Anti-glomerular basement membrane antibody-mediated nephritis complicating transplantation in a patient with Alport's syndrome.

Loss of an allograft caused by anti-GBM antibody-mediated nephritis is a rare complication of renal transplantation in Alport's syndrome. We describe a patient in whom this occurred. He belongs to the subgroup of patients with hereditary nephritis and deafness with an abnormal Goodpasture antigen, and he developed a high level of circulating anti-GBM antibodies within 20 days of transplantation of a kidney with a presumably normal Goodpasture antigen. The antibody titer fell, only to rise again when he developed evidence of acute infection with CMV. Coincident with this second rise in antibody titer he developed an anti-GBM antibody-mediated crescentic nephritis with resultant loss of graft function and transplant nephrectomy. This case provides support for the hypothesis that the abnormality in the basement membrane in some patients with Alport's syndrome involves the Goodpasture antigen, and raises the possibility that viral infection may have triggered autoantibody production.

The functional basis for nephrographic patterns in acute tubular necrosis.

There is considerable evidence that the principal functional abnormality in patients with acute reversible renal failure (ARF, or "acute tubular necrosis") is reduced cortical perfusion with diminished glomerular filtration. However, in such patients, high-dose intravenous urograms most commonly show an immediate obvious nephrogram. Since nephrographic density is believed to depend on the filtration of contrast medium into the tubular lumen, it is difficult to reconcile the early development of the nephrogram if glomerular filtration is reduced. Extensive experiments with both mercuric chloride and glycerol rat models of ARF have confirmed rapid intraluminal accumulation of contrast medium, albeit in reduced amounts. Studies using the normally filtered compound sodium nitroprusside and its precipitation as "prussian blue" suggest rapid transtubular diffusion from peritubular capillaries in kidneys with ARF. This, it is suggested, is also the mechanism for the rapid intraluminal ingress of contrast media and explains the early appearance of nephrogram. The less common intravenous urogram finding in ARF of a slowly developing and increasingly dense nephrogram may then represent cases with only slight tubular necrosis with predominant reduction in glomerular filtration.

Acquired cystic disease of kidney with multiple renal and urothelial neoplasms.

Acquired polycystic disease with concurrent development of renal cell tumours is a well documented complication of long term haemodialysis. We present a case of acquired polycystic disease with renal cell tumours and multifocal urothelial tumours. Only two similar cases have been recorded.

Immunohistological characterisation of amyloid deposits in renal biopsy specimens.

The amyloid deposits in 21 renal biopsy specimens were subjected to a detailed immunohistochemical analysis using a panel of antibodies against recognised constituents of tissue amyloid. This was a retrospective study of material originally submitted during the investigation of various renal abnormalities and studied by a routine protocol including histochemistry, electron microscopy, and immunofluorescence. The presence of an amyloid was confirmed in all 21 cases. Seventeen cases contained P component and either amyloid A (AA) (11 cases) or an immunoglobulin light chain associated amyloid (six cases). Four cases contained amyloid material with unusual immunohistochemical findings; one case had AA and P-component (PC) in the interstitium, one case had lambda light chain and beta-2 microglobulin, one case had kappa light chain and Clq, and one case had lambda light chains only. It was possible, therefore, to identify precisely the amyloid constituents and thereby "type" the amyloid by immunohistochemical means. The availability of the antibodies used and their application using these techniques could simplify the confirmation of clinically suspected amyloidosis.

Systemic amyloidosis of beta 2 microglobulin type.

A patient receiving haemodialysis for 15 years developed systemic amyloidosis of beta 2 microglobulin type. Noticeable deposits of amyloid were present in the myocardium, intervertebral discs, joint cartilages and tendons. Less conspicuous amounts were present in blood vessel walls in the lungs, liver, adrenal glands and brain, and within the stroma of the prostate, testis and kidney, often with foci of calcification.

Long-hours home haemodialysis--the best renal replacement therapy method?

