Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.
Nah Byung-Sik;Chung Woong-Ki;Ahn Sung-Ja;Nam Taek-keun;Yoon Mi-Sun;Song Ju-Young
Progress in Medical Physics
/
v.16
no.2
/
pp.82-88
/
2005
In this study, the physical compensator made with the high density material, Cerrobend, and the electronic compensator realized by the movement of a dynamic multileaf collimator were analyzed in order to verify the properness of a design function in the commercial RTP (radiation treatment planning) system, Eclipse. The CT images of a phantom composed of the regions of five different thickness were acquired and the proper compensator which can make homogeneous dose distribution at the reference depth was designed in the RTP. The frame for the casting of Cerrobend compensator was made with a computerized automatic styrofoam cutting device and the Millennium MLC-120 was used for the electronic compensator. All the dose values and isodose distributions were measured with a radiographic EDR2 film. The deviation of a dose distribution was $\pm0.99 cGy\;and\;\pm1.82cGy$ in each case of a Cerrobend compensator and a electronic compensator compared with a $\pm13.93 cGy$ deviation in an open beam condition. Which showed the proper function of the designed compensators in the view point of a homogeneous dose distribution. When the absolute dose value was analyzed, the Cerrobend compensator showed a $+3.83\%$ error and the electronic compensator showed a $-4.37\%$ error in comparison with a dose value which was calculated in the RTP. These errors can be admtted as an reasonable results that approve the accuracy of the compensator design in the RTP considering the error in the process of the manufacturing of the Cerrobend compensator and the limitation of a film in the absolute dosimetry.
Objective: The result of finite element analysis depends on material properties, structural expression, density of element, and boundar or loading conditions. To represent proper elastic behavior, a finite element model was made using Hounsfield unit (HU) values in CT images. Methods: A 13 year 6 month old male was used as the subject. A 3 dimensional visualizing program, Mimics, was used to build a 3D object from the DICOM file which was acquired from the CT images. Model 1 was established by giving 24 material properties according to HU. Model 2 was constructed by the conventional method which provides 2 material properties. Protraction force of 500g was applied at a 45 degree downward angle from Frankfort horizontal (FH) plane. Results: Model 1 showed a more flexible response on the first premolar region which had more forward and downward movement of the maxillary anterior segment. Maxilla was bent on the sagittal plane and frontal plane. Model 2 revealed less movement in all directions. It moved downward on the anterior part and upward on the posterior part, which is clockwise rotation of the maxilla. Conclusion: These results signify that different outcomes of finite element analysis can occur according to the given material properties and it is recommended to use HU values for more accurate results.
Purpose: This study is to analyze the clinical and radiological results after arthroscopic reduction and internal fixation of intra-articular fractures of lateral tibial plateau. Materials and Methods: The subject of the study are the 13 cases of the patients visited orthopedics surgery during March year 2000 to August year 2004 because of intra-articular fractures of lateral tibial plateau and were treated with arthroscopic reduction and internal fixation. X-rays and CT or MRI were both carried out to identify the precise pattern of fracture and the degree of depression which showed out to be all type 2 by Schatzker fracture classification. And in 9 of the cases, autogenous and allogenous bone grafts were given as bone loss were severe. The average age was 48, age group between 31 and 66, and average follow up period of about 38 months ($13{\sim}65months$). Radiological ratings were given by comparing the X-rays of degree of joint congruency before and after the operation, functional ratings by analyzing IKDC score and Lysholm score. Combined injuries observed after arthroscopy were posterior cruciate ligament injury in 1 case, meniscus injury in 4 cases and medial collateral ligament in 2 cases. Results: During follow up, X-rays showed well-maintained reduction of articular surface in all cases and no complications such as joint depression, fracture reduction loss, angular deformity or malunion were found. Average Lysholm score at last follow up was 87 points ranging from 65 to 97, in 8 of the cases excellent, 3 good, 1 fair and 1 poor according to Lynsholm classification. Average IKDC score was 92 (from 82 to 99). Conclusion: Not only does arthroscopic reduction of lateral tibial plateau fracture bring exact reduction of articular surface, but also, is considered to be a good way of operation to diagnose and treat combined injuries of knee joint using arthroscopy.
