Presentation and outcome of Marfan's syndrome patients with dissection and thoraco-abdominal aortic aneurysm.

BACKGROUND: In Marfan's syndrome, there is a paucity of data regarding intervention criteria for surgery of the dissected thoraco-abdominal aorta. METHODS: A retrospective analysis of 22 Marfan's patients with distal aortic dissection managed between September 1999 and April 2006 was performed. Serial diameters and linear expansion rates were calculated from imaging studies and the outcome of intervention was analysed. RESULTS: There were 14/22 male patients (median age 38 years), and 18 had prior aortic surgery. Surgery was recommended in 20 patients and undertaken in 19 (1 died prior to operation). Of the operated patients, 2 presented with rupture, 2 with airway obstruction, 1 with intermittent paraplegia and 14 underwent planned surgery for increased expansion rate or pain. All patients had residual type A or chronic type B dissection. The median aortic dimension at surgery was 6.7 cm (interquartile range (IQR) 5.5-8.2). The preoperative mean expansion rate increased from 0.5 cm/year to 1.7 cm/year (p<0.001), prior to operation. Fifteen patients underwent Crawford Extent II, two underwent Extent I and two underwent Extent III repair. Profound hypothermia and CSF drainage was used in 16 and 18 patients, respectively. There was no early mortality, paraplegia or renal failure. At a median postoperative follow-up of 56 months (range 6-86), the survival of the operated cohort was 90%. CONCLUSIONS: Thoraco-abdominal aortic aneurysm repair in Marfan's syndrome can be performed with good outcomes. Intervention should be based on size or accelerated expansion. Any role of endovascular management needs careful consideration.     

Alterations of paraoxonase and platelet-activating factor acetylhydrolase activities in patients on peritoneal dialysis.

OBJECTIVE: The more atherogenic lipid profile seen in peritoneal dialysis (PD) patients cannot fully explain the increased incidence of atherosclerosis in this population. Oxidative modification of low-density lipoproteins (LDL) is considered to play a central role in the atherogenic process, whereas high-density lipoprotein (HDL) protects LDL from oxidation. On the other hand, it has been suggested that the LDL and HDL of PD patients are more resistant to oxidation than those of control subjects, while PD-HDL equally protects LDL from oxidation compared to control-HDL. Two HDL-associated enzymes have been shown to protect both LDL and HDL from oxidation: paraoxonase (PON1) and HDL-associated platelet-activating factor acetylhydrolase (HDL-PAF-AH). Furthermore, low PON1 activity and high total plasma PAF-AH concentration, which represents mainly the LDL-associated enzyme, have been shown to be independent risk factors for coronary artery events in the general population. However, there are limited data regarding possible alterations of these enzymes in PD patients. The aim of our study was to examine the possible alterations of PON1 and PAF-AH activities in patients undergoing PD. DESIGN: A cross-sectional study. SETTING: A university medical center. PARTICIPANTS: 56 PD patients of Caucasian origin and 86 matched controls were studied. MEASUREMENTS: In all subjects, serum PON1 activity toward paraoxon (paraoxonase) and phenylacetate (arylesterase), as well as total serum and HDL-PAF-AH activities were measured; PON1 genetic polymorphisms known to influence PON1 activity (Q192R and M55L) were determined. RESULTS: The PD patients exhibited significantly increased serum PON1 (paraoxonase) and PON1 (arylesterase) activities compared to controls, regardless of the PON1 polymorphisms or the levels of HDL cholesterol. Additionally, PD patients had significantly elevated activities of total serum PAF-AH and HDL-PAF-AH, independently of the levels of LDL or HDL cholesterol. The ratio of HDL-PAF-AH/ total PAF-AH, which has recently been suggested to be a potential marker of atherogenicity, was decreased in these patients compared to controls. Moreover, no difference in the prevalence of PON1 polymorphisms between PD patients and controls was found. CONCLUSION: The elevated activities of PON1 and HDL-PAF-AH could explain the increased resistance of PD-HDL to oxidation; the higher activity of total PAF-AH and the decreased HDL-PAF-AH/ total PAF-AH ratio could contribute to the increased incidence of atherosclerosis in these patients.     

A binocular machine vision system for three-dimensional surface measurement of small objects

Rendering three-dimensional information of a scene from optical measurements is very important for a wide variety of applications. However, computer vision advancements have not yet achieved the accurate three-dimensional reconstruction of objects smaller than 1 cm diameter. This paper describes the development of a novel volumetric method for small objects, using a binocular machine vision system. The achieved precision is high, providing a standard deviation of 0.04 mm. The robustness, of the system, issues from the lab prototype imaging system with the crucial z-axis movement without the need of further calibration and the fully automated volumetric algorithms.     

α<Subscript>2β</Subscript> adrenoreceptor 301–303 deletion polymorphism in polycystic ovary syndrome

α_2β adrenoreceptor 301–303 deletion polymorphism does not influence basal metabolic rate, insulin resistance or weight gain in Greek women with polycystic ovary syndrome.     

Increased sensitization in urban vs. rural environment – Rural protection or an urban living effect?

