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Biomedical subjects

Y Hiki

Publications and source records attributed to Y Hiki.

At least 109 records · Page 6Linked to original sources

Association of HLA-DRw8 and DQw3 with minimal change nephrotic syndrome in Japanese adults.

The HLA systems of forty Japanese patients with adult-onset nephrotic syndrome and biopsy-proven minimal change were investigated. HLA-DRw8 was found in 35% of the patients and HLA-DQw3 in 95%, compared to 12.6 and 63.1% of the control, respectively (DRw8: and Pc less than 0.01, RR = 3.74; DQw3: Pc less than 0.02, RR = 11.1). The phenotype frequencies of all but one HLA-DR antigens, DR4, DR5, DRw8, and DRw9 associating with DQw3, were observed to increase (patient vs. control: DR4, 65 vs. 41.4%; DR5, 10 vs. 4.3%; DRw9, 20 vs. 23%). These results suggest that HLA-DQw3 may be a primary genetic marker associated with a major susceptibility gene to adult-onset minimal change nephrotic syndrome in the Japanese population.

Adolescent↗

Coexistence of IgA nephropathy and membranous nephropathy.

Renal biopsy findings of a 27-year-old female with asymptomatic proteinuria and hematuria revealed two distinct glomerular alterations compatible with both IgA nephropathy and membranous nephropathy. The concomitant presence of two different primary glomerular diseases is very rare.

Adult↗

Renal retinal dysplasia with diffuse glomerular cysts.

A 32-year-old male with renal retinal dysplasia is presented. He also showed hearing loss and growth retardation. Laboratory data showed mild proteinuria, renal dysfunction and type-1 renal tubular acidosis. Computed tomography showed multiple cysts at the corticomedullary junction of both kidneys. Ocular examinations disclosed retinitis pigmentosa. On light microscopy of renal biopsy specimens, diffuse cystic dilatation of Bowman's space as well as dilated tubules with interstitial fibrosis and cellular infiltration were noted. Electron microscopy revealed a peculiar chromatin condensation of epithelial cell nuclei in Bowman's capsules, glomeruli and tubules. The association of renal retinal dysplasia with diffuse glomerular cysts has not, to our knowledge, been previously reported.

Acidosis, Renal Tubular↗

Strong association of HLA-DR2 and MT1 with idiopathic membranous nephropathy in Japan.

Fifty adult Japanese patients with biopsy-proven idiopathic membranous nephropathy (IMN) were typed for HLA-A, B, and DR antigens, and additional B cell alloantigens designated the MT system. The phenotype frequencies of HLA-DR2 (80.0 vs. 36.0%) and MT1 (96.0 vs. 49.5%) appeared to be significantly increased in the patient group when compared to those of a control. There were lower frequencies of DR4, DRw9, MT3, and MT4 in these particular patients. The decreases of MT3 and MT4 were statistically significant but those of DR4 and DRw9 were not. These results suggest that HLA-DR2 and MT1 antigens are related to the occurrence of IMN in the Japanese population.

Adult↗

Glomerular tissue injury in IgA nephritis.

The glomerular lesions in 129 cases of IgA nephritis were analyzed. The development of glomerular injury occurred in two ways, one being a chronic mesangial depositive and sclerosing lesion commonly found in most glomeruli, and the other an acute but local injury initiating from the local peripheral glomerular basement membrane abnormality, i.e., thinning and/or splitting, which was seen at least in one third of all cases. The activation of the local coagulatory process could add segmental glomerular changes including small crescents, adhesion, and local tuft necrosis to the mesangial lesion.

Adolescent↗

IgA nephropathy: prognostic significance of proteinuria and histological alterations.

