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We experienced a case of lupus nephritis with antiphospholipid antibody syndrome. A renal biopsy specimen from this patient showed various renal histological changes, but the results of urinalysis were almost normal. The patient was a 56-year-old woman diagnosed as having systemic lupus erythematosus with antiphospholipid antibody syndrome in 1983. In 1998, she had diarrhea and blood gas analysis showed metabolic acidosis. Therefore, she was admitted to our hospital and underwent renal function examination. The glomerular filtration rate was reduced(GFR: 40/ml/min), but urinalysis was almost normal. To examine her renal dysfunction, we performed open renal biopsy. Her renal tissues showed global glomerular sclerosis, mesangial cell proliferation and infiltration of cells in the tubulointerstitial area(WHO II). Furthermore, some arterioles showed organized thrombus formation and recanalization due to the antiphospholipid antibody syndrome. Renal biopsy of patients with lupus nephritis is useful not only for precise diagnosis, but also for the selection of appropriate treatment.

We experienced two cases of limb edema of unknown pathogenesis. No evidence was found concerning involvement of the kidneys, heart of other visceral organs. Case 1 was 22-year-old woman. Her white blood cell count increased to 13,100/microliter with 65.0% eosinophils. Case 2 was a 27-year-old woman. Her white blood cell count increased to 23,300/microliter with 67.0% eosinophils. In these cases, extensive diagnostic evaluations revealed no evidence of atopy, neoplasms, collagen-vascular disease, or parasitic infestation. We diagnosed these cases as episodic angioedema with eosinophilia. In both cases, the angioedema improved gradually in parallel with a decrease in the white blood cell count. This disorder is very rare, but it is very important to consider it in differential diagnosis especially for nephrologists.

In order to clarify the factors that lead patients in the terminal stage of malignant diseases to home care, we interviewed ten families of such patients. Among factors involving patients, a strong desire to stay at home and to spend time at home with the family were initially required. Adequate understanding of the patients and their diseases was also needed by families. Other factors include guaranteed medical services in emergencies and/or 24 hours/day 7 days/week care. Patients requested referrals to family physicians, visiting nurses or community health services to cover outpatient clinic functions. It is concluded that a strong desire to stay at home, adequate understanding by the family and guaranteed medical services in emergencies were three significant factors leading to home care of patients in the terminal stage of malignant diseases.

The gene transcription of 5-lipoxygenase (5-LO) and 5-lipoxygenase-activating protein (FLAP) in renal tissue of patients with IgA nephropathy and mesangial proliferative glomerulonephritis were analyzed by amplification of reverse transcribed mRNA with polymerase chain reaction (PCR). The level of expression was determined by analysis of the PCR products of 5-LO, FLAP, and beta-actin. Forty percent of the patients expressed both 5-LO and FLAP. Relative to beta-actin, FLAP expression (0.42 +/- 0.21) was higher than 5-LO (0.14 +/- 0.10). Comparison of clinical data showed that patients who expressed 5-LO and FLAP had a lower glomerular filtration rate and an increased level of blood urea nitrogen, serum creatinine, and proteinuria. The results suggest arachidonic acid metabolism by inflammatory cells in renal tissue may play an important role in human glomerulonephritis.

We designed this study to elucidate a possible involvement of genetic factors which influence the progression of renal dysfunction in patients with non-insulin dependent diabetes mellitus (NIDDM). A total of 328 patients was randomly selected in the Tokai University Hospital. They had been cared for in our hospital as NIDDM for the past five years, until April, 1989. Fifty-six patients with persistent albuminuria, and an equal number of patients without persistent albuminuria were included in this study. No significant differences were observed between the two groups in terms of age, estimated period of illness, blood pressure, body-mass index, HbAlc, blood glucose, total cholesterol, triglyceride level and mean blood pressure. The family histories obtained by questionnaires revealed that there might be some genetic predisposing factors leading to the onset of nephropathy in patients with NIDDM, especially in cases with paternal high blood pressure. It is suggested that paternal hypertension might be related to the development of nephropathy in patients with NIDDM.

