Wegener's granulomatosis occurring in siblings.
A brother and sister sibling pair with Wegener's granulomatosis (WG) are described. Previous reports of familial WG are reviewed.
Biomedical subjects
Publications and source records attributed to M Beaman.
A brother and sister sibling pair with Wegener's granulomatosis (WG) are described. Previous reports of familial WG are reviewed.
This study analyses the prognostic factors associated with survival in 70 patients who developed acute renal failure following surgery for an aortic aneurysm. Forty-nine patients (70 per cent) had surgery for a ruptured aortic aneurysm and 21 patients (30 per cent) had an elective procedure. Fifty-nine patients received haemodialysis. Thirty-three patients (47 per cent) survived the episode of acute renal failure. Six of these thirty-three patients died within three months of recovering from acute renal failure, giving an overall survival of 27/70 (39 per cent). A stepwise logistic regression analysis showed that the following factors significantly adversely affected survival: a need for inotropic support, ventilation for more than three days and age over 65 years. A model developed using these variables provided a basis for predicting outcome.
From 133 to 615 glomeruli were examined in sections of kidneys from each of 60 animals, representing six rodent models of proteinuria. Particular attention was paid to the position of segmental lesions. Lewis rats given sheep anti-rat glomerular basement membrane antibodies had lesions almost exclusively at the glomerulo-tubular junction. Wistar rats on a diet of 24 per cent casein or with subtotal nephrectomy and a diet of 24 per cent soya had lesions mainly at the hilum. Wistar rats given bovine serum albumin had global lesions but virtually no segmental lesions. Wistar rats given puromycin aminonucleoside had lesions at the glomerulo-tubular junction and global mesangial abnormalities shortly after the treatment but later developed segmental lesions at all parts of the glomerulus. Untreated BUF/Mna rats had lesions at the glomerulo-tubular junction early in life but later had lesions at all parts of the glomerulus. Untreated NZB/NZW hybrid mice had various types of glomerulonephritis and also had lesions at the glomerulo-tubular junction. These findings showed that (1) segmental lesions at the glomerulo-tubular junction, or glomerular tip, occur in experimental animals, a fact not previously reported, and these tip changes are a common feature in several different models of proteinuria; (2) hilar segmental lesions are seen in conditions with hyperfiltration of protein; and (3) segmental lesions at various parts of the glomerulus are seen in some models of proteinuria and probably indicate late effects of random toxic damage to the glomerulus. Thus, there are at least three different types of segmental glomerular lesions in experimental animals--tip, hilar, and random--with different morphology and pathogenesis. It is likely that these findings can be extended to human renal diseases with segmental glomerular lesions. This will help to clarify the controversial and unsatisfactory term focal segmental glomerulosclerosis.
Thirteen patients with end-stage renal disease aged 70 years have been successfully treated by chronic dialysis treatment. Despite many problems associated with the elderly, a good quality of life was achieved.
Antibody responses against pneumococcal capsular antigens and tetanus toxoid were measured in 14 patients with chronic renal failure who were managed by continuous ambulatory peritoneal dialysis (CAPD) or haemodialysis (HD) and in eight healthy controls. IgG antipneumococcal responses were predominantly of the IgG2 and to a lesser extent IgG1 subclasses, while the IgG response against tetanus toxoid was largely IgG1 with smaller amounts of IgG4 and IgG3. The post-immunisation serum levels of IgG1 and IgM antibody against both antigens were significantly reduced in the uraemic patients compared with controls (P less than 0.05). All the uraemic patients had normal levels of IgG, IgA and IgM in the serum, but elevated levels of IgG3 prior to immunisation. The mechanisms responsible for the asymmetric depression of antibody responses in uraemia are unclear and may account in part for the increased susceptibility to infection in these patients.
102 episodes of continuous ambulatory peritoneal dialysis (CAPD) peritonitis were studied prospectively during a 288-day period at The Queen Elizabeth Hospital, Birmingham. Organisms were isolated from 76% of the episodes, with coagulase-negative staphylococci, being the most commonly encountered organism (55%). Initial treatment consisted of intraperitoneal vancomycin and ceftazidime with subsequent adjustment on the basis of antibiotic sensitivities. With this regimen, 83% of the positive cultures became negative by 72 h, 9.8% of cases relapsed and removal of the CAPD catheter was necessary in 8 patients (7.8%). Overall, 92% of cases were cured. No adverse drug reactions were seen. This combination of antibiotics appears effective and safe in the treatment of CAPD peritonitis.
A percutaneous renal biopsy is an important investigative procedure in many patients with renal disease. The procedure described below is easy and safe to perform in competent hands, but requires experienced histological support if clinical management is to be optimal.
