• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.1
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• pp.10-14
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• 2010

Vitamin D-resistant rickets(VDRR) is hereditary disease manifesting marked hypophosphatemia caused by renal tubular loss of phosphate into urine and an associated decrease in the calcium and phosphorous ion product. VDRR is identified by clinical symptoms, such as in the limbs, gait disturbance, dwarfism, familial occurrence, bowlegs, and knock-knees, as well as by laboratory findings. Dental findings are enlarged chambers and extension of the pulp horns into the cusp tips in both the primary and the permanent dentition in contrast to the vitamin D-deficient rickets. The major oral manifestations are multiple spontaneous abscesses in a caries-free dentition. We reported the clinical features and treatment of a 6-year-old boy with vitamin D-resistant rickets referred to the department of pediatric dentistry in Kyung Hee University for multiple spontaneous periapical abscesses and gingival fistula without severe dental caries.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.12
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• pp.6501-6504
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• 2012

Background: Multiple myeloma is the most common malignant plasma cell dyscrasia and ranks second among primary haematological malignancies. This study describes the epidemiologic, clinical and pathologic profile of monoclonal gammopathies seen in the University Hospital of the West Indies (UHWI), a tertiary care referral centre. Materials and Method: A retrospective analysis of 85 cases diagnosed at UHWI over the 5-year period 2003-2007 was conducted. The cases were identified from the bone marrow records as well as the computerized database of the Medical Records Department. Clinical presentation, family and personal history and demographic data were retrieved. Haematological and biochemical results were also analyzed. Results: There were 85 patients diagnosed with monoclonal gammopathies. The M:F ratio was 1.2:1 and the mean age was $65.7{\pm}1.3$ years. Eighty percent of the patients had skeletal pain and 40% experienced weight loss. Of the patients experiencing bone pain 56.7% had multiple lytic lesions, 26.7% had pathological fractures and 26.7% had compression fractures. Seventy-four patients (87.1%) had a haemoglobin level <12.0 g/dL with 52.9% having values <8.0 g/dL. Renal impairment was evident at diagnosis in 36.5%. Hypercalcemia was seen in 26.5% and hyperuricemia in 45.9%. Of the 79 patients who had serum protein electrophoresis performed, 77.2% had at least one monoclonal band and of these 24.6% had a monoclonal protein also present on urine protein electrophoresis. Conclusions: The demographic profile in this group of patients is largely similar to other studies in predominantly Caucasian populations; however there was a notable increase in prevalence of severe disease at presentation, with the majority of patients presenting at the most advanced stage. It is probable that these differences reflect socioeconomic factors and not merely inherent ethnic variation in disease biology.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.20
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• pp.8793-8796
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• 2014

Objective: To explore the influence of serum vascular endothelial growth factor (VEGF) level on therapeutic outcome and diagnosis/prognostic value in patients with cervical cancer. Materials and Methods: A total of 37 patients diagnosed with cervical cancer by biopsy were selected and treated with concurrent chemoradiotherapy. Double-antibody sandwich enzyme-linked immunosorbent assay (ELISA) was adopted before treatment to assess VEGF levels, and its relationships with clinicopathological features and short-term therapeutic effects were analyzed. Results: The median VEGF level in 37 patients before treatment was 647.15 (393.35~1125.16) pg/mL. Serum VEGF levels in patients aged <50 years, in International Federation of Gynecology and Obstetrics (FIGO) stage IIIa~IVa, with lymph node metastasis and tumor size >4 cm were significantly increased (P<0.05). The complete remission (CR) rate was 48.7% (18/37), partial remission (PR) rate was 35.1% (13/37), stable disease (SD) rate was 13.5% (5/37) and progressive disease (PD) rate was 2.70% (1/37), so the objective remission rate (ORR) after treatment was 83.8% (31/37). Logistic regression analysis showed that tumor size and serum VEGF level before treatment were independent risk factors affecting the therapeutic outcome, and the higher the level of serum VEGF, the worse the prognosis when tumor size>4 cm. Some 56.8% of patients manifested with myelosuppression, 37.8% with leucopenia, 24.3% with thrombocytopenia, 5.41% with diarrhea, 46.0% with nausea and vomiting, 21.6% with hair loss and 8.11% with hepatic and renal injury during the treatment. Conclusions: Serum VEGF level may reflect the degree of malignancy of cervical cancer and predict therapeutic effect, which is of great importance to cancer diagnosis and prognosis.

