A case of rapidly progressive glomerulonephritis associated with bucillamine-treated rheumatoid arthritis.
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Biomedical subjects
Publications and source records attributed to K Morozumi.
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Lupus anticoagulant (LAC) is an acquired inhibitor of prothrombin activator complex, which probably interferes with the phospholipid portion. Characteristically, LAC prolongs the partial prothrombin time, but only slightly prolongs the prothrombin time. It is a paradoxical fact that LAC is characterized by thrombosis. It was initially described in patients with SLE, but recently, it has been described as occurring with other autoimmune disorders. Patients with LAC have been treated with steroid and aspirin, anti platelet agents or warfarin. Steroid and aspirin therapy has been reported useful for habitual abortion associated with LAC. In our study, 11 patients, whose prior pregnancies resulted in habitual abortion (41 abortions), received intentional prednisolone (40 mg/day) and aspirin (81 mg/day) therapy before further pregnancies. The doses of both agents were decreased gradually, and the therapy with prednisolone (10-15 mg/day) and aspirin (40.5 mg/day) was maintained during the pregnancy period. The outcome of pregnancy was successful in 7 out of 10 pregnancies. To evaluate the relationship between LAC and glomerulopathy, we examined the renal biopsy from 5 LAC cases without SLE. In pathological findings, there were 3 of with mild proliferative GN and 2 cases of minor glomerular abnormality. There were no characteristic findings in LAC nephropathy.
Familial juvenile nephronophthisis (FJN) is a hereditary renal disease, characterized by a juvenile onset and the development of medullary cysts and progressive renal damage. The pathogenesis of FJN remains unknown, and at present, no rational therapy other than renal transplantation is available. We describe two cases in siblings in whom there were no extrarenal complications, such as retinopathy or central nervous system involvement. Both patients display juvenile onset of the disease and end-stage renal failure. The brother received a kidney from his father, and the sister received a kidney from her mother. Recurrence of the underlying disease has not so far been found in the transplanted kidney.
Cyclosporine (CSA)-associated arteriolopathy (CAA) is the second most frequent morphological diagnosis in renal allografts and its final outcome remains unclear. The present study was performed to clarify the morphological outcome of CAA by follow-up histological analysis after stopping CSA. Furthermore, the clinical management of patients showing CAA is discussed. Most of the patients came from our early experience with CSA between 1981-1983 when CSA doses and trough levels were high. Twenty recipients were divided into two groups according to the presence of CAA after stopping CSA: group A (n = 9) showed persistent CAA and group B (n = 11) showed no CAA. The majority of the patients, including five incomplete remission in group A, showed obvious improvement of CAA even if the arterioles were severely affected. Improvement of CAA was noted a few months after stopping CSA or after lower dose CSA therapy. There were no significant differences in CSA blood levels or duration of CSA therapy between the groups. The severity of preexistent CAA was significantly greater in group A. Only two patients who died from malignant tumor showed exacerbation of CAA. Eight patients died and eight grafts were lost, seven due to vascular rejection and one to hemolytic uremic syndrome-like CAA. Poor renal function was also noted in four cases with functioning graft owing to vascular rejection even though the improvement of CAA was evident. The complete regression of CAA and the remodelling of arterioles showing well preserved vascular patency were frequently found after stopping or reducing the dose of CSA.(ABSTRACT TRUNCATED AT 250 WORDS)
Anti-phospholipid antibody syndrome (APS) is characterized by thrombocytopenia, thrombosis and/or abortion. Steroid and aspirin therapy has been reported useful for habitual abortion associated with APS. Eleven patients, whose prior pregnancies resulted in habitual abortion (41 abortions), were received a intentional prednisolone (40 mg/day) and aspirin (81 mg/day) therapy before further pregnancies and both agents were decreased gradually. We maintained prednisolone (10-15 mg/day) and aspirin (40.5 mg/day) during pregnancy period. After the treatment, the outcome of pregnancy was successful in 7 of 10 pregnancies. The weight of newborn infants ranged from 1980 to 2980 g and apgar score ranged from 4 to 9 points. No side effect was observed, and fetal malformation and/or adrenal insufficiency was not recognized in any infants. In conclusion, an intentional prednisolone plus aspirin therapy is useful to prevent habitual abortion in patient with APS.
