We report four cases of hyalinosis cutis et mucosae. They developed hosrseness in their infancy and all had past history of frequent skin infections with accompanying varioliform scars. On physical examination, all the patients had beaded papules along their eyelid margins, hoarseness, varioliform scars and various skin and mucous membrane infiltrations. Pathologic examinations performed on skin infiltrations and eyelid papules revealed diastase resistant periodic acid Schiff positive materials deposited mainly in upper dermis. We have given three of four patients 1g/day of dimethyl sulphoxide from 6 to 12 months with no significant side effects nor remarkable clinical improvement.
Amylases ; Cicatrix ; Dermis ; Dimethyl Sulfoxide ; Eyelids ; Hoarseness ; Humans ; Lipoid Proteinosis of Urbach and Wiethe* ; Mucous Membrane ; Periodic Acid ; Physical Examination ; Skin

No abstract available.
Incidence* ; Nurseries* ; Parenteral Nutrition, Total* ; Pharmacy* ; Sepsis*

No abstract available.
Hepatolenticular Degeneration*

Melanosis coli is the brownish pigmentation of the colon associted with the ingestion of anthraquinone compounds as iaxatives. The brownish discoloration of the colon mucosa is due to accumulation of macrophage containing lipofuscin pigment in the lamina propria. This is the one of the complications of laxative abuse, but the pigments disappear by withdrawing the anthraquinone. We report a case of malanosis coli histologically confirmed by fibersigmoidoscopic biopsy in a 70-year-old female patient consuming anthraquinone compound for twelve months with a review of the literature.
Aged ; Biopsy ; Colon ; Eating ; Female ; Humans ; Lipofuscin ; Macrophages ; Melanosis* ; Mucous Membrane ; Pigmentation

Carcinosarcoma of the female genital tract, also called malignant mixed mullerian tumor (MMMT), is a rare and relatively aggressive tumor with various homologous and heterologous components. There have been various studies to find prognostic factors and to investigate the histogenesis, including tissue culture, electron microscopy, and immunohistochemical studies. We investigated carcinomatous, sarcomatous, and transitional areas of 6 cases of carcinosarcoma of the uterus and ovary by using epithelial and mesenchymal markers. Immunohistochemical profiles of the transitional areas were significantly different from those of carcinomatous and sarcomatous areas. Immunoreactivities for cytokeratin and epithelial membrane antigen (50% and 22.2%) were weaker than those of carcinomatous areas (95.2% and 100%), but stronger than those of sarcomatous areas (11.1% and 5.6%)(p<0.01). In transitional areas, vimentin, smooth muscle actin and S-100 protein were more strongly expressed than in carcinomatous areas, but more weakly expressed than in sarcomatous areas (p<0.01, p<0.01, and p=0.018, respectively). Myoglobin was entirely negative in carcinomatous areas and immunoreactive in minor portions of transitional and sarcomatous areas (22.2% and 16.7%, respectively). These results suggest that the transitional areas are between the carcinomatous and sarcomatous nature in differentiation, further supporting that the carcinosarcomas of the female genital tract may arise, through metaplastic change, from a type of carcinoma.
Actins ; Carcinosarcoma* ; Female* ; Humans ; Immunohistochemistry ; Keratins ; Metaplasia ; Microscopy, Electron ; Mucin-1 ; Muscle, Smooth ; Myoglobin ; Ovary ; S100 Proteins ; Uterus ; Vimentin

Purpose: To evaluate whether vascular uptake on 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18F-FDG PET/CT) during the clinically inactive state of Takayasu arteritis (TAK) is associated with disease relapse. Materials and Methods: Patients with TAK who underwent 18F-FDG PET/CT during the clinically inactive state of the disease between 2006 and 2019 were included. Clinically inactive disease was defined as a status not fulfilling the National Institutes of Health (NIH) criteria for active disease in TAK. Relapse was defined as recurrence of clinically active disease after a clinically inactive period, requiring change in the treatment regimen. Vascular uptake on 18F-FDG PET/CT was assessed using target/background ratio (TBR), calculated as arterial maximum standardized uptake value (SUV)/mean SUV in venous blood pool. Multivariable Cox regression analysis was performed to identify factors associated with relapse. Results: A total of 33 patients with clinically inactive TAK were included. During a median observation period of 4.5 (0.9–8.1) years, relapse occurred in 9 (27.3%) patients at median 1.3 (0.7–6.9) years. Notably, TBR [1.5 (1.3–1.8) vs. 1.3 (1.1–1.4), p=0.044] was significantly higher in patients who relapsed than in those who did not. On multivariable Cox regression analysis, the presence of NIH criterion 2 [adjusted hazard ratio (HR): 7.044 (1.424–34.855), p=0.017] and TBR [adjusted HR: 11.533 (1.053–126.282), p=0.045] were significantly associated with an increased risk of relapse. Conclusion Vascular uptake on 18F-FDG PET/CT and the presence of NIH criterion 2 are associated with future relapse in patients with clinically inactive TAK.

Purpose: To evaluate whether vascular uptake on 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18F-FDG PET/CT) during the clinically inactive state of Takayasu arteritis (TAK) is associated with disease relapse. Materials and Methods: Patients with TAK who underwent 18F-FDG PET/CT during the clinically inactive state of the disease between 2006 and 2019 were included. Clinically inactive disease was defined as a status not fulfilling the National Institutes of Health (NIH) criteria for active disease in TAK. Relapse was defined as recurrence of clinically active disease after a clinically inactive period, requiring change in the treatment regimen. Vascular uptake on 18F-FDG PET/CT was assessed using target/background ratio (TBR), calculated as arterial maximum standardized uptake value (SUV)/mean SUV in venous blood pool. Multivariable Cox regression analysis was performed to identify factors associated with relapse. Results: A total of 33 patients with clinically inactive TAK were included. During a median observation period of 4.5 (0.9–8.1) years, relapse occurred in 9 (27.3%) patients at median 1.3 (0.7–6.9) years. Notably, TBR [1.5 (1.3–1.8) vs. 1.3 (1.1–1.4), p=0.044] was significantly higher in patients who relapsed than in those who did not. On multivariable Cox regression analysis, the presence of NIH criterion 2 [adjusted hazard ratio (HR): 7.044 (1.424–34.855), p=0.017] and TBR [adjusted HR: 11.533 (1.053–126.282), p=0.045] were significantly associated with an increased risk of relapse. Conclusion Vascular uptake on 18F-FDG PET/CT and the presence of NIH criterion 2 are associated with future relapse in patients with clinically inactive TAK.

No abstract available.
Child* ; Humans ; Lung Diseases, Interstitial*

We report a 58-year-old man who developed synchronous gastric non-Hodgkin`s lymphoma (NHL) and renal cell carcinoma. He presented with epigastric discomfort for 2 months. Endoscopic finding of the stomach disclosed a large inegular ulceration with nodular margin on the upper body. Constrast enhanced CT scan of the abdomen showed an ulceration and focal wall thickening in the greater curvature side of stomach, and an enhanced bulging mass in the left kidney incidentally. The tissue obtained by radical proximal gastrectomy and nephrectomy showed diffuse large B-cell lymphoma on stomach and chromophobic type of renal cell carcinoma on kidney. To our knowledge, this is the first report of synchronous gastric NHL and renal cell carcinoma in Korea.
Abdomen ; Carcinoma, Renal Cell* ; Gastrectomy ; Hodgkin Disease* ; Humans ; Kidney ; Korea ; Lymphoma ; Lymphoma, B-Cell ; Middle Aged ; Nephrectomy ; Stomach ; Tomography, X-Ray Computed ; Ulcer

No abstract available.