BACKGROUND: The purpose of this study was to evaluated the usefulness of diffusion-weighted magnatic resonance imaging for monitoring the response to radiation therapy in metastatic bone marrow of the spines. MATERIALS AND METHOD: Twenty-one patients with metastatic bone marrow of the spine were examined with MRI. Diffusion-weighted and spin-echo MRI were performed in 10 patients before and after radiation therapy with or without systematic chemotherapy, and performed in 11 patiemts after radiation therapy alone. Follow up spin-echo and diffusion-weighted MRI were obtained at 1 to 6 months after radiation therapy according to patients' condition. The diffusion-weighted imaging sequence was based on reversed fast imaging with steady-state precession(PSIF). Signal intensity changes of the metastatic bone marrows before and after radiation therapy on conventional spin-echo sequence MRI and diffusion-weighted MRI were evaluated. Bone marrow contrast ratios and signal-to-noise ratio before and after radiation therapy of diffusion-weighted MRI were analyzed. RESULTS: All metastatic bone marrow of the spinal bodies were hyperintense to normal bone marrow of the spinal bodies on pretreatment diffusion-weighted MRI and positive bone marrow contrast ratio(p<0.001). and hypointense to normal spinal bodies on posttreatment diffusion-weighted MRI and negative bone marrow contrast ratio(p<0.001). The signal to noise ratio after treatment decreased comparing with those of pretreatment. Decreased signal intensity of the metastatic bone marrows on diffusion-weighted MRI began to be observed at average more than one month after the initiation of the radiation therapy. CONCLUSION: tThese results suggest that diffusion-weighted MRI would be an excellent method for monitoring the response to therapy of metastatic bone marrow of the spinal bodies. However, must be investigated in a larger series of patients with longer follow up period.
Bone Marrow ; Drug Therapy ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging* ; Signal-To-Noise Ratio ; Spine*

Cantú syndrome (CS, OMIM 239850) is a very rare autosomal dominantly inherited genetic disease characterized by congenital hypertrichosis, neonatal macrosomia, a distinct facial features such as macrocephaly, and cardiac defects. Since the first description by Cantú et al. in 1982, about 50 cases have been reported to date. Recently, two causative genes for CS has been found by using exome sequencing analyses: ABCC9 and KCNJ8 . Most cases of clinically diagnosed CS have resulted from de novo mutations in ABCC9. In this study, we report three independent Korean children with CS resulting from de novo ABCC9 mutations. Our patients had common clinical findings such as congenital hypertrichosis, distinctive facial features. One of them showed severe pulmonary hypertension and hypertrophic cardiomyopathy, which require medical treatment. And, two patients had a history of patent ductus arteriosus. Although two of our patients had shown early motor developmental delay, it was gradually improved during follow-up periods. Although CS is quite rare, there are the concerns about development of various cardiac problems in the lifetime. Therefore, an accurate diagnosis followed by appropriate management and genetic counseling should be provided to CS patients.
Cardiomyopathy, Hypertrophic ; Child ; Databases, Genetic ; Diagnosis ; Ductus Arteriosus, Patent ; Exome ; Follow-Up Studies ; Genetic Counseling ; Humans ; Hypertension, Pulmonary ; Hypertrichosis ; Megalencephaly

OBJECTIVE: The purpose of this study was to make a guideline of uterine artery embolization for the treatment of uterine leiomyomas accompanying with adenomyosis in Korea. MATERIALS AND METHODS: We performed the retrospective study for 37 women who had uterine leiomyomas accompanying with adenomyosis. Bilateral uterine artery embolization was performed in 37 patients (age range 25-65) during 17 months with pain, hypermenorrhea, urinary frequency etc due to leiomyomas. Ultrasound imaging was performed before the procedure and at mean 6.9 months after the procedure. RESULTS: All procedures were technically successful. Mean clinical follow-up was 12.8 months. Minor complication occurred in 82% patients after the procedure. After imaging follow-up (mean, 6.9 months postprocedure), median uterine volume decreased 34.4%, and dominant myoma volume decreased 86%. There was no statistical difference in uterine volume reduction and dominant myoma size reduction whether occluding agents was polyvinyl alcohol, polyvinyl alcohol plus gelfoam, and gelfoam, and whether ultrasound measured Resistance Index value before the procedure was low or high. CONCLUSION: Primary candidates for uterine artery embolization include those with symptomatic uterine leiomyomas who no longer desire fertility but wish to avoid surgery or are poor surgical risks. To our study, uterine volume reduction and dominant myoma size reduction in patients who had adenomyosis were similar to previous other studies in patients who had not adenomyosis. Therefore adenomyosis should not be considered as a contraindication for uterine artery embolization. Because there is little data about subsequent reproductive potential after this procedure, it should not be routinely advocated for infertile women. Further investigation is warranted for occluding agents and Resistance Index.
Adenomyosis* ; Female ; Fertility ; Follow-Up Studies ; Gelatin Sponge, Absorbable ; Humans ; Korea ; Leiomyoma* ; Menorrhagia ; Myoma ; Polyvinyl Alcohol ; Retrospective Studies ; Ultrasonography ; Uterine Artery Embolization

