Parry-Romberg syndrome(Progressive hemifacial atrophy), described in the last century by Parry(1825) and Romberg(1846), is a very rare disorder characterized by a slowly progressive and self-limited unilateral(rarely bilateral) atrophy of the faces affecting variably the skin, subcutaneous fat tissues, musculature, connective tissue, cartilage and bones. And this disorder is usually accompanied by contralateral Jacksonian epilepsy, trigerminal neuralgia, and changes in the eyes and hair. The onset is slow and progressive, starting at 5-15 years of age and lasting from 2-10 years, ending with the face being "burned out". There are a few cases of this disease which presented during the neonatal period. This disorder seems to affect females more than males, and its etiology and incidence has yet to be determined. Trauma, infection with a slow virus, sympathetic dysfunction, immunological abnormality and cranial vascular malformation are proposed causes. No typical or consistent neuropathologic findings occur. No specific treatment for the syndrome exists; however, various reconstructive surgical procedures can have in reasonably good cosmetic effects, as well as antiinflammatory or immunosuppressive treatment. We report a case of Parry-Romberg syndrome, which was presented at 1 month of age, and has progressd to contralateral hemiparesis.
Atrophy ; Cartilage ; Connective Tissue ; Epilepsy ; Facial Hemiatrophy* ; Female ; Hair ; Humans ; Incidence ; Infant, Newborn* ; Male ; Neuralgia ; Paresis ; Reconstructive Surgical Procedures ; Skin ; Subcutaneous Fat ; Vascular Malformations

Idiopathic myelofibrosis (IMF), which is characterized by marrow fibrosis, leukoerythroblastic anemia, teardrop poikilocytosis and splenomegaly due to extrumedullary hematopoiesis, has known to have no form of therapy. On the ground of the possibility of reversing collagen deposion in IMF using 1, 25dihydroxycholecalciferol [1, 25(OH)2D3], we report here our observations of 5 patients (M:F=1:4) with IMF before and after treatment with 0.5 microgram/day of alfacalcidol, precursor of 1, 25(OH)2D3. In 3 fo 5 patients the hemoglobin rose and in 4 of 5 the platelet count increased. Follow-up marrow examination revealed that marrow trephine reticulin fibrosis decreased according as the amelioration of clinical and laboratory findings. But these did not persist except one patient in spite of the sustained use of alfacalcidol. Our results suggest that alfacalcidol may have a therapeutic role in some patients with IMF. More extensive studies will be clarify the action of alfacalcidol in IMF.
Anemia, Myelophthisic ; Bone Marrow ; Child* ; Collagen ; Fibrosis ; Follow-Up Studies ; Hematopoiesis ; Humans ; Platelet Count ; Primary Myelofibrosis* ; Reticulin ; Splenomegaly

Eight cases of chondroblastoma were studied by analyzing the clinical and pathologic findings. The age of eight cases ranged from 17 to 38 years old(median age, 22.7 years old). The tumors developed in the femur (3 cases), patella (2 cases), tibia( 1 case), fibula (1 case), and ulna (1 case). The mean diameter of tumors was 4.0 cm (range, 1.5 to 8.0 cm). Grossly, tumors showed grayish brown solid area with foci of secondary aneurysmal bone cyst. Histologically, the tumor cells were round or polygonal in shape with nuclear groove. And there were chondroid differentiation(7 cases), mitosis(3 cases), calcific deposits(3 cases), secondary aneurysmal bone cyst(4 cases), hemosiderin deposits(4 cases), necrosis(3 cases), vascular invasion(1 caes) and foamy histiocytes and cholesterol cleft(1 cases). All cases showed no metastasis to lymph node and distant organ. Seven cases (87.5 %) were immunoreactive for S-100 protein. None were immunoreactive for cytokeratin.
Aneurysm ; Bone Cysts ; Cholesterol ; Chondroblastoma* ; Femur ; Fibula ; Hemosiderin ; Histiocytes ; Keratins ; Lymph Nodes ; Neoplasm Metastasis ; Patella ; S100 Proteins ; Ulna

