Beckwith-Wiedemann sydrome is a multisystemic pattern of congenital anomalies with overgrowth. This syndrome is first described independently by Beckwith in 1963 and by Wiedemann in 1964. There is wide spectrum of clinical manifestations, including prenatal or postnatal overgrowth, neonatal hypoglycemia, macroglossia, visceromegaly, omphalocele, hemihypertrophy and a predisposition for embryonal tumors, most frequently Wilms' tumor. We experienced a case of Beckwith-Wiedemann syndrome who developed left adrenal cortical neoplasm of indeterminate malignant potential.
Beckwith-Wiedemann Syndrome ; Hernia, Umbilical ; Hypoglycemia ; Macroglossia ; Wilms Tumor

Alterations in the expression of genes that control the cell cycle may be of critical importance in tumorigenesis and malignant transformation. The major regulatory events leading to cell proliferation occur in Gl phase of cell cycle, and the deregulated expression of Gl cyclins is related to oncogenesis. Cyclins Dl and E play important roles in the progression of cell through Gl phase of the cell cycle. Amplification and/or overexpression of the cyclin Dl gene and aberrant expression of cyclin E has been described in various forms of human cancer. However, the role of cyclins Dl and E in cervical cancer has been poorly defined. In this study, we examined the expression of cyclins Dl and E by Northern blot technique and the status of human papil- lomavirus(HPV) type 16 and 18 by polymerase chain reaction in 25 cases of cervical carcinoma to explore the relationship between cyclins Dl and E and cervical cancer. We found cyclin Dl expression showed down-regulated expression in cervical cancer but cyclin E expression was increased in cancer group. Other clinicopathological prognostic factors were not correlated with cyclins D1 and E expression. Further study based on larger numbers of cases with correlation of cyclins D1 and E status and survival data will be needed to elucidate the use of cyclin expressions as prognostic factor.
Blotting, Northern ; Carcinogenesis ; Cell Cycle ; Cell Proliferation ; Cyclin E ; Cyclins* ; Humans ; Polymerase Chain Reaction ; Uterine Cervical Neoplasms

BACKGROUNG AND OBJECTIVES: It was reported that low molecular weight heparin (LMWH) was more effective than unfractionated heparin in patients with acute coronary syndrome. Recent studies have shown that the pathophysiology of restenosis in stented lesions was different from those of nonstented lesions. Treatment strategies designed to limit cellular proliferation that were ineffective in nonstented lesions may be efficacious in reducing in-stent restenosis. This study was aimed to compare the clinical and angiographic results of LMWH (nadroparin) after coronary stenting with those of conventional ticlopidine regimen. MATERIALS AND METHODS: Patients were eligible for inclusion if they had angina and/or objective evidence of myocardial ischemia, and a significant (>50%) stenosis that was documented on a recent coronary angiogram. After stenting, prospective randomized comparison study was performed. Patients were randomly assigned to either nadroparin (200 IU/kg, sc, bid) or ticlopidine (250 mg bid) plus aspirin (200 mg qd) treatment groups. Repeat coronary angiography (KERN=*)was performed at 236+/-90days after stenting, and quantitative coronary angiographic analysis (QCA) was done. RESULTS: Intracoronary stent implantation was performed in eighty five lesions in eighty one patients (ticlopidine:40, nadroparin:41). There was no significant difference in any baseline clinical/angiographic variables between the two treatment groups. There were no subacute stent thrombosis, infarction and death in both groups. Six-month event-free survival was 36 (90%) in the ticlopidine group and 35 (85.4%) in the nadroparin group. Follow-up quantitative angiographic data such as late loss (1.35+/-0.70 vs 1.32+/-0.69), loss index (0.53+/-0.70 vs 0.56+/-0.23) and restenosis rate (36% vs 25.8%) were not different between ticlopidine and nadroparin groups. CONCLUSION: Effects of nadroparin were not different from those with ticlopidine therapy in the prevention of restenosis and subacute stent thorombosis after coronary stenting. Clinical outcomes between two strategies were similar. Low molecular weight heparin may be an alternative to ticlopidine in patients that ticlopidine cannot be administered because of severe adverse effects.
Acute Coronary Syndrome ; Aspirin ; Cell Proliferation ; Constriction, Pathologic ; Coronary Angiography ; Disease-Free Survival ; Follow-Up Studies ; Heparin ; Heparin, Low-Molecular-Weight ; Humans ; Infarction ; Myocardial Ischemia ; Nadroparin* ; Prospective Studies ; Stents* ; Thrombosis ; Ticlopidine*

