Myeloperoxidase-antineutrophil cytoplasmic antibody-associated glomerulonephritis with membranous nephropathy in remission.

A 47-year-old woman developed pulmonary hemorrhage and an increase in proteinuria during remission of membranous nephropathy. Renal biopsy revealed crescentic glomerulonephritis. She also had a high perinuclear antineutrophil cytoplasmic antibody level, so a diagnosis of myeloperoxidase-antineutrophil cytoplasmic antibody-associated glomerulonephritis was made. After immunosuppressive therapy was started, the pulmonary hemorrhage resolved and her proteinuria decreased. Renal biopsy was repeated after treatment and showed histological improvement. This case suggests that there may be a relationship between membranous nephropathy and myeloperoxidase-antineutrophil cytoplasmic antibody-associated glomerulonephritis.

Hyperimmunoglobulin E syndrome associated with nephrotic syndrome.

A 21-year-old man was admitted to Kure National Hospital with nephrotic syndrome in September 1996. He had suffered from an intractable pruritic skin rash and recurrent subcutaneous abscesses caused by the hyperimmunoglobulin E syndrome since the age of 18 months. Renal biopsy gave a diagnosis of membranoproliferative glomerulonephritis. Steroid therapy decreased urinary protein loss and hypoproteinemia, and his pruritic skin rash was improved. Patients with hyperimmunoglobulin E syndrome have a defective immune response, especially to Staphylococcus aureus infection. Continuous antigen stimulation may have caused this patient's renal histological damage as in immune complex glomerulonephritis.

Evaluation of the PD adequest program in Japanese patients on peritoneal dialysis.

To perform adequate peritoneal dialysis, it is necessary to individualize the dialysis regimen. PD Adequest is a software program based on the three-pore model that can be used to predict solute clearance and ultrafiltration volume during peritoneal dialysis. We evaluated the ability of this program to predict the solute clearance and ultrafiltration volume in Japanese patients on peritoneal dialysis. The weekly creatinine clearance and weekly urea Kt/V were determined in 45 patients. The PD Adequest was used to simulate their current dialysis regimens and the predicted values of these parameters were calculated. Strong positive correlations were obtained between the actual and predicted weekly creatinine clearance (r = 0.993) and weekly urea Kt/V (r = 0.991). In conclusion, this program is potentially useful for designing peritoneal dialysis regimens in Japanese patients, even though they have a smaller body mass than Canadians and Americans for whom it was designed.

Familial congenital hypoplasia of the thumb--report on a family.

Congenital hypoplasia of the thumb is not a rare condition in clinical practice, but a familial occurrence of this condition is exceedingly rare. We report a family with familial congenital hypoplasia of the thumb. In three generations, five members were proved to be affected and another two were presumed to be affected. To our surprise, a younger sister of the propositus had tetralogy of Fallot and triphalangeal thumbs on both hands.

Light and electron microscopic observation in a case of congenital renal proximal tubular dysfunction.

We report a case of congenital renal proximal tubular dysfunction (CRPTD) accompanied by IgA nephropathy. The mesangial matrix was slightly increased with depositions of IgA and C3. Podocytes contained many clear vacuoles. Cuboidal cells, as well as squamous cells, lined the parietal epithelium of Bowman's capsule, although the functional or pathological significance of the cuboidal cells is unknown. The proximal tubular cells appeared to have a less-developed apical endocytic complex, basal infoldings and fewer lysosomes. These morphological changes may reflect proximal tubular dysfunction.

A case of nephrotic syndrome with rapid spontaneous remission in an elderly patient.

In July 1994, a 70-year-old woman was diagnosed as having nephrotic syndrome with proteinuria of 8 to 10 g/day and a serum albumin level of 1.8 g/dl. She was hospitalized in August 1994 for investigation. The urinary findings then normalized, with urinary protein and occult blood both negative and total urinary protein excretion at 0 g/day. A renal biopsy was performed, and spontaneous remission of minimal change nephrotic syndrome was diagnosed. This is an interesting case involving rapid remission of minimal change nephrotic syndrome in an elderly patient.

Serum soluble interleukin 2 receptor in patients with IgA nephropathy.

