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Severe middle vault deformity with disturbed nasal form and function is one of the most challenging procedures to correct in a secondary rhinoplasty. Reconstructing the deformity with autologous septal cartilage would be the primary choice of most surgeons, if it were always available. However in certain cases the lack of a sufficient quantity of autologous cartilage has forced surgeons to explore other viable options. This paper discusses our experience with the combined use of spreader and dorsal onlay grafts from various materials in the reconstruction of severe middle vault deformity in 110 patients. In follow up, (between 6 and 42 months; mean 21 months) all patients were noted to have improved in both aesthetics and function with no major complications noted. In summary, this study proposes that any engrafting material can be used safely when the proper surgical principals and technique are employed.  相似文献   
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Clinical Rheumatology - The outcomes of COVID-19 in patients with axial spondyloarthritis (ax-SpA) have not been explored in detail. Tumour necrosis factor inhibitors (TNFi) are commonly used for...  相似文献   
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Purpose:The aim of this study was to evaluate the structural and functional changes occurring in patients with branch retinal vein occlusion (BRVO) according to the distance of the affected arteriovenous (AV) crossing to the centers of the fovea and optic disc by optic coherence tomography angiography (OCTA).Methods:Forty-five patients with unilateral BRVO and 45 age- and sex-matched healthy controls were included in this retrospective observational study. Images of the macula (3 mm × 3 mm) and affected AV crossing sites were obtained by OCTA. The fovea-AV crossing distance (FAVD), optic disc-AV crossing distance (DAVD), and optic disc-fovea distance (DFD) were measured.Results:The FAVD/DFD ratio was positively correlated with the vessel density in the superficial and deep affected hemifields (r = 0.430, P < 0.05 and r = 0.308, P < 0.05, respectively) and negatively correlated with the superficial foveal avascular zone and logarithm of the minimum angle of resolution (logMAR) visual acuity (r = –0.412, P < 0.05 and r = –0.356, P < 0.05, respectively). The DAVD/DFD ratio was not correlated with the logMAR visual acuity, superficial FAZ area or vessel densities in the affected hemifield (all P > 0.05).Conclusion:The affected AV crossing site that was further away from the fovea had better visual acuity and quantitative microvascular parameters in the affected hemifields. However, this correlation was not observed for the distance between the affected AV crossing site and the optic disc.  相似文献   
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Multiple sulfatase deficiency is biochemically characterized by the accumulation of sulfated lipids and acid mucopolysaccharides. The gene sulfatase-modifying factor 1 (SUMF1), recently identified, encodes the enzyme responsible for post-translational modification of a cysteine residue, which is essential for the activity of sulfatases. We describe clinical findings and mutation analysis of four patients. The patients presented with hypotonia, developmental delay, coarse face, ichthyosis, and hepatosplenomegaly. The diagnosis was made through clinical findings, enzymatic assays, and mutation analysis. We were detected to be homozygous for a novel missense mutation c. 739G > C causing a p.G247R amino acid substitution in the SUMF1 protein.Key Words: Child, multiple sulfatase deficiency, sulfatase-modifying factor 1 gene  相似文献   
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Silent myocardial ischemia is defined as an ischemic episode without chest pain but with transient ST abnormalities during stress testing or Holter monitoring. With Holter monitoring the prevalence of silent myocardial ischemia in hypertensive patients without coronary artery disease is between 25% and 73%. Simultaneous recording of ambulatory 24-h ECG and 24-h ambulatory blood pressure measurements (ABPM) with the option of additional ST-triggered blood pressure measurement is useful to detect silent ischemia and triggers of silent ischaemia. It is surprising that only a few combined 24-h Holter/ABPM devices are on the market, and in turn only three devices allow additional triggered blood pressure measurements. The paper provides an overview of studies investigating hypertensive patients with Holter monitoring for the detection of ST segment depression indicating myocardial ischaemia. Furthermore, requirements for combined devices allowing simultaneous ambulatory 24-h ECG and ABPM are defined.  相似文献   
