Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases.

Sixty patients with idiopathic retroperitoneal fibrosis presenting between 1965 and 1984 are reviewed. Their mean age at presentation was 56 years and the male:female ratio was 3:1. The commonest presenting symptoms were flank and abdominal pain, weight loss, nausea and polyuria. Physical examination was usually normal, expect for the presence of hypertension. Anaemia and elevation of erythrocyte sedimentation rate were usually present. Proteinuria was found in less than a third of patients at presentation and significant bacteriuria was uncommon. The correct diagnosis was made or suspected in very few patients before referral. The cumulative actuarial survival rate was 86% at 1 year and 78% at 2 years. Seventeen patients died; they were significantly older and more uraemic at the time of referral than those who survived. A few patients did well with either corticosteroid therapy or ureterolysis alone. In the majority, both operation and steroid treatment were necessary. In bilateral obstruction with residual function in both kidneys, bilateral ureterolysis proved superior to unilateral operation (each followed by steroid therapy) in conserving renal function. Operation alone or steroid therapy alone should be considered in cases where steroids or surgery respectively present particular hazards. The less traumatic unilateral operation should be considered in poor risk patients and in those whose renal function is absent on one side. In many survivors, disease activity has persisted for many years. Life-long follow-up is recommended.

Correction of anaemia following renal transplantation: serial changes in serum immunoreactive erythropoietin, absolute reticulocyte count and red-cell creatine levels.

Improvement of erythropoiesis following successful human renal transplantation in eight patients was monitored by sequential measurements of haemoglobin, red-cell creatine, absolute reticulocyte count and estimates of serum immunoreactive erythropoietin (siEp). SiEp increased in five patients after transplant, in three cases almost immediately after a return to normal of plasma creatinine. The increase in siEp was followed by a rise in the absolute reticulocyte count and red-cell creatine and finally by an increase in the haemoglobin level. As the haemoglobin approached normal levels a decline in the absolute reticulocyte count preceded a fall in siEp levels. Red-cell creatine also fell, though more gradually than the reticulocyte count. Acute graft rejection (in two patients) was associated with a fall in siEp. Chronic rejection (in one patient) was associated with persistent increases in siEp, reticulocyte count and red-cell creatine; this patient subsequently developed erythrocytosis.

Rapid correction of red-cell nucleotide abnormalities following successful renal transplantation.

Erythrocyte levels of 5' mono-, di- and triphosphates of adenosine, inosine and guanosine, together with NAD+ (H) and NADP+ (H) were measured before and serially after renal transplantation. The high levels of ATP and guanosine triphosphate (GTP) fell to normal within 3 d of achieving a normal plasma creatinine. The decline in ATP and GTP levels paralleled the fall in plasma creatinine. Acute graft rejection was accompanied by a rise in ATP and GTP, both of which returned rapidly to normal with resolution of the rejection episode. Radiolabelling of red cells confirmed that the metabolism of the existing red-cell population was corrected so that observed changes in nucleotide concentrations were not due to a new cohort of cells.

Clinical use of minoxidil (Loniten).

The powerful peripheral vasodilator minoxidil, in a dose of 5-40 mg daily, controlled the previously refractory blood pressure in 45 out of 47 patients for periods up to fifty-seven months. The majority of the previous polypharmacy was withdrawn, leaving most of the patients taking a beta-blocking drug, minoxidil and a diuretic. Severe sodium retention leading to congestive cardiac failure necessitated the withdrawal of the drug in 3 patients. In one patient the drug was discontinued because of postural hypotension, and it was withdrawn in 2 female patients because of hirsutism. Five patients were removed from the trial as they were started on chronic maintenance haemodialysis or received a renal transplant. Significant glucose intolerance developed in one patient, requiring the addition of an oral hypoglycaemic agent. The 4 deaths that occurred were not directly related to treatment. Following the oral administration of 5 mg minoxidil, an obvious reduction in both the systolic and diastolic pressure was seen in two hours.

An outbreak of Nocardia asteroides infection in a renal transplant unit.

An outbreak of Nocardia asteroides infection occurred in the nephro-urological intensive care unit, St. Peter's Group of Hospitals, London in 1979. In is suggested that the outbreak has been due to patient-to-patient transmission. Five months before the outbreak. N. asteroides was first isolated from nephrostomy urine from an asymptomatic patient who had undergone several urological operations. Subsequently six of the seven patients admitted for renal transplant over a three month period developed proven infections. The first transplant patient had an abdominal abscess and the other five had only pulmonary disease with obvious radiological lesions. Bacteriological diagnosis was made in three patients by examination of sputum and in the other three from bronchoscopic specimen, pleural fluid and pus from an abscess respectively. Five of the six renal transplant patients were treated with amoxycillin and erythromycin. Clinical and radiological improvement was observed and no patient died as a result of the infection. In the intensive care unit air and dust samplings were positive for N. Asteroides. The unit was closed and fumigated with formaldehyde and when the air sampling continued to be negative the unit was then re-opened.

