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Biomedical subjects

F J Bruns

Publications and source records attributed to F J Bruns.

At least 19 recordsLinked to original sources

Evaluation of platelet hyperfunction in hemodialysis patients receiving recombinant human erythropoietin.

There are data suggesting that recombinant human erythropoietin (rHuEPO) may induce thromboses in hemodialysis patients, possibly due to alterations in platelet function. In an earlier study, we found evidence of platelet hyperfunction in several patients 4 to 8 weeks following the start of rHuEPO therapy, which was begun shortly after hemodialysis was initiated. Studies were performed to examine the effects of rHuEPO on whole blood platelet aggregation and adenosine triphosphate (ATP) release independent of changes in hematocrit or the uremic state. Eight hemodialysis patients without and four with a history of vascular access clotting had platelet aggregation tests performed at baseline while receiving rHuEPO, off rHuEPO for 2 weeks, and 4 to 6 weeks after restarting the drug. While the plasma EPO level decreased significantly after the 2-week period off rHuEPO (P < 0.0001), the hematocrit did not change at any of the three time periods. Whole blood platelet aggregation in response to adenosine diphosphate (ADP), collagen, and ristocetin was not significantly altered on or off rHuEPO in either patient group. Platelet hyperfunction, determined by aggregation or ATP release either spontaneously or in response to low-dose ADP or ristocetin, was not seen in any patient. These data suggest that the increase in access clotting is not the result of platelet hyperfunction induced directly by rHuEPO.

Adenosine Diphosphate

Potentiation of uremic bleeding by hereditary storage pool disease.

This study demonstrates that specific bleeding tests can separate the thrombocytopathy of uremia alone from the bleeding disorders caused by uremia superimposed on preexisting platelet dysfunction. The case history of a uremic patient with exaggerated bleeding tendencies is presented. The findings in this patient are compared with the clinical characteristics and platelet function studies of nine other patients with chronic renal failure. The index and other uremic patients were similar except for the clinical bleeding and results of platelet function studies. The patient's nonocclusive bleeding time and measured blood loss during bleeding time tests were increased compared with the other uremic controls. In addition, her platelet aggregation in response to collagen was lower than that of the other uremic subjects. Repeat studies following renal transplantation were consistent with hereditary storage pool disease. An underlying platelet disorder may potentiate the hemostatic defects of uremia. The diagnosis should be suspected in patients with frequent and severe bleeding manifestations. Renal transplantation led to control of clinical bleeding.

Adult

Sustained remission of membranous glomerulonephritis after cyclophosphamide and prednisone.

OBJECTIVE: To determine the effect of cyclophosphamide and prednisone on progressive renal failure and on nephrotic features in patients with membranous glomerulonephritis. DESIGN: Prospective, nonrandomized time series. SETTING: Outpatient clinic at a university medical center. PATIENTS: Eleven consecutive patients with biopsy-proven membranous glomerulonephritis and rising plasma creatinine levels over at least 6 months. INTERVENTION: Cyclophosphamide and prednisone in ten patients and cyclophosphamide alone in one patient. MEASUREMENTS AND MAIN RESULTS: In ten patients treated with both agents, the median plasma creatinine rose 53 mumol/L (0.6 mg/dL) over the months before treatment from 141 to 194 mumol/L (1.6 to 2.2 mg/dL) (95% CI, 27 to 141 mumol/L; P = 0.002). After combined therapy for 6 months, the median plasma creatinine fell to 133 mumol/L (1.5 mg/dL) for a median decline of 62 mumol/L (0.7 mg/dL) (CI, 44 to 150 mumol/L; P = 0.006). Pretreatment plasma creatinine levels, which ranged from 159 to 371 mumol/L (1.8 to 4.2 mg/dL), decreased in the ten patients by 6 months and remained stable in seven of the eight patients followed 24 to 54 months after therapy was completed. The median urine protein excretion decreased by 9.6 g/d with 12 months of therapy in the ten patients from 11.9 to 2.3 g/d (CI, 6.0 to 15.1 g/d; P less than 0.001). The median plasma albumin rose by 14 g/L from 24 to 38 g/L (CI, 11 to 19 g/L; P less than 0.001). The median plasma cholesterol fell by 3.26 mumol/L (140 mg/dL) from 10.45 to 6.52 mumol/L (405 to 252 mg/dL) (CI, 1.42 to 7.16 mumol/L; P = 0.01). One patient who had a relapse 30 months after completing therapy responded to re-treatment with renal function and nephrotic variables returning toward normal. The eleventh patient received cyclophosphamide alone and had a course similar to that of the combined therapy group. CONCLUSION: Cyclophosphamide plus prednisone can promote prolonged remissions in membranous glomerulonephritis even when renal function is already declining.