A study was undertaken on the specificity of circulating IgA antibodies in patients with IgA nephropathy detected by immunofluorescence using avidin-biotin complexes. Renal biopsy specimens and serum samples were obtained from 33 patients with IgA nephropathy, 14 other glomerular diseases and 3 normal renal tissues. These renal specimens were treated with citrate buffer (pH 3.2), and then incubated with serum samples obtained from the same and other patients with IgA nephropathy, other glomerular diseases or healthy adults at 37 degrees C for 30 min. The specimens were incubated with biotin conjugated gout F(ab')2 anti-human IgA antiserum at 37 degrees C for 30 min, and then with fluorescein-labeled avidin at 37 degrees C for 30 min. It was found that IgA antibodies in the sera from patients with IgA nephropathy specifically combined with the autologous glomerular mesangial areas, but only 25.7% of them combined with allogeneic renal tissues of IgA nephropathy patients. Confirmatory findings were obtained using an automatic image analyzer. However, these IgA antibodies did not combine with the renal tissues from patients with other glomerular diseases or normal renal tissues. In parallel studies, in order to distinguish IgA nephropathy from other glomerular diseases before renal biopsy, the renal specimens from patients with IgA nephropathy were also incubated with serum samples obtained from 42 patients with proteinuria and/or hematuria before renal biopsy. It was demonstrated that the incidence of IgA binding in IgA nephropathy was significantly higher than that in other glomerular diseases prior to renal biopsy.(ABSTRACT TRUNCATED AT 250 WORDS)

A 63-year-old man with diabetes mellitus for 15 years was admitted to our hospital in 1990 because of end-stage renal failure. Five days after beginning continuous ambulatory peritoneal dialysis (CAPD) he developed an eosinophilic peritonitis (EP). With protein loss in the dialysate and a decreased serum albumin level, the patient developed ankle edema. The patient was treated with glycyrrhizin, and his EP resolved. It is suggested that an allergic background may play an important role in the development of EP in patients on CAPD.

A 72-year-old patient was referred to our hospital because of a hypoglycemic crisis possibly due to an insulin secreting-tumor. Although its localization was assumed to be at the head of the pancreas as assessed by transhepatic venography with sampling, no mass lesion was detected by selective angiography, ultrasound, computerized axial tomography, magnetic resonance imaging and 67Ga-citrate scan. The patient was treated with diazoxide, because of poor localization and his age, insulin secretion was improved after both 30 and 60 min in the 75 g oral glucose tolerance test. The total amount of urinary C-peptide excretion decreased from 70.8 to 29.2 mcg/day. Thereafter, no hypoglycemic symptoms were observed during his admission. No side effects except mild liver damage were observed.

A study of the detection of alpha 2-pregnancy associated-glycoprotein (alpha 2-PAG) by double immunofluorescence in cultured human peripheral blood mononuclear cells (MNC) was performed. MNC was obtained from four healthy adults and isolated on a Ficoll-Hypaque density gradient. MNC was incubated with RPMI-1640 with or without pokeweed mitogen in 5% CO2/air at 37 degrees C for six days. MNC was initially stained with rhodamine-conjugated antihuman IgA, IgM, and IgG antisera, and then stained with FITC-conjugated antihuman alpha 2-PAG antisera. It was concluded that alpha 2-PAG was not produced by IgA, or by IgM and IgG producing cells in healthy adults.

Double immunofluorescent studies on IgA, poly (MAC) or C3 in glomeruli from patients with IgA nephropathy are described. Renal biopsy specimens were obtained from 12 patients with IgA nephropathy, four patients with proliferative glomerulonephritis (PGN) and two normal human kidney (NHK). These specimens were incubated with monoclonal anti-poly C9 (membrane attack complex; MAC) and then stained with FITC-labelled goat anti-mouse immunoglobulin (Ig) antiserum. After washing with phosphate buffered saline (PBS) (pH 7.4), the sections were stained with rhodamine-labelled rabbit anti-human IgA antiserum and examined by fluorescence microscopy. The sections were also stained with FITC-labelled goat anti-human C3 antiserum and then stained with rhodamine-labelled rabbit anti-human IgA antiserum. Markedly combined depositions of IgA and poly C9 or C3 in glomeruli were observed in patients with IgA nephropathy. There was a significant correlation between the deposition of poly C9 and the grading of histopathological injuries in such patients. There was also a significant correlation between the deposition of poly C9 in the extraglomerular vascular vessels and the ageing in patients with IgA nephropathy, PGN and NHK. It appears that the deposition of poly C9 might detect directly the activities of complement in glomeruli from patients with IgA nephropathy.

