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

Afferent loop syndrome(ALS) is caused by obstruction of the afferent loop after subtotal gastrectomy with Billroth II gastrojejunostomy. Prompt diagnosis of ALS is important as perforation of the loop occurs. The aim of this study is to ascertain the value of sonography and CT to diagnose ALS. We describe the radiologic findings in ten patients with ALS. The causes of ALS, established at surgery, included cancer recurrence (n=4), internal hernia(n=4), marginal ulcer (n=1), and development of cancer at the anastomosis site(n=1). Abdominal X-ray and sonography were performed in all cases, upper GI series in five cases and computed tomography in two cases. The dilated afferent loop was detected in only two cases out of ten patients in retrospective review of abdominal X-ray. ALS with recurrence of cancer was diagnosed in three cases by upper GI series. Of the cases that had sonography, the afferent loop was seen in the upper abdomen crossing transversely over the midline in all ten patients. The causes of ALS were predicted on the basis of the sonograms in three of the five cancer patients. In two cases of computed tomography, the dilated afferent loop and recurrent cancer at the remnant stomach were seen. Our experience suggests that the diagnosis of afferent loop syndrome can be made on the basis of the typical anatomic location and shape of the dilated bowel loop in both sonography and computed tomography.
Abdomen ; Afferent Loop Syndrome* ; Diagnosis ; Gastrectomy ; Gastric Bypass ; Gastric Stump ; Gastroenterostomy ; Humans ; Peptic Ulcer ; Recurrence ; Retrospective Studies

The most common causes of conjugated hyperbilirubinemia after the first or second week of life are neonatal hepatitis and biliary atresia. Since these entities represent variable expressions of same pathologic process and have similar clinical, biochemical, and histologic features, differential diagnosis is extremely difficult. We prospectively studied 28 jaundiced infants by sonography and hepatobiliary scintigraphy. Final diagnoses included 12 biliary atresia and 16 neonatal hepatitis. Visualization of a normal-sized common bile duct or gallbladder was compatible with the diagnosis of neonatal hepatitis. Non-visualized or atrophic gallbladder no sonography coupled with non-visualization of bowel activity on scintigraphy was highly suggestive of biliary atresia. We believe that sonography plays valuable role in the initial evaluation of the infants with persistent neonatal jaundice. The combined use of sonography and hepatobiliary scintigraphy provides the most valuable in formation in suspected biliary atresia for prompt surgical treatment.
Biliary Atresia ; Common Bile Duct ; Diagnosis* ; Diagnosis, Differential ; Gallbladder ; Hepatitis ; Humans ; Hyperbilirubinemia ; Infant ; Infant, Newborn ; Jaundice, Neonatal* ; Prospective Studies ; Radionuclide Imaging*

To assess the pathological basis of the sonographic changes of the gallbladder wall in cholecystitis, thesonographic appearances of the gallbladder wall were analysed in 17 patients with acute cholecystitis and 27patients with chronic cholecystitis, and correlated with pathological specimens removed at surgery. In acutecholecystitis, a thin sonolucent layer within the echogenic gallbladder wall corresponds to subserosal edema,hemorrhage and inflammatory cell infiltration: in chronic cholecystitis it corresponds to subserosal edema,hemorrhage and inflammatory cell infiltration: in chronic cholelcystitis it corresponds to subserosal edema,hemorrhage and inflammatory cell infiltration, in chronic cholelcystitis it corresponds to muscular hypertrophy.Indistinctness and/or a low echogenicity rind along the inner margin reflects mucosal sloughing or obliteration ofthe mucosal folds. Uniformly decreased echogenicity of the wall is caused by severe inflammatory cell infiltrationwith sloughing of the mucosa or obliteration of the mucosal folds. These sonographic singns are considered to bevaluable sings of cholecystitis.
Cholecystitis ; Cholecystitis, Acute ; Gallbladder ; Humans ; Mucous Membrane ; Ultrasonography

Liposarcoma is extremely rare malignant tumor which develops primary in the orbit. Here, we report a case of primary orbital liposarcoma in 17 year old female patient. She visited our hospital due to decreased visual acuity and protrusion of Rt. eyeball. Malignant tumor was suspected by many examination. So exenteration was performed and tumor mass was confirmed as liposarcoma by pathologic examination. She was received cobalt 60 radiation therapy, but recurrent tumor mass appeared at the orbital apex 1 year after operation.
Adolescent ; Cobalt ; Female ; Humans ; Liposarcoma* ; Orbit* ; Visual Acuity