This study was designed to investigate the effect of cholestyramine on the formation of pigment gallstones in high carbohydrate diet-fed hamsters and whether that effect occurred because of cholecystokinin action. Forty seven hamsters were divided into three groups: group I(n = 16) was fed on normal rodent chow(43% carbohydrate), group II(n = 14) was fed on a high CHO diet(65% carbohydrate), group III(n = 17) was fed on a high CHO diet containing 4% cholestyramine. Gallstones developed in 0% of group I, 42.9% of group II and 5.9% of group III(P< 0.05, group II vs III). To evaluate the chronic status of cholecystokinin level, the wet weight of pancreas and the average area of pancreatic acinar in microscopic high power field were measured. There was no significant difference between group II and group III in pancreatic weight and average area of pancreatic acinar(P> 0.05). In gallbladder bile analysis, there was also no significant difference between group II and group III in cholesterol, phospholipid, total calcium, total bilirubin and bile acid levels. In conclusion, cholestyramine decreases the frequency of pigment gallstone formation in high CHO diet-fed hamsters, but it is not clear whether the mechanism of cholestyramine decreasing the gallstone formation is due to the action of cholecystokinin.
Animal ; Bilirubin/metabolism ; Cholecystokinin/*analysis ; Cholelithiasis/*pathology ; Cholesterol/metabolism ; Cholestyramine/*administration & dosage ; Dietary Carbohydrates/*administration & dosage ; Female ; Gallbladder/*metabolism/pathology ; Hamsters ; Male ; Mesocricetus ; Organ Weight ; Pancreas/physiopathology ; Phospholipids/metabolism ; Pigmentation ; Support, Non-U.S. Gov't

PURPOSE: It is the general conception that chronic prostatitis is initiated by urethritis. However, in the actual clinical situations, many male patients without histories of urethritis. To evaluate the possible relationship, a review of patients with chronic prostatitis was carried out. MATERIALS AND METHODS: Three hundred consecutive patients with prostatitis were reviewed retrospectively concerning the duration of prostatits, any history of urethritis, and the history of intercourse, including that with a high-risk partner(e.g., prostitute). RESULT: A history of urethritis was found in 216 of the patients(72%),but 30 patients(10%) denied both high-risk sexual intercourse and urethritis. In 54 patients (18%), there was no history of urethritis but a recognized sexual encounter with a high-risk partner. CONCLUSIONS: Having urethritis may not be an important factor in chronic prostatitis. This information could be most comforting to those who are afflicted with chronic prostatitis and concerned about the nature of their disease. Chronic prostatitis can be explained simply as an inflammation of prostate, not a dreadful end result of sexually transmitted disease.
Coitus ; Fertilization ; Humans ; Inflammation ; Male ; Prostate ; Prostatitis* ; Retrospective Studies ; Sexually Transmitted Diseases ; Urethritis*

PURPOSE: It is the general conception that chronic prostatitis is initiated by urethritis. However, in the actual clinical situations, many male patients without histories of urethritis. To evaluate the possible relationship, a review of patients with chronic prostatitis was carried out. MATERIALS AND METHODS: Three hundred consecutive patients with prostatitis were reviewed retrospectively concerning the duration of prostatits, any history of urethritis, and the history of intercourse, including that with a high-risk partner(e.g., prostitute). RESULT: A history of urethritis was found in 216 of the patients(72%),but 30 patients(10%) denied both high-risk sexual intercourse and urethritis. In 54 patients (18%), there was no history of urethritis but a recognized sexual encounter with a high-risk partner. CONCLUSIONS: Having urethritis may not be an important factor in chronic prostatitis. This information could be most comforting to those who are afflicted with chronic prostatitis and concerned about the nature of their disease. Chronic prostatitis can be explained simply as an inflammation of prostate, not a dreadful end result of sexually transmitted disease.
Coitus ; Fertilization ; Humans ; Inflammation ; Male ; Prostate ; Prostatitis* ; Retrospective Studies ; Sexually Transmitted Diseases ; Urethritis*

Enterococcus hirae infection is rare in humans. We describe a case of pyelonephritis that was caused by Enterococcus hirae in a 57-year-old woman. E. hirae was isolated from both blood and urine. This is the 2nd case report on bacteremia caused by E. hirae in Korea.
Bacteremia ; Enterococcus ; Female ; Humans ; Korea ; Middle Aged ; Pyelonephritis

Enterococcus hirae infection is rare in humans. We describe a case of pyelonephritis that was caused by Enterococcus hirae in a 57-year-old woman. E. hirae was isolated from both blood and urine. This is the 2nd case report on bacteremia caused by E. hirae in Korea.
Bacteremia ; Enterococcus ; Female ; Humans ; Korea ; Middle Aged ; Pyelonephritis

In 1861 Rokitanski laid down the law that the pyloric ring served as a barrier to the duodenal spread of gastric cancer. Although this theory was generally accepted, direct spread of gastric carcinoma into the duodenum has been reported. We report a case of gastric cancer spreading into the duodenum that was diagnosed by endoscopic duodenal biopsy and review the literature.
Biopsy ; Duodenum* ; Endoscopy ; Jurisprudence ; Stomach Neoplasms