• The Korean Journal of Nuclear Medicine
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• v.38 no.6
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• pp.522-527
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• 2004

Purpose: The purpose of this study was to evaluate the usefulness of single photon emission computed tomography (SPECT) of bone using Tc-99m MDP in the diagnosis of osteoporotic vertebral fractures. Materials and Methods: Thirty two patients with osteoporotic vertebral fracture were included in this study (mean age: $67{\pm}8$, male: 5, female: 27). Seventy nine vertebral fractures were detected (38 thoracic/thoracolumbar lesions and 41 lumbar lesions), which were classified by type of deformity (wedge, biconcave or compression). The patterns and locations of increased uptakes were examined and analyzed. Results: Forty seven wedge fractures, 20 biconcave fractures and 12 compression fractures were found. Diffuse and asymmetric uptakes were common in fractured bodies. More than one uptake were examined in 69 posterior elements of fractured vertebrae (87.3%) including 40 of 47 wedge fractures (85.1%), 17 of 20 biconcave fractures (85.0%) and 12 of 12 compression fractures (100%). Wedge fractures were predominant fracture in thoracic/thoracolumbar spine whereas incidence of biconcave or compression type war similar to that of wedge fracture in lumbar spine (p=0.04). Spinous process uptake was more frequently seen in lumbar lesions than thoracic/thoracolumbar lesions (p=0.009). Facet joint uptake in biconcave fracture was more common in lumbar spine (92.3%) than thoracic/thoracolumbar spine (57.1%). Spinous process uptake in biconcave fracture was also more frequently detected in lumbar spine (p=0.043). Conclusion: Bone SPECT was useful in the evaluation of osteoporotic vertebral fracture, especially posterior elements of vertebrae.

• Tuberculosis and Respiratory Diseases
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• v.58 no.6
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• pp.570-575
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• 2005

Introduction : Diffuse interstitial lung diseases (DILD) comprise of a large group of lung diseases with diverse etiologies. They are classified into four categories based on the etiology and pathological findings. In Korea, epidemiological data on DILD has never been reported in a prospective manner. Method : From May 2002 to April 2004, total 487 patients with DILD were prospectively registered at Samsung Medical Center. The prospective observational analysis of the etiologies, its classification based on 2002 ATS/ERS (American Thoracic Society/European Respiratory Society) guidelines, as well as diagnostic tests and the retrospective analysis of the treatment modalities were carried out. Any infectious and malignant causes were excluded. Results : 1) The patients were classified into idiopathic interstitial pneumonia (IIP) in 269 patients (55.2%), known causes of DILD in 168 patients (34.5%), sarcoidosis in 27 patients (5.5%), other forms of DILD in 14 patients (2.9%), and undetermined DILD in 9 patients (1.9%). 2) The diagnostic test showed that most patients had undergone chest high resolution computed tomography (HRCT) and pulmonary function test (PFT) (97%, 89%). Transbronchial lung biopsy (TBLB) and surgical lung biopsy (SLB) were performed in limited patients (38%, 29%). 3) Among 269 patients with IIP, 220 (82%) had idiopathic pulmonary fibrosis (IPF) while 23 (9%) had nonspecific interstitial pneumonia. SLB was carried out in 36% of patients with IIP. 4) Symptomatic supportive care was given to 67% of IPF, but specific medical treatment including corticosteroids was administered to 89% of non-IPF patients. Conclusion : A nationwide registry of DILD patients is required to determine the annual incidence, etiology, and practice pattern of diagnosis and treatment in Korea.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.790-798
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• 1999

Background: As the early outcome after coronary artery bypass grafting(CABG) has been stabilized, neurologic complication has now become one of the most important morbidity. The aim of this study was to find out the risk factors associated with the neurologic complications after CABG. Material and Method: In 351 patients who underwent CABG, the incidence and features of neurologic complications, with associated perioperative risk factors, were retrospectively reviewed. Neurologic complication was defined as a new cerebral infarction confirmed by postoperative neurologic examination and radiologic studies, or delayed recovery of consciousness and orientation for more than 24 hours after the operation. Result: Neurologic complications occurred in 18 patients(5.1%), of these nine(2.6%) were diagnosed as having new cerebral infarctions(stroke). Stroke was manifested as motor paralysis in four patients, mental retardation or orientation abnormality in four, and brain death in one. Statistical analysis revealed the following variables as significant risk factors for neurologic complications by both univariate and multivariate analyses: cardiopulmonary bypass longer than 180 minutes, atheroma of the ascending aorta, carotid artery stenosis detected by Duplex sonography, and past history of cerebrovascular accident or transient ischemic attack. Age over 65 years, aortic calcification detected by simple X-ray, and intraoperative myocardial infarction were significant risk factors by univariate analysis only. Neither the severity of carotid artery stenosis nor technical modifications such as cannulation of the aortic arch or single clamp technique, which were expected to affect the inciden e of neurologic complications, had significant relationship with the incidence. Conclusion: This study confirmed the strong association between neurologic complications after CABG and atherosclerosis of the arterial system. Therefore, to minimize the incidence of neurologic complications, systematic evaluation focused on atherosclerotic lesions of the arterial system followed by adequate alteration of operative strategy is needed.