PURPOSE: Metabolic syndrome (MetS) is a major public health problem related to increased risks of cardiovascular disease and type 2 diabetes. We investigated whether recommended levels of leisure time physical activity (LTPA) decrease the risk of MetS in a community-based prospective cohort study (Ansung and Ansan cohort).MATERIALS AND METHODS: A total of 3910 adults (1890 men, 2020 women) without MetS examined in 2001–2002 (baseline) were included in this study and followed up from 2013 to 2014. We measured LTPA energy expenditure using the standard metabolic equivalent value [metabolic equivalent of task (MET)-h/wk]. Individuals were categorized into five groups (physically inactive, 0.1 to <7.5, 7.5 to <15.0, 15.0 to <22.5, 22.5 to <40.0, and ≥40.0 MET-h/wk).RESULTS: The mean follow-up period was 11.8 years. Among the cohort, 482 men (25.5%) and 541 women (26.8%) were diagnosed with newly developed MetS. Multivariate logistic analysis revealed a 4.4% lower MetS risk among those performing two to three times the recommended minimum of 7.5 MET-h/wk [multivariate relative risk (RR), 0.956; 95% confidence interval (CI), 0.654–1.398] and a 21.9% lower risk among those performing three to four times the physical activity minimum (multivariate RR, 0.781; 95% CI, 0.574–1.063). This association was only significant in females performing three to four times the minimum (crude RR, 0.655; 95% CI, 0.432–0.994).CONCLUSION: We noted that physical activity at more than 2 times the recommended minimum prevents MetS. Healthcare professionals should encourage inactive adults to be physically active during their leisure time to take advantage of its health benefits.
Adult ; Cardiovascular Diseases ; Cohort Studies ; Delivery of Health Care ; Energy Metabolism ; Female ; Follow-Up Studies ; Gyeonggi-do ; Humans ; Insurance Benefits ; Korea ; Leisure Activities ; Male ; Metabolic Equivalent ; Motor Activity ; Prospective Studies ; Public Health

Background: G protein-coupled receptor 40 (GPR40) is a key molecule in diabetes and fatty liver, but its role in endothelial dysfunction remains unclear. Our objective in this study was to determine whether GPR40 agonists protect endothelial cells against palmitatemediated oxidative stress. Methods: Human umbilical vein endothelial cells (HUVECs) were used to investigate effects of various GPR40 agonists on vascular endothelium. Results: In HUVECs, AM1638, a GPR40-full agonist, enhanced nuclear factor erythroid 2–related factor 2 (NRF2) translocation to the nucleus and heme oxygenase-1 (HO-1) expression, which blocked palmitate-induced superoxide production. Those antioxidant effects were not detected after treatment with LY2922470 or TAK875, GPR40-partial agonists, suggesting that GPR40 regulates reactive oxygen species (ROS) removal in a ligand-dependent manner. We also found that palmitate-induced CCAAT/enhancer‐binding protein homologous protein expression; X-box binding protein-1 splicing, nuclear condensation, and fragmentation; and caspase-3 cleavage were all blocked in an NRF2-dependent manner after AM1638 treatment. Both LY2922470 and TAK875 also improved cell viability independent of the NRF2/ROS pathway by reducing palmitate-mediated endoplasmic reticulum stress and nuclear damage. GPR40 agonists thus have beneficial effects against palmitate in HUVECs. In particular, AM1638 reduced palmitate-induced superoxide production and cytotoxicity in an NRF2/HO-1 dependent manner. Conclusion GPR40 could be developed as a good therapeutic target to prevent or treat cardiovascular diseases such as atherosclerosis.

Minimally invasive surgery is being widely accepted in various fields of surgery. Although several appendectomy techniques have been reported but, there is no standardization. We report here the experiences of transumbilical endoscopic appendectomy in humans. Between July 2008 and September 2010, ten patients with appendicitis successfully underwent transumbilical endoscopic appendectomies. There were 7 cases of suppurative, 2 cases of gangrenous and 1 case of perforated in operative findings. The ages of the patients were 13-56 years (mean age, 32.7 +/- 15.4 years). Under general anesthesia, a 15-mm port was inserted through the umbilicus and then a two-channel endoscope was inserted in the peritoneal cavity. After appendix identification, counter-traction of the appendix with a direct abdominal wall puncture using a straight round needle prolene was performed to achieve good visualization of the operative field. Tissue dissection was performed using an endoscopic needle knife. Tissue grasping and resected appendix retrieval were done with endoscopic forceps. The average operation time was 79.5 +/- 23.6 minutes (range, 45 to 110 minutes). No procedures were converted to laparoscopic or open appendectomy. Hospital stay was 4-6 days. All patients completely recovered without complications. As it is highly maneuverable, we believe transumbilical endoscopic appendectomy can be a feasible method. And, as surgeons want to proceed from laparoscopic surgery to natural orifice transluminal endoscopic surgery, this procedure could be a triable method.
Abdominal Wall ; Anesthesia, General ; Appendectomy* ; Appendicitis ; Appendix ; Endoscopes ; Endoscopy ; Hand Strength ; Humans ; Laparoscopy ; Length of Stay ; Natural Orifice Endoscopic Surgery ; Needles ; Peritoneal Cavity ; Polypropylenes ; Punctures ; Surgical Instruments ; Surgical Procedures, Minimally Invasive ; Umbilicus

