Cho, Sujin;Seo, Go Hun;Kim, Yoon-Myung;Kim, Gu-Hwan;Yoo, Han-Wook;Lee, Beom Hee
Journal of The Korean Society of Inherited Metabolic disease
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v.18
no.1
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pp.13-17
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2018
Methylmalonic acidemia is an autosomal recessive disorder caused by complete (mut0) or partial (mut-) deficiency of methylmalonyl-CoA mutase (MUT) or by defects in the synthesis of adenosylcobalamin (cblA, cblB, cblD variant 2). Long term complications of methylmalonic acidemia include tubulointerstitial nephritis with progressive renal failure, intellectual impairment, pancreatitis, and growth failure. We report a case of methylmalonic acidemia in a girl who diagnosed at 6 days after birth. She has developed recurrent metabolic crises with hyperammonemia and metabolic acidosis. In addition, she suffered from the chronic complications including tubulointerstitial nephritis, electrolyte imbalance associated with renal dysfunction, growth failure and fracture of femur shaft. At the age of 10 years, hypercalcemia and severe osteoporosis were noted, and pamidronate therapy was given for two years, which relieved hypercalcemia and osteoporosis.
Magnetic fluid, a new type of magnetic material, is a colloidal liquid constituted of nano-scale ferromagnetic particles suspended in carrier fluid. Magnetic fluid sealing is one of the most successful applications of magnetic fluid. As a new type of seal offering the advantages of no leakage, long life and high reliability, the magnetic fluid seal has been widely utilized under vacuum- and low-pressure-differential conditions. In practical applications, for improved pressure capacity, a multistage sealing structure is always used. However, in engineering applications, a uniform distribution of magnetic fluid under each tooth often cannot be achieved, which problem weakens the overall pressure capacity of the seals. In order to improve the pressure capacity of magnetic fluid seals and broaden their applications, the present study theoretically and experimentally analyzed the degree of non-uniform distribution of multistage magnetic fluid seals. A mathematical model reflecting the relationship between the pressure capacity and the distribution of magnetic fluid under a single tooth was constructed, and a formula showing the relationship between the volume of magnetic fluid and its contact width with the shaft was derived. Furthermore, the relationship of magnetic fluid volume to capacity was analyzed. Thereby, the causes of non-uniform distribution could be verified: injection of magnetic fluid; the assembly of magnetic fluid seals; the change of magnetic fluid silhouette under pressure loading; the magnetic fluid sealing mechanism of pressure transmission, and seal failure. In consideration of these causes, methods to improve the pressure capacity of magnetic fluid seals was devised (and is herein proposed).
Pile foundation is a typical form of bridge foundation and viaduct, and large-diameter rock-socketed piles are typically adopted in bridges with long span or high piers. To investigate the effect of a mountain slope with a deep overburden layer on the bearing characteristics of large-diameter rock-socketed piles, four centrifuge model tests of single piles on different slopes (0°, 15°, 30° and 45°) were carried out to investigate the effect of slope on the bearing characteristics of piles. In addition, three pile group tests with different slope (0°, 30° and 45°) were also performed to explore the effect of slope on the bearing characteristics of the pile group. The results of the single pile tests indicate that the slope with a deep overburden layer not only accelerates the drag force of the pile with the increasing slope, but also causes the bending moment to move down owing to the increase in the unsymmetrical pressure around the pile. As the slope increases from 0° to 45°, the drag force of the pile is significantly enlarged and the axial force of the pile reduces to beyond 12%. The position of the maximum bending moment of the pile shifts downward, while the magnitude becomes larger. Meanwhile, the slope results in the reduction in the shaft resistance of the pile, and the maximum value at the front side of the pile is 3.98% less than at its rear side at a 45° slope. The load-sharing ratio of the tip resistance of the pile is increased from 5.49% to 12.02%. The results of the pile group tests show that the increase in the slope enhances the uneven distribution of the pile top reaction and yields a larger bending moment and different settlements on the pile cap, which might cause safety issues to bridge structures.