• Journal of Chest Surgery
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• v.40 no.6 s.275
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• pp.414-419
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• 2007

Background: Replacing the ascending aorta is a standard surgical option for treating acute type A aortic dissection. But replacing the aortic arch has recently been reported as an acceptable procedure for this disease. We compared the effects of aortic arch replacement for treating acute type A aortic dissection with the effects of ascending aortic replacement. Material and Method: From 2002 to 2006, 25 patients undewent surgical treatment for acute type A aortic dissection, 12 patients undewent ascending aortic replacement and 13 patients underwent aortic arch replacement. Among the aortic arch group, an additional distal stent-graft was inserted during the operation in 5 patients. 19 patients (11 arch replaced patients and 8 ascending aortic replaced patients) were followed up at the out patient clinic for an average of $756{\pm}373$ days. All the patients undewent CT scanning and we analyzed their distal aortic segments. Result: 4 patients who underwent ascending aortic replacement died, so the overall mortality rate was 16%. Among the 11 long term followed-up arch replacement patients, 2 patients (18.1 %) developed distal aortic dilatation and one of them underwent thoracoabdominal aortic replacement later on. However, among the 8 the ascending aortic replaced patients, 5 patients (62.5%) developed distal aortic dilatation. Conclusion: Aortic arch replacement is one of the safe options for treating acute type A aortic dissection. Aortic arch replacement for treating acute type A aortic dissection could contribute to a reduced distal aortic dilatation rate and fewer secondary aortic procedures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.158-165
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• 2002

Purpose: We designed this retrospective study to establish the incidence of diagnosic delay in children diagnosed with acute appendicitis and to identify associated factors with delayed diagnosis and its impact on the clinical course. Methods: All cases of children under 15 years of age who underwent appendectomy from 1996 to 2001 at Gangneung Asan Hospital were reviewed. We reviewed signs and symptoms, type of health professional first contacted, the advice given by the health professional and a history of appendicitis in first degree relatives. Diagnostic period is the time elapsed between first complaints and definitive diagnosis. Delay was defined as diagnostic period exceeded the 48 hours. Postoperative course and complications were also reviewed. Results: Incidence of diagnostic delay differed by whether diarrhea and fecalith on X-ray were present. Also children whose parents were advised to observe them at home were more likely to have a diagnostic delay. In almost half of the cases in delayed group, initial diagnosis was not acute appendicitis but gastroenteritis. The perforation rate in non-delayed group was 22%, whereas 87% in delayed group. The delayed group showed a higher number of postoperative complication and a longer hospitalization period. Conclusions: Diarrhea with abdominal pain and fever in children should not be dismissed as gastroenteritis, respiratory infections or other common disorders. Our study suggests that physicians have a responsibility to prevent diagnostic delay and resultant perforation of acute appendicitis in children by having a high index of suspicion about acute appendicitis.

• Journal of Life Science
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• v.17 no.12
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• pp.1766-1770
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• 2007

We describe here a case of malignant mixed osteogenic tumor of the mammary gland with alveolar carcinomatous appreance. A firm, 2 to 2.5cm (in diameter) mass under the 5th nipple, showing the structure of extraosseous osteogenic sarcoma, was removed from the left 5th mammary gland of 12-year-old female dog. When investigated under the microscope, the osteoid material undergoing mineralization was surrounded by numerous scattered osteoblasts and a few osteoclastic cells throughout the osteoid tumorous stroma. The osteoid lesions were continuous with hypercellular myoepithelial cells of a very immature character with several mitotic figures. In addition, there were also carcinomatous tubules and alveoli, with invading cells into peripheral stroma, surrounded by myoepithelial cells in the mammary gland. In these lesions, emanating cords of tumor cells appear to be continuous with the myoepithelial cell layer of a duct. The presence of all these cell types suggests the existence of a common malignant origin, the stem cell being differentiated into epithelial carcinomatous and mesenchymal sarcomatous chondral and osteogenic tissues.

