• Childhood Kidney Diseases
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• v.2 no.2
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• pp.161-168
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• 1998

Purpose : Lots of congenital anomalies of urinary tract including hydronephrosis are detected in fetus and neworn by popular use of prenatal ultrasonography. But there are little data available in Korea about natural course of hydronephrosis diagnosed antenatally by ultrasonography. So we intended to help management of these patients by analizing the follow up data of the neonates with hydronephrosis diagnosed antenatally. Methods : We evaluated 22 patients with neonatal hydronephrosis(33 renal units) who were diagnosed prenatally and confirmed postnatally. Especially patients with suspected ureteropelvic junction obstruction were followed regulary with renal ultrasonography and diuretic renography for 8-24 months. Results : 1) The etiologies of neonatal hydronephrosis diagnosed prenatally were suspected ureteropelvic junction obstruction($69.9\%$), vesicoureteral reflux($15.1\%$), primary megaureter($3.0\%$), double ureter with ureterocele($3.0\%$), ureteral stricture($3.0\%$), multicystic dysplastic kidney(3.0$\%$), and ureterovesical junction obstruction(3.0$\%$). 2) The follow up results of 23 renal units of suspected ureteropelvic junction obsruction: Except 4 renal units with palpable abdominal mass, of the remained 19 renal units, 14 units($73.6\%$) were improved spontaneously, 3 units($15.7\%$) remained stable, only2 units($10.5\%$) were aggravated. Conclusion : We concluded that in most cases of hydronephrosis there is no need for immediate surgery, and that nonoperative approach, using serial ultrasonography and diuretic renogram, is safe management of neonatal hydronephrosis diagnosed anteratally.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1236-1240
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• 2008

Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with behavioral and learning problems to a lethal disease characterized by multiple malformations. Here, we describe a newborn male with ambiguous genitalia who was diagnosed to have type II SLOS during the neonatal period. A clinical examination revealed low levels of unconjugated estriol in the maternal serum, and a variety of fetal ultrasound anomalies, including prenatal growth retardation. After birth, the infant was diagnosed to have congenital heart disease (Tetralogy of Fallot with severe pulmonary artery stenosis), cleft lip and palate, micrognathia, postaxial polydactyly, ambiguous genitalia, and cataracts. Clinical investigation revealed extremely low plasma cholesterol levels and the presence of mutation (homozygote of p.Arg352Gln) in the DHCR7 gene. The patient underwent palliative heart surgery (to widen the pulmonary artery) and received intravenous lipid supplementation. Cholesterol levels increased slightly, but not to normal values. The patient died from cardiopulmonary failure and sepsis 72 days after birth. This report provides the first description of a Korean patient with SLOS confirmed by verification of DHCR7 gene mutation and illustrates the need for early recognition and appropriate diagnosis of this disease.

• Radiation Oncology Journal
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• v.11 no.1
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• pp.59-67
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• 1993

Between December 1979 and September 1989, 23 patients with craniopharyngioma who underwent surgery and postoperative radiation therapy were retrospectively evaluated to assess the efficacy of this management at the Department of Therapeutic Radiology, Seoul National University Hospital. Total removal of tumor was attempted in all patients. Of these, surgeons tried total removal in eight patients, but revealed residual mass by postoperative CT, and partial removal was done in 15 patients. The morphology of tumor on the operative finding was grouped into three types: cystic 13 ($57{\%}$), solid 4 ($17{\%}$), and mixed 6 ($26{\%}$). Cystic type was predominant in$\leq$20 years old group. Actuarial overall survival rates at 5 and 10 years were $95{\%}$ and $81{\%}$ respectively and actuarial tumor control rates were $74{\%}$ and $50{\%}$. Surgical extent was not related to the survival rates (p=0.41). Pediatric and adolescent patients (age of$\leq$20 years) had a trend of better survival than that of adult patients (p=0.10). The results indicated that limited surgical excision followed by radiation therapy is recommended when total excision is not possible.