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Fourteen immunosuppressed patients with Pneumocystis carinii infection presented in two clusters that were separated by 2 years. The diagnosis in all cases was made early by alveolar lavage with cytology. The first group of seven patients was immunosuppressed with cyclophosphamide or azathioprine and prednisolone. All recovered with high dose co-trimoxazole. The second group of seven patients was on prednisolone and cyclosporin A. Despite identical treatment three patients died and a further two who survived lost their grafts from rejection. Our data suggest that cyclosporin A adversely affects the prognosis from Pneumocystis carinii infection and raises the question of prophylactic co-trimoxazole in these patients. The clustering of Pneumocystis carinii infection suggests the possibility of nosocomial transmission although in this study we were unable to implicate person-to-person spread of infection.
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The effect of the nephrotic syndrome induced by puromycin aminonucleoside (PA) in rats on specific antibody responses to 2,4 dinitrophenyl (DNP) conjugated to either spider crab haemocyanin (MSH), a T cell-dependent antigen, or hydroxyethyl starch (HES), a T cell-independent type 2 antigen were studied. The serum IgG anti-DNP levels following immunization with both antigens were reduced in nephrotic animals compared with controls while IgM anti-DNP antibody titres were higher. The half-life of IgG anti-DNP antibodies passively transferred into non-immunized nephrotic rats was markedly reduced while the half-life of anti-DNP antibodies of the IgM class was comparable to that in controls. Low serum IgG and elevated IgM levels were seen in nephrotic animals compared to controls. Antibody-forming cells specific for DNP were demonstrated by immunohistology on rat spleens and the numbers of both IgG and IgM-producing cells were found to be significantly increased (P less than 0.05) in nephrotic animals in response to both DNP-HES and DNP-MSH. These data indicate that in nephrotic rats the alteration seen in the serum immunoglobulin levels is not attributable to reduced antibody production but increased catabolism of serum IgG antibodies.
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1. The nephrotic syndrome was induced in inbred female Wistar rats by the intravenous injection of puromycin aminonucleoside (PA) (5 mg/100 g body weight). 2. One group (n = 12) received superoxide dismutase (SOD) (15 mg/kg body weight), a second group (n = 12) received polyethylene glycol coupled catalase (PEG-catalase) (5000 i.u./kg body weight) and the third (n = 9) saline (150 mmol/l NaCl) via the intraperitoneal route, in addition to the PA. 3. SOD and PEG-catalase reduced the 24 h urine protein on days 8 and 15 compared with unmodified puromycin treated animals and this difference was significant on day 15 for SOD (P less than 0.05) and for PEG-catalase (P less than 0.01). Glomerular filtration rate, as measured by the creatinine clearance, was lower in the PEG-catalase group but did not differ significantly from the saline treated group. 4. These data suggest that superoxide anion and hydrogen peroxide, or their reaction products, are involved in the glomerular injury of puromycin nephropathy.
We describe four patients with seropositive rheumatoid arthritis who developed proteinuria and microscopic haematuria. Renal biopsy demonstrated a mesangial proliferative glomerulonephritis with mesangial deposits of IgA. These data suggest a possible causal relationship between rheumatoid arthritis and IgA nephropathy.
We report a 53 year old man with chronic renal failure on continuous ambulatory peritoneal dialysis. Following eight episodes of severe peritonitis over a 2 year period, he died and was found to have widespread AA amyloid at post-mortem.
During the four-year period 1981-1984, 250 patients with severe acute renal failure were treated at one centre. There were seven obstetric cases (2.8 per cent) 118 'surgical' cases (47.2 per cent) and 125 medical cases (50 per cent). This is a different pattern from that seen in the majority of earlier reports. In 60 of the 125 medical patients the aetiology of the acute renal failure could only be determined by renal biopsy. This series suggests that with changing medical practice (particularly the improvement in resuscitation) and an ageing population, the pattern of causes of acute renal failure is altering. It also highlights the value of renal histology as a guide to diagnosis and treatment in patients with unexplained acute renal failure.
The glomerular tip nephropathy is a cause of the nephrotic syndrome and has distinct pathological features. Glomerular tufts appear normal on light microscopy except for a segmental lesion invariably present in all glomeruli at the origin of the proximal tubule. Data on twenty adults whose renal biopsies demonstrated this lesion and who were followed for a mean of 7.4 years are analyzed. Eighteen patients were treated with steroids; ten of these had complete remission of proteinuria and seven a significant reduction of their proteinuria. Ten patients had moderately impaired renal function (serum creatinine greater than 120 mumol/l) at presentation, eight received steroids and achieved a reduction in serum creatinine. The prognosis was good, with no patient developing chronic renal failure requiring dialysis.