Bupropion for Blau syndrome

Blau syndrome (BS) is an autosomal dominantly inherited disease characterized by granulomas and arthritis. The gene mutated in BS was recently found to be CARD15. Mutations in this gene also occur in about 20% of patients with Crohn's disease (CD), though with different mutations than in the Crohn's patients. We are not aware of any cure or specific treatment for BS. We have found that bupropion is effective for CD, and we now suggest that bupropion be considered for treatment of BS.     

Transforming growth factor-alpha and epidermal growth factor receptor in colonic mucosa in active and inactive inflammatory bowel disease

Transforming growth factor-alpha (TGF-alpha) is overexpressed in colonic carcinomas and promotes mucosal wound healing. It may be implicated in chronic inflammatory bowel disease (IBD). We analyzed the expression of TGF-alpha and its receptor, epidermal growth factor receptor (EGF-r), in the colonic mucosa of patients with Crohn's disease (CD) or ulcerative colitis (UC), in active or inactive stages, as compared with controls. Proteins and mRNA were detected in biopsies from the right and left colon and in surgical colonic specimens. Immunoblot analysis revealed TGF-alpha protein as a 29 kDa band. This band was normally expressed in uninvolved colonic mucosa of patients with CD or UC whether in active or inactive stages, but decreased or absent in involved mucosa of active IBD, even when TGF-alpha mRNA and EGF-r protein were detected. In the unaffected mucosa of CD, the intensity of TGF-alpha immunoreactivity was similar to that of controls in the right colon but stronger (P = 0.05) in the left colon. There was no TGF-alpha overexpression in dysplastic regions. In conclusion, in active IBD disease, the decreased TGF-alpha protein amount seems not only related to epithelial cell loss but reflects a down-regulation at least at the protein level. We speculate that TGF-alpha does not play a role within the active stage but may be implicated later in the repair process.     

An ordered comparative map of the cattle and human genomes

A cattle-human whole-genome comparative map was constructed using parallel radiation hybrid (RH) mapping in conjunction with EST sequencing, database mining for unmapped cattle genes, and a predictive bioinformatics approach (COMPASS) for targeting specific homologous regions. A total of 768 genes were placed on the RH map in addition to 319 microsatellites used as anchor markers. Of these, 638 had human orthologs with mapping data, thus permitting construction of an ordered comparative map. The large number of ordered loci revealed > or =105 conserved segments between the two genomes. The comparative map suggests that 41 translocation events, a minimum of 54 internal rearrangements, and repositioning of all but one centromere can account for the observed organizations of the cattle and human genomes. In addition, the COMPASS in silico mapping tool was shown to be 95% accurate in its ability to predict cattle chromosome location from random sequence data, demonstrating this tool to be valuable for efficient targeting of specific regions for detailed mapping. The comparative map generated will be a cornerstone for elucidating mammalian chromosome phylogeny and the identification of genes of agricultural importance."Ought we, for instance, to begin by discussing each separate species-in virtue of some common element of their nature, and proceed from this as a basis for the consideration of them separately?" from Aristotle, On the Parts of Animals, 350 B.C.E.     

Comparison between nafarelin acetate andd-Trp6-LHRH for temporary pituitary suppression in in vitro fertilization (IVF) patients: A prospective crossover study

Purpose Nafarelin acetate is a new gonadotropin releasing (GnRH) agonist analogue with unique potency, intranasal administration, and convenient storage. Hence, nafarelin was considered as an alternative for temporary pituitary suppression in patients undergoing ovulation induction in IVF. A crossover treatment in a prospective study was performed including 40 women with bilateral obstructed tubes and normal ovarian function, treated in 80 ovulation induction cycles using the long protocol. Twenty patients used nafarelin acetate 600 g/daily in their first cycle and received d-Trp6-LHRH, 0.5 mg/daily, in their following cycle. The other 20 women used decapeptyl in their first cycle and received nafarelin in the second.Results Estradiol suppression was achieved by both d-Trp6-LHRH and nafarelin at equal time intervals. The average total number of ampoules (P=0.0005) and the length of administration of hMG required for ovarian stimulation (P=0.0002) and the time interval between GnRHa initiation to oocyte retrieval (P=0.04) was significantly lower in nafarelin cycles. The number and the distribution between large and small follicles as well as the average number of oocytes retrieved did not differ between the two GnRH analogues.Conclusion Our results demonstrate that nafarelin acetate is comparable to d-Trp6-LHRH for temporary pituitary suppression used for controlled ovarian stimulation in IVF patients. However, using nafarelin ovarian stimulation was achieved with fewer ampoules of hMG, administered for a shorter period of time, thus with a lesser cost.     