• Journal of Chest Surgery
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• v.16 no.4
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• pp.498-505
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• 1983

It has been over 20 years since successful operations of Cardiac valves at the Department of Thoracic and Cardiovascular surgery, college of medicine, Yonsei University. About six hundreds of patients with severely symptomatic valvular heart disease have had valve operations with complete loss or sharp decrease in their cardiac symptoms since 1956. As the number of cardiac patient increases, reoperation on valves assumes greater importance. To define the group of patients undergoing reoperations on valves and the factors influencing their survival, we have reviewed our experiences of the reoperation on valves at the Yonsei University, Severance Hospital. This is a report of 29 cases which was undergone secondary or more surgery for valvular heart disease from 1966 to 1983. The primary operations includes 159 cases of open heart surgery from 1966 to 1975 and 476 cases from 1976 to march, 1983. The secondary operations are classified into groups of secondary valvuloplasty or valvotomy [8 cases], prosthetic valve replacement following valvuloplasty or valvotomy [14 cases] and prosthetic valve rereplacement [2 case] for such as calcification, degeneration and perforation of the cusps and paravalvular leakage, of the bioprosthetic valves. The leading indication for reoperation of mitral valve was restenosis or stenoinsufficiency, The indications of aortic valve replacement was active bacterial endocarditis, medically uncontrollable prosthetic endocarditis or paravalvular leakage. Overall death rate of the reoperation was 17.4% [5 death among the 29 patients] and the leading causes of death were myocardial failure, arrhythmia, cerebral embolism, acute renal failure due to low output syndrome. And it was followed by sepsis associated with active prosthetic endocarditis. The death rate of reoperation was 4.3% in the elective cases except urgent cases and the death rate of overall cardiac valve except reoperation cases was 4.1% in the last two years. Although the general mortality of reoperation was high, both mortality rates were comparable except emergency cases due to urgent preoperative patient’s condition.

• Journal of Chest Surgery
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• v.29 no.6
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• pp.626-631
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• 1996

The forty patients with carcirLoma of the esophagus or cardia seen at National Medical Center between November 1983 and April 1994 underwent surgical exploration. The esophagogastrectomy was carried out in 29 of 40 patients, one case through right thoracotomy, the others through left thoracotomy. Two patients underwent colon bypass surgeries due to upper esophageal cancer Transhiatal esop agectomy was performed In one case. Feeding gastrostomy or feeding jejunostomy were performed in 8 patients due to the advanced stage or malnutrition. In this report, we evaluated the long-term results in the 28 patients who underwent esophagogastrectomy for palliation through the left thoracotomy. There were 25 men(89%) and 3 women(11 %), and the mean age was 58.65$\pm$7.15 years(range, 46 to 73 years). The most frequent preoperative symptoms included dysphagia (22), weight loss (15), chest pain (6), vomiting (1), and hoarsness (1). Twenty-three patients had sqamous cell cancers of mid-and lower esophagus and five adenocarcionomas of cardia. One patient died in the hospital within 30 days of the op- eration for a hospital mortality rate of ).7%, Cause of death was sepsis due to anastomotic leakage. There were five additional complications in five patients; acute respiratory distress syndrome (1), post-op- erative bleeding (1), diaphragmatic hernia (1), acute renal failure (1) and late raft stenosis (1). The one year, 1틴o years, and three years acturial survival rate were 75.6$\pm$9.5%, 43.2$\pm$ 11.6%, 21.6: 10.5$\circledcirc$ re- spectively. The average survival was 21.8 months. The data from this study suggest that esophagogastrectomy through the left thoracotomy can achieve resonable long-term palliation for carci- noma of the esophagus. The operation can be performed with a low operative mortality and few serious postoperative complications.

• The Korean Journal of Pain
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• v.10 no.2
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• pp.246-249
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• 1997

Hiccup is defined as an abrupt involuntary contraction of the diaphragm and intercostal muscles with sudden closure of the glottis 35 msec after onset. The term "intractable" is given to those hiccups with a duration ranging from 24 hours to more than 25 years. Short hiccup bouts are mostly associated with gastric distention or alcohol intake, resolved spontaneously or with simple remedies. In contrast, intractable hiccup is a rare but disabling condition which can induce depression, weight loss and sleep deprivation. Although the pathophysiologic mechanisms of hiccup are still poorly understood, wide variety of pathological conditions such as: brain tumor, abdominal tumor, myocardial infarction, renal failure, abdominal surgery etc., can cause intractable hiccup. A 58-year-old male who had suffered from hepatocellular cancer was consulted from medical department due to intractable hiccup. Initial treatment modalities with administrations of metoclopramide and chlorpromazine and nerve block including phrenic nerve block, cervical epidural block and glossopharyngeal nerve block were not effective. Administration of midazolam and baclofen however achieved desirable effect.

• Journal of Gastric Cancer
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• v.10 no.4
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• pp.241-246
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• 2010