Discriminant analysis of clinical markers before renal biopsy in patients with IgA nephropathy is described. Sixty eight patients with IgA nephropathy (IgA nephropathy group) and 66 patients with other chronic glomerulonephritis (non-IgA nephropathy group) were examined. The discriminant analysis was applied to separate those two groups by using twenty clinical parameters as well as binding capacity of serum IgA to the glomeruli of renal specimens. Binding of serum IgA of patients to the glomeruli obtained from patients with IgA nephropathy was performed using avidin-biotin immunofluorescence. Among twenty clinical markers, the levels of serum IgA and creatinine, and degree of microhematuria in IgA nephropathy group were significantly higher than those in non-IgA nephropathy group Furthermore, the positive incidence of serum IgA binding of IgA nephropathy group was significantly higher than that of serum IgA binding of non-IgA nephropathy group. The correct classification rate were 79.10% using five clinical markers including serum IgA, microhematuria, serum C4, quantitation of proteinuria and degree of proteinuria. It is indicated that the levels of serum IgA and the binding of serum IgA to the glomeruli were considered to be major markers for clinical diagnosis of patients with IgA nephropathy It was concluded that the discriminant analysis before renal biopsy was useful for diagnosis of IgA nephropathy.

The solubilization of renal deposits of IgG and other serum proteins by fresh human sera or human gamma-globulin was studied in patients with diabetic nephropathy. Renal biopsy specimens were from 10 patients with diabetic nephropathy. These specimens were incubated with fresh sera obtained from the same patients and healthy adults or human gamma-globulin at 37 degrees C for 1 hr in plastic test tubes. The sections were stained with FITC-labeled heavy chain specific anti-human IgG, alpha 1-antitrypsin, haptoglobin and beta-lipoprotein antisera, and then examined with a fluorescent microscope. It was demonstrated that fresh human sera or gamma-globulin significantly solubilized glomerular deposits of IgG and other serum proteins in patients with diabetic nephropathy. It was postulated that solubilization of protein deposits in glomeruli requires the same substances detected in vitro in the kidney tissues from patients with diabetic nephropathy.

A study on the significance of the measurement of fibrinopeptide A (FPA) and fibrinopeptide B beta 15-42 (FPB beta) in urine and plasma before and after urokinase (UK) administration in patients with chronic glomerulonephritis is described. FPA and FPB beta in urine and plasma were measured by radioimmunoassay. The levels of fibrinogen degradation product (FDP) in urine and sera were also determined before and after the UK administration. UK was administered at a dose of 48,000 IU/20 ml in saline intravenously, followed by an intravenous infusion of 250 ml of 5% glucose for one hour. It was demonstrated that the levels of FPB beta in urine and plasma showed a significant increase after UK administration in patients with chronic glomerulonephritis. However, there was no significant difference in the levels of FDP in urine and sera before and after UK administration. It was concluded that the measurement of FPB beta in urine and plasma may be useful for evaluating the efficacy of UK therapy in patients with chronic glomerulonephritis.

A twelve year old girl with rapidly progressive glomerulonephritis (RPGN) is reported. First symptoms of renal failure developed in August 1987 just five months after a routine school urinalysis which detected no abnormalities. The patient developed end stage renal failure within 2 months of onset. Continuous ambulatory peritoneal dialysis (CAPD) was introduced to manage the renal failure, and open renal biopsy was performed simultaneously in order to elucidate the underlying renal disease in this patient. Microscopic examination of the biopsy specimen demonstrated marked formation of crescents in about 90% of the glomeruli, and some of the glomeruli were already sclerotic. Granular depositions of IgG(trace), IgA(1+), IgM(3+), properdine(3+), Clq(3+) and C3(3+) were observed by immunofluorescence staining. A definite diagnosis of rapidly progressive glomerulonephritis was made as the cause of renal failure in this patient. It was suggested that open renal biopsy is useful in diagnosing the underlying kidney disease of end stage renal failure in cases where the clinical course is relatively short.

