10 mM). The anti-alpha-galactosyl antibodies purified, by affinity chromatography, from sera of 10 normal children, 10 pathological controls and four children with mesangial IgA deposits without hematuria belonged to IgG class. In contrast, both IgG and IgA anti-alpha-galactosyl antibodies were detected in six of six patients with mesangial IgA deposits and hematuria. The IgA content of immune complexes detected in those patients decreased after incubation of sera with alpha-galactosyl-glucoside, but not with D-glucose.(ABSTRACT TRUNCATED AT 250 WORDS)

Scanning electron microscopy of acellular glomeruli in human glomerulonephritis: application of a technique.

Scanning electron microscopy (SEM) has been employed in studies of human and experimental glomerulonephritis (GN), a major contribution being the elucidation of podocyte morphology and the process of podocyte foot process retraction in proteinuric conditions. Application of SEM in GN has been limited however by an emphasis on cell surface alterations while the site of major disease processes, the glomerular basement membrane, has remained hidden from view. A previously reported technique for the preparation of acellular glomeruli from fresh tissue has been adapted for use on frozen human renal biopsies. One case of minimal change disease (MCD) and one case of membranous glomerulonephritis (MGN) have been studied. The acellular glomerular basement membrane of MCD has a lightly textured or granular surface whereas in MGN a striking reticular network of basement membrane has formed perpendicular to the native basement membrane. The immune complexes have been removed. This technique provides a graphic visualization of GBM alterations occurring in glomerulonephritis and is applicable to the study of human as well as experimental model of glomerulonephritis.

Circulating immune complex in glomerulonephropathy associated with hepatitis B virus infection.

Urinary abnormalities combined with positive serum hepatitis B virus surface antigen (HBsAg) were found in 9 children. Most of these patients were found by mass urine screening of school children. Renal biopsy findings revealed 6 cases of membranous glomerulonephropathy (MGN) and 3 cases of minimal change disease. Immune deposits consisting of hepatitis B virus e antigen (HBeAg), IgG and C3 were detected in the glomerular capillary walls by immunofluorescent study in patients with MGN. Circulating immune complex was positive at a rate of 67% (6/9 cases) by the C1q-binding assay and at a rate of 56% (5/9 cases) by the protein-A precipitation test in patients with persistent urinary abnormalities and hepatic dysfunction.