BACKGROUND: The effects of a newly synthesized potassium channel opener, KR-30816((-)(nitro-2-hydroxymethyl-2-methy-2H-1-benzopyran-4-y1)pyridine oxide) on the action potential of papillary muscles of guinea pigs and the ATP-sensitive potassium channel current(IKATP) of single ventricular muscle cells of rats were examined to make clear its action mechanism of the KATPchannel. METHODS: We used the conventional microelectrode and the excised inside-out patch configuration. RESULTS: KR-30816 caused a shortening of the action potential duration in dose-dependent manner, which was inhibited by glibenclamide(3microM). Before run-down of the K+channel, KR-30816 activated the cardiac ATP-sensitive K+ channel only in the presence of ATP and shifted the dose-response relation curve between [ATP]i and the channel activity to the right in parallel. After run-down of the KATP channel, KR-30816 did not after the channel opening either in the absence or in the presence of UDP. CONCLUSION: These results suggest that KR-30816 antagonizes the inhibitory effect of ATP on the KATPchannel in a competitive manner, thereby enhancing the channel openings.
Action Potentials ; Adenosine Triphosphate ; Animals ; Guinea Pigs ; Heart ; Microelectrodes ; Muscle Cells ; Papillary Muscles ; Potassium Channels* ; Potassium* ; Rats ; Uridine Diphosphate

No abstract available.
Animals ; Cerebellum* ; Inositol 1,4,5-Trisphosphate* ; Inositol* ; Rats*

BACKGROUND: For Neer type IIB fracture of distal clavicle with coracoclavicular ligament injury, various surgical treatments have been used in literatures. However, there was no consensus on the optimal treatment. The aim of this study is to report the clinical and radiological results of open reduction and internal fixation of unstable distal clavicle fracture and suture augmentation of disrupted coracoclavicular ligament. METHODS: A prospective study was performed in 23 patients with Neer type IIB distal clavicle fracture in Seoul Medical Center, Eulji Hospital, and National Medical Center. Firstly, suture anchors are inserted in the base of coracoid process and preliminary reduction was achieved by tie-off of three suture limbs around the clavicle. Then, the final fixation was completed with anatomical locking plate. Bony union and the distance between coracoclavicular ligaments were evaluated. Clinical results and complications including stiffness and secondary procedures were evaluated. RESULTS: Bony union was achieved in all cases except one (22 of 23). At mean 14.9 months, no significant difference in the mean coracoclavicular distance was observed compared to uninjured shoulder (8.2 +/- 7.9 mm versus 7.3 +/- 3.4 mm, p=0.14). Pain visual analogue scale, American Shoulder and Elbow Surgeons score, Constant score, and Disabilities of the Arm, Shoulder and Hand score were 0.5, 83.4, 78.5, and 6.2, respectively. Revision surgery was performed in one case of nonunion. Four patients who complained of skin irritation underwent implant removal. CONCLUSIONS: In cases of an unstable distal clavicle fracture with coracoclavicular ligament disruption, satisfactory clinical results were obtained by locking plate fixation and coracoclavicular ligament suture augmentation concurrently.
Arm ; Clavicle* ; Consensus ; Elbow ; Extremities ; Hand ; Humans ; Ligaments* ; Prospective Studies ; Seoul ; Shoulder ; Shoulder Fractures ; Skin ; Suture Anchors ; Sutures*