Degenerative pannus arising from the atlanto-axial articulation is a rare entity, which is associated with chronic atlanto-axial instability in degenerative arthropathies. Due to cervical instability and the mass effect on the spinal cord, it can produce severe neck pain and compressive myelopathy. We report a case of cervical myelopathy resulting from non-rheumatoid atlanto-axial subluxation. The posterior stabilization provided excellent neurological improvement and pannus regression. Early recognition of this condition is important as the clinical condition will deteriorate without surgical interventions.
Neck Pain ; Osteoarthritis ; Spinal Cord ; Spinal Cord Compression ; Spinal Cord Diseases

OBJECTIVE: The purpose of this study was to establish guidelines for using uterine artery embolization for the treatment of uterine leiomyomas accompanied by adenomyosis in Korea. This study was performed to investigate 1) the effects of uterine embolization on leiomyoma accompanied by adenomyosis, 2) the outcome of uterine embolization according to the embolization materials used, 3) the relationship between the Resistance Index (RI) and the change in uterine volume. MATERIALS AND METHODS: We performed a retrospective study on 37 women who had uterine leiomyomas accompanied by adenomyosis. Bilateral uterine artery embolization was performed over a period of 17 months in 37 patients (age range 25 - 65) experiencing pain, hypermenorrhea, urinary frequency, etc. due to leiomyomas. Ultrasound imaging was performed before the procedure and at mean 6.9 months after the procedure. RESULTS: All procedures were technically successful. Mean clinical follow-up time was 12.8 months. Minor complications occurred in 82% patients following the procedure. After follow-up imaging, the median uterine volume decreased by 34.4% and the dominant myoma volume decreased by 86%. There was no statistical difference in the uterine volume reduction and the dominant myoma size reduction, whether the occluding agent was polyvinyl alcohol, polyvinyl alcohol plus gelfoam, or gelfoam, and whether the ultrasound measured Resistance Index value before the procedure was low or high. CONCLUSION: Primary candidates for uterine artery embolization include those with symptomatic uterine leiomyomas, who no longer desire fertility, but who wish to avoid surgery or are poor surgical risks. According to the results of our study, adenomyosis should not be considered as a contraindication for uterine artery embolization. However, because little data is available about the subsequent reproductive potential following this procedure, it should not be routinely advocated for infertile women. Further investigation is warranted regarding the choice of occluding agent and the role of the Resistance Index.
Adult ; Arteries ; *Embolization, Therapeutic ; Female ; Human ; Leiomyoma/*therapy ; Middle Age ; Uterine Neoplasms/*therapy ; Uterus/*blood supply

Veno-occlusive disease of the liver was first reported by Chiari in 1899. Pyrrolizidine-containing substances, chemotherapeutics and hepatic radiation injury can cause this disorder. Bone marrow and renal allograft recipients are at risk for the development of veno-occlusive lesions. Veno-occlusive disease produces a syndrome of painful hepatomegaly, jaundice and fluid accumulation. The disease affects central vein and zone 3 of the liver acinus. We discuss a patient with tender hepatomegaly and high fever due to veno-occlusive lesion. We mis-diagnosed this case as acute cholecystitis because of clinical symptoms and radiological findings of GB wall thickening. During the laparotomy, she was found to have a congested liver and dilatation of superficial lymphatics of the liver surface. The Liver biopsy showed centrilobular congestion and hepatocyte necrosis in acinar zone 3. We could not determine the etiological factor in this patient. We considered that she suffered a mild form of veno-occlusive disease and recovered spontaneously within 1 month.
Allografts ; Biopsy ; Bone Marrow ; Cholecystitis, Acute ; Dilatation ; Estrogens, Conjugated (USP) ; Fever ; Hepatic Veno-Occlusive Disease* ; Hepatocytes ; Hepatomegaly ; Humans ; Jaundice ; Laparotomy ; Liver ; Necrosis ; Radiation Injuries ; Veins

Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder of the primitive hematopoietic stem cells. CML is characterized by the overproduction of myeloid cells, which results in marked splenomegaly and leukocytosis. CML presented by multiple chloromas is extremely rare. Multiple chloromas in the skin and brain are quite rare as the initial presentation of CML. These rare manifestation should alert clinicians to include CML in the differential diagnosis of patients presenting with multiple non-pruritic skin nodules or neurologic symptoms. Dasatinib has promising therapeutic potential for managing intracranial leukemic disease. Here, we report the case of a patient who visited the hospital with multiple chloroma which is unusual presentation of CML, and treated with dasatinib successfully.
Brain ; Diagnosis, Differential ; Hematopoietic Stem Cells ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Leukocytosis ; Myeloid Cells ; Myeloproliferative Disorders ; Neurologic Manifestations ; Sarcoma, Myeloid* ; Skin ; Splenomegaly ; Dasatinib