Adult

Long-term follow-up of aggressively treated idiopathic rapidly progressive glomerulonephritis.

PURPOSE: We wanted to examine the long-term effects of aggressively treating idiopathic rapidly progressive glomerulonephritis (RPGN), with a particular focus on clinically characterizing the patient population, assessing the short- and long-term effects of therapy on renal function, and determining complications of the therapy. PATIENTS AND METHODS: Twenty-three consecutive patients with RPGN were treated and followed from one to 11 years. On renal biopsy, 13 had immune complexes, eight had no immune complexes, and two had antiglomerular basement membrane deposits. All had greater than 25 percent crescents and 19 of 23 had greater than 50 percent crescents. Every patient responded on a short-term basis to either large-dose pulse methylprednisolone or plasma exchange, with reduction of the mean plasma creatinine level from 6.5 +/- 2.0 mg/dl to 2.9 +/- 1.0 mg/dl (p less than 0.001). Each patient received oral prednisone and all but one received cyclophosphamide. RESULTS: Three died of non-renal causes. Fifty percent of the remaining 20 patients maintained stable renal function for at least two years. Four of nine patients followed-up for longer than two years had a relapse, but all responded again to therapy. No characteristic clinical symptoms predicting relapse were found, although nearly all had hematuria and proteinuria. Complications of therapy were frequent and may have contributed to death in two patients. CONCLUSION: Thus, long remissions are seen in most patients with RPGN treated aggressively.

Adult

Hypercalcemia in patients with advanced chronic renal failure not yet requiring dialysis.

Six patients with progressive chronic renal failure not yet requiring dialysis and not consuming supplemental calcium or vitamin D developed hypercalcemia. Three had proven and 1 suspected tertiary hyperparathyroidism, 1 parathyroid carcinoma and 1 aplastic bone. None of the 3 patients who underwent bone biopsy had heavy bone aluminum staining. The patients with proven parathyroid-mediated hypercalcemia had marked elevation of C-terminal parathyroid hormone and alkaline phosphatase values and, when performed, radiographs consistent with osteitis fibrosa. When these findings are absent or the diagnosis is otherwise uncertain, a bone biopsy may provide a definitive diagnosis and guide management.

Adult

Rupture of ovarian cyst: massive hemoperitoneum in continuous ambulatory peritoneal dialysis patients: diagnosis and treatment.

Two women on continuous ambulatory peritoneal dialysis (CAPD) developed recurrent episodes of hemoperitoneum while in the reproductive age group. Initially, both were thought to have mechanical problems with the peritoneal catheter system. A laparotomy was performed in the first patient, and a bleeding ovarian cyst was identified. The second patient had ovarian cysts documented by ultrasound. Thus, abdominal pain and bloody dialysate should not just be ascribed to catheter-related problems. The second patient's midcycle bleeding was suppressed with birth control pills.

Adult

Genital edema in patients on continuous ambulatory peritoneal dialysis. Report of 3 cases and review of the literature.

