Photodynamic therapy (PDT) with 5-aminolaevulinic acid (ALA) is a novel treatment for high-grade dysplasia (HGD) in Barrett's esophagus (BE). Our aim was to evaluate the effectiveness of differing light doses. Patients with HGD in BE received oral ALA (60 mg/kg) activated by low (500 J/cm), medium (750 J/cm), high (1,000 J/cm), or highest (1,000 J/cm x2) light dose at 635 nm. Follow-up was by regular endoscopy with quadrantic biopsies. Twenty-four patients were treated. Successful eradication of HGD was significantly correlated with light dose (log rank, p < 0.01). Six of eight patients (75%) treated with the highest light dose, one of two treated with high dose (50%), two of nine (22%) receiving medium light dose, and zero of five receiving low light dose had successful eradication of HGD (median follow-up 45 months, range 1-78 months). No skin photosensitivity or esophageal strictures occurred. The efficacy of ALA-PDT for eradication of HGD in BE is closely related to the light dose used. With a drug dose of 60 mg/kg and light at 635 nm, we recommend a minimum light dose of 1,000 J/cm of esophagus. This dose appears safe.
A 54 year old woman with neurofibromatosis type 1 (NF-1) was found to have multiple coronary aneurysms. Intraoperative intravascular ultrasound (IVUS) revealed severe coronary disease proximal to the aneurysm that had not been apparent angiographically. An IVUS picture of one of the giant coronary aneurysms is also shown. The vascular manifestations of neurofibromatosis and the causes of coronary aneurysms are reviewed. (Postgrad Med J 2001;77:45-47) Keywords: coronary aneurysm; neurofibromatosis; intravascular ultrasoundThe vascular manifestations of neurofibromatosis were first described in 1944 by Reubi. 1 Vascular abnormalities can aVect the aorta or iliac, mesenteric, renal, and cerebral arteries. There have been two previous reports of coronary aneurysms causing myocardial infarction in neurofibromatosis, 2 3 a report of coronary ectasis and stenosis causing myocardial infarction, 4 and a report of myocardial infarction presumed to have resulted from vasospasm. 5 We report here the first case of multiple coronary aneurysms in association with neurofibromatosis, and present the first published IVUS picture of a giant coronary aneurysm.
Case reportA 54 year old woman presented to our hospital with a five month history of exertional chest pain. She had a past history of neurofibromatosis type 1 (NF-1), characterised by axillary freckling and multiple café au lait macules and neurofibromata, and carcinoma of the breast, treated by mastectomy. The diagnosis of NF-1 had never been genetically confirmed. An exercise test was positive at a low workload, and we proceeded to left heart catheterisation which showed inferior akinesia, severe mitral regurgitation, and two circular calcified lesions in the region of the left anterior descending coronary artery (LAD) and the distal right coronary artery near the crux. There was occlusion of the LAD, occlusion of the circumflex artery, and aneurysmal dilatation of the right coronary artery, which also filled the distal LAD through collaterals (figs 1 and 2).Transoesophageal echocardiography showed restriction of the P2/P3 portions of the posterior mitral valve leaflet in systole with severe mitral regurgitation, consistent with an ischaemic aetiology.She was referred for coronary artery bypass grafting and mitral valve repair. At operation two calcified aneurysms were seen at the origin of the LAD and in the mid right coronary artery. Intraoperative intravascular ultrasound (IVUS) showed severe disease of the right coronary artery proximal to the aneurysm, which had not been visible on the coronary angiogram, followed by aneurysmal dilatation, reaching 8 × 9 mm at its largest internal diameter (fig 3).
DiscussionNF-1 is a congenital hereditary disease with generalised neuroectodermal and mesodermal dysplasia that aVects the skin, nervous system, skeleton, and vascular system. Diagnosis is based on the presence of at least two of the following seven criteria 6 : x Six or more café au lait macules, over 5 mm in prepubertal or 15 mm in postpubertal individu...
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