The percentages of glycosylated proteins or albumin, or glycohemoglobin A1 (HbA1) in sera from patients with noninsulin dependent diabetes mellitus (NIDDM) were measured to elucidate whether such measurements are useful for evaluation of the mean levels of blood glucose during short intervals in these patients. Forty patients with NIDDM and twenty healthy adults were examined. The percentages of glycosylated proteins or albumin were quantitated by a Glyc-Affin system and those of HbA1 were measured by electrophoresis. It was indicated that the percentages of glycosylated proteins or albumin, and HbA1 in sera from patients with NIDDM were significantly higher than those in sera from healthy adults. There was a significant correlation between the percentages of HbA1 in sera and the mean levels of fasting blood sugar (FBS) during short intervals in patients with NIDDM. It was concluded that the measurement of HbA1 is a useful method for evaluation of the mean levels of blood glucose during short intervals in patients with NIDDM compared with those of glycosylated proteins or albumin.

In this report, two cases of metastatic renal tumor are described. The first case is a 58-year-old female who had branchiogenic carcinoma. She had found a left neck tumor, before asymptomatic macrohematuria developed. The second case is a 25-year-old female who had choriocarcinoma. She had no urological symptoms. In both cases, unilateral nephrectomy was performed. Seventy nine cases of metastatic renal tumor in survivals have already been reported.

A study on the clinical effects of urokinase in patients with IgA nephropathy is described. Three different methods of administration, including single, continuous, and mixed administration, were employed in this study. Measurements of plasminogen, plasmin and alpha 2-plasmin inhibitor levels in plasma were performed during the course of urokinase administration in patients with IgA nephropathy and chronic proliferative glomerulonephritis. Measurements of alpha 1-anti-trypsin and alpha 2-macroglobulin levels were also performed in these patients. Urinalysis was performed both before and after administration of urokinase. It was demonstrated that a single shot of urokinase induced a significant fibrinolytic activity in patients with IgA nephropathy, and that a single shot of urokinase was effective in improving proteinuria and/or hematuria in patients with IgA nephropathy. It is concluded that a single shot of urokinase may be useful for treatment of patients with IgA nephropathy.

The detection of molecular weights of IgA-circulating immune complexes (CIC) in sera is described. Measurement of IgA-, IgG- and IgM-CIC in sera was also performed. Serum samples were obtained from nine patients with IgA nephropathy, nine patients with chronic proliferative glomerulonephritis without IgA deposits (PGN) and 19 healthy adults. Serum samples of these patients were subjected to high performance liquid chromatography (HPLC) and then divided into 50 fractions. Measurement of IgA-, IgG- and IgM-CIC in such fractions was performed using solid-phase anti-C3 Facb EIA. The levels of IgA-CIC in sera from patients with IgA nephropathy were significantly higher than those from patients with PGN or healthy adults. The peak values of molecular weights of IgA-CIC in sera from patients with IgA nephropathy ranged from 2.6 X 10(5) to 3.0 X 10(5) daltons (mean +/- SD; 2.9 +/- 2.0). It was suggested that the increase of IgA-CIC in sera from patients with IgA nephropathy may be mainly due to dimers and/or polymers.

Clinical effects of dipyridamole and carbazochrome sodium sulfonate in patients with IgA nephropathy are described. Oral administration of 300 mg of dipyridamole and 180 mg of carbazochrome sodium sulfonate per day was employed in the present study. Urinalysis and renal function tests, i.e. serum creatinine (s-Cr), blood urea nitrogen (BUN), glomerular filtration rate (GFR) and phenolsulfonphtalein (PSP) tests, were performed before and after the administration of dipyridamole. It was demonstrated that the administration of dipyridamole was effective in reducing the level of proteinuria in the patients. The administration of carbazochrome sodium sulfonate was not effective in reducing the proteinuria level. It was concluded that the administration of dipyridamole may be useful for treatment of patients with IgA nephropathy.

