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991.
    
Pyruvate dehydrogenase complex (PDHC) deficiency is a mitochondrial disorder. We report two cases of PDHC deficiency with clinical symptoms and brain imaging findings reminiscent of FOXG1 syndrome, suggesting a phenotypic overlap of these disorders.  相似文献   
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We encountered a surgical case of middle aortic syndrome (MAS) in a 56-year-old man who had resistant hypertension. Computed tomography showed severe stenosis of the abdominal aorta from below the superior mesenteric artery to above the inferior mesenteric artery. Although bilateral renal artery stenosis was confirmed, renal function was within normal limits. A 10-mm vascular prosthetic graft was used to perform a descending aorta to left external iliac artery bypass. His hypertension was well controlled without medication. This extra-anatomic bypass may be a simple and useful approach for treating MAS if it is not necessary to reconstruct the renal artery or visceral artery.  相似文献   
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This study presents a comprehensive investigation of the anatomical features of the levator ani muscle. The levator ani is a critical component of the pelvic floor; however, its intricate anatomy and functionality are poorly understood. Understanding the precise anatomy of the levator ani is crucial for the accurate diagnosis and effective treatment of pelvic floor disorders. Previous studies have been limited by the lack of comprehensive three-dimensional analyses; to overcome this limitation, we analysed the levator ani muscle using a novel 3D digitised muscle-mapping approach based on layer-by-layer dissection. From this examination, we determined that the levator ani consists of overlapping muscle bundles with varying orientations, particularly in the anteroinferior portion. Our findings revealed distinct muscle bundles directly attached to the rectum (LA-re) and twisted muscle slings surrounding the anterior (LA-a) and posterior (LA-p) aspects of the rectum, which are considered functional parts of the levator ani. These results suggest that these specific muscle bundles of the levator ani are primarily responsible for functional performance. The levator ani plays a crucial role in rectal elevation, lifting the centre of the perineum and narrowing the levator hiatus. The comprehensive anatomical information provided by our study will enhance diagnosis accuracy and facilitate the development of targeted treatment strategies for pelvic floor disorders in clinical practice.  相似文献   
996.
    

Purpose

We investigated whether myocardial 123I-metaiodobenzylguanidine (123I-MIBG) scintigraphy predicts impairment of myocardial functional reserve in response to dobutamine stress in patients with idiopathic dilated cardiomyopathy (DCM).

Methods

Forty DCM patients (LVEF 39?±?12 %) underwent myocardial 123I-MIBG scintigraphy, echocardiography, and cardiac catheterization. Myocardial 123I-MIBG uptake was quantified as the delayed heart to mediastinum (H/M) ratio and washout rate (WR). Local denervation was evaluated on polar map images. LV dP/dt max and T1/2 were determined from left ventricular pressure curves at baseline and during dobutamine infusion (15 μg/kg?/min). Patients were classified into two groups as follows: group A comprised 21 patients showing a delayed H/M ratio of <1.9 (median value); group B comprised 19 patients showing a delayed H/M ratio of ≥1.9.

Results

The percentage change in heart rate (%HR), LV dP/dt max (%LV dP/dt max), and T 1/2 (%T 1/2) from baseline to dobutamine stress were significantly more reduced in group A than in group B (39.3?±?20.2 %, 55.2?±?24.1 %, p?<?0.01; 102.3?±?46.3 %, 152.0?±?72.3 %, p?<?0.05; 38.7?±?15.3 %, 46.9?±?15.4 %, p?<?0.05, respectively). No significant differences between the two groups were observed in the echocardiographic parameters or baseline cardiac catheterization parameters. Significant correlations were found between delayed H/M ratio and %HR (r?=?0.35, p?<?0.05), %LV dP/dt max (r?=?0.45, p?<?0.05) and %T 1/2 (r?=?0.34, p?<?0.05). Significant inverse correlations were also found between WR and %HR (r?=??0.37, p?<?0.05), %LV dP/dt max (r?=??0.60, p?<?0.0001), and %T 1/2 (r?=??0.34, p?<?0.05). SPECT images revealed enhanced denervation from the inferoposterior to anterior wall in accordance with the advancement of global denervation.

Conclusion

Reduced 123I-MIBG uptake and increased washout were related to impairment in adrenergic myocardial functional reserve in idiopathic DCM.  相似文献   
997.
998.
    
Strontium lithium orthosilicate hydride Sr2LiSiO4H was synthesized by the reaction of Sr2SiO4 with LiH at 700 °C in a H2 rich atmosphere. Rietveld refinement of the neutron powder diffraction pattern revealed that Sr2LiSiO4H is isostructural to Sr2LiSiO4F (space group P21/m) and its channel-like structure preferentially accommodates H ions over F ions. In addition, Sr2LiSiO4H is stable in air and its Eu2+-doped analog exhibits yellow photoluminescence with an emission band at 544 nm and a broad excitation band ranging from 250 to 450 nm. These bands were observed in the longer wavelength region when compared with those displayed by Sr2LiSiO4F:Eu2+. The red shift, which is induced by H substitution, is consistent with the constrained density functional theory calculations, predicting the photo-excitation and emission energies of 4f–5d transitions. The present study reports the synthesis of stable oxyhydrides acting as phosphor hosts for rare earth ions. The phosphor hosts exhibit large nephelauxetic effects owing to the presence of H ligands.

Strong nephelauxetic effect on Eu2+ ion in Sr2LiSiO4H: enhancement of Eu 5d centroid shift by hydride ligand coordination.  相似文献   
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Objectives: Although Gelatin-Resorcin-Formalin (GRF) glue is widely used in surgery for acute aortic dissection, late complications possibly due to the glue, such as late aortic root redissection, have also been reported. We have experienced similar complications, some of which required redo surgeries, and these cases are reviewed.Methods: Twenty-six consecutive patients who underwent surgery for acute type A aortic dissection using GRF glue, from December 1996 to February 2001, were retrospectively studied, with a special focus on any late complications and any reoperation.Results: Of the 21 patients who survived and were followed as outpatients, false aneurysms were found in 5 patients (21%) at 24–42 (mean 34) months following the initial surgery. Of these, 2 patients required resternotomy because of the increasing aneurysm diameter. In both cases, the aortic root was redissected at the site of the GRF glue use where the anastomosis between the aortic root and the prosthesis had widely opened and had become the aneurysm entry point. Significant aortic regurgitation was noticed in 3 patients (14%, 1 of whom showed a false aneurysm), and 2 of these underwent reoperation for aortic root redissection.Conclusions: A high incidence of aortic root redissection with false aneurysm and/or aortic insufficiency was found following the surgery for acute aortic dissection using GRF glue. These patients should be carefully followed for years after surgery.  相似文献   
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