Drash syndrome, which was first reported by Denys et al. in 1967 is a complex disorder which associates a nephropathy, Wilms' tumor, and male pseudohermaphroditism. The common denominator is a nephropathy. The nephropathy may be associated with either genital abnormalities or Wilms' tumor, and these associations are called incomplete form of Drash syndrome. This syndrome appears early in life and the first sign usually is genital ambiguity. The nephropathy presents with proteinuria, hematuria and hypertension, and eventually progresses to end stage renal failure. Renal biopsy may reveal a variety of glomerular and interstitial changes. Wilms' tumor may appear as s mass on ultrasound or it may not be recognized until nephrectomy or even autopsy. We report on a boy with nephropathy and genital abnormalities. A nephrotic syndrome with hypertension was present when first seen at 15 days of age. The karyotype was 46, XY and external genitalia was ambiguous. The nephrotic syndrome and signs of renal insufficiency persisted and he died at the age of 40 days. Histopathologic findings of kidney at autopsy revealed those of diffuse mesangial sclerosis. The case was presented with brief review of literatures.
46, XY Disorders of Sex Development ; Autopsy ; Biopsy ; Denys-Drash Syndrome* ; Disorders of Sex Development ; Genitalia ; Hematuria ; Humans ; Hypertension ; Karyotype ; Kidney ; Male ; Nephrectomy ; Nephrotic Syndrome ; Proteinuria ; Renal Insufficiency ; Sclerosis ; Ultrasonography ; Wilms Tumor

Varieties of telepathology system had been developed and in use, but their functional capability and diagnostic accuracy are considered to be inferior to those of conventional optical microscope. This study is intended to find out: 1) the diagnostic accuracy and reproducibility rate according to the input devices and the video signals; 2) any potential technical problems of the telepathology system; 3) any possible physical and psychological impacts. We devised a virtual telepathology system using our existing microscope equipped with CCD camera unit that has no restriction of network speed. Total fifty-five surgical pathology cases from 11 different organs were selected. Three pathologists were involved in making diagnoses. The resulting diagnostic accuracies were: 1 CCD camera with composite video signal was 86.2%; 3 CCD camera with composite video signal was 93.1%; 3 CCD camera with component video signal was 95.0%. The 3 CCD camera with component video signal resulted in 95.0% diagnostic accuracy and was superior to 1 CCD camera with composite video signal. Some technical problems noted during this study were: the visual field of the virtual telepathology system was smaller by 43% than that of microscope; the difference of cell sizes between microscope and monitor; low resolution of image. Some physical and psychological symptoms were noted.
Cell Size ; Diagnosis ; Pathology, Surgical ; Telepathology* ; Visual Fields

No abstract available.
Pericardial Effusion* ; Radionuclide Imaging* ; Technetium Tc 99m Medronate*

BACKGROUND: McKeown first described two autopsy cases of esophageal small cell carcinoma (SMC) in 1952; about 230 cases have since been reported in the literature. Small cell carcinoma has been reported to account for 0.4% to 7.6% of all esophageal malignancies. SMC of the esophagus as regarded as having a poor prognosis with frequent systemic dissemination. Choice of treatment remains controversial. MATERIAL AND METHOD: From August 1987 to December 1998, a review of the records and histologic sections of 8 patients with primary small cell carcinoma of the esophagus seen in 11 years was undertaken. RESULT: Small cell carcinoma of the esophagus constituted 1.5% of all esophageal cancers. The median age was 61.5 years (range from 42 to 71 years). Seven patients were male, tumor was mainly located in the middle and lower thirds (6 cases) of the esophagus. Pure SMC is 5 cases, and mixed SMC is 3 cases. Operative procedure were as follow: transthoracic esophagectomy with thoracic or cervical reconstructon in 7 patients, transhiated esophagectomy with cervical reconstruction in one. The operative death was none. Adjuvant chemotherapy was performed in 7 patients except one who had poor general condition. Recurrence was observed in 4 patients (mediastinal LN, abdominal LN, SCN, bone). The overall median survival was 15.9 months. Only one patient survived for more than 5 years. CONCLUSION: We considered that esophageal SMC should be regarded as a systemic disease, and multimodality treatment including chemotherapy should be used. Surgery may be offered in selected patients to manage local disease as part of a chemotherapy based treatment program.
Autopsy ; Carcinoma, Small Cell* ; Chemotherapy, Adjuvant ; Drug Therapy ; Esophageal Neoplasms ; Esophagectomy ; Esophagus* ; Humans ; Male ; Prognosis ; Recurrence ; Surgical Procedures, Operative

Acetabular fractures assume great clinical importance because acetabular is weight bearing joint in the lower extremity. The undisplaced fractures of the acetabulum gave satisfactory results with closed method, but the treatment of displaced fractures have been controversial. A clinical analysis was performed on the 72 patients with displaced acetabular fractures, who had been treated at Soonchunhyang University Hospital from January 1980 to December 1989. The results obtained were as follows. 1. The prevalent age ranged from 21 to 50 years, which comprised 86.1% of all, and the ratio between male and female was 3:l. 2. The most common cause of injury was traffic accident, which comprised 76.4%. 3. According to Letournel's classification, simple fracture was 38 cases (52.8%), associated fracture was 34 cases (47.2%) and hip dislocation was associated in 28 cases (38.9%) and most common was posterior (20.8%). 4. Closed treatment was done in 47 cases and open treatment in 25 cases. 5. Results of open treatment group were better than closed treatment group in both clinical and roentgenographic study. 6. The complication were occured in 14 (29.8%) out of 47 cases of closed treatment group, and 10 (40.0%) out of 25 cases of open treatment group. 7. Not only choice of treatment but also determination of surgical approach require accurate assessment of the fracture pattern in this region of complex bony architecture by careful radilogic analysis.
Accidents, Traffic ; Acetabulum ; Classification ; Female ; Hip Dislocation ; Humans ; Joints ; Lower Extremity ; Male ; Methods ; Weight-Bearing

