The incidence of hypercalcemia in terminal cancer patients is rare but if it is not treated, the consequence is dismal. Hydration, diuretics, corticosteroids are not so effective for the treatment of hypercalcemia in this situation. Pamidronate (synthetic bisphosphonate analogue of pyrophosphonate) decreases the activity and the life span of the osteoclasts. It results in decrease in resorption of bone mineral, directly and indirectly. There are only a few reports on the usage of pamidronate in children. We experienced a case with hypercalcemia in patient with Wilms tumor who was treated with pamidronate. A 5-year-old male patient was admitted to Ajou University Hospital due huge abdominal mass. Abdominal CT revealed a huge mass originating from right kidney (size: 18 11 8 cm) with lung metastasis. The chemotherapy according to National Wilms Tumor Study (NWTS)-4 was started but there was no response after 4 months. Right nephrectomy with mass removal was done and the pathologic finding revealed diffuse anaplastic type. The serum calcium level increased to be 16.7 mg/dL at 7 days after operation which was not responsive to hydration and furosemide. Pamidronate was given at the dose of 1 mg/kg and the serum calcium decreased to 8.0 mg/dL 24 hours later. The pulmonary lesion progressed and the patient died 4 months after operation but the serum calcium level remained within normal range thereafter.
Adrenal Cortex Hormones ; Calcium ; Child ; Child, Preschool ; Diuretics ; Drug Therapy ; Furosemide ; Humans ; Hypercalcemia* ; Incidence ; Kidney ; Lung ; Male ; Neoplasm Metastasis ; Nephrectomy ; Osteoclasts ; Reference Values ; Tomography, X-Ray Computed ; Wilms Tumor*

The treatment of moya moya disease, a chronic occlusive cerebrovascular disease of unknown etiology, isn't settled ut various operative methods to maximize cerebral revascularization have been reported. Two cases in children treated surgically are presented, one with cerebroarteriosynangiosis and the other with encephalo-duro-arterio-synangiosis(EDAS). The methods of cerebral revascularization are discussed in detail.
Cerebral Revascularization ; Child* ; Humans ; Moyamoya Disease*

The purpose of this study is to investigate the concentration of plasma choline of Korean and to clarify the relationship between plasma choline concentration and choline intake. Plasma choline concentration of 30 young adults (15 males, 15 females) aged 20 - 30 years living in Deajeon metropolitan city are analyzed and their dietary choline intake. Choline content of one day meal was directly analyzed with the use of enzymatic method. Plasma choline concentration from more than 12 hr fasting blood was analyzed by using HPLC-MS. Choline intakes of male subjects were in the range of 253.51 - 1724.14 mg and those of female subjects were in the range of 240.85 - 938.06 mg. Mean intakes of choline were 634.53+/-353.68 mg in male subjects and 473.99+/-183.76 mg in female subjects. Plasma choline concentration of total subjects was in the range of 5.08 - 14.01 micro mol/L. Mean plasma choline concentration was 9.19+/-2.05 micro mol/L in male subjects and 8.11+/-1.70 micro mol/L in female subjects. Plasma choline concentration did not show significant correlation with choline intake in male and total subjects, but showed positive correlation with choline intake in female subjects (p<0.05). This result shows that more studies on large scaled samples are needed.
Choline* ; Daejeon ; Fasting ; Female ; Humans ; Male ; Meals ; Plasma* ; Young Adult*

Early repolarization is a common electrocardiographic (ECG) feature found in young adults, men and athletes, and has been considered to be a benign feature for the last several decades. But recent studies suggest that early repolarization may be related to idiopathic ventricular fibrillation and sudden cardiac death. We report a young man, 35 years old, who had life threatening ventricular fibrillation and sudden cardiac arrest. He was evaluated for cardiac causes of ventricular fibrillation. There was no explanation other than that his ECG showed an early repolarization pattern so we treated him with implantable cardioverter defibrillator. Thus, we suggest that early repolarization may be related with life threatening ventricular arrhythmia.
Arrhythmias, Cardiac ; Athletes ; Death, Sudden, Cardiac ; Defibrillators ; Electrocardiography ; Humans ; Male ; Ventricular Fibrillation* ; Young Adult