For the success of dental implant, accurate radiographic evaluation is prerequisite for planning the location of the osseointegrated implants and avoiding injury to vital structures. CT/MPR(computed tomography/multiplanar reformation) shows improved visualization of inferior alveolar canal. In order to obtain cross-sectional images parallel to the teeth, the occlusal plane is used to orientate for the axial plane. If the direction of axial plane is not parallel to the occlusal plane, the reformatted cross-sectional scans will be oblique to the planned fixture direction and will not show the actual dimension of the planned fixture's location. If the available bone height which measured in the cross-sectional view is much greater than the actual available bone height, penetration of canal may occur. The aim of this study is to assess the effect of the axial plane to measurement of available bone height for dental implant in computed tomography of the mandible. 40 patients who had made radiographic stents and had taken CT were selected. The sites that were included in the study were 45 molar regions. In the central panoramic scan, the length from alveolar crest to superior border of inferior alveolar canal(available bone height, ABH) was measured in direction of reformatted cross-sectional plane(uncorrected ABH). Then, length from alveolar crest to superior border of canal was measured in direction of stent(corrected ABH). The angle between uncorrected ABH and corrected ABH was measured. From each ABH, available fixture length was decided by $Br{{\aa}}nemark$ system. The results were following ; the difference between two ABHs was statistically significant in both first and second molar(p< 0.01). The percentage of difference more than 1 mm was 8.7% in first molar and 15.5% in second molar. The percentage of difference more than 2 mm was 2.0% in first molar and 6.6% in second molar. The maximum value of difference was 2.5 mm in first molar and 2.2 mm in second molar. The correlations between difference of 2 ABHs and angle was positive correlations in both first and second molar. The correlation coefficient was 0.534 in first molar and 0.728 in second molar. The second molar has a stronger positive correlation. The percentage of disagreement between 2 fixture lengths from two ABHs was 24.4% in first molar and 28.9% in second molar.
Kang Joon Kyu;Kim Hyung Tai;Park In Duk;Chung Young Mi;Lee Cheol Joo
Journal of Chest Surgery
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v.38
no.6
s.251
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pp.410-414
/
2005
There are several options for choosing a graft in CABG, we routinely chose LIMA for LAD and great saphenous vein for other target vessels. To evaluate the posoperative graft patency, we have studied the results using a 16 slices multi-detector computed tomography. Material and Method: From 1995 to 2003, 80 CABG patients who did not complain any event of MACE have been examined by 16-MDCT, mostly in an out patient clinic. Result: There were 61 men and 19 women. MDCT was used as early as 7 days to 9 years post-operatively with a median follow-up period of 6.5 years, and mean follow-up peiod of $31.5\pm25.4$ months. Mean age was $58.4\pm12.6$ years old in men and $61.5\pm17.2$ years old in women. 72180 patients received LIMA to LAD, and all other patients received vein grafts for bypass. The target vessel of vein grafts were 8 in LAD, 47 in RCA, 60 in diagonals, and 61 in obtuse marginals. Among them 42 sequential anastomoses were performed. The mean graft number was $3.1\pm1.8$ grafts. 5 year graft patency rate of each grafts was as followings; $93.1\%$ in LIMA to LAD, $94.9\%$ in vein to diagonals, $92.1\%$ in vein to obtuse marginals, and $79.2\%$ in vein to RCA. Sequential grafting showed better graft patency than the isolated grafting $(95.2\%\;vs\;78.7\~95.0\%)$. Conclusion: In this study, CABG with LIMA and saphenous veins showed satisfactory longterm results. 16-MDCT provided good images for follow-up study after CABG. Additionally, as radiologic tools (64-MDCT, MRI) improve more in the future, they can be used for diagnosing preoperative anatomical coronary disease as well as cardiac functions.