• Journal of Preventive Medicine and Public Health
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• v.26 no.3 s.43
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• pp.400-411
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• 1993

The aim of this study is to analyze the variations among hospitals and hospital groups in resource use and procedures of diagnostic and therapeutic process, such as laboratory tests, radiologic examinations, tissue diagnosis, timing of surgery after admission, the time required for operation. The study was performed for five procedures including cesarean section (C/S), appendectomy, cholecystectomy, cataract extraction, and pediatric pneumonia. The 2,316 subjects were selected from medical insurance claims list, and from this list 413 cases were sampled for medical record review. The patterns of resource utilization and process of treatment were described according to hospitals and characteristics of hospital groups. The major results were as follows : 1. The numbers of laboratory and radiologic tests showed significant difference among hospitals and hospital groups. In case of hospital groups, we could find tendencies of more tests with increasing hospital bed size. 2. In general, the proportion of operative cases evaluated by tissue diagnosis postoperatively among all operations ranged from 28.3% to 100%. The proportion varied among hospital groups, of which general hospital A group(more than 15 specialty) showed the highest proportion. 3. Post-admission delay until operation and the time required for operative procedure were not invariable among hospitals and hospital groups. The duration of operation in tertiary hospitals was slightly shorter than general hospitals, with varying statistical significance. We could find that probably there were differences of quality among hospitals in some components of procedures, which suggested that the implementation of quality assurance activities would be mandatory. In this study, we simply described the patterns of resource utilization and some features of clinical process, with institution of the need for advanced studies with in-depth analyses for each component of diagnosis and treatment procedures.

• Childhood Kidney Diseases
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• v.11 no.2
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• pp.294-298
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• 2007

Moyamoya disease is a progressive cerebrovascular disorder with stenosis or occlusion of the bilateral internal carotid arteries with abnormal vascular networks at the base of the brain. Previous reports have shown that there are extracranial vascular involvements in Moyamoya disease, especially in the renal artery. We report a 7-year-old patient with Moyamoya disease associated with renovascular hyper tension, who presented in infancy with seizures and hemiparesis. Renal angiography showed multiple stenoses of the right renal artery. Although renal artery stenosis in Moyamoya disease has been effectively treated with balloon angioplasty, stent implantation, or surgery, bat-loon angioplasty could not be done in this patient due to multiple stenoses. His blood pressure was successfully controlled with medical treatment, and remained normotensive during the follow up period of 6 months.

• The Korean Journal of Nuclear Medicine
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• v.33 no.1
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• pp.57-64
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• 1999

Purpose: The purpose of this study was to evaluate the accuracy of Tc-99m DTPA diuretic renal scans in children with dilated upper urinary tract. Materials and Methods: We reviewed diuretic renal scans of 14 pediatric patients (age range: 3 days to 4 years) with unilateral hydronephrosis diagnosed by ultrasonography. Diuretic renal scan was done using Tc-99m DTPA and standardized protocol. In 3 neonates, diuretic renal scans were performed within 1 week and 3-7 months after birth. Results: Six patients required Pyeloplasty and eight were managed conservatively. All 6 patients requiring Pyeloplasty were diagnosed as having ureteropelvic junction obstruction in the diuretic renal scan. In these 6 patients, post-operative renal scans at 3-12 months after surgery were converted to nonobstructive pattern in 5 and a nonfunctioning pattern in 1. In 3 patients who underwent diuretic renal scan within 1 week after birth, nonobstructive patterns of initial scan were converted to obstructive patterns in the follow-up scan. However, all patients with nonobstructive diuretic renal scans performed after the neonatal period did well on serial ultrasonography and showed favorable clinical outcome without progression to obstruction. Conclusion: Tc-99m DTPA diuretic renal scan with standardized protocol is useful in assessing suspected ureteropelvic junction obstruction in children as an initial diagnostic or post-operative follow-up modality. Nonobstructive or indeterminate scan results in the neonatal period requires follow-up scan to monitor development of the obstructive pattern.