Intra-osseus gas formation in osteomyelitis of vertebrae and pelvis by Klebsiella pneumoniae]

A 59-year-old woman had persistent sepsis after abdominal operations because of a volvulus and subsequently a retroperitoneal abscess, in spite of antibiotic treatment against Klebsiella pneumoniae, which grew in blood cultures. During abscess drainage, a haemorrhage from the infrarenal part of the aorta had occurred; in view of a presumed aortitis this part had been replaced by a bifemoral bypass. Computer tomography revealed intraosseous formation of gas in vertebrae and pelvis. At operation, abscesses were drained and necrotomy and sequestrotomy of the bone were performed. Cultures of the pus from the iliac crests showed K. pneumoniae. The antibiotic management was changed; the wounds were flushed regularly. After exposure of the wounds still draining after 6 months and vascular surgery because of occlusion of the bypass after 7 months, the patient recovered well. She died 2 years later from a cerebral haemorrhage.     

MR-guided percutaneous nephrostomy of the nondilated upper urinary tract in a porcine model

OBJECTIVE: The purpose of this study was to assess the safety and feasibility of MR-guided percutaneous nephrostomy of the nondilated contrast-enhanced upper urinary tract in a porcine model. SUBJECTS AND METHODS: Six MR-guided percutaneous nephrostomies of the nondilated upper urinary tract were performed in four domestic farm pigs (body weight range, 20-25 kg) using a 0.2-T system. Ten minutes after IV administration of 0.2 mmol/kg of gadodiamide and 0.4 mg/kg of furosemide, a fast T1-weighted sequence was used to guide insertion of an 18-gauge MR-compatible needle into a predetermined calix. After confirmation of needle position using a turbo spin-echo T1-weighted sequence, a 0.035-inch catheter coated with superparamagnetic iron oxide was inserted during MR monitoring. Insertion was followed by tract dilatation and insertion of a 4-French sheath. The final position of the sheath was confirmed by injection of diluted superparamagnetic iron oxide into the collecting system. RESULTS: Needle insertion was successful for all six procedures, with no complications. Instrumentation time ranged from 4 to 13 min (mean, 6+/-4 min). Sheath placement was successful in five of six kidneys. Placement time ranged from 6 to 28 min (mean, 16+/-9 min). CONCLUSION: MR-guided percutaneous nephrostomy of the nondilated contrast-enhanced upper urinary tract in a porcine model is feasible and safe.     

Low-flow vascular malformations in the head and neck: safety and feasibility of MR imaging-guided percutaneous sclerotherapy--preliminary experience with 14 procedures in three patients

Fourteen percutaneous sclerotherapy procedures with magnetic resonance (MR) imaging guidance were performed in three patients with low-flow vascular malformations. All targeted vascular malformation compartments were filled with sclerosing agent without complications in a mean procedural time of 29 minutes. Follow-up imaging demonstrated reduction in size of the treated portions in all patients. In conclusion, sclerotherapy with MR imaging guidance can be performed safely and allows monitoring of injection.     

Massive osteolysis of the skull base

Gorham's vanishing bone disease is a rare disorder characterized by massive osteolysis. Its etiology is unknown. We report the case of a 14-year-old girl with an unusually aggressive course and extensive involvement originating within the skull base. To our knowledge this pattern has not been described previously. MR findings are discussed. Received: 10 January 1996 Accepted: 3 July 1996