Successful renal transplantation in primary hyperoxaluria.

A successful live related renal transplant in a 29-year-old male patient with Type 1 primary hyperoxaluria, who remains well 32 months postoperatively, is described. The plasma oxalate and exchangeable oxalate pool before transplantation were 160 mumol/1 and 4429 mumol respectively. Since the transplant these have been greatly reduced although they remain elevated above the normal by a factor of 2. Pyridoxine therapy and the avoidance of oxalate-rich foods have been effective in maintaining these reduced levels and the 24-hr urinary oxalate excretion has also been maintained close to normal levels on this regime. After review of the previously reported transplants in patients with well documented primary hyperoxaluria and from the experience with this patient, the following guidelines for successful renal transplantation in primary hyperoxaluria are suggested: transplants should only be carried out in those who have shown a response to adequate pyrodoxine therapy; frequent haemodialysis pre-operatively and during periods of oliguria postoperatively is necessary; oxalate-rich foods should be avoided and a high fluid intake should be maintained after transplantation. If these guidelines are followed there is no contra-indicatin to live related renal transplants in primary hyperoxaluric patients.

Single sample estimates of renal clearances.

Thirty adult patients were investigated with 36 125I Hippuran and 40 51Cr EDTA single injection clearance studies. "Volumes of distribution" obtained from single samples have been correlated with the slope clearances and regression equations have been determined. From these equations it is possible to determine glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) from single plasma samples and the method is sufficiently accurate for routine application.

Plasma level and renal clearance of oxalate in normal subjects and in patients with primary hyperoxaluria or chronic renal failure or both.

1. Plasma oxalate has been measured by a radioisotopic method applicable to all concentrations of plasma oxalate and renal function, and also by an enzymatic method which was only applicable to raised concentrations of plasma oxalate. 2. Where the two methods could be applied simultaneously, the agreement between them was good. 3. Plasma oxalate was 86% ultrafiltrable at concentrations of up to 44 micromol/l. 4. Oxalate clearance and the exchangeable oxalate pool were also measured. The ratio of oxalate clearance to creatinine clearance was greater than unity in most normal subjects and patients. 5. These methods were used in normal subjects and in patients with primary hyperoxaluria and/or chronic renal failure. A raised plasma oxalate concentration was found in both conditions. Chronic renal failure is probably the most common cause of a raised plasma oxalate.

Monotherapy with labetalol for hypertensive patients with normal and impaired renal function.

1 Labetalol was given to 41 hypertensive patients in a divided dosage of 150--2,400 mg daily for periods ranging from 1--64 months. 2 Monotherapy with labetalol was adequate in 12 out of 19 patients with essential hypertension and in 15 out of the 22 with renal hypertension. 3 Following a single dose of labetalol 200 mg orally a hypotensive response was seen between 1.5 and 2 hours. 4 In the doses used there was no exercise or postural hypotension. 5 No reduction in overall renal function attributable to labetalol was seen.

Monotherapy with labetalol for hypertensive patients with normal and impaired renal function.

1 Labetalol was given to 41 hypertensive patients in a divided dosage of 150-2,400 mg daily for periods ranging from 1-64 months. 2 Monotherapy with labetalol was adequate in 12 out of 19 patients with essential hypertension and in 15 out of the 22 with renal hypertension. 3 Following a single dose of labetalol 200 mg orally a hypotensive response was seen between 1.5 and 2 hours. 4 In the doses used there was no exercise or postural hypotension. 5 No reduction in overall renal function attributable to labetalol was seen.

Development of a lymphocytic lymphoma during immunosuppressive therapy with azathioprine for systemic lupus erythematosus with renal involvement induced by phenylbutazone.

A case of systemic lupus erythematosis (SLE) with renal involvement after the administration of phenylbutazone is described. The patient subsequently developed a lymphocytic lymphoma following two years treatment with azathioprine and prednisone. Although the association between SLE, immunosuppressive therapy and the development of lymphomas is well documented, the increased incidence of malignant disease in uremic patients is less well recognized. The varied factors that may have contributed to the development of the lymphoma in this case are discussed, together with a review of the literature.