Two hundred and eighty-six patients (190 males and 96 females) with end-stage renal failure (ESRD) started haemodialysis (HD) at Withington Hospital between 1 January 1968 and 31 December 1986. Of these, 152 (53.1%) were successfully transplanted, while 134 had only HD or one transplant lasting < 3 months (i.e. total HD interruption < 3 months). For the whole group, the probabilities of being alive on long-hours home HD at 10 and 20 years were 58.7% and 33.2%, respectively. Mean gross mortality 1968-1986 was 6.5 +/- 3.2% per year. The main causes of death were cardiovascular (36.6%), infection-related (19.2%) and malignancy (9.6%). Males and younger cohorts had a significantly (p < 0.05) higher probability of being alive on long-hours home HD than did females and older cohorts. Eighty-two patients (29% of the total group) survived more than 10 years, of whom 54 were still alive at 1 January 1996: 44 continuing on HD while the other ten had been successfully transplanted. In these 54 patients, mean 24-h ambulatory blood pressure recorded at the date of the study was 117.6/68.9 mmHg; mean BP for the last 5 years on HD was 136.4/81.2 mmHg. Only four (7.4%) were regularly taking antihypertensive medication. Left ventricular hypertrophy (LVH) (by ECG) was present in 64.8% of the 54 patients; its prevalence by echocardiography (LVM index > 130 g/m2 for men and > 110 g/m2 for women) was 77.5%. Only 10 (18.5%) had symptoms or clinical signs of ischaemic heart disease and/or peripheral vascular disease. None had cardiac failure symptoms NYHA class 3-4. Our data show a low incidence of all-cause and cardiovascular mortality, confirming those from the Tassin unit in France, and make a medical case for extended haemodialysis treatment hours.

Treating paracetamol overdose by charcoal haemoperfusion and long-hours high-flux dialysis.

After serious paracetamol overdose, charcoal haemoperfusion was used to remove paracetamol from the circulation, aiming to reduce the severity of subsequent hepatic damage. Daily long-hours high-flux dialysis was given to patients with grade III-IV hepatic encephalopathy, and also to those at risk of developing encephalopathy. We reviewed patients treated in this manner who had not received N-acetylcysteine within the first 15 h after overdose. From January 1983 to January 1993, 73 patients with serious paracetamol overdose were seen, of whom 51 received charcoal haemoperfusion and/or high-flux dialysis. Patients who were admitted within the first 42 h after overdose and who received haemoperfusion and/or dialysis had significantly lower peak levels of prothrombin time, bilirubin and creatinine than those who were admitted after 42 h. Mortality was also lower amongst patients admitted before 42 h, at 2/18 (11%) vs. 15/33 (45%), p < 0.05.

Kinetics of uptake and elimination of silicic acid by a human subject: a novel application of 32Si and accelerator mass spectrometry.

Silicon is possibly important in human physiology in protecting against the toxic effects of aluminium, but the kinetics of uptake and excretion of silicic acid, the bioavailable form, are not well characterised. We have used 32Si as a tracer in a human uptake experiment to determine a gastrointestinal uptake factor for silicic acid, and to elucidate the kinetics of renal elimination. Urine collections were made for extending intervals from 2 to 12 h over 2 days following ingestion by a single human subject of a neutral silicic acid solution containing tracer levels of 32Si (t1/2 approximately 150 y). Silicon was isolated as SiO2 and the 32Si content determined by accelerator mass spectrometry (AMS), using a gas-filled magnet technique to eliminate a prolific isobaric interference from 32S. Silicon uptake appears to have been essentially complete within 2 h of ingestion. Elimination occurred by two simultaneous first-order processes with half-lives of 2.7 and 11.3 h, representing around 90% and 10%, respectively, of the total output. The rapidly eliminated 32Si was probably retained in the extracellular fluid volume, whilst the slower component may represent intracellular uptake and release. Elimination of absorbed 32Si was essentially complete after 48 h and was equivalent to 36% of the ingested dose. This establishes only a lower limit for gastrointestinal absorption as, although there was no evidence for longer term retention of additional 32Si, the possibility could not be excluded by these results.

Desferrioxamine in the treatment of aluminum overload.