Serum soluble interleukin 2 receptor (IL-2 R) was determined by the ELISA method in 29 cases of IgA nephropathy and 50 healthy controls. The results showed that the value in IgA nephropathy cases was significantly higher than that in healthy controls. Furthermore, among the cases of IgA nephropathy, the value was significantly higher in the groups with hypertension, elevated serum IgA and depressed creatinine clearance than in that of the corresponding controls. These findings suggest that serum soluble IL-2 R can serve as a prognostic index of IgA nephropathy.

Myeloperoxidase antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in a very elderly patient with generalized vasculitis at autopsy.

An 86-year-old woman was admitted with hemoptysis and rapid deterioration of renal function. Renal biopsy disclosed necrotizing crescentic glomerulonephritis. Based on positivity for serum myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA), MPO-ANCA-associated glomerulonephritis was diagnosed. Steroid pulse therapy was performed, but the patient died after the second course. Autopsy revealed renal vasculitis with fibrinoid necrosis extending to the level of the arcuate arteries. Vasculitis was also observed in the liver, adrenal gland, uterus, and spleen, suggesting the presence of microscopic polyarteritis. This case demonstrates the broad spectrum of MPO-ANCA-positive vasculitis and suggests the need for a more effective therapy suitable for very elderly patients.

A case of aortitis syndrome and IgA nephropathy: possible role of human leukocyte antigens in both diseases.

A 51-year-old woman, who had both aortitis syndrome (Takayasu arteritis) and IgA nephropathy, presented with hypertension, fever, a high erythrocyte sedimentation rate, high C-reactive protein and serum IgG levels, proteinuria, and renal dysfunction. Renal arteriography showed stenosis and poststenotic dilatation at the origin of the right renal artery, as well as tortuosity of the left renal artery branches and marked atrophy of the left kidney. Renal biopsy showed IgA nephropathy with deposits of IgA, C3, and fibrinogen in the glomeruli and arteriolosclerosis. The present patient had human leukocyte antigen (HLA)-B 52, which is reported to be related to the aortitis syndrome, as well as HLA-DR 4, which is possibly related to IgA nephropathy, suggesting that HLA status may be involved in the pathogenesis of both diseases.

An immunohistochemical study of extracellular matrix components and integrins in human glomerular diseases.

The localization of extracellular matrix components and their cell surface receptors (integrins) was studied in 130 subjects in order to clarify their participation in the progression and aggravation of various types of nephritis. Included in the study were 2 normal subjects, 14 patients with minimal change disease, 2 patients with minimal change nephrotic syndrome, 65 patients with IgA nephropathy, 18 patients with mesangial proliferative glomerulonephritis, 15 patients with membranous glomerulonephritis, 5 patients with membranoproliferative glomerulonephritis and 9 patients with systemic lupus erythematosus (SLE). The distribution of fibronectin (FN), vitronectin (VN), laminin (LN), heparan sulfate proteoglycan (HSPG), type III, IV, V, VI collagen, fibronectin receptor (FNR) and vitronectin receptor (VNR) in the glomerulus was studied employing the indirect immunoperoxidase method. FN, LN, type IV, V and VI collagen, FNR and VNR were found to be distributed in the expanded mesangial region in IgA nephropathy, mesangial proliferative glomerulonephritis and membranoproliferative glomerulonephritis. Deposition of VN was observed in some of the patients. In membranous glomerulonephritis and membranoproliferative glomerulonephritis, the distribution of FN, LN, type IV collagen, FNR and VNR was increased in the thickened loop wall and VN deposition was also observed. Quantitative and functional changes in the extracellular matrix and integrins, therefore, appear to participate in the progression and aggravation of glomerulonephritis.

In vitro immunoglobulin production by peripheral blood mononuclear cells as a prognostic factor in IgA nephropathy.

To determine whether immune system disorders are involved in the exacerbation of IgA nephropathy, the immunoglobulin production of peripheral blood mononuclear cells obtained from 45 IgA nephropathy patients was measured and then compared with that of healthy individuals. The level of IgA production was classified into an elevated group and a non-elevated group and comparisons were made with various clinical factors considered to be related to exacerbation of this disease. The results indicated that although there was no significant difference in immunoglobulin production of the peripheral mononuclear cells between IgA nephropathy cases and healthy individuals in the group not stimulated with pokeweed mitogen (PWM), the group stimulated with PWM revealed a production of IgA, IgG, and IgM which was significantly elevated (P less than 0.01). Also, within the group stimulated with PWM, hypertension, severe proteinuria and microscopic hematuria, elevated BUN and serum creatinine values, decreased 15-min PSP and creatinine clearance values, severe histological damage, and severe IgA deposition were observed more in the elevated IgA production group than in the non-elevated group. These findings suggest that an elevated IgA production plays an important role in the excerbation of this disease.