Retroperitoneal cystic lymphangiomas are uncommon tumors usually found incidentally at surgery, autopsy, orlymphography. When clinically significant, they usually present early in life as a palpable abdominal mass. Wediscribe an unusual case of a six-years old boy who was operated for a cystic lymphangioma of the right axilla atthe age of five months, and, six years later, was found to have a retroperitoneal cystic lymphangioma accompainedwith motor weakness of the lower extremities.
Autopsy ; Axilla ; Humans ; Lower Extremity ; Lymphangioma, Cystic ; Male

No abstract available.
Eustachian Tube*

A case of xanthoma of the liver in a 61-year-old Korean woman with multiple myeloma, hyperlipidemia and xanthoma of the skin is described. Microscopically, the liver showed a multiple xanthomatous collection of foamy histiocytes as well as diffuse sinusoidal infiltration of the foam cells. This hepatic accumulation of foam cells seems to be related to hyperlipidemia of the patient. The mechanism of hyperlipidemia in multiple myeloma is discussed.
Case Report ; Female ; Human ; Hyperlipidemia/blood/*complications ; Liver Diseases/blood/*complications ; Middle Age ; Multiple Myeloma/blood/*complications ; Xanthomatosis/blood/*complications

PURPOSE: Metallothionein is an intracellular cystein-rich thiol-containing protein. Increased metallothionein content in tumor cells has been suggested to be a mechanism of resistance to cisplatin. In most of previous studies evaluating the role of metallothionein in cisplatin resistance, tumor cells were usually exposed to cadmium to increase metallothionein content. Therefore, cisplatin resistance of the cells may be related to cadmium exposure itself, which induces various changes in cell characteristics, but not to increased metallothionein content. The purpose of this study is to evaluate the role of metallothionein content alone in cellular resistance to cisplatin without exposure of cells to cadmium. MATERIALS AND METHOD: We measured the toxicity of cisplatin in mouse NIH/3T3 cells that vary in their content of metallothionein as a consequence of transfection with a plasmid that result in the constitutive expression of metallothionein. MT cells were derived from NIH/3T3 cells by transfection with a plasmid containing the genome of bovine papilloma virus and the mouse metallothionein-I, derived by the promoter for the glucose-regulated protein of 78kD. Control cells were similary transfected with bovine papilloma virus-based plasmids with the gene for metallothionein inverted and thus separated from the promoter (TM), or deleted, along with promoter (BPA). The number of copies of the plasmid were similar in each kind of transfected cells. Expression of metallothionein required neither selection nor maintenance of cells in the presence of heavy metals. RESULTS: Synthesis of metallothionein was 15-fold greater in the MT cells than in the TM or BPA cells. The concentration of cisplatin sufficient to reduce the cells per well by one-half (IC-50) was 0.40+/-0.075 uM in MT cells. In TM and BPA cells, it was 0.36 0.035 uM and 0.423+/-0.032 uM. There were no significant differences in IC-50 between three cell lines. CONCLUSION: In spite of large differences between MT and control cells in their cellular content of metallothionein, no differences in resistance to cisplatin were observed.
Animals ; Cadmium ; Cell Line ; Cisplatin* ; Genome ; Metallothionein* ; Metals, Heavy ; Mice* ; Papilloma ; Plasmids ; Transfection

BACKGROUND: The preparation of conventional smears (CS) from mucoid samples, despite mucolysis, can pose difficulties for cytotechnologists or cytopathologists. In recent years, liquid-based cytology (LBC) devices have been developed in attempts to improve the cytopreparation process. LBC improves both sample collection and sample preparation. Cell block preparations (CB) can be made from residual tissue fluids, and are a useful adjunct to smears. METHODS: We retrospectively reviewed 3 preparations from 209 patients whose diagnosis was later confirmed via bronchoscopic biopsy, fine needle aspiration, gun biopsy or operation. Each case was categorized into one of three groups: "negative," "atypical or suspicious" and "malignant." RESULTS: When conflating the "atypical" and "malignant" categories into a "positive" category, the sensitivity of each preparation was 74.4% in LBC, 72.9% in CS, and 76.5% in CB preparations. Specificity was 98.7%, 94.7% and 98.7%, respectively. By combining LBC and CB, the sensitivity is 78.2%. CONCLUSIONS: Among three different preparation methods, sensitivity is highest in the CB method. LBC has many advantages in evaluating cell morphology and by combining CB method, the sensitivity can be improved slightly. The application of all three methods may prove helpful when one or another method proves diagnostically inconclusive.
Biopsy ; Biopsy, Fine-Needle ; Humans ; Lung ; Lung Neoplasms ; Retrospective Studies ; Sensitivity and Specificity