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Summary:Primary or AL amyloidosis results from a plasma cell dyscrasia in which fibrillar light chain protein deposition leads to organ failure and death. Standard treatment for AL amyloidosis has been oral melphalan and prednisone. However, this form of treatment modifies the natural history of this lethal disease only marginally, extending median survival from 13 months following diagnosis to 17 months. At Boston University Medical Center, we have developed treatment protocols using high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM/SCT) to treat AL amyloidosis, and we have treated over 200 patients with HDM/SCT during the past six years. This extensive experience has shown that patients with AL amyloidosis, despite multisystem involvement and compromised organ function can tolerate this aggressive form of treatment. Furthermore, HDM/SCT results in durable hematologic responses in a substantial proportion of patients, and such responses are associated with clinical improvement, decreased amyloid-related organ dysfunction, and prolonged survival. However, toxicity from treatment is high (overall peri-transplant mortality, 14%), particularly for those patients with clinically significant cardiac involvement. For this reason, we believe a multidisciplinary management approach is essential when using HDM/SCT for treatment of AL amyloidosis. Based on our experience, we believe that HDM/SCT is the treatment of choice for patients with AL amyloidosis who have a good performance status and limited cardiac involvement at the time of diagnosis. HDM/SCT offers the best chance for hematologic remission, prolongation of survival, and reversal of amyloid-related disease. At the same time, we believe that HDM/SCT should continue to be examined in the context of clinical trials, directed at developing approaches to broaden the applicability of this therapy by minimizing toxicity and to increase the likelihood of

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A 13-year-old boy with aortic coarctation and Klippel-Feil syndrome is reported. This association has not been described previously.The triad of low occipital hairline, limitation of neck movement, and fusion of cervical vertebrae was first described by Klippel and Feil (1912) and has come to bear their name. Since then a large number of cases have been described, some with associated congenital anomalies. These include 'Sprengel's' shoulder, rib anomalies, syringomyelia, and cardiac malformations (Menkes, 1974).Case report A 13-year-old boy was admitted for investigation of coarctation of the aorta. At the age of 2 he underwent a laparotomy for bleeding Meckel's diverticulum and the diagnosis of coarctation of the aorta was made at that time. He had no symptoms and school performance was normal. There was no family history of heart disease and his parents and younger brother were well.On examination he was an intelligent, prepubertal boy, height 147 cm (30th centile) and weight 32 kg (10th centile). A short neck (Fig. 1) and low occipital hairline were noted but facial appearance was normal. There were no abnormalities of the palmar creases. Other relevant physical findings were limited to the cardiovascular system-radial pulses were equal and normal, femoral pulses were absent. The apex beat was not displaced and no thrills were palpable. A grade 2 systolic ejection murmur was audible all over the praecordium and over the scapulae. One collateral vessel was palpable over the right scapula. Blood pressure was 120/70 mmHg (16.0/9.3 kPa) in both arms. Fundi were normal.Chest x-ray film showed early rib notching and there was fusion of the second and third ribs on the right.Electrocardiogram showed left ventricular hypertrophy by voltage criteria.

SUMMARY Nocardia asteroides was isolated from the prosthetic valve of a 64-year-old woman who had died of endocarditis after aortic valve replacement. Multiple blood cultures had all been negative. This organism is increasingly being recognised as a pathogen and is usually sensitive to sulphonamides. These should be tried in culture negative endocarditis unresponsive to standard therapy.Case report A 64-year-old woman had an aortic valve replacement with a Bjork-Shiley prosthesis for severe calcific aortic valve disease. Immediate postoperative recovery was good and the prosthetic valve functioned well.Six months after operation she experienced anorexia, weight loss, and night sweats. She was admitted to The General Hospital, Burton-onTrent, 3 weeks after the onset of symptoms.Examination revealed 3 splinter haemorrhages, no clubbing, clear prosthetic heart sounds, no hepatosplenomegaly, and normal urine. There was an intermittent pyrexia ranging from 36°C to 38 5°C. Haemoglobin 13'0 g/dl. Leucocytes 15 600 (80% neutrophils). Erythrocyte sedimentation rate 101 mm/h. Urea and electrolytes and liver function tests normal. Chest x-ray film showed cardiomegaly and pulmonary congestion but no evidence of focal infection.Blood cultures were sterile, but in view of the possibility of an infected prosthesis she was started on gentamicin and ampicillin. Despite this therapy, her pyrexia persisted, the erythrocyte sedimentation rate remained high, and she became progressively more anaemic.Three weeks after admission, she developed aortic systolic and diastolic murmurs and was transferred to Dudley Road Hospital, Birmingham, under the care of Dr J. Mackinnon.On examination she was anaemic with splinter haemorrhages, collapsing pulse, cardiomegaly, and 'Present address:

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