Multicystic dysplastic kidney (MCDK) is a relatively common developmental anomaly in infants and children and has a good prognosis. In contrast, a malignant rhabdoid tumor of the kidney (MRTK) is one of the most lethal neoplasms of early life. However, the presentation of such a lethal tumor combined with multicystic dysplasia has not been reported to date. In this report, we describe a case of MRTK in a 5-yr-old girl who also had multicystic dysplasia. She was previously diagnosed with MCDK at birth due to a huge palpable mass on the right side of the abdomen. The right kidney was extensively replaced by numerous grossly dilated, variable-sized cysts. Microscopically, the tumor cells show a diffusely infiltrative growth pattern, which revealed large non-cohesive, round-to-polygonal tumor cells with vesicular nuclei. Some tumor cells had eccentric nuclei and large, round, eosinophilic cytoplasmic inclusions. There were metanephrons present, with the central ureteric bud and peripheral branches surrounded by condensing mesenchyma, immature glomeruli, and metaplastic cartilage in the adjacent parenchyma. To our knowledge, this is the first combined case of the two aforementioned diseases and this case may, in fact, suggest a new disease entity.
Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Kidney Neoplasms/*complications/*diagnosis ; Multicystic Dysplastic Kidney/*complications/*diagnosis ; Prognosis ; Rhabdoid Tumor/*complications/*diagnosis

No abstract available.
Bone Marrow* ; Chimerism* ; Hematologic Neoplasms* ; Recurrence*

Minerals play an important role in the body as essential nutrients. However, it is not easy to meet DRIs because food sources of minerals are limited. Recently, consumption of bottled natural water has been increasing in Korea due to water pollution and distrust of tap water. The present study was conducted to investigate mineral contents in bottled natural water and their intakes among Korean adults. We analyzed eight minerals in seven kinds of bottled natural water by ICP-spectrometry and conducted a survey on the intake status of water and bottled natural water with 400 Korean young adults. The mean contents of Ca, Mg, Fe, Zn, Cu, Mn, Se, and Mo in bottled natural water were 22.45+/-22.48 mg/L, 10.59+/-9.97 mg/L, 0.27+/-0.18 microgram/L, 2.06+/-1.48 microgram/L, 5.47+/-0.70 microgram/L, 1.43+/-0.37 microgram/L, 1.90+/-0.96 microgram/L, and 3.34+/-0.79 microgram/L, respectively. The mean age, height, weight, and BMI were 22.76 years, 174.94 cm, 68.64 kg, and 22.41 kg/m2 for males (n=150) and 21.25 years, 162.04 cm, 51.05 kg, and 19.46 kg/m2 for females (n=250), respectively. The respective daily intakes of total water and bottled natural water as water itself were 670.30 ml and 212.20 ml for males and 488.04 ml and 132.72 ml for females. The daily intakes of Ca, Mg, Fe, Zn, Cu, Mn, Se, and Mo from bottled natural water were 4.76 mg, 2.25 mg, 0.06 microgram, 0.44 microgram, 1.16 microgram, 0.30 microgram, 0.40 microgram, and 0.71 microgram for males and 2.98 microgram, 1.41 mg, 0.04 microgram, 0.27 microgram, 0.73 microgram, 0.19 microgram, 0.25 microgram, and 0.44 microgram for females, respectively. Overall, the contents of Ca, Mg, and Se in bottled natural water were relatively high and the daily intakes of these minerals were 0.4~1.0% of the DRIs.
Adult ; Drinking ; Female ; Humans ; Korea ; Male ; Minerals ; Water ; Water Pollution ; Young Adult

PURPOSE: The objective of this study was to evaluate the effect of microthreads on removal torque and bone-to-implant contact (BIC). METHODS: Twelve miniature pigs for each experiment, a total of 24 animals, were used. In the removal torque analysis, each animal received 2 types of implants in each tibia, which were treated with sandblasting and acid etching but with or without microthreads at the marginal portion. The animals were sacrificed after 4, 8, or 12 weeks of healing. Each subgroup consisted of 4 animals, and the tibias were extracted and removal torque was measured. In the BIC analysis, each animal received 3 types of implants. Two types of implants were used for the removal torque test and another type of implant served as the control. The BIC experiment was conducted in the mandible of the animals. The P1-M1 teeth were extracted, and after a 4-month healing period, 3 each of the 2 types of implants were placed, with one type on each side of the mandible, for a total of 6 implants per animal. The animals were sacrificed after a 2-, 4-, or 8-week healing period. Each subgroup consisted of 4 animals. The mandibles were extracted, specimens were processed, and BIC was analyzed. RESULTS: No significant difference in removal torque value or BIC was found between implants with and without microthreads. The removal torque value increased between 4 and 8 weeks of healing for both types of implants, but there was no significant difference between 8 and 12 weeks. The percentage of BIC increased between 2 and 4 weeks for all types of implants, but there was no significant difference between 4 and 8 weeks. CONCLUSIONS: The existence of microthreads was not a significant factor in mechanical and histological stability.
Animals ; Biomechanics ; Dental Implants ; Mandible ; Osseointegration ; Swine ; Tibia ; Tooth ; Torque

