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*

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

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

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

Glomus tumors are mesenchymal neoplasms that are composed of modified smooth muscle cells of the normal glomus body. Most glomus tumors are benign and they occur in the distal extremities, and particularly the subungual lesions that occur in the hand, the wrist and the foot. We report here on a case of a solid type glomus tumor that had an uncertain malignant potential with a juxtacortical location at the distal tibia, and there were no neoplastic erosion of the cortical surface and no periosteal reaction. The tumor cells showed mild nuclear atypia and moderate mitotic activity (3-5/10HPF). Prominent intranuclear cytoplasmic pseudoinclusions were also observed. This case is interesting due to the very unusual tumor location of the juxtacortical area of the long bone, the atypical histologic features and the unique cytological finding of cytoplasmic intranuclear inclusions throughout the tumor cells.

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

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 role of the anaphase-promoting complex (APC) is to promote the degradation of mitotic cyclins and other substrates involved in sister chromatid adhesions. The APC appears to be responsible for the degradation of cyclin B and may have a potential role in the loss of control concerning cell proliferation in mammalian cells. However, a direct link between the defects in the APC components and oncogenesis has not been estabilished. This study investigates the relationship between APC expression and variable prognostic factors in invasive ductal carcinoma of the breast. METHODS: We evaluated 108 cases of invasive ductal carcinoma surgically resected from January, 1996 to May, 2000 at Wonju Christian Hospital, Wonju College of Medicine, Yonsei University. Immunohistochemical stains for APC, estrogen receptor, and Ki-67 were done in paraffin sections using the avidin-biotin complex method. The results were compared with clinical and pathologic parameters and flow cytometric DNA analysis factors. RESULTS: Forty cases (37.0%) showed immunopositive reactions for APC. The APC positivity in histologic grades 1, 2, and 3 were 28 cases (84.4%), 33 cases (60.0%), and 7 cases (35.0%), respectively (p=0.0011). The APC expressions in cases with the number of mitosis of less than 10, 10-19, and more than 20 per 10 high power fields, were noted in 37 cases (75.5%), 26 cases (63.4%), and 5 cases (27.8%), respectively (p=0.0016). The mean value of the Ki-67 labeling index was 221.7 in the APC-positive group and 317.9 in the APC-negative group (p= 0.0091). DNA flow cytometric analysis revealed higher APC expressions in cases with diploid patterns (p=0.0095). The APC expression rate increased significantly with decreasing histologic grade, with decreasing mitotic activity, in cases with a low Ki-67 labeling index, and those in the diploid group (p<0.05). The APC expression was not statistically correlated with clinical stage, tumor size, and estrogen receptor status. CONCLUSIONS: These findings suggest that positive APC expression may be considered as a good prognostic factor of invasive ductal carcinoma, and loss of APC expression may be related with the progression of breast cancer.
Breast Neoplasms ; Breast* ; Carcinogenesis ; Carcinoma, Ductal* ; Cell Proliferation ; Chromatids ; Coloring Agents ; Cyclin B ; Cyclins ; Diploidy ; DNA ; Estrogens ; Gangwon-do ; Humans ; Mitosis ; Paraffin ; Siblings