This paper describes pathological and clinical investigations of glomerular lesions in bucillamine-induced nephropathy by analyzing biopsy materials from 9 patients with rheumatoid arthritis (RA). There was no specific predisposition for nephrotic syndrome induced by bucillamine in clinical profiles related to age, onset, duration of disease, sex, activity and dose of bucillamine. In light-microscopic, electron-microscopic and immunofluorescent findings, the characteristic changes were similar to those of idiopathic membranous glomerulonephritis (MGN). After discontinuance of bucillamine, the nephrotic syndrome improved slowly with or without corticosteroid therapy. Results confirmed that the most common lesion of nephrotic syndrome associated with bucillamine therapy in RA is MGN. We recommend that corticosteroid therapy should be restricted to cases with severe proteinuria.
A new type of idiopathic glomerular disease is reported in a 49-year-old Italian woman who presented with uncharacteristic renal symptoms, i.e., hypertension and slight proteinuria. Clinical investigation excluded a familial renal disease and more specifically nail-patella syndrome. Diagnostic renal biopsy by light microscopy showed a picture similar to membranoproliferative glomerulonephritis. The enlarged glomeruli were lobulated, the peripheral basement membranes were thickened by the deposition of light-microscopically undefined material, cell proliferation was lacking. By electron microscopy, the material was nonhomogenous, partly granular partly fibrillar, containing typical collagen fibers. The latter were identified as collagen type III, to a lesser extent collagen type I. Review of the literature resulted in 12 similar or identical cases reported from Japan and one additional case reported in a white American female. Evidence of systemic disease is lacking. Etiology and pathogenesis are elusive. A progressive deterioration of renal function must be expected. Collagen type III glomerulopathy is suggested as term of this new type of idiopathic glomerular disease.
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We carried out a retrospective study to investigate the clinical and pathological findings in 31 patients with rheumatoid arthritis (RA). In clinical findings, 17 patients showed nephrotic syndrome, five had isolated proteinuria, two had proteinuria and hematuria and seven had renal failure. In pathological findings, there were 16 patients with membranous nephropathy (MN), two with proliferative glomerulonephritis (DPGN), two with minor glomerular abnormality (MGA), six with amyloidosis, 2 with tubulointerstitial nephritis, and three patients had accompanying lupus nephritis. Eleven of 16 with MGN had been treated with gold, bucillamine or D-penicillamine, so they were diagnosed as drug induced MGN. In the other five patients, we could not decide which drugs induced the nephropathy. The 2 cases of MGA were associated with nephrotic syndrome and acute renal failure, which were caused by non-steroidal antiinflammatory drugs. There were two cases of non-Ig A DPGN, which was regarded as the native nephropathy in RA. The three cases with lupus nephritis were diagnosed as systemic lupus erythematosus by the criteria of the American Rheumatism Association (ARA). In conclusion, the nephropathy in patients with RA was varied and renal biopsy was a useful examination.
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We studied four cases of Crow-Fukase syndrome with renal dysfunction. Kidney specimens obtained by needle biopsy showed glomerular lesions resembling those seen in conditions characterized by microangiopathy. Common glomerular findings by light microscopy were mesangial expansion and narrowing of the capillary lumina. An enlarged subendothelial space and mesangial area with deposition of amorphous material as well as swelling and vacuolization of endothelial cells were observed by electron microscopy. In an active phase, severe mesangial edema and segmental mesangiolysis, and in a late stage, mesangial cell interposition and sclerosis were seen. Tests by immunofluorescence microscopy for the presence of immunoglobulins A, M, G, lambda and kappa light chains, C3, and C4 were negative. Decay accelerating factor was found in glomeruli and in the vascular pole. Other findings included lymph node angiosclerosis, peripheral nerve microangiopathy and hemangioma formation with endothelial cell proliferation. These observations suggest that chronic endothelial injury constitutes the basic pathology of Crow-Fukase syndrome. Hemodialysis was required to manage anasarca in three of the patients although serum creatinine levels were below 5.0 mg/dl. Urinalysis revealed mild abnormalities and did not reflect the severity of the glomerular lesion. Corticosteroids given to three of the patients were effective in controlling fever and the lymphadenopathy; in two cases the corticosteroids induced a recovery of renal function. Thus Crow-Fukase syndrome may be due to chronic endothelial injury; the clinical symptoms and renal involvement respond to corticosteroid therapy.