PURPOSE: Although the influence of Notch signaling on several types of malignancies has been studied, the role of Notch signaling in clear cell renal cell carcinoma (ccRCC) remains unclear. In this study, we evaluated the levels of Notch1 and Jagged1 and their significance in ccRCC. MATERIALS AND METHODS: Tumor tissue and matched normal adjacent kidney tissue from 49 ccRCC cases were obtained. The expression of Notch1 and Jagged1 was analyzed using real-time polymerase chain reaction (PCR) and Western blotting. Tissue samples were divided into several groups according to clinicopathological features, and the relative expression of Notch1 and Jagged1 was assessed. RESULTS: Real-time PCR revealed increased Notch1 expression in tumor tissues compared with that in adjacent normal tissues (p=0.044). Based on the pathological stage, a significant difference in Notch1 expression was observed between tumor and normal kidney tissues in pT2 and pT3 ccRCC (pT2, p=0.041; pT3, p=0.001). Notch1 expression in ccRCC relative to that in normal tissue was higher in later-stage ccRCC and larger ccRCC. Notch1 expression showed significant positive correlation with the maximal diameter of the primary renal tumor (mRNA, p<0.001; protein, p=0.001). High Notch1 expression was associated with recurrence and disease-specific death, although the difference was not significant. Jagged1 level was not significantly correlated with any of the factors examined. CONCLUSIONS: Notch1 may play a significant role in the tumorigenesis and progression of ccRCC. Notch signaling may be a potential target for chemopreventive or adjuvant therapeutics for ccRCC.
Biomarkers ; Blotting, Western ; Carcinogenesis ; Carcinoma, Renal Cell* ; Kidney ; Real-Time Polymerase Chain Reaction ; Recurrence ; Tissue Array Analysis

The annual incidence of a first episode of deep vein thrombosis or pulmonary embolism (PE) in the general population is 120 per 100,000. Cancer is associated with an approximately 4- to 7-fold higher risk of thrombosis. Adrenocortical carcinoma (ACC) is a rare type of malignancy, accounting for 0.02% of all cancers reported annually. Approximately 40% of ACCs are nonsecretory. Most patients with nonsecreting tumors have clinical manifestations related to tumor growth (e.g., abdominal or flank pain). Often the adrenal mass is detected by chance via radiographic imaging. As a result, most ACC patients are diagnosed at an advanced stage and have a poor prognosis. Herein, we report a case of a 54-year-old woman who was admitted to our emergency department complaining of dyspnea. She was diagnosed with ACC accompanied by thrombi in the pulmonary artery and inferior vena cava. We performed a left adrenalectomy and administered adjuvant radiotherapy. The patient is currently receiving warfarin and adjuvant mitotane therapy. She was incidentally diagnosed with ACC, with PE as the initial manifestation.
Accounting ; Adrenalectomy ; Adrenocortical Carcinoma ; Dyspnea ; Emergencies ; Female ; Humans ; Incidence ; Middle Aged ; Mitotane ; Prognosis ; Pulmonary Artery ; Pulmonary Embolism ; Radiotherapy, Adjuvant ; Thrombosis ; Vena Cava, Inferior ; Venous Thrombosis ; Warfarin

BACKGROUND: The glucose uptake rate is the limiting step in glucose utilization and storage. The failure of insulin to stimulate glucose uptake in muscle appears to be a primary defect of insulin resistance. This study was undertaken to examine the effect of physiological hyperinsulinemia on the phosphorylation of the insulin receptor (IR-beta), insulin receptor substrate (IRS), Akt kinase and GSK-3 in isolated skeletal muscle, in people with type 2 diabetes (n=9) and control subjects (n=11). METHODS: 75g OGTT and euglycemic hyperinsulinemic clamp test were done. And vastus lateralis muscle was obtained before and 30 min into the euglycemic clamp. Western blots were performed for tyrosine phosphorylation of insulin receptor substrate (IRS) and phosphorylation of the insulin receptor(IR-beta), Akt and GSK-3. RESULT: There were no statistical differences in the mean age, BMI and body fat between the control subjects and diabetic patients. The fasting blood sugar and HbA1c in controls and diabetic patients were 98.+/-1.3 and 208.1+/-16.5 ng/dl, and 5.4+/-0.5 and 9.2+/-0.6%, and 1.4+/-0.2 in the control subjects, and 72.2+/-52.3% (p<0.01) and 10.2+/-6.3 (p<0.01) in the diabetic patients, respectively. The insulin resistance from the euglycemic hyperinsulinemic clamp tests were 8.2+/-0.6 mg/kg/min and 3.7+/-1.1 ng/kg/min in the control subjects and in the diabetic patients, respectively (p<0.01). Compared with the normal controls, insulin-stimulated IR phosphorylation was no different to that in the diabetic patients. However, insulin-stimulated IRS phosphorylation, insulin-stimulated Akt phosphorylation and insulin-stimulated GSK-3 phosphorylation were reduced in the diabetic patients compared with the normal controls by 24, 43 and 25%, respectively (p<0.05). CONCLUSION: In korean type 2 diabetic patients, the insulin resistance may be due to the impairment of the upstream insulin signal molecular network. Further studies will focus on determining whether these signaling defects are the cause of the development of insulin resistance, or secondary to the altered metabolic state, associated with type 2 diabetes mellitus
Adipose Tissue ; Blood Glucose ; Blotting, Western ; Diabetes Mellitus, Type 2 ; Fasting ; Glucose ; Glucose Clamp Technique ; Glucose Tolerance Test ; Glycogen Synthase Kinase 3 ; Humans ; Hyperinsulinism ; Insulin ; Insulin Resistance ; Muscle, Skeletal* ; Phosphorylation ; Phosphotransferases ; Quadriceps Muscle ; Receptor, Insulin ; Signal Transduction* ; Tyrosine

