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31.
Introduction
We analyzed clinical factors related to uptake on a Tc-99 m HDP bone scan of the accessory navicular (AN).Materials and Methods
We retrospectively reviewed patients who had been examined by an orthopedic surgeon and underwent bone scan due to suspected symptomatic AN. A three-point grading system was used to evaluate uptake on bone scan. Relationships between grade, symptoms, age, gender, symptom duration, and bone size were analyzed.Results
In total, 73 ANs (30 asymptomatic, 43 symptomatic) were enrolled. The majority of asymptomatic ANs had no uptake but some had grade 1 (n?=?8) or 2 (n?=?2) uptake. All asymptomatic ANs with uptake remained asymptomatic during follow-up. For the asymptomatic ANs, larger bones showed a higher grade. With a cut-off value of size ≤6.8 mm, there is no chance of uptake. All symptomatic ANs showed uptake on bone scan. For symptomatic ANs, larger size and shorter pain duration were related to a higher grade. Age, gender, and left-/right-sideness were not related to grade. Multiple regressions revealed that only uptake grade, not size or symptom duration, was the significant risk factor for a symptomatic AN. With a cut-off value of grade <1, a symptomatic AN could be ruled out with a negative predictive value of 100 %.Conclusion
Bone scanning is useful for symptomatic ANs with a high negative predictive value. Higher grade is related to larger size and shorter pain duration. For asymptomatic ANs, grade was related to size but did not predict symptom development.32.
33.
Glucocerebrosidase is a lysosomal enzyme responsible for hydrolysis of glucosylceramide to ceramide and glucose. Mutations disrupting the function of this enzyme cause autosomal recessive Gaucher disease. This disease is very heterogeneous. The clinical heterogeneity is due to a large number of mutations within the gene encoding glucocerebrosidase. To date 36 mutations have been described in Gaucher disease. In this part we present the mutations and review the more common ones. We also review the glucocerebrosidase natural activator, designated saposin C and mutations in its gene, associated with Gaucher disease. © 1994 Wiley-Liss, Inc. 相似文献
34.
Firat Okmen Huseyin Ekici Sabahattin Anil Ari 《Journal d'obstetrique et gynecologie du Canada》2018,40(11):1466-1467
Background
Candida species are harmless commensals of hosts, including humans, but they can cause infection when the immune system is compromised. Infections with non-albicans species can occur, ranging from urinary tract infections to sepsis, especially among patients in intensive care units.Case
The patient, a 37-year-old woman, presented with severe abdominal pain, fever, and vomiting. The patient's symptoms and fever continued in spite of treatment with antibiotics, and she underwent exploratory laparotomy. Cyst content culture results showed that Candida kefyr was present in the cyst.Conclusion
To the best of our knowledge, this is the first case report of a tubo-ovarian abscess caused by C. kefyr. Rare pathogens can be found in patients with a tubo-ovarian abscess, so culture of the abscess material is important for determining subsequent treatment, particularly in women who require an operation for tubo-ovarian abscess. 相似文献35.
Eric Lemonnier Gaëlle Robin Cline Degrez Roman Tyzio Marine Grandgeorge Yehezkel Ben‐Ari 《Acta paediatrica (Oslo, Norway : 1992)》2013,102(6):e288-e290
We report that daily administration of the diuretic NKCC1 chloride co‐transporter, bumetanide, reduces the severity of autism in a 10‐year‐old Fragile X boy using CARS, ADOS, ABC, RDEG and RRB before and after treatment. In keeping with extensive clinical use of this diuretic, the only side effect was a small hypokalaemia. A double‐blind clinical trial is warranted to test the efficacy of bumetanide in FRX. Conclusion: This single case report showed an improvement of the scores of each test used after 3 months of treatment. Double‐blind clinical trials are warranted to test the efficacy of bumetanide in FRX. 相似文献
36.
Ethical considerations for enzyme replacement therapy in neuronopathic Gaucher disease 总被引:1,自引:0,他引:1
Elstein D, Abrahamov A, Zimran A. Ethical considerations for enzyme replacement therapy in neuronopathic Gaucher disease. Clin Genet 1998: 54: 179–184. 0 Munksgaard, 1998
Enzyme replacement therapy for Gaucher diseases, the most prevalent lysosmal storage disease, was originally approved by the FDA for type I patients and has proven to be both safe and effective in reducing hepatosplenomegaly and improving the hematological parameters. However, the use of enzyme treatment in both neuronopathic forms has heretofore been on an investigational or trial basis, with reports of progression of neurological deterioration even at very high doses. To date, there are no guidelines for clinicians with regard to enzyme replacement therapy in the neuronopathic forms of metabolic diseases. Herein, we discuss strategies derived from the literature ub-his treatment of very premature babies and from the Jewish Halachic point of view. In conclusion, we describe recommendations for the ethical treatment and/or withdrawal of treatment. as well as practical guidelines for dosage regimens, in children with neuronopathic Gaucher disease. 相似文献
Enzyme replacement therapy for Gaucher diseases, the most prevalent lysosmal storage disease, was originally approved by the FDA for type I patients and has proven to be both safe and effective in reducing hepatosplenomegaly and improving the hematological parameters. However, the use of enzyme treatment in both neuronopathic forms has heretofore been on an investigational or trial basis, with reports of progression of neurological deterioration even at very high doses. To date, there are no guidelines for clinicians with regard to enzyme replacement therapy in the neuronopathic forms of metabolic diseases. Herein, we discuss strategies derived from the literature ub-his treatment of very premature babies and from the Jewish Halachic point of view. In conclusion, we describe recommendations for the ethical treatment and/or withdrawal of treatment. as well as practical guidelines for dosage regimens, in children with neuronopathic Gaucher disease. 相似文献
37.