We report a case showing typical diabetic nodular glomerulosclerosis without retinopathy or other apparent clinical findings of DM except for impaired glucose tolerance. The 57-year-old man had a family history but no personal history of DM. In an extensive examination for DM, the results of funduscopy, daily profile of serum glucose and hemoglobin Alc were entirely within normal limits. However, the oral glucose tolerance test was abnormal. A renal biopsy showed typical nodular lesions (Kimmelstiel-Wilson's lesions). Previously, the interesting feature of transient proteinuria had been recognized. Although hypocellular nodular lesions by light microscopy are characteristic of diabetic nephropathy, renal amyloidosis, carbon disulfide intoxication, multiple myeloma and light chain disease, we concluded that the present lesions had resulted from diabetic nephropathy based on the family history, patient history, impaired glucose tolerance, immunofluorescent findings and electron microscopic observations.
A case of IgD lambda type multiple myeloma that developed acute renal failure was presented. Histopathologic studies revealed apparent myeloma cast nephropathy with typical giant cells and light chain deposition along tubular basement membrane and inter-peritubular interstitium. Glomerular deposits of light chain were absent by electron and immunofluorescent microgram. The presented case showed a very uncommon histopathologic findings of unusual deposits of light chain, to tubulo-interstitium except for glomeruli, in addition to vertically rare IgD myeloma. A sixty-year old female who was suffered from acute renal failure and treated by hemodialysis for one month was admitted to our institute. Physical examinations revealed ill-apparent and emaciated uremic patient on admission. Laboratory and radiological studies demonstrated IgD lambda type monoclonal protein and numerous punched out appearance of skull bone. The diagnosis of IgD lambda myeloma was confirmed with aspiration bone marrow test. Anti-myeloma therapy of melphalan and prednisolone have started, however, fatal arrhythmia suddenly suffered the patient and resuscitation attempts failed to get any success one week after admission. Renal necropsy was performed and specimens were studied by light, immunofluorescent and electron microscope.(ABSTRACT TRUNCATED AT 250 WORDS)
Polyarteritis nodosa (PN) is a disease characterized by vasculitis of multiple organs, and it is complications of kidney and heart are fatalistic factor. Myocardial infarction and pericarditis are well known as cardiac involvement of PN. The pericardial cysts are uncommon disease and it originated in the mesenchymal tissue during fetal life. There has been no report that pericardial cyst undergoing with PN. The present paper was the first report about case with enlargement of pericardial cyst caused by vasculitis. A 64-years old female was referred for acute renal failure and mediastinal tumor. The diagnosis of PN was confirmed with the renal biopsy showing the typically histological features of necrotizing angitis, renal angiography showing vascular irregularity in the renal arterioles and clinical features such as fever, hypertension and skin lesion. The mediastinal tumor was identified with enlargement of pericardial cyst diagnosed by computer tomography (CT) and magnetic resonance imaging (MRI), and ultrasonic cardiography (UCG) revealed pericardial effusion. Enlargement of pericardial cyst, pericardial effusion and acute renal failure were due to vasculitis as a partial manifestation of PN. Under the steroid administration, sequentially recovery of renal function and disappearance of pericardial cyst and pericardial effusion were observed.
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Clinical effectiveness of cefoperazone (CPZ) was studied for 46 elderly patients with respiratory infections. 1. Clinical efficacy rate of CPZ was 80.6% (29/36). 2. Adverse reactions occurred in 2 patients as systemic rush and fever. Laboratory abnormalities were observed in 5 patients. These included elevation of transaminase value. 3. The serum concentration of CPZ at 12 hours after administration of 2 g CPZ was not reflected in creatinine clearance and total bilirubin, and those 2 values appeared to have little effects on CPZ excretion. 4. The administration of CPZ at 4 g/day for less than 3 weeks had no effects on the renal function. As a result of this study, CPZ was found to have little undesirable effect on renal functions and to be a useful drug for the treatment of respiratory infections in elderly patients.
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