Severe genital edema is a well-described complication of continuous ambulatory peritoneal dialysis (CAPD). Leakage of dialysate fluid from defects in the peritoneum may occur from clinically detectable and undetectable inguinal hernias, defects at the catheter insertion site, or other defects in the peritoneal membrane. We describe 3 patients (who underwent five surgical procedures), illustrating the complexity of the problem. In 2 patients, unsuccessful surgical repairs (1 catheter replacement, 1 hernia repair) were performed based on misleading radiologic findings using intraperitoneal contrast without computerized tomography (CT) scanning. The use of CT scanning with intraperitoneal contrast led to the correct diagnosis and prompt surgical correction in both patients so studied. One patient had leakage from a clinically undetectable inguinal hernia, and the other had a peritoneal defect at the Tenckhoff insertion site which was only demonstrated following a period of upright posture with a 3-liter exchange. Our experience and a review of the literature convinces us that a CT scan of the abdomen utilizing radiocontrast material added to the dialysate accurately identifies the site of leakage in CAPD patients who develop genital edema. Thus, the CT scan can help avoid unnecessary surgery and prolonged hospitalization in CAPD patients who develop genital edema.

Adult

Autosomal dominant polycystic kidney disease: presentation, complications, and prognosis.

Fifty-three symptomatic adults with autosomal dominant polycystic kidney disease were studied retrospectively for a mean follow-up of 12 years (range 10 months to 33 years). Diagnosis was confirmed by either x-ray, ultrasound, laparotomy, or autopsy. Commonest presenting clinical findings were flank pain (30%), hypertension (21%), symptomatic urinary tract infection (UTI) (19%), gross hematuria (19%), and palpable masses (15%). A total of nine patients (17%) progressed to end-stage renal disease. Change in renal function measured using the reciprocal of plasma creatinine plotted against time was linear for each individual patient with a maximum functional decline of 0.7 mg/dL/yr (slope = -0.07). Past the age of sixty renal failure was uncommon. Easily controlled hypertension developed in 64% attended by mild retinopathy. UTIs were common (53%), often recurrent (61%), precipitated by instrumentation in 6 of 14 patients (43%), leading to death in two (33%). Renal calculi were extremely common (34%) and had no defined metabolic cause. The presence of hematuria (64%), gross or microscopic, bore no relationship to the decline in renal function. Pregnancy was normal in these patients with no increase in fetal or maternal morbidity or mortality. We conclude the following: Renal functional deterioration is linear, less than previously reported, and bears no relationship to hematuria. Hypertension is common, easily treated, and causes minor end-organ damage. Renal calculi are frequent. Urinary tract instrumentation often induces infection with considerable morbidity and mortality and must be avoided. Pregnancy is not contraindicated if renal function is normal. The prognosis for survival in this disease is better than previously reported.

Adolescent

Plasmapheresis as sole therapy in a patient with essential mixed cryoglobulinemia.

An 82-year-old woman with essential mixed cryoglobulinemia type II (IgM K IgG) presented with moderate renal failure and nephritic syndrome. Mesangiocapillary glomerulonephritis with mesangial and subendothelial granular deposits containing IgG, IgM, and C3 in conjunction with small-vessel vasculitis was seen on renal biopsy. Renal symptomatology preceded by a period of 10 months the development of leg ulcers and purpura. The onset of the skin lesions was accompanied by an acute decline of renal function and an increase in liver alkaline phosphatase. Plasmapheresis with a 50% plasma exchange each week over 12 weeks led to improvement in renal function, healing of leg ulcerations, disappearance of purpura, and a return to the baseline of alkaline phosphatase in association with the disappearance of circulating cryoglobulins.

Aged

The use of streptokinase to treat renal artery thromboembolism.

The effects of streptokinase are difficult to determine. Furthermore, it has toxic side effects, and renal function may not recover from its use. However, because of favorable experiences with this drug in the early treatment of venous thromboembolism and following myocardial infarction, as well as the favorable findings with early perfusion in the dog model, the use of local streptokinase may be justified if the infusion is begun early, preferably within four to six hours.

Aged

Disseminated listeriosis presenting as acute hepatitis. Case reports and review of hepatic involvement in listeriosis.