• Journal of Oral Medicine and Pain
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• v.33 no.4
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• pp.317-322
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• 2008

The moderners of industrial society suffer from various stresses, which bring about increase in prevalence of temporomandibular disorders, oral mucosal disease and chronic neuropathic pain, therefore, the number of patients seeking help of those symptoms tend to increase. The purpose of this study was to discuss the importance of oral medicine related disease by investigating questions that appeared in cyber consultation of Kangnung National University Dental Hospital web site. Among the nearly 2000 questions, the rate of questions related to oral medicine was 20.92%, and the rate of questions related to other departments were oral and maxillofacial surgery 16.87%, conservative dentistry 16.67%, orthodontics 14.02%, prosthodontics 12.25%, periodontics 8.36%, pediatric dentistry 4.93%, preventive dentistry 2.08% and otherwise questions 3.90%. Among the 403 questions related to oral medicine, the frequent questions were oral mucosal diseases 44.17%, temporomandibular disorders 41.19%, halitosis 4.47%, xerostomia 3.23%, other orofacial pain 2.23%, forensic dentistry 1.49% and otherwise questions 2.98%. From the higher rate of questions related to oral medicine compared with other fields of dentistry, we would consider that the people are considerably concerned about the oral medicine related disease, such as oral mucosal diseases and temporomandibular disorders.

• Journal of Dental Anesthesia and Pain Medicine
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• v.15 no.2
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• pp.77-83
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• 2015

Background: To prepare for possible emergency situations during dental treatment, it is helpful to know how often and what kinds of emergencies may arise. This study set out to evaluate the incidences, causes, treatments, and outcomes of emergency situations in the outpatient clinic of a dental teaching hospital in Korea. Methods: We retrospectively reviewed the records of patients who had experienced an emergency situation and emergency response team activated in a selected outpatient clinic between November 2004 and November 2013. Specific information about the emergency cases was collected, including the patient characteristics and the frequency, types, treatments, and outcomes of the emergency situations. Results: We identified 35 instances of emergency situations in 2,890,424 patients (incidence = 0.012 per 10,000 outpatients). The number of cases was as follows: 10 (28.6%) in the Department of Periodontics, 10 (28.6%) in the Department of Oral and Maxillofacial Surgery, 6 (17.1%) in the Department of Oral and Maxillofacial Radiology, 4 (11.4%) in the Department of Prosthodontics, 2 (5.7%) in the Department of Conservative Dentistry, 2 (5.7%) in the Department of Pediatric Dentistry, and 1 (2.9%) in the Department of Orthodontics. Three (8.6%) of the emergency situations arose before treatment, 22 (62.9%) during treatment, 7 (20.0%) after treatment, and 2 (5.7%) in a patient's guardian. Conclusions: In accordance with the growing elderly population and more aggressive dental procedures, the number of emergency situations may increase in the future. We recommend that clinicians keep in mind airway management and the active control of emergency situations.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1014-1031
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• 2022

The Kasai portoenterostomy is the first-line treatment for the restoration of the flow of bile to the small intestine in patients with biliary atresia. Various complications can occur after Kasai portoenterostomy, including ascending cholangitis, biliary cirrhosis, and portal hypertension. Of these potential complications, ascending cholangitis in the most common. In cases of patients having uncontrolled complications due to progressive liver cirrhosis, portal hypertension, or progressive hyperbilirubinemia, liver transplantation is the indicated as treatment plan. Lifelong follow-up, particularly involving imaging studies, is important for the identification of various complications arising from biliary atresia after Kasai portoenterostomy. Additionally, imaging studies play a crucial role in the evaluation of potential liver donors and recipients. US is a key imaging modality utilized in the management of patients who undergo Kasai portoenterostomy, while CT and MRI are imperative to obtaining an accurate diagnosis.

• Journal of the Korean Society of Radiology
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• v.84 no.5
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• pp.1158-1162
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• 2023

Ortner's or cardiovocal syndrome is hoarseness attributable to left recurrent laryngeal nerve (RLN) palsy associated with mechanical compression of the nerve by pathologically enlarged cardiovascular structures. Ortner's syndrome is a rare condition, and to our knowledge, only a few cases have been reported in Korea. Furthermore, this condition is extremely uncommon in pediatric patients with thyrotoxicosis-related RLN paralysis. We report a case of reversible Ortner's syndrome in an adolescent who presented with secondary pulmonary hypertension related to thyrotoxicosis.