Edwards syndrome is first introduced by Edwards and characterized by facial anomalies, multiple cardiovascular, gastrointestinal, urogenital, and skeletal malformations. It results from triplication of part or all of chromosome 18 in some or all of the patient's cells. It has an incidence of 1 in 4,500 live births or less and short life expectancy. Recently we experienced a case of 3-day-old female new born infant with this syndrome. Post mortem examination showed progeric face with prominent occiput, large flabby ears, microphthalmia, and micrognathia, bilateral clenched hands with flexion contraction of middle fingers, and bilateral rockerbottom feet. Internal examination revealed horseshoe kidney, esophageal atresia with tracheoesophageal fistula, two accessory spleens, and multiple cardiac anomalies. A trisomy 18 was confirmed by the cytogenetic study.
Infant ; Male ; Female ; Infant, Newborn ; Humans ; Incidence

Most renal carcinomas are thought to originate from the epithelial cells of proximal convoluted tubules in the renal cortex. Collecting duct carcinoma is a recently recognized histological variety of renal cell carcinoma considered to arise from the epithelium of the collecting ducts. It is important to distinguish the collecting duct carcinoma from ordinary renal cell carcinoma, because which has an aggressive clinical course with early metastasis and death. But in patients, the natural course of the disease and its response to treatment have not been clearly established. Herein we report a case of collecting duct carcinoma of kidney in 38-year-old woman who had generalized edema and left flank dull pain. The preoperative diagnosis was left renal cell carcinoma on physical examination and radiologic finding, and left radical nephrectomy was done.
Adult ; Carcinoma, Renal Cell* ; Diagnosis ; Edema ; Epithelial Cells ; Epithelium ; Female ; Humans ; Kidney* ; Neoplasm Metastasis ; Nephrectomy ; Physical Examination

BACKGROUND/AIMS: Although interferon-a(IFNa) is currently the most effective antiviral agent for treating patients with chronic hepatitis C, its efficacy is not always reliable. Factors suggested to infruence outcome of IFN-a therapy for chronic hepatitis C are histological activity, level of viremia and HCV genotype, etc. The aim of this study was to determine the relationship between several pretreatment factors and response to IFN-a therapy in patients with chronic HCV infection. METHODS: Fifty-four patients with chronic HCV infection(47 with chronic hepatitis and 7 with liver cirrhosis) who received IFN-a(2a or 2b) therapy(3 6 MU, three times a week, for 3 12 months) were included. Level of serum HCV RNA(50 patients), HCV genotype(27 patients) and IgM anti- HCV(21 patients) during pretreatment period were assayed. RESULTS: Overall, 19(35%) subjects achieved sustained response(SR), 12(22%) had transient response(TR) and 23(43%) did not respond (nonresponse;NR). Mean age of patients with SR, TR and NR was 46+ 10, 51+ 7.5 and 54+ 9.7 years, respectively(p<0.05 between SR and NR). Among 30 patients with biopsy-proven chronic hepatitis, 13(43%) achieved SR;but only one(14%) in 7 patients with liver cirrhosis. Mean serum HCV RNA level(X10' copies/ml) was higher in nonresponders(7,7+ 13.0) compared with SR(2.3+ 2. 7) or TR(3.1+ 4.9), although statistically insignificant HCV genotyping in 27 patients revealed type la in 5(18.5%), 1b in 14(52%), 2a in 5(18.5%), 2b in 1(3.7%) and 4 in 2(7%), respectively. In non-1b patients, SR rate was significantly higher than 1b patients(69.2% vs. 21.4%, p=0.03). Although IgM anti-HCV was positive in 12(57%) among 21 patients studied, the positive rate and the titer of IgM anti-HCV was not significantly different in three groups. CONCLUSION: Our results suggest that in patients with chronic hepatitis C, infection with genotype 1b, old age, high serum HCV RNA level and the presence of cirrhosis would predict poor response to IFN therapy.
Fibrosis ; Genotype ; Hepatitis C, Chronic ; Hepatitis, Chronic ; Humans ; Immunoglobulin M ; Interferons* ; Liver ; Liver Cirrhosis ; RNA ; Viremia

No abstract available.
Radionuclide Imaging*

A statistical observation was made on 267 in-patients with age distribution and operative procedures in the Department of Urology, National Seoul Hospital, during the period from January 1. 1976 to December 31, 1978.
Age Distribution ; Seoul ; Surgical Procedures, Operative ; Urology*