BACKGROUND: Emergence agitation after sevoflurane anesthesia in children can be prevented by midazolam. Alternative splicing of the GABAA receptor changes with age. Therefore, we hypothesized that alternative splicing of the gamma2 subunit affects the GABA current when applying sevoflurane and midazolam. METHODS: We performed the whole-cell patch clamp technique on human embryonic kidney 293 cells that were transfected with alpha1beta2gamma2L or alpha1beta2gamma2S. The concentration-response relations were recorded for midazolam and sevoflurane, and the co-application responses were measured at concentrations of 1.5 nM, 15 nM and 300 nM of midazolam and 0.5%, 2.0% and 4.0% of sevoflurane. Each GABA current was compared with that produced by 5 microM of GABA. RESULTS: The concentration-response relationships for midazolam and sevoflurane were dose-dependent without any differences between the alpha1beta2gamma2L and alpha1beta2gamma2S subtypes. 1.5 nM and 15 nM of midazolam did not significantly enhance the current after treatment with 0.5% sevoflurane for both subtypes. The current after treatment with 2.0% sevoflurane was enhanced by 1.5 nM midazolam for the alpha1beta2gamma2S subtype, but not for the alpha1beta2gamma2L subtype. In the case of 2.0% sevoflurane with 15 nM of midazolam, and 4.0% sevoflurane with 300 nM of midazolam, the GABA currents were significantly enhanced for both subtypes. CONCLUSIONS: These results show that the difference in the gamma2 subunit cannot explain the emergence agitation after sevoflurane anesthesia in children in vitro. This suggests that co-application of sevoflurane and midazolam enhances the GABA current according to the alternative splicing of the gamma2 subunit and the concentration of both drugs.
Alternative Splicing ; Anesthesia ; Child ; Dihydroergotamine ; gamma-Aminobutyric Acid ; Humans ; Kidney ; Methyl Ethers ; Midazolam

In the past, computer-aided design/computer-aided manufacturing (CAD/CAM) technology was the closed system that users had to use the components of only one manufacturer. At present, it has changed to the open system with the flexibility to select and use the components of various manufacturers' components according to their needs. Despite the development of dental materials and prostheses manufacturing methods, denture manufacturing has followed conventional manufacturing methods for nearly 100 years. However, studies on CAD/CAM fabricated denture have been recently carried out to overcome the disadvantages of conventional denture manufacturing. Some commercialized products using milling or 3D printing have already been applied clinically. This case report confirms the possibility of CAD/CAM dentures using 3D face scan and compared them to conventionally fabricated dentures.
Dental Materials ; Denture, Complete* ; Dentures ; Pliability ; Printing, Three-Dimensional ; Prostheses and Implants

BACKGROUND: The main source of carbohydrate in the Korean diet is rice, which is usually served in a rice bowl. This study investigated the impact of a meal plan using smaller rice bowls on dietary energy intake and macronutrient composition in overweight or obese patients with type 2 diabetes mellitus. METHODS: A total of 67 women with type 2 diabetes were enrolled in our study. We divided these participants into three groups: a normal-weight group (NW; body mass index [BMI] < 23 kg/m2; n = 17), an overweight group (OW; 23 < or = BMI < 25 kg/m2; n = 24) and an obese group (OB; BMI > or = 25 kg/m2; n = 26). Three-day dietary records were analyzed for total energy intake (TEI) and macronutrient composition both before enrollment and two weeks after patients received instruction in a dietary plan based on using a small (200 mL) rice bowl. RESULTS: After the intervention, TEI decreased in the OW and OB groups. Decreased carbohydrate (NW, -4 +/- 5%; OW, -4 +/- 5%; OB, -3 +/- 6%) and increased fat intakes were found in all three groups, which complies with Korean Diabetes Association recommendations. The protein proportion of TEI significantly increased only in the OW group. Body weight decreased both in the OW and OB groups. CONCLUSION: A short-term, small-rice-bowl-based meal plan was effective for body weight control and macronutrient balance in overweight or obese women in Korea with type 2 diabetes.
Body Mass Index ; Body Weight ; Diabetes Mellitus, Type 2 ; Diet, Diabetic ; Diet ; Diet Records ; Energy Intake ; Female ; Humans ; Korea ; Meals ; Overweight

