Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases.

OBJECTIVES: This study investigated the long-term effects of bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist, in patients with pulmonary arterial hypertension (PAH) exclusively related to connective tissue diseases (CTD). METHODS: A total of 53 patients with PAH related to connective tissue diseases (PAH-CTD) in World Health Organization (WHO) functional class III received bosentan 62.5 mg twice a day for 4 weeks and then 125 mg twice a day for 44 weeks in this open non-comparative study. Assessments at weeks 16 and 48 included WHO class, clinical worsening, quality of life (Short-Form Health Survey (SF-36) and health assessment questionnaire (HAQ) modified for scleroderma), and survival (week 48 only). Safety and tolerability were monitored throughout the study. RESULTS: At week 48, WHO class improved in 27% of patients (95% CI 16-42%) and worsened in 16% (95% CI 7-29%). Kaplan-Meier estimates were 68% (95% CI 55-82%) for absence of clinical worsening and 92% (95% CI 85-100%) for survival. Overall changes in quality of life were minimal. There were no unexpected side effects observed during the study. CONCLUSIONS: In most patients, bosentan was associated with improvement or stability of clinical status. The 92% estimate for survival at 48 weeks is a significant achievement in this patient population.

Percutaneous drainage of splenic abscesses: an effective and safe procedure.

Percutaneous drainage of splenic abscesses has not yet become a common procedure due to the potential risk of bleeding and the danger of damage to neighbouring organs. We present our experience of percutaneous drainage in eight patients with splenic abscesses. Four patients were treated by therapeutic percutaneous needle aspiration of the fluid collection, and four by percutaneous trocar catheter insertion. All procedures were guided by ultrasound or computed tomography. The procedure was successful in all eight patients with no complications.

External carotid occlusive disease as a cause of facial pain.

A 47 year old man suffered an acute left hemiparesis after several weeks of right-sided facial pain. Right carotid angiography revealed internal carotid artery thrombosis and severe occlusion of external carotid branches supplying facial structures. An ischaemic aetiology for the facial pain is suggested.

Prepyloric diaphragm, an unusual case of gastric outlet syndrome.

The prepyloric diaphragm is a rare entity, with well-established anatomicopathological features but of unknown etiology. Approximately half of the 180 cases described occurred in children. The clinical presentation may be a chronic partial obstruction as in pyloric stenosis, or a complete gastric outlet obstruction when the orifice of the diaphragm is very small or nonexistent. Despite the variable clinical picture, radiological and endoscopic examinations may reveal typical pathognomonic features. With cases being reported more frequently, the treatment had changed from a wholly surgical approach to the introduction of endoscopic web incision and conservative treatment. We report a case diagnosis during surgery, and review the pertinent literature.

Purification and characterization of a novel 5' exodeoxyribonuclease from the yeast Saccharomyces cerevisiae.

We have isolated from yeast cells an exonuclease which preferentially attacks double-stranded DNA from the 5' ends producing 5'-mononucleotides as reaction products. A second typical product is a full-length single-stranded DNA complement, suggesting that the enzyme hydrolyzes one DNA strand in a processive manner before it associates with another DNA substrate to initiate a new reaction cycle. Its biochemical properties suggest that the enzyme is unlike the yeast exonucleases which have been reported so far. However, the new exonuclease is strikingly similar to the well characterized 5' exonuclease of bacteriophage lambda.

Orthotopic renal autotransplantation in the treatment of renovascular hypertension.

Severe hypertension non-responsive to medical therapy was discovered in a 27-year-old woman in the third trimester of pregnancy. Renal arteriography after delivery revealed bilateral renal artery stenosis. The right kidney was smaller than the left one. The differential renal function studies, rather than the renal vein renin measurements, identified which side was causing the hypertension. An attempt at primary repair of the right renal artery failed. Orthotopic renal autotransplantation, using saphenous vein grafts that were interposed between the renal veins and the inferior vena cava, and between the segmental renal arteries and the abdominal aorta, reduced the blood pressure to normal values.

Structure of the human type I DNA topoisomerase gene.

We describe the molecular organization of the human gene coding for type I DNA topoisomerase. The coding sequence is split into 21 exons distributed over at least 85 kilobase pairs (kb) of human genomic DNA. The sizes of the 20 introns vary widely between 0.2 and at least 30 kb and all contain the sequence elements known to be required for pre-mRNA splicing. Several of the intron sequences separate exons encoding parts of the enzyme that are highly conserved between human and yeast suggesting that at least some of the exons may code for individual, structurally, or functionally important domains of the enzyme. We also describe the promoter sequence of the human topoisomerase I gene and show that it is composed of distinct functional elements.

Transcutaneous drainage of abscesses of the liver guided by computed tomography scan.

Six instances of pyogenic abscess of the liver, diagnosed and localized by computed tomography scan, showed dramatic improvement after treatment by needle aspiration and antibiotics or precutaneous continuous aspiration and antibiotics. An intercostal trocar drain, 18F, was used to obtain optimal drainage also of viscous purulent material and debris. After drainage was begun, improvement was rapid and without complication or mortality. We have collected from the literature 59 patients with abscesses of the liver treated by the same methods with a surprisingly low mortality, 1.5 per cent. It seems that the continuous percutaneous drainage of the pyogenic abscess of the liver, guided by computed tomography scan or ultrasonography, may be the method of choice and that open surgical drainage should be limited to special instances.