A retrospective study of 166 patients with IgA nephropathy was undertaken to clarify possible correlations between clinical and histological features, and the severity and prognosis of the disease. At the time of biopsy, impaired renal function, with creatinine clearance (Ccr) below 90 ml/min was found in 61 cases. At the final examination, after a mean follow-up period of 34 months, 82 patients had impaired renal function, 12 of these patients went into terminal renal failure requiring hemodialysis treatment. The presence of proteinuria of more than 1.0 g/day was closely correlated with impairment of renal function both at the time of biopsy and at the final observation. An unfavorable outcome was also anticipated in the presence of hypertension. In contrast, microhematuria, macrohematuria or high serum IgA levels did not appear to be related to the outcome. Histologically, sclerotic lesions such as mesangial or global sclerosis, interstitial fibrosis and tubular atrophy, and some active changes such as mesangial hypercellularity and tuft adhesion were more frequent and severe in patients with impaired renal function. Impressive localization of IgA and C3 in the mesangium as well as in capillary loops was observed more often in these patients. These results clearly indicate that IgA nephropathy may follow a slowly progressive course in about half of the patients, and that marked proteinuria and severe histological changes appear to correlate closely with an unfavorable course.

Adult↗

Study of lupus nephritis: its classification and the significance of subendothelial deposits.

This study describes the clinical and pathological characteristics of 74 patients with lupus nephritis classified according to renal biopsy findings using light, electron and immunofluorescent microscopy, and further, assesses the significance of subendothelial deposits in evaluating disease activity. In membranous lupus nephritis (14 cases), many cases showed normal renal function even with the nephrotic syndrome, although five cases had little or no urinary abnormalities. Glomerular cellular proliferation was very mild and subepithelial deposits with a few mesangial deposits were the main pathological alterations. Mesangial proliferative lupus nephritis (17 cases) clinically had very mild renal disease. Renal biopsies in this group revealed mesangial deposits with slight cellular proliferation. Although clinical features of mild diffuse proliferative lupus nephritis (16 cases) were similar to those of mesangial lupus nephritis, glomerular loop deposits were seen in addition to mesangial deposits. In moderate diffuse proliferative lupus nephritis (17 cases), renal function was slightly decreased, moderate proteinuria with haematuria were found, and C3 level was low. Renal biopsies showed active proliferative changes, and subendothelial deposits were frequently seen. In severe diffuse proliferative lupus nephritis (10 cases), the duration from onset of SLE to renal biopsy was short. Impairment of renal function, and nephrotic syndrome with haematuria and hypocomplementemia were frequent. Only three patients survived in this group. Renal biopsies demonstrated highly active proliferative and necrotizing changes, and electron microscopy showed massive subendothelial and mesangial deposits accompanied by subepithelial and intramembranous deposits. The amount of subendothelial deposits correlated with those of mesangial deposits and subepithelial deposits in the cases with diffuse proliferative lupus nephritis. Urinary protein loss and histologic activity showed statistically significant correlations with the amount of subendothelial deposits, but C3 levels and creatinine clearance revealed negative correlations with those deposits.

Adult↗

Nephrotic syndrome with diffuse mesangial IgM deposits.

The renal biopsies of three cases of nephrotic syndrome were examined by light, electron and immunofluorescent microscopy. The histological appearances were characterized by mild mesangial proliferation and extensive diffuse and global deposition of IgM in the mesangial areas, with some in the capillary walls. C3 and C1q were also identified in two of the cases. Electron microscopy revealed scanty electron dense deposits in the mesangial areas and subendothelial spaces. All three patients had a nephrotic proteinuria but no hematuria. None had hypertension or renal dysfunction throughout their clinical courses. They received steroid therapy and achieved complete remission within one month after the initial treatment. Two of them, however, have had frequent relapses whenever the dosage of steroid was gradually reduced. One case continues to be free of proteinuria. These cases indicate that a distinct disease may exist, because of the uniform immunofluorescent findings. We wish to designate it as IgM nephropathy.

Adult↗

Prednisone treatment of non-nephrotic patients with idiopathic membranous nephropathy. A prospective study.