OBJECTIVES: In this study, the cognitive characteristics, especially priming effect, of above 50 years old age group who had less than 25 points in K-MMSE were investigated. METHODS: In this study, an implicit memory test measuring priming effect was made and adminis-tered to old age people who had less than 25 points in K-MMSE in screening session. To control the effect of age, educational level and intelligence, demographic variables were measured, and the subtests of KWIS, vocabulary and block design were administered. Descriptive statistical analysis of the two priming measures and correlational analysis between variables were done. To test the effect of cognitive functioning on priming effect multiple regression analysis was done. RESULTS: Correlational analysis revealed priming score obtained from correct identification response was positively correlated with K-MMSE and ADAS-Cog. And priming score obtained from mean reaction time was positively correlated with age and negatively correlated with vocabulary substest of KWIS. The regression analysis results indicated general cognitive functioning measured by ADAS-Cog has significant effect on priming score obtained from correct identification response, whereas age has significant effect on priming score obtained from mean reaction time. CONCLUSIONS: The results revealed that as cognitive deterioration progresses priming effect which identify primed stimulus correctly diminish, and as one grow older within age 50 to 70, priming effect which identifies primed stimulus quickly increase.
Humans ; Intelligence ; Mass Screening ; Memory ; Middle Aged ; Mild Cognitive Impairment* ; Reaction Time ; Vocabulary

Congenital absence of the gall bladder without extrahepatic biliary atresia is an extremely rare congenital malformation with a reported incidence ranging between 0.01 and 0.02%. It is thought to be occurred from failure of the gallbladder bud to develop in utero and frequent association with other malformations. Agenesis can be randomly discovered at autopsy or operations for symptoms suggestive of biliary tract disease. The authors report a case of agenesis of the gallbladder without extrahepatic biliary atresia in a neonate which was incidentally found at laparotomy for presumed duodenal obstruction, with a review of the literature.
Autopsy ; Biliary Atresia* ; Biliary Tract Diseases ; Duodenal Obstruction ; Gallbladder* ; Humans ; Incidence ; Infant, Newborn ; Laparotomy ; Urinary Bladder

Here we describe a case of Twiddler's syndrome in a patient with an implanted cardioverter-defibrillator who tinkered with the device, shifting it to a vertical position and causing her skin to protrude in various locations. Fluoroscopy was used to determine what direction the device was rotating in during specific body postures and physical activities. Inappropriate shocks were delivered whenever the patient pushed the device down toward her sternum. The syndrome did not recur following training of the patient in a lead-revision procedure that involved shifting the device down vertically toward her lateral side with her hands.
Defibrillators ; Fluoroscopy ; Hand ; Humans ; Motor Activity ; Posture ; Shock ; Skin ; Sternum

Childhood obesity seems to contribute to the development of vascular inflammation and the progression of arterial wall changes. High-sensitivity C-reactive protein (hs-CRP) has recently emerged as a useful biomarker for vascular inflammation associated with atherosclerosis. The objectives of this study were to evaluate the association of the serum hs-CRP level with ultrasonic findings of early atherosclerosis, carotid intima-media wall thickness (IMT) and brachial flow-mediated dilation (FMD), in obese children. Thirty eight obese children and 45 sex/age-matched healthy control children were recruited. Serum CRP levels were measured by the high-sensitive latex turbidimetric immunoassay, and we measured carotid IMT and brachial FMD using high-resolution B-mode ultrasonography. Obese children had significantly higher hs-CRP levels (1.40+/-0.74 mg/L vs. 0.55+/-0.49 mg/L, p<0.01), as well as increased IMT (0.52+/-0.09 mm vs. 0.41+/-0.07 mm, p<0.01) and impaired FMD (7.35+/-7.78% vs. 20.34+/-16.81%, p<0.01) compared to healthy controls. Serum hs-CRP correlated positively with IMT (r=0.413, p<0.05) and inversely with FMD (r=-0.350, p<0.05) in the obesity group. Measurement of the serum hs-CRP level is a simple, cheap, and highly reproducible assay and correlates with IMT and FMD in obese children. Thus, it would be a useful marker for evaluating and estimating the degree of atherosclerosis in children.
Sensitivity and Specificity ; Risk Factors ; Risk Assessment/*methods ; Reproducibility of Results ; Prognosis ; Obesity/*blood/*complications/diagnosis ; Male ; Humans ; Female ; C-Reactive Protein/*analysis ; Biological Markers/blood ; Atherosclerosis/*blood/complications/*diagnosis ; Adolescent