Effective use of portions of the supernumerary digit to correct polydactyly of the thumb.

The purpose of surgery for polydactyly of the thumb is to ablate the supernumerary digit and to reconstruct a thumb with adequate function that approximates normal size and shape. A further aim is to prevent the occurrence of deformity in the future. To achieve these goals, one good thumb is reconstructed using portions from the supernumerary digit as follows: To increase the diameter of the reconstructed thumb, a skin flap is formed from the supernumerary digit. A portion of the joint from the supernumerary digit is used to correct joint deviation in the reconstructed thumb. The collateral ligament and abductor muscle are detached from the supernumerary digit with the bone fragment into which they are inserted, and transplanted together with the bone fragment into the reconstructed thumb. Osseous tissue to be used in open wedge osteotomy to correct longitudinal deviation is obtained from the phalanx of the supernumerary digit.

In situ hybridization analysis of cytomegalovirus and adenovirus DNA in immunoglobulin A nephropathy.

Viruses are thought to play a role in the occurrence of IgA nephropathy. In fact, cytomegalovirus (CMV) antigens have been detected in the glomerular mesangium. To clarify this relationship, we used in situ hybridization with biotin-labeled DNA probes of CMV and adenovirus (AV) in 40 patients with IgA nephropathy to determine the presence or absence of virus in affected renal tissue. Renal tissue DNA from these patients did not hybridize with the CMV or AV probes. Our results fail to support the theory that consecutive regional renal infections with CMV and/or AV are involved in the pathogenesis of IgA nephropathy.

Immunohistochemical studies of extracellular matrix components and integrins in IgA nephropathy.

Localization of adhesive glycoproteins as the extracellular matrix and their cell surface receptors (integrins) were studied in an attempt to clarify their roles in the progression and aggravation of IgA nephropathy. The relationship between their localization and factors associated with clinical progression was then investigated in 65 patients with IgA nephropathy. The indirect immunoperoxidase method was used to study the distribution of fibronectin (FN), vitronectin (VN), fibronectin receptor (FNR) and vitronectin receptor (VNR). In all cases, FN was present in the glomeruli, mainly confined to the mesangial region, and in some cases, it was observed along the glomerular capillary loops. An expanded mesangial region accompanied higher FN distribution. FNR was present in the mesangial region and glomerular capillary loops, and was higher in the expanded mesangial region. VN was positive in 51 of 65 cases. In all cases, VNR was present in the mesangium and glomerular capillary loops, and its distribution was more predominant in and along the capillary loops than in the mesangial region. The expanded mesangium accompanied higher VNR distribution. There was a significant correlation in distribution between all factors with the exception of VN and FNR. FN, FNR and VNR increased significantly as a degree of histological damage. However, VN was not significantly associated with the degree of histological damage in positive and negative groups. The distribution of FN, FNR and VNR was associated with clinical aggravation factors although the distribution of VN was not. In conclusion, the distribution and functional alteration of FN and integrins in the glomeruli appear to be involved in the progression and exacerbation of IgA nephropathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Serum soluble interleukin-2 receptor in patients with glomerulonephritis.

Serum soluble interleukin-2 receptor (IL-2R) concentrations were determined using the ELISA method in 55 cases of glomerulonephritis. These patients can be classified as 29 cases of IgA nephropathy, 10 cases of membranous glomerulonephritis and 16 cases of mesangial proliferative glomerulonephritis. Our results showed that serum soluble IL-2R concentrations in glomerulonephritis cases were significantly higher than those in healthy controls. Among the different types of glomerulonephritis cases, however, no significant differences in serum soluble IL-2R were observed. While we found a significant positive correlation of serum soluble IL-2R to BUN and creatinine, we also found a significant negative correlation between serum soluble IL-2R and creatinine clearance. These findings suggest that serum soluble IL-2R can serve as an indicator of exacerbation of glomerulonephritis.