No abstract available.
Urinary Tract Infections* ; Urinary Tract*

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

No abstract available.
Rectal Prolapse* ; Surgical Procedures, Operative*

Primary sarcomas of the liver are rare. A case of primary malignant fibrous histiocytoma of the liver is reported. A 55-year-old male was admitted with epigastric pain. An abdominal computed tomographic scan disclosed a 10cm, low-density area in the left lobe of the liver. Histological examination of the resected tumor showed bundles of spindle cells arranged in a storiform pattern. In some areas, many bizarre giant cells were scattered. Immunohistochemically, tumor cells were positive for alpha 1-antitrypsin and alpha 1-antichymotrypsin, and weakly positive for vimentin. The tumor cells did not express cytokeratin, desmin or alpha fetoprotein.
alpha 1-Antichymotrypsin ; alpha 1-Antitrypsin ; alpha-Fetoproteins ; Desmin ; Giant Cells ; Histiocytoma, Malignant Fibrous* ; Humans ; Keratins ; Liver* ; Male ; Middle Aged ; Sarcoma ; Vimentin

PURPOSE: Prader-Willi Syndrome(PWS) is caused by absence of paternal contributions of the chromosome region 15q11-q13. To detact this region, high resolutional cytogenetic analysis, FISH with probe at PWS critical region or microsatellite polymorphism can be used. The gene for the small nuclear ribonucleoprotein polypeptide N(SNRPN) is not expressed in patients with PWS. We conducted molecular analysis with RT-PCR with SNRPN primers to find out more useful diagnostic tool in PWS. METHODS: Four patients with obesity and other characteristics of PWS were studied. The exprssion of SNRPN and control gene were studed by RT-PCR from peripheral lymphocytes. RESULTS :The SNRPN expression in reverse transcribed RNA from blood were easily detected in normal control but not in patients with suspected Parder-Willi Syndrome. CONCLUSION: We conclude that SNRPN expression study is a useful diagnostic method for detection of Prader-Willi Syndrome.
Cytogenetic Analysis ; Humans ; Lymphocytes ; Microsatellite Repeats ; Obesity ; Pathology, Molecular* ; Prader-Willi Syndrome* ; Ribonucleoproteins, Small Nuclear ; RNA ; snRNP Core Proteins*

A few cases of reticulate acropigmentary disorders have been reported in Korea. Most of them were reticulate acropigmentation of Kitamura, but not acropigmentation symmetrica of Dohi. A 21 year-old male patient diagnosed as acropigmentation symmetrica of Dohi had been previously treated with various treatment methods unsuccessfully. Re-treatment with the Q-switched alexandrite laser(755 nm, 100nsec) showed a temporary improvement but a relapse of the lesion was experienced again. We describe a case of acropigmentation symmetrica of hi treated with the Q-switched alexandrite laser.
Humans ; Korea ; Lasers, Solid-State* ; Male ; Recurrence

Dental porcelain should have adequate strength and similar esthetics as natural teeth. Recently esthetics has become a high priority in clinical dentistry. Thus the evaluation of color stability of dental porcelain has become an important part in dental research. The purpose of this study was to evaluate the difference in color stability of 3 types of porcelain (Vintage, VMK 95, CERAMCO II). The porcelain were divided into groups by 2 types of treatment (Glazing or non-glazing and thermocycling or non-thermocycling). The porcelain wee dyed with methylene blue and distilled water, and then the color stability was evaluated with a spectrophotometer. The results were as follow ; 1. The color change of porcelain increased in the order of VMK 95, Vintage, CERAMCO II. But there was no significant difference. 2. The color change decreased in the glazing group compared to the non-glazing group(p<0.05). 3. The color change increased after thermocycling But there was no significant difference. 4. The color change according to dye method was significantly larger with methylene blue copared to distilled water(P<0.05).
Crowns* ; Dental Porcelain* ; Dental Research ; Dentistry ; Esthetics ; Methylene Blue ; Tooth ; Water

Calcification and ossification of colon cancer is frequently encountered, especially in the mucinous carcinoma. However, cartilage formation or enchondral ossification has rarely been described in human colon cancer. This report describes a case of a 59-year-old man with retroperitoneal metastasis of mucinous adenocarcinoma of colon, which showed a widespread heterotopic ossification through membranous or enchondral ossification. The ossification appeared in apposition to tumor cell nests and in the organized mucin pool. In our knowledge, this is the first case showing enchondral ossification in gastrointestinal carcinoma in Korea.
Adenocarcinoma* ; Adenocarcinoma, Mucinous ; Cartilage ; Colon* ; Colonic Neoplasms ; Humans ; Korea ; Middle Aged ; Mucins ; Neoplasm Metastasis ; Ossification, Heterotopic

No abstract available.
Polyethylene*

We report a rare case of adreno-hepatic fusion in a 63-year-old man with a traumatic hepatic rupture. The adrenal tissue was located beneath the Glisson's capsule of the liver, and measured 3.5x2x0.3 cm. On histologic examination, the ectopic tissue was composed of both adrenal cortex and medulla surrounded by a delicate capsule of connective tissue.
Adrenal Cortex ; Choristoma ; Connective Tissue ; Humans ; Liver ; Middle Aged ; Rupture