Three successive pregnancies in a patient with Takayasu's arteritis.

Takayasu's arteritis is a rare non-specific obliterative panarteritis of unknown origin that occurs predominantly in young Asian and Oriental females of childbearing age and has been encountered in the UK. With the exception of a few large series from the Far East, much of the information on Takayasu's arteritis in pregnancy comes from isolated case reports with no long term follow-up after pregnancy. We report a patient with this condition who had three pregnancies during a 4-year follow-up period with no serious complications.

Biochemical diagnosis of phaeochromocytoma: two instructive case reports.

The biochemical features of two patients with phaeochromocytomas illustrate the inadvisability of depending on a single group of analytes for the diagnosis. The first case presented as a surgical emergency with retroperitoneal haemorrhage. Biochemical diagnosis was difficult since total 24 hour urinary free catecholamine excretion was within normal limits in two out of three samples, and only marginally raised in the third with an atypical preponderance of adrenaline. Plasma catecholamine concentrations were also normal. But urinary excretion of the catecholamine metabolites, metadrenaline and 4-hydroxy-3-methoxy mandelic acid (HMMA), was consistently raised. In contrast, the second patient presenting with headache and labile hypertension showed normal metabolite excretion in the face of grossly increased free noradrenaline excretion and raised plasma noradrenaline concentrations. It is therefore recommend that, as well as urinary free catecholamines, one group of their main metabolites, the 3-methoxy amines (normetadrenaline and metadrenaline) or HMMA, should routinely be measured whenever a phaeochromocytoma is suspected.

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.

Urokinase: a treatment for relapsing peritonitis due to coagulase-negative staphylococci.

Four consecutive patients with relapsing peritonitis due to coagulase-negative staphylococci have been successfully treated by the addition of urokinase to their treatment regime, having failed to respond to appropriate antibiotic therapy alone. The organisms isolated from each episode of peritonitis in an individual patient were shown to be identical by antibiotic sensitivity, phage typing, slime production and immunoblot analysis. The action of urokinase is unknown but it may act by fibrinolysis, allowing antibiotics access to a source of infection previously protected by fibrin. The technique described is a simple, safe and effective treatment of relapsing peritonitis due to coagulase-negative staphylococci, and its use can markedly reduce the morbidity associated with this infection.

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.

Treatment of fracturing renal osteodystrophy by desferrioxamine.

Aluminium removal by desferrioxamine chelation has been demonstrated in three long-term haemodialysis patients with dialysis encephalopathy and fracturing renal osteodystrophy. Aluminium concentrations in serum and in both bone marrow and bone trabeculae, determined separately in transiliac biopsy specimens, fell significantly over the treatment period. Bone aluminium removal was confirmed by specific histochemical staining. In two patients osteomalacia disappeared, and in two patients osteitis fibrosa emerged but improved in one following vitamin D therapy. We conclude that desferrioxamine is capable of mobilising aluminium from bone and that the calcification defect in fracturing renal osteodystrophy may be overcome.

Surgical management of idiopathic retroperitoneal fibrosis.

The management of 10 patients with idiopathic retroperitoneal fibrosis causing obstructive uropathy is presented. In all cases the obstruction was relieved by ureterolysis and corticosteroid therapy was not used during the initial treatment. In 9 patients the operation was well tolerated but one, who developed a pyonephrosis after pre-operative ureteric drainage, died of septiceamia 15 days post-operatively. In 2 patients further explorations and ureterolysis were necessary for recurrent fibrosis which may have been related to urinary leakage. A policy for the management of these cases is presented which recommends the use of silastic tubing splints after ureteric mobilisation and the use of an omental wrap.

The hourly rate of urinary amylase excretion, serum amylase, and serum lipase. I. In control subjects and patients with renal disease.

The rates of urinary amylase excretion/hour and the levels of serum amylase and lipase were measured in 190 normal subjects and patients with no evidence of renal or gastrointestinal disorder. The hourly rate of urinary amylase excretion/hour was found to have an upper limit of normal of 69 IU. The upper limit of normal for the serum amylase was 251 IU/litre and for the serum lipase 1.6 units/ml. Raised levels of serum amylase and lipase with a normal rate of urinary amylase excretion/hour were found in chronic renal failure.

The hourly rate of urinary amylase excretion, serum amylase, and serum lipase. II. Patients with gastrointestinal and pancreatic disorders.

Gastrointestinal disease other than hepatobiliary and pancreatic disorders was associated with hourly rates of urinary amylase excretion above the limits of normal for control subjects (88 IU/hour compared with 69 IU/hour). In hepatobiliary disease, excretion rates of more than 88 but usually less than 190 IU/hour were sometimes found. Whilst rates of urinary amylase excretion were not helpful in the diagnosis of chronic pancreatitis or carcinoma of the pancreas, levels above 190 IU/hour were found in acute pancreatitis at a time when the serum levels were also diagnostic. After the acute episode the rate of urinary amylase excretion was moderately elevated for up to six days but did not reach diagnostic levels. Persistent elevation of serum amylase and lipase levels and hourly rates of urinary amylase excretion for more than six days suggested that a pseudocyst had developed. In acute pancreatitis the level of serum lipase was more frequently raised and persisted so for longer than either the serum or urinary amylase. Although the hourly rate of urinary amylase excretion is of little value alone, when performed in conjunction with evaluating the serum amylase and lipase it may provide useful additional evidence of pancreatic disease and it could be useful in the diagnosis of relapsing chronic pancreatitis.

Infections of Scribner and Brescia arteriovenous shunts.

The incidence of infections of Scribner shunts and Brescia fistulae in patients receiving intermittent haemodialysis was compared. In both groups infection was most commonly due to Staphylococcus aureus and was associated especially with carriage of this organism on the skin of the limb where the shunt or fistula was sited. The incidence of local infection was much less with the Brescia fistula but this was accompanied by a relatively higher incidence of septicaemia.