A clinical observation was made on 150 patients of urolithiasis who admitted to the Department of Urology, National Seoul Hospital during the 5 years period from January, 1976 to December. 1980. The results were as follows; 1. The incidence of the patients with urolithiasis was 29.2% of the in-patients (513 cases) and male to female ratio was 2.75 : 1. 2. The great majority of urinary calculi occurred it patients between the ages of 21 and 40 (64.7%). 3. A seasonal occurrence was highest during summer on 34.0%. 4. The locational distribution of urinary calculi were 59.4% in the ureter, 28.8% in the kidney, 9.3 % in the bladder, and 3.3% in the urethra. 5. The most favored predilection site of ureteral stone was lower third in 57.3% of all cases. 6. The pyuria was showed in 66.7% and microscopic hematuria was revealed in 54.0%. 7. The clinical symptoms of upper urinary tract lithiasis showed flank pain in 82.4%, hematuria in 32.1%. nausea and vomiting in 25.2%, but, in lower urinary tract, frequency was 84.2%. hematuria and painful urination were each 63.2%. 8. The most surgical intervention was ureterolithotomy in 25.3%. 9. The results of chemical analysis of 76 urinary calculi showed the mixed type of calcium phosphate and calcium oxalate in 38.2% (29 cases), calcium phosphate in 18.4% (14 cases), calcium oxalate in 15.8% (12 cases), the mixed type of calcium phosphate and ammonium phosphate in 11.8% (9 cases) and calcium carbonate in 5.3% (1 cases). The major component of urinary calculi was calcium-phosphate oxalate.
Ammonium Compounds ; Calcium ; Calcium Carbonate ; Calcium Oxalate ; Female ; Flank Pain ; Hematuria ; Humans ; Incidence ; Kidney ; Lithiasis ; Male ; Nausea ; Pyuria ; Seasons ; Seoul ; Ureter ; Urethra ; Urinary Bladder ; Urinary Calculi ; Urinary Tract ; Urination ; Urolithiasis* ; Urology ; Vomiting

A clinical observation was made on forty-three cases of benign prostatic hypertrophy, admitted to the Department of Urology, National Seoul Hospital during the period from January 1, 1977 to December 31, 1980. The results were summerized as follows. 1. The incidence of benign prostatic hypertrophy was 10.9% to 396 total in-patients and 34.4% to male in-patients 50 years old or more. 2. Most of patients were in the 7th and 8th decades of life (83.7%) with a mean age of 73.5 years old. 3. The mean average hospital period was 12.4 days. 4. The mean period passed away from initial symptoms to visit was 2.8 years. Common symptoms and signs were frequency in 35 cases (81.4%), dysuria with small stream in 25 cases (58.1%), acute urinary retention in 23 cases (53.5%) and lower abdominal fulling sense and discomfortness in 13 cases (3.2%). 5. The mean volume of residual urine was 430ml. 6. Hematuria it 28cases (65.1%) and pyuria was revealed in 12 cases (27.9%). 7. I.V.P was performed in 34 cases and hydronephrosis was found in 5 cases (14.7%) and hydroureter in 4 cases (11.8%). Cystourethrography was performed in 31 cases, and elevated bladder base in 23 cases (74.2%) and trabeculation of bladder in 21 cases (67.7%). 8. Endoscopy was performed in 32 cases, The common findings were trabeculation in 28 cases (87.5%) and inflammations in 25 cases(78.1%). 9. Associated conditions with B.P.H. were hypertension in 6 cases (14.0%) and pulmonary tuberculosis in 5 cases (16.1%). 10. Management was done with retropubic prostatectomy in 16 cases, transurethral prostatectomy in 3 cases and suprapubic transvesical prostatectomy in 3 cases. 11. The mean weight of the removed adenoma was 34.2gm. 12. The mean duration of the indwelling catheter was 8.2 days. 13. The mean amount of transfused blood was 1. 78 pints. 14. Postoperative complications were urinary infection in lt cases (63.6%), temporary incontinence in 12cases (54.5%), epididymitis in 3 cases (13.6%), urethral stricture and rebleeding in each 2 cages (9.1%), delayed wound healing and pyrexia in each 1 case (4.5%). The mortality rate was 0%.
Adenoma ; Catheters, Indwelling ; Dysuria ; Endoscopy ; Epididymitis ; Fever ; Hematuria ; Humans ; Hydronephrosis ; Hypertension ; Incidence ; Inflammation ; Male ; Middle Aged ; Mortality ; Postoperative Complications ; Prostatectomy ; Prostatic Hyperplasia* ; Pyuria ; Rivers ; Seoul ; Transurethral Resection of Prostate ; Tuberculosis, Pulmonary ; Urethral Stricture ; Urinary Bladder ; Urinary Retention ; Urology ; Wound Healing