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

The infantile polycystic kidney disease is rare fetal urinary tract anomaly. It is inherited with an autosomal recessive pattern and recurrence rate is 25%. The gene locus is on chromosome 6p. The pathogenesis of infantile polycystic kidney is the primary defect of the collecting ducts. The ultrasonographic finding of infantile polycystic kidney is oligohydramnios, bilaterally symmetrical enlarged kidneys with maintenance of their reinform shape. The differential diagnosis with adult polycystic kidney disease is important. The massive enlargement of the kidneys is rarely seen in adult polycystic kidney disease and the examination of the parents and other members of the family is helpful to confirm the adult polycystic kidney disease. If there is severe renal involvements, stillbirth or neonatal death secondary to pulmonary hypoplasia would be developed. If it were diagnosed before viability, termination of pregnancy is recommended. In a fetus at risk, diagnosed after viability, pregnancy termination is also recommended since this condition is uniformly fatal. We present a case of infantile polycystic kidney.
Diagnosis, Differential ; Female ; Fetus ; Humans ; Kidney ; Oligohydramnios ; Parents ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Dominant ; Pregnancy ; Recurrence ; Stillbirth ; Urinary Tract

BACKGROUND: Alterations in ventricular loading conditions lead to changes in action potential duration via mechanoelectrical feedback. A decrease in load immediately leads to prolongation of repolarization. QT interval and QT dispersion were measured to determine the long-term effect of changes in ventricular systolic load on the ventricular repolarization. METHOD: Corrected QT interval and QT dispersion were measured in 26 patients before and 3-6 months after valvuloplasty for pulmonary stenosis. To determine the effect of ventricular load on ventricular repolarization, patients were divided in 2 groups; Group 1 was those patients with a greater than 30 mmHg and Group 2 was those patients with a less than 30 mmHg decrease in right ventricular systolic pressure. RESULTS: Corrected QT interval (412.6+/-14.5 msec vs 426.4+/-16.8 msec, p < 0.05) and QT dispersion (35.0+/-7.3 msec vs 45.7+/-14..1 msec, p < 0.05)were increased significantly only in Group 1 after vlavuloplasty. CONCLUSION: It suggested that mechanoelectrical interactions are operative for long duration in humans that changes in ventricular load after successful pulmonary valvuloplasty showed long-term effect on the ventricular repolarization.
Action Potentials ; Balloon Valvuloplasty* ; Blood Pressure ; Humans ; Pulmonary Valve Stenosis*

Small hepatoma is defined as hepatocellular carcinoma less than 3cm in maximum diameter and fewer than 3 innumber. To assess the ability of ultrasound to detect small hepatomas, a prospectively study was done in a groupof patients with HBsAg-positive chronic hepatitis and liver cirrhosis. Herein, we present 4 hypoechoic smallhepatomas detected on ultrasound and emphasize the role of real-time ultrasonography as a practical test formonitoring hepatoma high-risk, subjects.
Carcinoma, Hepatocellular ; Hepatitis, Chronic ; Humans ; Liver Cirrhosis ; Mass Screening ; Prospective Studies ; Ultrasonography

Author analyzed 154 cases of transabdominal ultrasonography of normal adults who had normal urinalysis, no complaint of symptoms related with urinary system and no evidence of prostate disease. The maximum anteroposterior, transverse and longitudinal diameters of prostate were measured. The weight of prostate was computed using the formular for the weight of ellipsoid object (W=SGx4/3 pie r1.r2.r3, w; weight, SG; specificgravity about 1.05 gm/ml, r; radious of prostate in any dimension). The results were as follows; 1. The averagesized of prostate were 4.12±0.68 cm in transverse diameter, 3.46±0.49 cm in longitudinal diameter and 2.36±0.40cm in AP diameter. Transeverse diameter was the longest and AP diameter was the shorteset in all cases. 2. Theaverage weight of prostate in each age groups were 19.25±6.78 gm in the 3rd decade, 16.64±5.18 gm in the 4th decade, 16.28±5.31 gm in the 5th decade, 22.31±6.95gm in the 6th decade and 13.06±8.18 gm in over the 7th decade. The average weight of prostate in groups more than 50 years of age were significantly greater than youngerage groups (p<0.005). Total average weight was 18.96±6.78 gm. 3. The distribution of prostate weighting over 30gm in each groups were 14.29%(5/35) in the 3rd decade, 0%(0/34) in the 4th decade, 2.63%(1/38) in the 5th decade,20%(6/30) in the 6th decade and 23.5%(4/17) in the 7th decade.
Adult ; Humans ; Prostate ; Ultrasonography ; Urinalysis