18 adults with nonnephrotic proteinuria showed a milder form of idiopathic membranous nephropathy on renal biopsy. These patients were allocated to two groups, 2 years' steroid treatment or nontreatment. 8 of 9 tested patients showed complete disappearance of proteinuria in an average time of 2.4 months after starting the treatment, but in 9 patients of the nontreatment group proteinuria continued for at least 1 year. All five repeated biopsy specimens obtained after subsidence of proteinuria in the treatment group revealed a distinctive regression of glomerular basement membrane alterations. Though the study was not a controlled one, these results suggest that steroid therapy in idiopathic membranous nephropathy is beneficial, particularly in patients with lesser degrees of basement membrane lesions.

Adolescent↗

Strong association of HLA-DR4 with benign IgA nephropathy.

103 adult patients with biopsy-proved IgA nephropathy were typed for HLA-A and HLA-B antigens and 80 of these cases were typed for HLA-DR antigens. A significant association with HLA-DR4 was clearly noted (pc less than 0.04). The high phenotype frequency (PF) of BW35 was observed in the patients, but not statistically significant (pc less than 0.2). Two groups, the stable group and progressive group, were selected from all patients according to their clinical courses. The PF of HLA-DR4 was significantly higher in the stable group than the progressive group (pc = 0.005). The PF of BW35 was also higher in the stable group, but this difference was not statistically significant (pc = 0.6). The frequency of the combination of HLA-BW35 and DR4 increased significantly as compared to that in the control group. Further, the patients who had both of these antigens showed favorable courses. These results suggest that the HLA-DR4 antigen, especially a combination of HLA-BW35 and DR4 antigens are related to the occurrence of benign IgA nephropathy.

Adult↗

Effect of methylprednisolone on progressive Masugi nephritis in the rabbit. I. Suppression of antibody production and crescent formation.

Experiments were undertaken to clarify whether a large dose of methylprednisolone (MPSL) could have any suppressive effect on progressive Masugi nephritis in the rabbit. Progressive crescentic Masugi nephritis could be induced with high reproducibility by preimmunization with a small amount of nephrotoxic duck gamma-globulin incorporated with complete Freund's adjuvant, followed by an intravenous injection 4 days later. Two groups of rabbits treated with 80 mg/kg of MPSL either before or after the development of proteinuria, showed a significant decrease in both antibody titers and serum creatinine levels during treatment. Histologically, the prominent diffuse intracapillary proliferation and crescent formation observed in controls, were markedly diminished. Accumulations of monocytes in the intra- and extracapillary space were also decreased. These results suggest that suppression of antibody production by a large dose of MPSL is one of its most fundamental actions, and can prevent the processes leading to crescentic glomerular lesions.

Animals↗

Radical resection and allograft replacement in the treatment of bone tumors.

Resection and allograft transplantation in the treatment of bone tumors was introduced in the United States by Parrish in the mid 1960's, based on the demonstration by several investigators that fresh frozen allografts were well tolerated by the host. Since 1971, we have performed 63 such procedures in 60 patients who have had segmental resections for malignant and aggressive bone tumors. Analysis of the results in 46 patients who have been followed longer than 1 year (mean followup of 2.7 years) show that none of the patients have an identifible immunologic reaction to the graft. Twenty-five (53.5%) of the patients had an uneventful recovery and achieved functional status without complication ("good" resuls). An additional eleven patients (23.9%) who exhibited late complications of allograft fracture, infection, or nonunion required further surgery to achieve the same degree of functional restoration as the former group ("satisfactory" results), thus raising the total of good and satisfactory results to 78.2%. Seven of the patients (15.2%) were gross failures in that two required amputation, two had the grafts removed and three have died. Although these data suggest that the procedure is a successful one for treatment of certain types of bone tumors, continued follow-up is required to ascertain the long term results. The technique should be reserved for patients with aggressive or low grade tumors, in which resection can be performed without excessive tissue sacrifice and who will not require adjuvant chemotherapy.

Adolescent↗