PURPOSE: Williams syndrome (WS) is an autosomal dominant genetic disorder, caused by deletion at chromosome 7q11.23. Although WS is characterized by congenital heart disease (CHD), mental retardation, and distinctive facial dysmorphism, clinical features may vary according to a patient's age, making the diagnosis more difficult. We reviewed clinical, psychosocial, and behavioral developmental manifestations of WS patients according to age. METHODS: We retrospectively studied nine children with WS from March 2003 to February 2009 at NHIC Ilsan Hospital Developmental Disorder Clinic. Patients were divided into three groups by age: infancy, preschool age and school age. Patients' facial dysmorphism, motor, speech, and cognitive development and associated symptoms were analyzed. RESULTS: Prominent clinical features of the infants group were feeding difficulty, hypotonia, constipation, sleep disturbance, hernia with facial features which were not characteristic. The preschool age group showed developmental delays, had very friendly personalities, and hyperacusis. Their typical facial features included long philtrum, prominent lips, and teeth malocclusion. The school age group showed mental retardation, learning disorders, and typical facial dysmorphism, as well as short stature. Although overly sociable, they were not able to adapt to school life. Eight patients out of nine showed CHD. CONCLUSION: WS can be suspected when an infant primarily presents with hypotonia, feeding difficulty, failure to thrive with CHD, and occasional hypercalcemia. When a patient in early childhood presents with delayed development with good verbal language ability and has hyperacusis, WS must be suspected. When an older or adolescent child has unique learning disabilities and has facial dysmorphism, short stature, and a loquacious personality, WS should also be suspected.
Adolescent ; Child ; Constipation ; Failure to Thrive ; Heart Diseases ; Hernia ; Humans ; Hyperacusis ; Hypercalcemia ; Infant ; Intellectual Disability ; Language ; Learning Disorders ; Lip ; Malocclusion ; Muscle Hypotonia ; Retrospective Studies ; Tooth ; Williams Syndrome

Inflammatory myofibroblastic tumor, also known as inflammatory fibrosarcoma, has been frequently diagnosed as inflammatory pseudotumor. Although there are six cases reported as inflammatory pseudotumors or inflammatory myofibroblastic tumors in the lung, no cases of pulmonary inflammatory myofibroblastic tumor with features of inflammatory fibrosarcoma have been reported in Korea. We experienced a case of inflammatory myofibroblastic tumor (inflammatory fibrosarcoma)characterized by high cellularity, severe nuclear pleomorphism, necrosis, or increased mitoticcounts. A 31-year-old male patient with a solitary pulmonary nodule on the routine chest x-rayreceived a right lower lobectomy. The tumor was an ovoid solid mass with multifocal necrosis, showing diffuse irregular proliferation of spindle cells with high cellularity and focal nuclear pleomorphism, admixed with dense lymphoplasmacytic cells. Although spindle cells are focally immunoreactivefor smooth muscle actin, the ultrastructural examination failed to demonstrate smooth muscledifferentiation. In cases of inflammatory myofibroblastic tumor (inflammatory fibrosarcoma), a completeexcision and close follow-up without radical surgery, radiation, or chemotherapy are needed.
Actins ; Adult ; Drug Therapy ; Fibrosarcoma ; Follow-Up Studies ; Granuloma, Plasma Cell ; Humans ; Korea ; Lung* ; Male ; Muscle, Smooth ; Myofibroblasts* ; Necrosis ; Solitary Pulmonary Nodule ; Thorax

PURPOSE: The purpose of this study was to evaluate the role of immediate postoperative radiotherapy (RT) in adult patients with a low-grade oligodendroglioma (LODG). MATERIALS AND METHODS: A total of 74 patients, older than 15 years, were treated in our institution between April 1990 and March 2006 for newly diagnosed LODGs. After surgery, 43 patients were treated with immediate RT with a total dose of 54~55.8 Gy with 1.8 Gy fractions (RT group) and 31 patients were followed with no adjuvant RT (OP group). All patients were closely observed until tumor progression or death with frequent work-ups including neurological examinations and MRI. Primary endpoints were overall survival and progression-free survival. The median follow-up duration of survivors was 6.2 years in the RT group and 5.8 years in the OP group. RESULTS: Median progression-free survival was 13.2 years in the RT group and 4.6 years in the OP group; multivariate analysis confirmed improved outcome with the use of immediate RT (hazard ratio, 0.22; 95% confidence interval-CI, 0.09~0.55; p<0.001). Median overall survival was 14.9 years in the RT group and 9.8 years in the OP group; the use of adjuvant RT was also associated with a trend toward better overall survival after immediate RT based on multivariate analysis (hazard ratio, 0.3; 95% CI, 0.08~1.17; p=0.082). No severe RT related complications were observed. CONCLUSION: Immediate RT following surgery appears to be an effective treatment modality for supratentorial LODGs. However, the potential benefit of adjuvant RT for overall survival needs to be tested prospectively in the future.
Adult ; Brain Neoplasms ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Multivariate Analysis ; Neurologic Examination ; Oligodendroglioma ; Survivors