This study is standard surgical measurement of the size of kidney in normal Korean population that might be basic data of renal size in comparison with radiographic and ultrasonographic measurement for clinical implies. The 235 cases of donors for renal transplantation were measured by the length, width, thickness, weight and the outer diameter of renal vessels as well as identification of branches of renal vein at the time of donor nephrectomy. These data were tested to statistically significant by T- test, ANOVA, Pearson correlation analysis and multivariate analysis. The mean values of renal size were 11.6+/-0.87 (9.2-15.4cm) x 6.1+/-0.81 (4.4-10.0cm) x 4.9+/-0.75(2.2-7.1cm) for male 11.7+/-0.77 (9.8-14.0cm) x 6.2+/-0.85 (4.5-10.0cm) x 5.O+/-0.77 (2.8- 7.1cm) and for female 11.4+/-0.96 (9.2-15.4cm) x 6.0+/-0.75 (4.4-8.2cm) x 4.8+/-0.7 (2.2-7.0cm). The mean value of renal weight were 183.1+/-36.92 (115.0-370.0gm) : for male 188.2+/-40.10 (120.0-370.0gm) and for female 177.6+/-32.41 (115.0-300.0gm). The width, thickness and weight of kidney were larger in male than in female (p<0.05). I found out that the age of donors did not affect the renal length, width, thickness and weight (p>0.05). The renal length, width, and weight increased proportional to the body weight (p<0.05). The renal thickness and weight were different in each range of the body height (P<0.05). The body weight had correlation with renal length (r=0.25), thickness (r=0.32), weight (r=0.36, p<0.001) and width (r=0.16, p<0.05). The body height was correlated well with renal length (r=0.20), thickness (r=0.18) and weight (r=0.25, p<0.05). The body weight was the most reliable factor affecting renal size and weight in multivariate analysis (p<0.05). The mean number of branches of renal vein was 0.36+/-0.63 (0.0-2.0) in right kidney and 3.12+/-0.76 (1.0-6.0) in left one. The mean diameter of the renal artery was 0.61+/-0.132 (0.3-1.0cm) and the mean diameter of the renal vein 1.46+/-0.132 (0.50-2.20cm). The radiographic measurement of renal size was larger than our surgical measurement. The mean renal length and width in radiographic measurement(IVP) were 12.1+/-0.70cm in right kidney, 12.1+/-0.65cm in left one and 6.4+/-0.54cm in right kidney, 6.8+/-0.45cm in left one versus 11.6+/-0.87cm, 11.6+/-0.89cm and 6.1+/-0.81cm, 6.1+/-0.84cm in surgical measurement. Surgical measurements of renal size of donors at the time of renal transplantation were real renal size despite of radiographic and ultrasonographic measurement and provided basic standard data for Korean population that might be utilized in approaching renal disease.
Adult* ; Body Height ; Body Weight ; Female ; Humans ; Kidney ; Kidney Transplantation ; Male ; Multivariate Analysis ; Nephrectomy ; Renal Artery ; Renal Veins ; Tissue Donors

No abstract available.
Endocarditis*

An eccrine squamous syringometaplasia (ESS) is defined as a mature squamous metaplasia of the eccrine ducts. The clinical and pathological features of an ESS are presented. Syringometaplasia is a rare lesion, mostly occuring in the extremities, and as far as we know, no report on the ESS has been published in Korean literature. We experienced a case of an ESS occured in a 15 year-old male, who had a tender erythematous plaque in the right knee. The histologic examination revealed some scattered nests of metaplastic squamous epithelium in the deep dermis, associated with acute nonspecific panniculitis. The importance of the ESS is that it histologically simulates the well differentiated squamous cell carcinoma. The histopathologic findings were discussed and a brief review of the literature was made.
Adolescent ; Carcinoma, Squamous Cell ; Dermis ; Epithelium ; Extremities ; Humans ; Knee ; Male ; Metaplasia ; Panniculitis