• Journal of Korean Neurosurgical Society
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• v.29 no.3
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• pp.379-388
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• 2000

Objective : Although transoral transpharyngeal approach is a very useful method for the lesions of craniovertebral junction, it is not frequently used because of anatomical unfamilarity, risk of cerebrospinal fluid(CSF) leakage, and resultant postoperative meningitis. To evaluate the usefulness of transoral transpharyngeal approach for various lesions of craniovertebral junction, clinical characteristics and the results of this approach are investigated. Methods : Transoral transpharyngeal approaches were performed in eight cases between 1996 and 1999. Among them, there were three basilar invaginations due to congenital anomalies, two odontoid type I fractures, two atlantoaxial dislocations, and one pseudotumor. Surgical methods included five cases of anterior decompression and posterior fusion, two anterior approaches for decompression and one transoral approach for biopsy. Results : This procedure allowed immediate clinical improvement in all cases. In seven patients with preoperative motor deficit showed a progressive neurological improvement. The follow-up plain x-rays demonstrated successful bony fusion in all patients. Only one patient suffered from postoperative wound dehiscence, but she completely recovered after wound revision. There was no complication of postoperative CSF leakages. Conclusions : Transoral transpharyngeal approach for the ventral lesions of craniovertebral junction, can be used as a relatively simple and effective method.

• Journal of the Korean Arthroscopy Society
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• v.14 no.3
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• pp.180-187
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• 2010

Purpose: Rotator cuff tears involving the subscapularis are less common than those involving the superior and posterior rotator cuff. The purpose of the present study was to report the clinical results of repair of isolated traumatic tears of the subscapularis tendon. Materials and Methods: Fifteen patients (13 males, 2 females; mean age 46.2 years; range 35 to 52) with unilateral ruptures of the subscapularis tendon after trauma who underwent arthroscopic repair between February 2003 and October 2008 were reviewed retrospectively. All the cases were isolated tears of the subscapularis without the involvement of any other rotator cuff tendon and were followed for at least two years (mean 28 months). The entire subscapularis was involved in 9 cases and the tear was localized to the upper two thirds in 6 cases. The preoperative and postoperative status of patients with isolated subscapularis tears were analyzed using the Constant Score, American Shoulder and Elbow Society Index (ASES Index) and postoperative integrity was determined through magnetic resonance imaging. Results: The average clinical outcome scores and strength were all improved significantly at the time of the final follow-up. The constant shoulder score improved from 41.5 to 81.3 points (P<0.05) compared to before surgery and ASES index improved from 46.4 to 89.6 points (P<0.05) postoperatively. Thirteen patients (87%) were satisfied with the result of the treatment. The total tears were significantly more improved by surgery than the partial tears. In 12 of 15 patients (80%) were judged to reveal healed tendon on magnetic resonance imaging at a mean of 13 months postoperatively. The postoperative score was significantly lower for the patients with a failed repair than it was for those with an intact repair (P<0.05). Conclusion: Repair of traumatic isolated subscapularis tears through arthroscopic techniques effectively restores patient function with regard to pain, mobility, strength and postoperative tendon integrity. The postoperative integrity of the repair correlates with the functional results and the total tears were more improved by surgery than the partial tears, but future studies may be needed.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.62-67
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• 2018

Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.15-21
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• 2021

Purpose: Urea cycle disorder (UCD) is an inherited inborn error of metabolism, acting on each step of urea cycle that cause various phenotypes. The purpose of the study was to investigate the long-term clinical consequences in different groups of UCD to characterize it. Methods: Twenty-two patients with UCD genetically confirmed were enrolled at Pusan National University Children's hospital and reviewed clinical features, biochemical and genetic features retrospectively. Results: UCD diagnosed in the present study included ornithine transcarbamylase deficiency (OTCD) (n=10, 45.5%), argininosuccinate synthase 1 deficiency (ASSD) (n=6, 27.3%), carbamoyl-phosphate synthetase 1 deficiency (CPS1D) (n=3, 13.6%), hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHHS) (n=2, 9.1%), and arginase-1 deficiency (ARG1D) (n=1, 4.5%). The age at the diagnosis was 32.7±66.2 months old (range 0.1 to 228.0 months). Eight (36.4%) patients with UCD displayed short stature. Neurologic sequelae were observed in eleven (50%) patients with UCD. Molecular analysis identified 37 different mutation types (14 missense, 6 nonsense, 6 deletion, 6 splicing, 3 delins, 1 insertion, and 1 duplication) including 14 novel variants. Progressive growth impairment and poor neurological outcomes were associated with plasma isoleucine and leucine concentrations, respectively. Conclusion: Although combinations of treatments such as nutritional restriction of proteins and use of alternative pathways for discarding excessive nitrogen are extensively employed, the prognosis of UCD remains unsatisfactory. Prospective clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth or neurological outcomes and decrease metabolic crisis episodes in patients with UCD.