Renal tissues from 19 patients with the minimal or slight stage of IgA nephropathy were examined for evidence of glomerular T cell or monocyte infiltration using monoclonal antibodies to identify T cells (OKT3, OKT11), T cell subsets (OKT4, OKT8), and monocytes and null cells (OKM1) by indirect immunofluorescence. Renal tissues from 12 patients at the same stage of mesangial proliferative glomerulonephritis without IgA deposition were also investigated by these procedures. Light microscopic examination of the same renal tissues was also performed. Reactive glomerular mononuclear cells were found to be numerous in patients with IgA nephropathy. The most prominent type of glomerular cells was OKT8 positive. T cell subsets and OKM1 positive cells in glomeruli from patients with IgA nephropathy were significantly increased as compared to those from patients with mesangial proliferative glomerulonephritis. Focal segmental proliferation of mesangial cells was observed in glomeruli which showed an accumulation of OKT8 positive cells. It is concluded that the immuno-regulatory mechanism involving T cells and/or monocytes might be one of the exacerbating factors in patients with IgA nephropathy.

A diabetic patient with renal insufficiency and a giant ventricular aneurysm was treated by continuous ambulatory peritoneal dialysis (CAPD). No apparent exacerbation of the patient's cardiovascular function was observed after starting CAPD therapy, although his ejection fraction remained low and his angina attacks persisted. It is suggested that CAPD therapy has no deleterious effects on the cardiovascular system of patients with ventricular aneurysms, and that the benefits outweigh the risks.

Siblings with IgA nephropathy and diffuse proliferative glomerulonephritis without mesangial IgA deposits (PGN) who had identical HL-A antigens are described. A 21-year-old woman suffered from IgA nephropathy and her 27-year-old sister showed diffuse proliferative glomerulonephritis (PGN) distinct from IgA nephropathy. These siblings had identical HL-A alloantigens which were A2, Aw24; Bw16, Bw35; Cw4, Cw7; DR4. Microhematuria and increased serum IgA levels were shown in their father and other sister but renal biopsies were not performed. The serotypes for the HL-A antigens of their father and other sister were A2, Aw24; Bw27, Bw35; Cw4; DR4 and A2; Bw16; Cw7; Dr2, respectively. It is demonstrated that IgA nephropathy and PGN share a common HL-A phenotype including DR4. It is postulated that the presence of DR4 antigens is not directly related to the occurrence of IgA nephropathy.

A case of mesangial IgM nephropathy associated with chronic renal failure is described. The patient did not reveal nephrotic syndrome during the clinical course. Renal biopsy specimens revealed typical features of mesangial IgM nephropathy when studied by light microscopy, electron microscopy and immunofluorescence staining. Mesangial IgM nephropathy is considered to be a heterogenous disorder, because this disorder is clinically characterized by nephrotic syndrome, mild proteinuria and/or hematuria or renal failure.

Clinical effects of a newly developed oral carbonaceous adsorbent (AST-120) in patients with advanced stages of chronic glomerulonephritis is described. Measurements of serum creatinine were performed before, during and after the administration of AST-120. The reciprocal of serum creatinine concentration was also plotted serially in each patient. The mean levels of the slope of the reciprocal serum creatinine vs time plot after administration of AST-120 was decreased compared with those before the administration of AST-120 but there was no statistical significance (p greater than 0.10). Further studies with more patients are needed to determine whether the administration of AST-120 may be useful for treatment of patients with chronic glomerulonephritis.

Detection of alpha 2-plasmin inhibitor (alpha 2-PI) and/or fibrinogen in glomeruli by immunofluorescence in 26 patients with IgA nephropathy was described. The present study showed that glomerular injuries such as glomerular adhesion to Bowman's capsule and the cellular and/or fibrous crescent were predominantly observed in glomeruli with alpha 2-PI and/or fibrinogen deposits in patients with IgA nephropathy. Alpha 2-PI coexisted with fibrinogen in glomeruli from patients with IgA nephropathy. It was postulated that the deposition of alpha 2-PI in vivo might lead to the accumulation of glomerular fibrinogen deposits in patients with IgA nephropathy. It was suggested that the depositions of alpha 2-PI and/or fibrinogen in glomeruli may be one of the exacerbative factors in glomeruli from patients with IgA nephropathy.