Patients with idiopathic pulmonary arterial hypertension usually undergo acute vasodilator tests with nitric oxide (NO) for haemodynamic evaluation and therapeutical planning. The aim of this study was to evaluate the link between the variation of N-terminal (NT)-pro-brain natriuretic peptide (BNP) levels and haemodynamic parameters during the acute vasodilator test.A total of 22 idiopathic pulmonary arterial hypertension patients who underwent acute vasodilator tests were studied. Blood samples were collected at baseline and after 30 and 60 min of NO inhalation. NT-pro-BNP levels were measured in each sample.A receiver-operating characteristic curve was used to evaluate the capability of the NT-pro-BNP level variation during NO inhalation in recognising nonresponders. To distinguish responders from nonresponders, the increase of the NT-pro-BNP (0% as cut-off value) determined a 50% specificity and 100% sensitivity (positive predictive value of 38% and a negative predictive value of 100%).These results suggest that N-terminal-pro-brain natriuretic peptide was able to distinguish nonresponder patients with the acute vasodilator test. N-terminal-pro-brain natriuretic peptide may be an interesting additional biological tool in the evaluation of idiopathic pulmonary arterial hypertension patients.

A 20 year old woman died of respiratory failure due to cicatricial pemphigoid ofthe trachea and bronchi. This is the first case with the lower airways affected to be reported.Cicatricial pemphigoid, or benign mucus membrane pemphigoid,'2 is a chronic bullous disease primarily affecting mucus membranes and less frequently the skin. It occurs from the second to the eighth decade of life, but is more common in the elderly. Its course is usually benign, though conjunctival scarring may cause visual impairment. The respiratory tract is rarely affected, and in previously reported cases disease has been limited to the larynx.'-2 We present a patient who died of respiratory failure caused by lesions in the trachea and bronchi. Case reportA 20 year old Bolivian woman was admitted to our hospital from another centre with mucosal ulceration and respiratory dysfunction. She had been healthy until September 1986, when a painful blistering eruption had developed in the mouth and vulva, which partially resolved with prednisolone. One month later typhoid fever was diagnosed, and she was treated with chloramphenicol, sulphamethoxazole, and trimethoprin. A few days later there was an abrupt onset of a generalised cutaneous bullous rash, thought to be erythema multiforme, so antibiotics were discontinued. The rash resolved but the mucosal lesions persisted. A lip biopsy specimen taken at the time was reported as showing the histological changes of pemphigus vulgaris, though no immunofluorescence studies were carried out. Prednisolone (70 mg/day) was reintroduced, again with partial improvement, but it was withdrawn after three weeks, after the onset ofdepressive psychosis. At this time the patient also began to complain of dyspnoea, cough, and wheeze. The respiratory symptoms progressively worsened; bron- Accepted 28 February 1989 chodilators and steroids afforded some relief but again steroids had to be discontinued, and she was transferred to our care.On admission she was afebrile but distressed, dyspnoeic, and cachectic, with painful ulceration and scarring of the vulva and mouth and blistering of the face and trunk. The conjunctivae were ulcerated and scarred, and early synechiae were present. There was prolonged expiration and wheeze, and chest radiography and computed tomography showed hyperinflated lungs and distended bronchi (fig 1). Arterial oxygen tension was 7-3 kPa and arterial carbon dioxide tension 8 kPa; FVC was 1 08 (pred 3 64)1, FEV, 0-53 1 (pred 2-99 1), PEF 108 1/min (pred 379 1/min), and FEF 25-75% 0-23 (pred 3 68) 1/s. The airways obstruction was unaffected by inhaled salbutamol. Oesophageal and tracheal ulcers surrounded by cicatricial areas were seen at endoscopy.Laboratory investigations showed mild anaemia, a total white cell count of 4-8 x 109/1, and reactive bone marrow. Antinuclear factor, lupus erythematosus cells, rheumatoid factor, circulating immune complexes and anti-basement membrane antibody were absent; serum complement and immunoglobulin concentrations were within normal limits.Biop...

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Aims: To study the relationship between myocardial release of cTnI and myocardial cell death as assessed by the amount of apoptosis and necrosis after cardiac surgery. Methods: Eighteen young pigs were operated on with standardized cardiopulmonary bypass (CPB). Release of cTnI in the cardiac lymph (CL), coronary sinus (CS), and arterial blood (A) was related to postoperative myocardial cell death by both necrosis and apoptosis. Apoptotic cells were detected by a TUNEL detection kit. Necrotic cells were counted by light microscopy. Results: In all animals, cTnI was significantly released and reached peak values observed simultaneously in A (cTnI, 20.1±2.6 ng/ml) (mean ±SEM), CS (19.5±3.2 ng/ml) and CL (5202±2500 ng/ml). Percentage of total myocardial cell death was 3.1±0.5%, including 1.2±0.35% necrosis and 1.9±0.5% apoptosis. cTnI release during and after CPB did not correlate with the degree of myocardial apoptosis or necrosis. Conclusion: Cardiac operations with CPB are related to myocardial cell damage including myocardial cell death due to both necrosis and apoptosis. As the loss of cTnI is not related to the amount of cell death, our results suggest that increased cardiac myocyte membrane permeability more than cell death is responsible for intraoperative and postoperative cTnI release.