Purpose: Laparoscopy-assisted gastrectomy (LAG) has become a technically feasible and safe procedure for early gastric cancer treatment. LAG is being increasingly performed in many centers; however, there have been few reports regarding LAG at low-volume centers. The aim of this study was to report our early experience with LAG in patients with gastric cancer at a low-volume center. Materials and Methods: The clinicopathologic data and surgical outcomes of 39 patients who underwent LAG for gastric cancer between April 2007 and March 2010 were retrospectively reviewed. Results: The mean age was 68.3 years. Thirty-one patients had medical co-morbidities. The mean patient ASA score was 2.0. Among the 39 patients, 4 patients underwent total gastrectomy and 35 patients underwent distal gastrectomy. The mean blood loss was 145.4 ml and the mean operative time was 259.4 minutes. The mean time-to-first flatus, first oral intake, and the postoperative hospital stay was 2.8, 3.1, and 9.3 days, respectively. The 30-day mortality rate was 0%. Postoperative complications developed in 9 patients, as follows: anastomotic leakage, 1; wound infection, 1; gastric stasis, 2; postoperative ileus, 1; pneumonia, 1; cerebral infarction, 1; chronic renal failure, 1; and postoperative psychosis, 1. Conclusions: LAG is technically feasible and can be performed safely at a low-volume center, but an experienced surgical team and careful patient selection are necessary. Furthermore, for early mastery of the learning curve for LAG, surgeons need education and training in addition to an accumulation of cases.

• Journal of Genetic Medicine
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• v.12 no.1
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• pp.44-48
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• 2015

Townes-Brocks syndrome (TBS) is a rare genetic disorder characterized by the classic triad of congenital anomalies of the anus, thumbs, and ears, with variable expressivity. Additionally, renal malformations, cardiac anomalies, and endocrine and eye abnormalities can accompany TBS, although less frequently. TBS is inherited in an autosomal dominant fashion; however, about 50% of patients have a family history of TBS and the remaining 50% have de novo mutations. SALL1, located on chromosome 16q12.1, is the only causative gene of TBS. SALL1 acts as a transcription factor and may play an important role in inducing the anomalies during embryogenesis. Clinical features of TBS overlap with those of other multiple anomaly syndromes, such as VACTERL syndrome, Baller-Gerold syndrome, Goldenhar syndrome, cat eye syndrome, and Holt-Oram syndrome. Consequently, there are some difficulties in differential diagnosis based on clinical manifestations. Herein, we report a Korean family with two generations of TBS that was diagnosed based on physical examination findings and medical history. Although the same mutation in SALL1 was identified in both the mother and the son, they displayed different clinical manifestations, suggesting a phenotypic diversity of TBS.

• Clinical and Experimental Pediatrics
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• v.62 no.11
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• pp.422-427
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• 2019

Background: Polyomavirus BK (BKV) infection is an important cause of graft loss in kidney transplant patients. Purpose: The purpose of this study was to evaluate clinical findings and risk factors for BKV in pediatric patients after kidney transplantation. Methods: This retrospective single-center study included 31 pediatric kidney transplant recipients from January 2002 to December 2017. Two patients received 2 transplantations during the study period, and each transplant was analyzed independently. Total number of cases is 33 cases with 31 patients. BKV infection was confirmed from blood samples via periodic quantitative polymerase chain reaction. Results: The mean age at kidney transplantation was 11.0±4.7 years, and the male-to-female ratio was 2.7:1. Three patients had a past medical history of high-dose chemotherapy and autologous stem-cell transplantation for solid tumors. Nine patients (27.3%) developed BKV infection. The median period from kidney transplantation to BKV detection in blood was 5.6 months. There was no statistically significant difference in estimated glomerular filtration rate between patients with and those without BKV infection. Among 9 patients with BKV viremia, 7 were treated by reducing their immunosuppressant dose, and BKV was cleared in 6 of these 7 patients. In the other 2 BKV-positive patients, viremia improved without immunosuppressant reduction. Conclusion: BKV infection is common in children with kidney transplantation and might not have affected short-term renal function in our patient sample due to early immunosuppressant reduction at the time of BKV detection.

• The Korean Journal of Physiology and Pharmacology
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• v.7 no.1
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• pp.39-45
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• 2003

Reactive oxygen species (ROS) have been suggested to be contributory factors in complications of diabetes mellitus. In the present study, we investigated the generation of superoxide, the lipid peroxide level measured as thiobarbituric acid reactive substances, the vasorelaxation of isolated thoracic aorta and the iNOS expression in kidney of streptozotocin induced diabetic rats. Sprague Dawley rats were divided into four groups: control, ascorbate (400 mg/kg rat weight daily in drinking water), diabetic (single dose of 50 mg of STZ/kg i.p.) and diabetic simultaneously fed with ascorbate for 12 wk. Rats in groups were studied at tri-weekly intervals (0 to 12 wk). Diabetic rats were evaluated periodically with changes of plasma glucose levels and body weight. The ascorbate supplimentation attenuated the development of hyperglycemia and weight loss induced by STZ injection in rats. In the present experimental condition, the ascorbate supplimentation had no significant effect on plasma glucose levels and changes in body weight of normal rate. The superoxide generation, formation of thiobarbituric acid reactive substance and iNOS expression in kidney were significantly increased in STZ-treated rats that were decreased by ascorbate supplimentation. The ascorbate supplimentation had no effect on vasorelaxation of isolated thoracic aorta. These results indicate that ascorbate supplimentation may exert an inhibitory effect on STZ-induced oxidative tissue damage through protection of pancreatic islet cells by scavanging reactive oxygen species. The ascorbate supplimentation may possibly attenuate the renal complication of diabetes mellitus.