PURPOSE: We evaluated 5 different rat models using different agents in order to establish a standard animal model for interstitial cystitis (IC) in terms of the functional and pathologic characteristics of the bladder. METHODS: Five IC models were generated in 8-week-old female Sprague-Dawley rats via transurethral instillation of 0.1M hydrogen chloride (HCl) or 3% acetic acid (AA), intraperitoneal injection of cyclophosphamide (CYP) or lipopolysaccharide (LPS), or subcutaneous injection of uroplakin II (UPK2). After generating the IC models, conscious cystometry was performed on days 3, 7, and 14. All rats were euthanized on day 14 and their bladders were obtained for histological and pro-inflammatory-related gene expression analysis. RESULTS: In the cystometric analysis, all experimental groups showed significantly decreased intercontraction intervals compared with the control group on day 3, but only the LPS and UPK groups maintained significantly shorter intercontraction intervals than the control group on day 14. The histological analysis revealed that areas with severe urothelial erosion (HCl, AA, and UPK) and hyperplasia (CYP and LPS), particularly in the UPK-treated bladders, showed a markedly increased infiltration of toluidine blue-stained mast cells and increased tissue fibrosis. In addition, significantly elevated expression of interleukin-1b, interleukin-6, myeloperoxidase, monocyte chemotactic protein 1, and Toll-like receptors 2 and 4 was observed in the UPK group compared to the other groups. CONCLUSIONS: Among the 5 different agents, the injection of UPK generated the most effective IC animal model, showing consequent urothelial barrier loss, inflammatory reaction, tissue fibrosis stimulation, and persistent hyperactive bladder.
Acetic Acid ; Animals ; Animals* ; Chemokine CCL2 ; Cyclophosphamide ; Cystitis, Interstitial* ; Female ; Fibrosis ; Gene Expression ; Humans ; Hydrochloric Acid ; Hyperplasia ; Immunization ; Injections, Intraperitoneal ; Injections, Subcutaneous ; Interleukin-6 ; Mast Cells ; Models, Animal* ; Peroxidase ; Rats* ; Rats, Sprague-Dawley ; Toll-Like Receptors ; Urinary Bladder ; Uroplakin II

BACKGROUND/AIMS: The aims of this study were to determine subgoups of functional dyspesia and to evaluate the short-term effect of cisapride in patients with functional dyspepsia in Korea. METHODS: 1025 patients, with a mean age of 42.6 years, with symptoms of functional dyspepsia, were recruited consecutively and upper gastrointestinal symptoms were investigated by interview in 41 hospitals in Korea. In an open, multicenter trial, 1025 patients received Smg of cisapride three times a day (TID) for at least .2 weeks for the treatment of symptoms of functional dyspepsia. When necessary, the dose of cisapride was increased to 10mg TID and the duration of therapy was extended to 4 weeks. RESULTS: The most frequently reported symptoms of functional dyspepsia were epigastric discomfort or fullness (85%), bloating (70%), belching (53%), early satiety (52%) and epigastric pain (46%) retrospectively. Subgroups of functional dyspepsia were as follows; dysmotility-like 73.5%, ulcer-like 39.7%, reflux-like 13.0%, and unspecified dyspepsia 14.0%. However, 33.2% of subjects with functional dyspepsia could be classified into more than one subgroup. Upper gastrointestinal symptoms were decreased to average 50.3% (range; 42.2 to 59.2%) after 2 weeks of cisapride treatment and to 25% (19.2 to 29.9%) after 4 weeks. cisapride therapy resulted in good or excellent improvement in 59.0% of the patients after two weeks, in 75% of patients after 4 weeks. Adverse events were occurred in 52 patients (5.8% of all patients), most commonly, loose stools or diarrhea (3.5%), abdominal pain (1.1%), and dizziness (0.3%). The majority of adverse events was mild and transient in nature and led to premature discontinuation of treatment in 4 patients. CONCLUSIONS: Although the majorities of patients with functional dyspepsia have dysmotility like symptoms in Korea, there is such overlap among the dyspepsia subgroups. Most patients responded well to a short therapeutic trial with cisapride without significant side effects.
Abdominal Pain ; Cisapride* ; Diarrhea ; Dizziness ; Dyspepsia* ; Eructation ; Humans ; Korea* ; Retrospective Studies