We report three cases of disseminated listeriosis that presented as acute hepatitis characterized by striking increase of liver function test values and fever. Peak serum transaminases (SGOT) for each of three patients were 5,380, 2,350, and 443 mu/ml respectively. The correct diagnosis was not suspected in any of the patients until blood and cerebrospinal fluid cultures obtained routinely in the course of evaluation for fever grew Listeria monocytogenes. When antibiotic therapy was instituted, serum transaminase values plummeted in two patients; these two were eventually cured of their infection. The third patient succumbed to his infection; postmortem examination showed miliary abscesses of the liver which revealed L. monocytogenes. Review of the literature for previous reports of hepatic involvement in adult patients with listeriosis shows that hepatitis is an unusual mode of presentation. However, since we observed these three cases over a one-year period, we suspect this may not be an uncommon occurrence.

Ampicillin

Rapid progressive glomerulonephritis: relapse after prolonged remission.

Three patients with epithelial cell crescents and rapid progressive glomerulonephritis (RPGN) had prolonged remission, presumably induced by treatment with maintenance oral prednisone and cyclophosphamide plus plasmapheresis. Patient 1 had anti-glomerular basement membrane-mediated RPGN, patient 2 had an immune complex disease, and patient 3 did not show any immune deposits. After a two-year remission in two patients and a greater than one-year remission in the third patient, renal function deteriorated. Epithelial cell crescents were again demonstratable on repeated renal biopsy specimens in each patient. One patient again received triple therapy, while the other two patients received megadoses of intravenous prednisolone sodium succinate in place of plasmapheresis. Each patient again entered a stable remission. These three cases demonstrate that RPGN may recur after prolonged remission in all three varieties of this syndrome. If the exacerbation is treated promptly, a second remission may be induced.

Aged

Infectious complications with plasmapheresis in rapidly progressive glomerulonephritis.

We recently used plasmapheresis to treat eight patients with rapidly progressive glomerulonephritis. Life-threatening infections developed in five patients, three of which were caused by opportunistic pathogens. In contrast, only two infections occurred in 21 patients with similar renal disease treated with immunosuppressive drugs but not with plasmapheresis. Although infectious complications of plasmapheresis therapy have not been amphasized previously, our experience, coupled with that of previous reports, suggests that serious infections will develop in one third of patients undergoing plasmapheresis for renal disease. Plasmapheresis should be used with caution in patients with rapidly progressive glomerulonephritis.

Adolescent

Propranolol-related bronchospasm in patients without history of asthma.

Three patients with no history of asthma or allergy developed bronchospasm while taking propranolol for hypertension. The bronchospasm was severe in all three and in one patient resulted in respiratory arrest. Since the bronchospasm was relieved with discontinuation of propranolol and supportive bronchodilator therapy, the bronchospasm was believed to be caused by propranolol. Furthermore, each patient was subsequently treated with other antihypertensive medications which, like propranolol, contain the stabilizer additive tartrazine. Bronchospasm did not recur, making it unlikely that tartrazine hypersensitivity caused this problem. Regardless of a negative history of asthma, therefore, life-threatening bronchospasm must be considered a possible complication of propranolol therapy.

Bronchial Spasm

Effect of early plasmapheresis and immunosuppressive therapy on natural history of anti-glomerular basement membrane glomerulonephritis: report of a 22-month follow-up.

A patient with anti-glomerular basement membrane (GBM)-mediated necrotizing and proliferative glomerulonephritis with crescents was treated with plasmapheresis, cyclophosphamide, and steroids. Treatment resulted in decreased circulating anti-GBM antibody and prompt improvement of renal function that remained stable for 15 months after all treatment was discontinued. Renal biopsies were performed initially, at seven and 17 months. Immunofluorescent examination showed that anti-GBM antibody continued to be present on GBMs although light and electron microscopic findings demonstrated a transformation to a form of sclerosing glomerulonephritis. To our knowledge, this patient's course is the first demonstration that early treatment with plasmapheresis and immunosuppressions may transform the histologic findings in anti-GBM-induced rapidly progressive glomerulonephritis, thereby altering the natural history of this disease.

Adrenal Cortex Hormones