In a population-based longitudinal cohort study, we tested the hypothesis that children growing up in a high-traffic polluted urban area (UA) in the Athens' basin have higher prevalence of allergies and sensitization when compared with those growing up in a Greek provincial rural area (RA). We recruited 478 and 342 children aged 8-10 living in the UA and the RA, respectively. Respiratory health was assessed by a parent-completed questionnaire in three phases: 1995-96 (phase 1), 1999-2000 (phase 2), 2003-04 (phase 3) and skin-prick testing to common indoor and outdoor aeroallergens was performed at phases 1 and 2. Reported asthma and eczema did not differ between the two areas, whereas reported hay fever was persistently more prevalent in the UA than in the RA (16.5%, 17.0%, 18.2% vs. 7.0%, 8.3%, 9.6%, respectively). Sensitization was more prevalent in the UA at both phases (19.0% vs. 12.1% in phase 1, 20.0% vs. 14.1% in phase 2). Residential area contributed independently to sensitization to >or=1 aeroallergens (OR: 0.29; 95% CI: 0.13-0.66; p = 0.003) and to polysensitization (OR: 0.28; 95% CI: 0.10-0.82; p = 0.020) in phase 1. These associations were independent of farming practices. No significant contributions were found in phase 2. Our results suggest that long-term exposure to urban environment is associated with a higher prevalence of hay fever but not of asthma or eczema. The negative association between rural living and the risk of atopy during childhood, which is independent of farming practices, implies that it is mainly driven by an urban living effect.     

Is Preservation of the Adrenal Vein Mandatory in Laparoscopic Adrenal-Sparing Surgery?

BACKGROUND AND OBJECTIVE: Adrenal tissue-sparing or partial adrenalectomy evolved initially for patients with bilateral synchronous adrenal surgical pathology to preserve vital adrenal volume. In the laparoscopic era, the exact criteria for performing such procedures laparoscopically have yet to be defined. Controversy exists regarding the importance of preserving the adrenal vein, main or accessory. The aim of this retrospective study was to present our short series of laparoscopic tissue-sparing adrenalectomies with vein preservation. Our main goal is not to support partial adrenalectomy as an alternative to total (this is already advocated by many surgeons) but to emphasize the vein-preserving technique. METHODS: Seven patients with peripherally located either aldosterone-producing adenomas (4 cases) or myelolipomas (4 cases) underwent laparoscopic lateral partial adrenalectomy. One patient harbored an aldosterone-producing adenoma and a myelolipoma as well. The main adrenal vein was identified and preserved in 6 patients and the accessory vein in one. RESULTS: No conversion to open adrenalectomy was necessary, and no perioperative morbidity or mortality occurred. Three adenoma patients are normotensive 44, 23, and 20 months postoperatively, while the fourth one's pressure is refractory. CONCLUSIONS: Surprisingly, total adrenalectomies preceded the partial ones, which is controversial compared with other procedures. Laparoscopic lateral partial adrenalectomy is a technically challenging tissue-sparing operation. Meticulous dissection allows preservation of the middle artery and main or accessory vein resulting in a functioning adrenal stump.     

Treatment of metastatic malignant melanoma with dacarbazine, vindesine and cisplatin

Twenty-seven patients with disseminated malignant melanoma were treated monthly with cisplatin (CDDP) 120 mg m-2 on day 1, vindesine (VDS) 3 mg m-2 on day 2 and dacarbazine (DTIC) 250 mg m-2 on days 2-6. None of them had received prior chemotherapy. All patients are evaluable for response and toxicity. There were five (19%) complete (CR) and seven (26%) partial (PR) responses for a total response rate of 45%. We conclude that the combination of DTIC, VDS and CDDP is capable of producing a relatively high rate of response in patients with advanced metastatic malignant melanoma, but responses are short.     

Analysis of SAA1 gene polymorphisms in the Greek population: rheumatoid arthritis and FMF patients relative to normal controls. Homogeneous distribution and low incidence of AA amyloidosis.

OBJECTIVE: To address whether or not the rarity of amyloidosis in Greek patients with rheumatoid arthritis (RA) is related to specific alleles of single nucleotide polymorphisms (SNPs) in the 5'-flanking region and the exon 3 of the SSA1 gene. METHODS: The genotypes of the -13T/C SNP in the 5'-flanking region of the SAA1 gene and the two SNPs within exon 3 of SAA1 (2995C/T and 3010C/T polymorphisms) were determined in 88 Greek patients with RA, 14 patients with familial Mediterranean fever (FMF) and 110 healthy controls. Linkage disequilibrium and haplotype frequencies involving -13T/C, 2995C/T and 3010C/T in these populations were tested and estimated, respectively. RESULTS: The genotypic distribution and allelic frequencies were similar in all groups tested. SNPs 2995 and 3010 were in linkage disequilibrium for all study populations (p < 0.05), whereas SNP -13 was not in linkage disequilibrium with either 2995 or 3010 (p > or = 0.05). Two major haplotypes presented in all patients with RA and FMF and controls: -13C; 2995T; 3010C (-13C; alpha) and -13C; 2995C; 3010T (-13C; beta). The -13T allele was linked with the gamma haplotype in Greek patients with RA and controls. The frequency of the -13T allele was found to be very rare in all groups tested. CONCLUSIONS: In conclusion, the rarity of the putative amyloidogenic -13T allele in Greek populations may be related to low prevalence of AA amyloidosis development in Greek RA patients.