Aluminum removal is essential in treating patients with aluminum accumulation. Currently the most effective method is chelation of aluminum with desferrioxamine (DFO). DFO administration has been shown to improve dialysis encephalopathy and dialysis bone disease. The optimum dose of DFO and the mode of administration have yet to be determined. Commonly between 40 and 80 mg/kg is given parenterally once weekly. The very high serum aluminum concentrations which develop do not appear to be toxic. The administration of small doses of DFO with each dialysis may also be advocated. Monitoring of serum aluminum levels and of iron status is advisable during DFO treatment. DFO may also play a role in evaluating tissue aluminum accumulation.

Echocardiographic findings in long-term, long-hour hemodialysis patients.

This article describes the echocardiographic structural and functional findings in a cohort of 30 patients on ten or more years of uninterrupted long-hour (24 hours per week dialysis schedule) hemodialysis (mean duration 187.7 months, range 120 to 299 months). Cardiac structural analysis was remarkable for the prevalence of LVH (76%), very rarely asymmetric (3%). Hemoglobin and (log) plasma renin activity were determinants of the LV wall thickness ratio (r = -0.57 and 0.54, p = 0.003 and 0.044 respectively). Markers of systolic contractile function were frequently normal (100% MVCFS; 85% FSI). Diastolic ventricular compliance was abnormal in 59% of patients. Blood pressure history appeared important in determining LVH, but office/ABPM measures of BP were not. Patients after parathyroidectomy (PTx) had a smaller LVPWTN (8.68 mm/m2 without PTx cf 7.01 mm/m2 after PTx, p = 0.036). Left ventricular cavity size was rarely enlarged (10%), with hemoglobin (r = -0.47, p = 0.012) and PTH (r = -0.65, p < 0.001) the major determinants of EDDN. Left atrial diameter was increased in 77% of patients. Cardiac valvular calcification was seen in 50% of patients. Our findings show that despite good BP control without recourse to antihypertensive drugs, LVH with good LV systolic function is very common in these long-survivors.

Hemosiderosis in a patient on regular hemodialysis: treatment by desferrioxamine.

Hemosiderosis following regular administration of parenteral iron was observed in a patient receiving maintenance hemodialysis. Infusions of desferrioxamine in doses of 2,3 and 4 g each resulted in the removal of approximately 45 mg of iron during dialysis. Desferrioxamine 2 g was infused thrice weekly during dialysis for twelve months. Body iron stores, as judged by liver iron and serum ferritin concentrations, fell by about half. This agrees well with the result calculated from the amount of iron administered and the amount removed during dialysis.

Bromocriptine improves reduced libido and potency in men receiving maintenance hemodialysis.

Twenty-five men on maintenance hemodialysis commenced a placebo-controlled, double-blind, crossover trial of bromocriptine for erectile impotence. Eight patients on bromocriptine and one on placebo failed to complete the trial due to side-effects and 2 were withdrawn following renal transplantation. Libido or the frequency and/or quality of erections or both improved in 11 of the remaining 14 patients during bromocriptine treatment. A significant (P less than 0.01) improvement in patients' perception of sexual performance occurred on bromocriptine compared with placebo. The 3 patients in whom potency failed to improve had the lowest serum testosterone concentrations. Bromocriptine is usually effective in the management of impotence in males receiving maintenance hemodialysis provided pretreatment serum testosterone is above the lower end of the normal range, but side-effects are relatively common.

Do anaemia co-ordinators have to be nurses?

The role of the anaemia co-ordinator has developed subsequent to the introduction of erythropoietin therapy for renal anaemia, and posts have been established at an increasing number of hospitals in the UK. While co-ordinators have previously tended to come from a nursing background, the post at our hospital has been held jointly by a pharmacist and a clinical nurse specialist since July 1997. This paper presents an informal evaluation of our experience of joint working, and has drawn on diary entries to outline the components of the service provided. The main focus is on the boundary negotiated between pharmacist and nurse responsibilities and expertise, involving as it does areas of potential conflict and complementarity. Through a critical examination of the assumptions and expectations associated with 'generic' pharmacist and nurse roles, we begin to clarify the respective contributions which the disciplines make to anaemia management.