Neurofibromatosis type I is one of the most common neurocutaneous syndrome which is inherited by autosomal dominant manner, characterized by cafe au-lait spots, axillary freckling, Lisch nodules in iris, multiple neurofibromas and bone involvement with pseudoarthrosis, bowing of the long bone. And Moyamoya disease is a specific disease characterized by progressive idiopathic stenosis and eventual occlusion of the large cerebral arteries at the circle of Willis. In response to the stenosis, an abnormal network of small collateral vessels develops, creating the "puff of smoke". Intracranial lesions associated with Neurofibromatosis type I include optic glioma, sphenoid wing dysplasia, "unidentified bright objects" and cerebrovascular lesions such as Moyamoya syndrome and aneurysm. Moyamoya syndrome is an uncommon association of neurofibromatosis type 1 and lung mass has not frequently been found with neurofibromatosis and moyamoya syndrome. We report a case with Neurofibromatosis type I with Moyamoya syndrome and ganglioneuroma in lung with reviewing literatures.
Aneurysm ; Cerebral Arteries ; Circle of Willis ; Constriction, Pathologic ; Ganglioneuroma ; Iris ; Lung ; Moyamoya Disease ; Neurocutaneous Syndromes ; Neurofibromatoses ; Neurofibromatosis 1 ; Optic Nerve Glioma ; Pseudarthrosis

BACKGROUND: Tricuspid regurgitation has been considered as a secondary lesion when it is combined with left valvular heart diseases. However, there have been some reports which show that tricuspid regurgitation keeps going and results in congestive heart failure even after a successful operation for left valvular heart disease. So far, there are no definite operation indications and predictive factors for the tricuspid regurgitation which is resulted from the left sided valvular heart disease. We designed this study to evaluate the effects of pulmonary artery pressure and left ventricular ejection fraction on the prognosis of tricuspid regurgitation, and to make an operation indication for the patients with secondary tricuspid regurgitation. MATERIAL AND METHOD: We reviewed the medical records of patients who underwent surgery for the left sided valvular heart disease with tricuspid regurgitation and were followed for more than 1 year with echocardiograms. There was a total of 114 cases. We compared the grades of tricuspid regurgitations and pulmonary artery pressures and left ventricular ejection fractions on the basis of echocardiograms which were checked preoperatively and on the last follow up. RESULT: There were 43 cases of tricuspid annuloplasty. In these patients, the grades of tricuspid regurgitations were improved in 42 cases (97.7%). But in 71 cases without annuloplasty, 29 cases (41%) were improved, 32 cases (45%) had no change, and 10 cases (14%) were aggravated. This finding shows significant differences in the prognoses of tricuspid regurgitations between the two groups (p<0.05). There was no difference in pulmonary artery pressures and ejection fractions between the patients who showed progression of tricuspid regurgitations and those who didn't (p>0.05). The improvements of tricuspid regurgitations are not statistically related to the changes of pulmonary artery pressures or left ventricular ejection fractions. CONCLUSION: This study shows that it is impossible to predict the prognoses of tricuspid regurgitations with preoperative pulmonary artery pressures or left ventricular ejection fractions. Also, the excellent results of tricuspid annuloplasty is proven in controlling the secondary tricuspid regurgitations. Therefore, when tricuspid regurgitation is detected preoperatively, the procedures to correct the tricuspid regurgitation at the time of the operation for the left-sided valvular heart disease must be considered positively, regardless of the grades of tricuspid regurgitations, to prevent significant tricuspid regurgitation that may develop later.
Follow-Up Studies ; Heart Failure ; Heart Valve Diseases* ; Humans ; Medical Records ; Prognosis* ; Pulmonary Artery ; Stroke Volume ; Tricuspid Valve Insufficiency*

BACKGROUND/AIMS: Despite of increasing numbers of reports on intraductal papillary mucinous tumor (IPMT), there is still difficulty in its' diagnosis, treatment and prediction of prognosis. The purpose of this multicenter study was to evaluate the clinico-pathological features of IPMT in Korea and suggest the prediction criteria of malignancy in IPMT. METHODS: We retrospectively reviewed the clinico-pathological data of 208 patients who underwent operations with IPMT between 1993 and 2002 at 28 institutes in Korea. RESULTS: Of the 208 patients with a mean age of 60.5+/-9.7 years, 147 were men and 61 were women. 124 patients underwent pancreatoduodenectomy, 42 distal pancreatectomy, 17 total pancreatectomy, 25 limited pancreas resection. Benign cases were 128 (adenoma (n=62), borderline (n=66)) and malignant cases were 80 (non-invasive (n=29), invasive (n=51)). A significant difference in 5-year survival was observed between benign and malignant group (92.6% vs. 65.3%; p=0.006). Of the 6 factors (age, location, duct dilatation, tumor appearance, main duct type, and tumor size) that showed the statistical difference in univariate analysis between benign and malignant group, we found three significant factors (tumor appearance (p=0.009), tumor size (p=0.023), and dilated duct size (p=0.010)) by multivariate analysis. CONCLUSION: Although overall prognosis of IPMT is superior to ordinary pancreatic cancer, more curative surgery is recommended in malignant IPMT. Tumor appearance (papillary), tumor size (> or =30 mm) and dilated duct size (> or = 12 mm) can be used as preoperative indicators of malig-nancy in IPMT.
Academies and Institutes ; Diagnosis ; Dilatation ; Female ; Humans ; Korea* ; Male ; Mucins* ; Multivariate Analysis ; Pancreas* ; Pancreatectomy ; Pancreatic Neoplasms ; Pancreaticoduodenectomy ; Prognosis ; Retrospective Studies

Local alignment is an important task in molecular biology to see if two sequences contain regions that are similar. The most popular approach to local alignment is the use of dynamic programming due to Smith and Waterman, but the alignment reported by the Smith-Waterman algorithm has some undesirable properties. The recent approach to fix these problems is to use the notion of normalized scores for local alignments by Arslan, Egecioglu and Pevzner. In this paper we consider the problem of finding all local alignments whose normalized scores are above a given threshold, and present a fast heuristic algorithm. Our algorithm is 180-330 times faster than Arslan et al.''s for sequences of length about 120 kbp and about 40-50 times faster for sequences of length about 30 kbp.
Molecular Biology