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PURPOSE: The aim of this study was to evaluate the changes in salivary gland function in patients with chronic renal failure (CRF) undergoing hemodialysis. METHODS: The group consisted of 23 patients with CRF (13 female, 10 male; mean age: 40 +/- 13 yr) and 14 healthy control subjects (mean age: 40 +/- 13 yr). All underwent dynamic salivary gland scintigraphy with gustatory stimulation. After intravenous administration of 99mTc pertechnetate, first, perfusion images at 2 seconds per frame were acquired for 1 minute, then dynamic images at 1 minute per frame were acquired for 45 minutes. At 30 minutes after injection, 10 ml lemon juice was given for 15 minutes as a gustatory stimulus. We obtained time-activity curves derived from regions of interest centered over the four major salivary glands. The following functional indices were calculated for each gland: the time of maximum radioactivity (Tmax) for the prestimulated period, the time of minimum radioactivity (Tmin), as an indicator of velocity of secretion after stimulation, and the Lem E5% value as an indicator of the secretion function. RESULTS: When the patients with CRF undergoing hemodialysis were compared to the controls, there were statistically significant differences in Tmax, Tmin and Lem E5% values for bilateral parotid glands, and Tmin values for bilateral submandibular glands (p < 0.05), there were no statistically significant differences in Tmax and Lem E5% values for bilateral submandibular glands. There were also significant differences in Tmax and Lem E5% values for bilateral parotid glands between mild oral problems and severe oral problems in patients with CRF (undergoing hemodialysis). CONCLUSION: In this study, prolonged Tmax and Tmin values, and decreased Lem E5% values for parotid glands and prolonged Tmin values for submandibular glands on salivary scintigraphy pointed out decreased parenchymatous and excretory function in patients with CRF undergoing hemodialysis.  相似文献   
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Higher technetium-99m methoxyisobutylisonitrile (MIBI) uptake in non-small cell lung cancer (NSCLC) has been reported to be associated with a positive response to chemotherapy. It has previously been found that in tumour cells, P-glycoprotein (Pgp) expression is of importance for tracer uptake. However, some studies have indicated that Pgp expression does not play an important role in (99m)Tc-MIBI uptake in NSCLC; indeed, a negative correlation between (99m)Tc-MIBI uptake and Pgp expression has been reported. Against the background of conflicting results, our aim was to evaluate the relationship between (99m)Tc-MIBI uptake, prognosis and Pgp expression in NSCLC. A total of 37 patients with NSCLC underwent (99m)Tc-MIBI single-photon emission tomography (SPET) before chemotherapy. In 19 patients both Pgp and p53 expression, and in two patients only p53 expression (due to the limited biopsy material), were measured with immunohistochemical staining. (99m)Tc-MIBI uptake was significantly higher in responders than in non-responders: 3.09+/-1.14 vs 2.24+/-0.88 ( P<0.03) and 3.09+/-1.08 vs 2.37+/-1.06 ( P<0.05) for the early ratio (ER) and the delayed ratio (DR), respectively. The wash-out rate (WR) of responders was not significantly different from that of non-responders. We found no significant differences in ER, DR and WR among the groups positive or negative for Pgp and p53 status. There was a significant positive correlation between the survival rate and both ER and DR: r=0.49 ( P=0.003) and r=0.40 ( P=0.018), respectively. Patients with ER and DR values above 3 showed significantly longer survival than those with values below 3: 14.7+/-8.5 months vs 7.3+/-5.1 months ( P<0.009) and 13.2+/-8.4 months vs 7.4+/-5.3 months ( P<0.04) for ER and DR, respectively. However, interestingly, and in contrast to expectations, patients with a Pgp score of +2 showed significantly longer survival (12.9+/-6.7 months) than those with Pgp scores of +1 (4.4+/-3.0 months) or - (negative) (3.8+/-2.2 months) ( P<0.009 and P<0.02, respectively). Our results suggest that in NSCLC, patients with higher (99m)Tc-MIBI uptake tend to show a positive response to chemotherapy, and patients with ER and DR values above 3 have a significantly better prognosis. We also found that Pgp expression seems to play only a minor role in (99m)Tc-MIBI uptake. Our finding that patients with ER and DR values above 3 have a better prognosis needs to be confirmed in larger series of patients.  相似文献   
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