Leena Paavolainen Keijo Häkkinen Ari Nummela Heikki Ruskol 《European journal of applied physiology》1994,69(2):119-126
The purpose of this study was to investigate neuromuscular and energy performance characteristics of anaerobic power and capacity and the development of fatigue. Ten endurance and ten sprint athletes performed a new maximal anaerobic running power test (MARP), which consisted ofn x 20-s runs on a treadmill with 100-s recovery between the runs. Blood lactate concentration [la–]b was measured after each run to determine submaximal and maximal indices of anaerobic power (P
3mmol·1
–1,P5mmol·1
–1,P10mmol·1
–1andP
max) which was expressed as the oxygen demand of the runs according to the American College of Sports Medicine equation: the oxygen uptake (ml·kg–1·min–1)=0.2·velocity (m·min–1) +0.9·slope of treadmill (frac)·velocity (m·min–1)+3.5. The height of rise of the centre of gravity of the counter movement jumps before (CMJrest) and during (CMJ) the MARP test, as well as the time of force production (t
F) and electromyographic (EMG) activity of the leg muscles of CMJ performed after each run were used to describe the neuromuscular performance characteristics. The maximal oxygen uptake (
max), anaerobic and aerobic thresholds were determined in the
max test, which consisted ofn x 3-min runs on the treadmill. In the MARP-testP
max did not differ significantly between the endurance [116 (SD 6) ml·kg–1·min–1] and sprint [120 (SD 4) ml·kg–1·min–1] groups, even though CMJrest and peak [la–]b were significantly higher and
max was significantly lower in the sprint group than in the endurance group and CMJrest height correlated withP
max (r=0.50,P<0.05). The endurance athletes had significantly higher mean values ofP
3mmol·1
–1andP
5mmol·1
–1[89 (SD 7) vs 76 (SD 8) ml·kg–1·min–,P<0.001 and 101 (SD 5) vs 90 (SD 8) ml·kg–1·min–1,P<0.01. Significant positive correlations were observed between theP
3mmol·l
–1and
max, anaerobic and aerobic thresholds. In the sprint group CMJ and the averaged integrated iEMG decreased andt
F increased significantly during the MARP test, while no significant changes occurred in the endurance group. The present findings would suggest thatP
max reflected in the main the lactacid power and capacity and to a smaller extent alactacid power and capacity. The duration of the MARP test and the large number of CMJ may have induced considerable energy and neuromuscular fatigue in the sprint athletes preventing them from producing their highest alactacidP
max at the end of the MARP test. Due to lower submaximal [la–]b (anaerobic sprinting economy) the endurance athletes were able to reach almost the sameP
max as the sprint athletes. 相似文献
38.
39.
Hietala EM Maasilta P Juuti H Nuutinen JP Harjula AL Salminen US Lassila R 《Thrombosis and haemostasis》2004,92(6):1394-1401
Platelets play a key role in (sub)acute thrombotic occlusion after stenting. We examined the possible differences between biodegradable polylactide (PLA) and stainless steel (SS) stents in platelet attachment and morphology after whole blood perfusion. PLA stents of different configurations (spiral/braided) and polycaprolactone-polylactide (PCL-PLA)-coatings, or SS stents were implanted into a PVC tube (? 3.2 mm), with or without precoating of the tube with type-I collagen. PPACK (30 microM)-anticoagulated blood with (3)H-serotonin prelabeled platelets was perfused (flow rate: 30 ml/min, 90 s) over the stents. Platelet deposition was assessed by scintillation counting and morphology by scanning electron microscopy (SEM). To examine coagulation activation, plasma prothrombin fragments (F1 + 2) were measured before and after the perfusion. Protein deposition on PLA/SS stents was assessed at augmented shear forces mimicking coronary flow (rate: 60 ml/min, 60 s) under minimal anticoagulation (PPACK 1 microM). More platelets deposited on PLA stents than on SS stents under all study conditions (p < 0.03). Under anticoagulation (PPACK 30 microM) the generation of F1 + 2 remained unaltered. Under higher flow rate and limited anticoagulation SS stents accumulated 3.27 +/- 0.75 microg and PLA stents 5.25 +/- 1.74 microg of protein (Mean +/- SD, p <0.95). Among all biodegradable stents, the braided PLA stent coated with PCL-PLA-heparin accumulated the fewest platelets (p < 0.02). In SEM, signs of platelet activation on braided heparin-coated PLA stents, when compared with uncoated braided PLA/SS stents, appeared modest. In conclusion, PCL-PLAheparin coating of biodegradable stents may enhance their hemocompatibility, expressed by less platelet deposition. Nevertheless, materials, design, and coating techniques of biodegradable stents must be further developed. 相似文献
40.
Digit representation is more than just hand waving 总被引:1,自引:0,他引:1
Thompson JC Abbott DF Wheaton KJ Syngeniotis A Puce A 《Brain research. Cognitive brain research》2004,21(3):412-417
Lip-reading and interpreting hand gestures help provide nonverbal information that aids speech comprehension in noisy environments and places emphasis on certain key utterances. In this fMRI study, we examined if viewing the similar semantic information presented by either finger movements or lip movements was processed by common or discrete brain regions. Subjects viewed videos of a hand conveying number information via finger movements and a face whose lip movements conveyed the same numerical information. Control stimuli consisted of meaningless finger and lip movements. Lip-reading numbers activated left posterior superior temporal sulcus (STS), while identifying numbers presented by fingers activated the intraparietal region (IPR) bilaterally. Conjunction analysis highlighted common activation in right IPR to numbers presented via fingers and lips. Our data indicate that left hemisphere decodes human movements conveying semantic information, although the specific brain region that is engaged may depend on the body part that is moving. 相似文献