Background: It is very important to determine an accurate staging of the non-small cell lung cancer(NSCLC) for an assessment of operability and it's prognosis. However, it is difficult to evaluate tumor involvement of mediastinal lymph nodes accurately utilizing noninvasive imaging modalities. PET is one of the sensitive and specific imaging modality. Unfortunately PET is limited use because of prohibitive cost involved with it's operation. Recently hybrid SPECT/PET(single photon emission computed tomography/positron emission tomography) camera based PET imaging was introduced with relatively low cost. We evaluated the usefulness of coincidence detection(CoDe) PET in the detection of metastasis to the mediastinal lymph nodes in patients with NSCLC. Methods: Twenty one patients with NSCLC were evaluated by CT or MRI and they were considered operable. CoDe PET was performed in all 21 patients prior to surgery. Tomographic slices of axial, coronal and sagittal planes were visually analysed. At surgery, mediastinal lymph nodes were removed and histological diagnosis was performed. CoDe PET findings were correlated with histological findings. Results: Twenty of 21 primary tumor masses were detected by the CoDe PET. Thirteen of 21 patients was correctly diagnosed mediastinal lymph node metastasis by the CoDe PET. Pathological N0 was 14 cases and the specificity of N0 of CoDe PET was 64.3%. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of N1 node was 83.3%, 73.3%, 55.6%, 91.7%, and 76.2% respectively. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of N2 node was 60.0%, 87.5%, 60.0%,87.5%, and 90.0% respectively. There were 3 false negative cases but the size of the 3 nodes were less than 1cm. The size of true positive nodes were 1.1cm, 1.0cm, 0.5cm respectively. There were 1 false positive among the 12 lymph nodes which were larger than 1cm. False positive cases consisted of 1 tuberculosis case, 1 pneumoconiosis case and 1 anthracosis case. Conclusion: CoDe PET has relatively high negative predictive value in the enlarged lymph node in staging of mediastinal nodes in patients with NSCLC. Therefore CoDe PET is useful in ruling out metastasis of enlarged N3 nodes. However, further study is needed including more number of patients in the future.
Kim, Ja Hyung;Kim, You Jeong;Lee, Byeong Seon;Ko, Tae Sung;Park, Young Seo
Clinical and Experimental Pediatrics
/
v.45
no.2
/
pp.232-239
/
2002
Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with renal cystic diseases in the Department of Pediatrics, Asan Medical Center from October 1989 to June 2001. Results : In 95 patients, there were 55 cases(58.0%) with multicystic dysplastic kidney(MCDK), 19 cases(20.0%) with simple renal cysts, 13 cases(13.7%) with hereditary polycystic kidney diseases( 7 with autosomal recessive type, 5 with autosomal dominant type, 1 with undetermined), 6 cases(6.3%) with renal cysts in tuberous sclerosis and 1 case(1.0%) with medullary cystic disease. All MCDK patients had no renal dysfunction and hypertension during the follow-up period. Three out of 13 with polycystic kidney diseases had progressed to end-stage renal disease during the follow-up period. One case with a simple cyst underwent laparoscopic malsupialization for decompression. Conclusion : Renal cystic diseases have diverse clinicopathologic features and variable prognosis. We emphasize that routine follow-up should be performed to prevent and to detect early treatable complication in renal cystic diseases. Therefore, their natural history and treatment need further investigation and long term follow-up is required.
The Journal of the Korean bone and joint tumor society
/
v.16
no.1
/
pp.21-26
/
2010
Purpose: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). Materials and Methods: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and fi ve were female. The mean age was 34 years. The mean follow-up period was 54 months. Results: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. Conclusion: Comprehensive understanding of clinical, radiological and pathological features of secondary chondro sarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.
Purpose: To assess the effect of extracranial-intracranial (EC-IC) bypass surgery on hemodynamic improvement, we evaluated serial regional cerebral hemodynamic change of the middle cerebral artery (MCA) in symptomatic patients with atherosclerotic occlusion of the internal carotid artery (ICA) or MCA using $^{99m}Tc$-ECD acetazolamide stress brain perfusion SPECT (Acetazolamide SPECT). Materials and Methods: The patients who had suffered a recent stroke with atherosclerotic ICA or MCA occlusion underwent EC-IC bypass surgery and Acetazolamide SPECT at 1 week before and three to six months after surgery. For image analysis, attenuation corrected images were spatially normalized to SPECT templates with SPM2. Anatomical automated labeling was applied to calculate mean counts of each Volume-Of-Interest (VOI). Seven VOIs of bilateral frontal, parietal, temporal regions of the MCA territory and the ipsilateral cerebellum were defined. Using mean counts of 7 VOIs, cerebral perfusion index and perfusion reserve index were calculated. Results: Seventeen patients (M:F =12:5, mean age $53{\pm}2yr$) were finally included in the analysis. The cerebral blood flow of the parietal region increased at 1 week (p = 0.003) and decreased to the preoperative level at 3-6 months (p = 0.003). The cerebrovascular reserve of the frontal and parietal regions increased significantly at 1 week after surgery (p<0.01) and improved further at 3-6 months. Conclusion: Cerebrovascular reserve of the MCA territory was significantly improved at early postoperative period after EC-IC bypass and kept improved state during long-term follow-up, although cerebral blood flow did not significantly improved. Therefore, cerebrovascular reserve may be a good indicator of postoperative hemodynamic improvement resulted from bypass effect.
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