Muscle spindle afferents from masseter muscle were labelled by the intra-axonal HRP injection and were processed for light microscopic reconstruction. Regions containing terminal arbors scattered in the central portion of the masseteric motor neuron pool (type I a) and those restricted to 2-3 small portion of it (type II) were selected and processed for electronmicroscopic analysis with serial sections. The shape of the labelled boutons was dome or elongated shape. Scalloped or glomerulus shape with peripherial indentation containing pre or postsynaptic neuronal propiles, which is occasionally found in the trigeminal main sensory nucleus and spinal dorsal horn, was not observed. Both type Ia and type II boutons had pale axoplasm and contained clear, spherical vesicles of uniform size(dia : 49-52nm) and occasionally large dense cored vesicles(dia : 87-118nm). The synaptic vesicles were evenly distributed throughout the boutons although there was a slight tendency of vesicles to accumulate at the presynaptic site. The average of short and long diameter(short D. + long D./2) of type I a bouton was smaller than that of type II bouton. All the labelled boutons, which showed prominent postsynaptic density, large synaptic area and multiple synaptic contact, made asymmetrical synaptic contact with postsynaptic neuronal propiles. Most of the type Ia and type II boutons made synaptic contact with only one neuronal propile and boutons which shows synaptic contact or more neuronal propiles was not observed. Most of the type Ia boutons(87.2%) were presynaptic to the soma or proximal dendrite and a few remainder(12.8%) made synaptic contact with dendritic shaft or distal dendrite. In contrast, majority of type II boutons showed synaptic contact with dendritic shaft and remainder with soma or proximal dendrite. In conclusion, terminal boutons which participate in the excitatory monosynaptic jaw jerk reflex made synaptic contact with more proximal region of the neuron, and showed very simple synaptic connection, compared with those from the primary afferenst in the other region of the central nervous system such as spinal dorsal horn and trigeminal main sensory nucleus which assumed to be responsible for the mediating pain, tactile sensation, sensory processing or sensory discrimination.
The accurate measurement of the femoral anteversion is important for the derotational osteotomy. To estimate femoral anteversion, following three major parameters are required; the neck axis, the long axis, and the knee axis. Conventional methods on the basis of 2D images are ambiguous to determine these major axes. As the femur has a complex 3 dimensional structure, the 3 dimensional model should be applied for accurate and reliable measurement of femoral anteversion. In this thesis, we model femur and define three parameters. The neck axis is defined from the femoral head and neck model. The long axis is determined from the cylindrical model of the femoral shaft. The knee axis is also determined from the model of femoral condyles. According to the definition of the femoral anteversion, the femoral anteversion is efficiently estimated from these models. 20 specimens were tested by the conventional 2D imaging method and 3D imaging method witch was developed by authors and the new 3D modeling method. The study provides accurate, fast and human factor free measurement for femoral anteversion.
It was revealed that the morphology and projection pattern of terminal arbors from single primary afferent are different among distinct fiber types, functional types and the different subdivision of trigeminal sensory nucleus complex(TSNC). But it was not identified the ultrastructural morphology and synaptic connections of terminal arbors from each primary afferent within TSNC. So we employed the intra-axonal horseradish peroxidase(HRP) injection technique to define the terminal arbors of primary afferent fiber from slowly adapting mechanoreceptors in the periodontal ligament of the cat, and examined 66 labeled terminal arbors within the rostrodorsomedial part(Vo.r) of the trigeminal nucleus oralis, electromicroscopically with 90nm serial sections. All the boutons labelled with HRP contained clear, spherical and uniform sized synaptic vesicles(diameter : $47.66{\pm}3.58nm$ ). Most of the labelled boutons were boutons en passant type and they were connected by unmyelinated axonal strand. In which neurofilament and microtubule was not developed but occasionally contained synaptic vesicle in contrast to the myelinated axon. The size of the labelled bouton was relatively small(long diameter : $1.46{\pm}0.24{\mu}m$, short diameter $0.85{\pm}0.26{\mu}m$, average diameter $1.15{\pm}0.24{\mu}m$) and the shape of which varied from dome to elongated shape, but scalloped glomerulus shape was not developed. Each primary ending in Vo.r made synapse with one or two neuronal propiles(average : $1.11{\pm}0.31$), of which, 89.4% of labelled boutons made synapse with only one neuronal pro pile, the remainder, 10.6% of labelled boutons, made synapse with two neuronal propile. So characteristically they made very simple synapse. Most of labelled boutons(80.03%) made asymmetrical synapse only with dendritic shaft or spine, and 6.1% of labelled boutons received symmetrical synapse from pleomorphic vesicle containing axonal ending(p-ending). So presynaptic inhibiton was relatively scarce. Synaptic triad, in which a p-ending is presynaptic both pre-and post-synaptic element of the axo-dendritic contact from the labelled primary ending was not observed.
Condition Monitoring System (CMS) has been used to detect unexpected faults of wind turbine caused by the abrupt change of circumstances or the aging of its mechanical part. In fact, it is a very hard work to do regular inspection for its maintenance because wind turbine is located on the mountaintop or sea. The purpose of this study is to find out distribution patterns of vibration signals measured from the main mechanical parts of wind turbine according to its operation condition. To this end, acceleration signals of main bearing, gearbox, generator, wind speed, rotational speed, etc were measured through the long period more than 2 years and trend analyses on each signal were conducted as a function of the rotational speed. In addition, correlation analysis among the signals was done to grasp the relation between mechanical parts. As a result, the vibrations were dependent on the rotational speed of main shaft and whether power was generated or not, and their distributions at a specific rotational speed could be approximated to Weibull distribution. It was also investigated that the vibration at main bearing was correlated with vibration at gearbox each other, whereas vibration at generator should be dealt with individually because of generating mechanism. These results can be used for improving performance of CMS that early detects the mechanical abnormality of wind turbine.
