1/4. Systemic sclerosis therapy with iloprost: a prospective observational study of 30 patients treated for a median of 3 years.iloprost is useful in the short-term treatment of severe Raynaud's phenomenon and ischaemic ulcers in patients with systemic sclerosis (SSc), but its long-term effects are largely unknown. The aim of this study was to report long-term outcome (median follow-up 36 months) in a prospective observational study of a cohort of 30 consecutive patients with SSc who received iloprost therapy with maintenance infusions every 3 weeks after an initial cycle of 5 consecutive days. At the end of the observation, compared to the pretreatment point, we observed complete healing of digital ulcers in 19/21 patients (90%), a decrease of the Raynaud's phenomenon visual analogue score from 10/10 (25th-75th percentile 7-10) to 5/10 (4-6.75) ( P <0.001) and, in patients with diffuse cutaneous involvement, of the modified Rodnan skin thickness score from 25.5 (16.5-31.5) to 16 (13.5-20) ( P = 0.02), minimal improvement of the health Assessment Questionnaire from 0.87 (0.68-1.37) to 0.75 (0.62-1.25), which was neither statistically nor clinically significant. The forced vital capacity was not significantly changed, but the diffusion capacity corrected for the alveolar volume decreased from 71% (54-76.7) of the expected value to 62% (51.5-71) ( P = 0.02). In one patient with limited SSc a positive effect on pulmonary hypertension was observed. Six patients, after a median of 25 months of treatment and healing of digital ulcers, discontinued the therapy; after a median of 10 months ulcers did not recur in five of these six. Other reasons for discontinuation were: tolerability (1), disease progression (normotensive renal crisis: 1), and death due to intracranial haemorrhage (1). This same patient had previously suffered a central retinal vein thrombosis. In conclusion, long-term therapy with iloprost in patients with SSc has a durable effectiveness on ischaemic ulcers and Raynaud's phenomenon, but it is not possible to conclude that the natural history of the disease was modified.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
2/4. A fine balance--one-lung ventilation in a patient with Eisenmenger syndrome.A 38-yr-old woman with an atrial septum defect and Eisenmenger syndrome was scheduled for a lung biopsy via thoracoscopy during one-lung ventilation. Fluids were given to increase central venous pressure to 8 mm Hg, an epidural catheter was inserted at the sixth thoracic intervertebral space and ropivacaine 0.3%, 6 ml were given. Careful balance of systemic and pulmonary vascular resistance is crucial in Eisenmenger syndrome, so norepinephrine (0.14 mg kg(-1) min(-1)) was infused before general anaesthesia was started with fentanyl 4 mg kg(-1), ketamine 2 mg kg(-1), pancuronium 1 mg and succinylcholine 2 mg kg(-1). Anaesthesia was maintained with propofol 4-8 mg kg(-1) h(-1). To control pulmonary artery pressure, ventilation was performed with oxygen 100% and nitric oxide 20 ppm. Surgery and anaesthesia course were uneventful and the patient was extubated. However, pleural haemorrhage required treatment with blood components, re-intubation on the second postoperative day and removal of the haematoma by mini-thoracotomy. A step-by-step approach using a balanced combination of regional and general anaesthesia, controlled fluid administration, norepinephrine and inhaled nitric oxide preserved a stable circulation even during one-lung ventilation. The diagnostic value of lung biopsy must be weighed against the possibility of life-threatening haemorrhage.- - - - - - - - - - ranking = 2keywords = haemorrhage (Clic here for more details about this article) |
3/4. Pulmonary vasoconstriction following intravenous nimodipine.A case is reported in which intravenous nimodipine therapy was associated with severe acute pulmonary hypertension in a patient with a subarachnoid haemorrhage. The patient had previously received enteral nimodipine without problems.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |
4/4. Congenital syphilis associated with persistent pulmonary hypertension of the neonate--a clinico-pathological case study.Congenital syphilis remains a significant clinical problem, especially in developing countries. We report a fatal case of congenital syphilis complicated by persistent pulmonary hypertension and hypoxic ischaemic encephalopathy. OBJECTIVE: To describe the association of congenital syphilis with persistent pulmonary hypertension of the newborn (PPHN). METHOD: Case report of a single patient. RESULTS: fatal outcome of one baby with congenital syphilis and associated PPHN despite maximal conventional treatment. Histological examination of the lungs revealed pulmonary oedema, intra-alveolar haemorrhages, localised bronchopneumonia and marked interstitial infiltrates of lymphocytes, plasma cells, macrophages and fibroblasts with interstitial fibrosis. Examination of peripheral pulmonary arteries revealed focal excessive muscularisation with increased adventitial connective tissue. DISCUSSION: Reviewing our own experience and available literature, this case study supports the infrequent association of congenital syphilis with PPHN. However, when it occurs, this combination appears to be fatal. CONCLUSION: More research is warranted to clarify the role of inflammatory mediators in congenital syphilis of the lung.- - - - - - - - - - ranking = 1keywords = haemorrhage (Clic here for more details about this article) |