Purpose: In the process of replantation of the amputated fingertips, the primary concern was given to survival of the amputees, while the functional aspect of digits after the surgery has been easily neglected. Although an internal fixation with a K-wire is often a part of replantation of the amputated fingertips, little consideration had been given to the study of relationship between distal interphalangeal joint fixation and post operative range of motion. A comparative study in relation to post operative range of motion was done on two different groups, one group with K-wire insertion and the other group without a K-wire insertion at the distal interphalangeal joint. Materials and Methods: The study was done on the cases of a single digit amputation conducted at our institute (the age in the range of 10 to 60) in about four-year of time span from March of 2005 to March of 2009. The cases with a thumb replantation, osteomyelitis or articular surface injury have been excluded from this study. The cases of both head and shaft fracture, except the insertion site of tendon, of distal phalanx of internal fixation with a single K-wire were reviewed for this study. A group of 24 cases without distal interphalangeal joint fixation in comparison to a group of 22 cases with distal interphalangeal joint fixation was reviewed to assess the postoperative range of motion at distal interphalangeal joint on the 6th week after the surgery. And, on the 30th month after the surgery, a group of 10 cases without distal interphalangeal joint fixation in comparison to a group of 10 cases with joint fixation was reviewed. A K-wire was removed in about 5 weeks after the fracture was reunited under the radiographic image, immediately followed by a physical therapy. Result: The active range of motion for a group without interphalangeal joint fixation was measured $49.0^{\circ}$ on average, while $28.6^{\circ}$ was measured for a group with interphalengeal fixation on the 6th week after the surgery. On the 30th month after the surgery, the active range of motion was measured $52.0^{\circ}$ and $55.0^{\circ}$ on average for a group without and with interphalangeal fixation respectively. Conclusion: In the process of replantation of the amputated fingertips, short-term(on the 6th week) improvement of postoperative active motion of range can be expected in the cases without distal interphalangeal fixation in comparison to the cases of interphalangeal joint fixation with a K-wire. However, there seems to be no difference on motion of range in a long-term (on the 30th month) follow up period.
Kim, Hyunwoo;Park, Dohyun;Park, Eui-Seob;Sunwoo, Choon
Tunnel and Underground Space
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v.24
no.4
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pp.297-307
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2014
Cavern Thermal Energy Storage system stores thermal energy in caverns to recover industrial waste heat or avoid the sporadic characteristics of renewable-energy resources, and its advantages include high injection-and-extraction powers and the flexibility in selecting a storage medium. In the present study, the structural stability of rock mass pillar between these silo-type storage caverns was assessed using a coupled thermal-mechanical model in $FLAC^{3D}$. The results of numerical simulations showed that thermal stresses due to long-term storage depended on pillar width and had significant effect on the pillar stability. A sensitivity analysis of main factors indicated that the influence on the pillar stability increased in the order cavern depth < pillar width < in situ condition. It was suggested that two identical caverns should be separated by at least one diameter of the cavern and small-diameter shaft neighboring the cavern should be separated by more than half of the cavern diameter. Meanwhile, when the line of centers of two caverns was parallel to the direction of maximum horizontal principal stress, the shielding effect of the caverns could minimize an adverse effect caused by a large horizontal stress.
The Journal of the Korean bone and joint tumor society
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v.16
no.2
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pp.80-86
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2010
Purpose: The skeleton is commonly affected by metastatic cancer. The purpose of this study was to evaluate the results of treating metastatic pathologic fractures in lower extremities using locking plates. Materials and Methods: Between 2004 and 2010, we evaluated 12 patients (13 cases) of metastatic pathologic fractures in lower extremities, treated with the locking plate. Mean patient age was 62.2 years (range, 50-81 years), the locations of the fractures were; proximal femur in 2 cases, femoral mid-shaft in 3, distal femur in 3, proximal tibia in 4, and distal tibia in 1 case. The interval to wheelchair ambulation, pain relief and complications were evaluated. Additionally, we assessed operation time and postoperative blood loss. Results: Mean time from operation to wheelchair ambulation was 3.2 days (range, 1-6 days). Mean VAS scores improved from a preoperative score of 8.1 points (range, 7-9 points) to a score of 2.7 points (range, 2-4 points) at 1 week postoperatively. No early complications associated with surgery were encountered. Mean operation time was 88.4 minutes (range, 70-105 minutes), and mean postoperative blood loss was 246.5 ml (range, 130-320 ml). Conclusion: Internal fixation of metastatic pathologic fractures using a locking plate in the lower extremity can be an effective treatment option in the meta- or diaphyseal area of long bones with massive bony destruction or poor bone stock by offering early ambulation, pain relief and low postoperative complications.
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