Rotor syndrome is a rare disease of hereditary hyperbilirubinemia transmitted with autosomal recessive trait. In general, Rotor syndrome shows direct hyperbilirubinemia and there has been several reports since Sons's report in 1966, in Korea. A 34-year-old female was admitted with the chief complaint of intermittent icteric sclera for 24 years. There was no family history of jaundice. Rotor syndrome was diagnosed by oral cholecystogram, BSP retention test, 99mTc-DISIDA scan, liver biopsy and electron microscopy study of liver biopsy specimen. We report this case with brief review of the literature.
Adult ; Biopsy ; Female ; Humans ; Hyperbilirubinemia ; Hyperbilirubinemia, Hereditary* ; Jaundice ; Korea ; Liver ; Microscopy, Electron ; Rare Diseases ; Sclera ; Technetium Tc 99m Disofenin

OBJECTIVE: To evaluate the effects of recombinant FSH (rFSH) and urinary FSH (uFSH) on the gene expressions of human endometrial stromal cells in vitro. METHODS: Endometrial tissue was obtained from a pre-menopausal women undergoing hysterectomy. Primary endometrial stromal cells were isolated and in vitro cultured with FBS-free DMEM/F-12 containing 0, 10, 100, and 1,000 mIU/ml of rFSH and uFSH for 48 hours, respectively. Total RNA was extracted from the cultured cells and subjected to real time RT-PCR for the quantitative analysis of progesterone receptor (PR), estrogen receptor alpha/beta (ER-alpha/beta), cyclooxygenase 2 (Cox-2), leukemia inhibitory factor (LIF), homeobox A10-1 and -2 (HoxA10-1/-2). RESULTS: Both hormone treatments slightly increased (< 3 folds) the expressions of PR, ER-beta and HoxA10-1/-2 gene. However, ER-alpha expression was increased up to five folds by treatments of both FSH for 48 hours. The LIF expression by the 10 mIU/ml of uFSH for 12 hours was significantly higher than that of rFSH (p<0.01). After 24 hours treatment of two kinds of hormones, the expression patterns of LIF were similar. The 100 and 1,000 mIU/ml of rFSH induced significantly higher amount of Cox-2 expression than those of uFSH, respectively (p<0.05). CONCLUSION: This study represents no adversely effect of exogeneous gonadotropins, rFSH and uFSH, on the expression of implantation related genes. We suggest that rFSH is applicable for the assisted reproductive technology without any concern on the endometrial receptivity.
Cells, Cultured ; Cyclooxygenase 2 ; Estrogens ; Female ; Gene Expression* ; Genes, Homeobox ; Gonadotropins ; Humans* ; Hysterectomy ; Leukemia Inhibitory Factor ; Receptors, Progesterone ; Reproductive Techniques, Assisted ; RNA ; Stromal Cells*

BACKGROUND: The testes are one of the most common extramedullary sites of relapse in boys with acute lymphoblastic leukemia(ALL). The reported incidence of isolated testicular relapse varies from 3 to 40%. If these patients are treated exclusively with testicular irradialion, a systemic relapse occurs within a few months. Recently, the use of intensive chemotherapy and testicular irradiation improved the survival rate for boys with testicular leukemia. So, we performed this study to identify clinical manifestations, disease free survival and prognostic factors of testicular leukemia in children. METHODS: We reviewed 33 patients of testicular leukemia among total 410 boys with ALL diagnosed at the Department of Pediatrics, Seoul National University Children's Hospital from Jan. 1970 to Aug. 1996. Testicular leukemia was confirmed by testicular biopsy in all 33 patients. These patients were treated with combined local testicular irradiation(2,400~2,500 cGy/8~12fractions) and systemic chemotherapy. Two patients, in whom testicular relapse was diagnosed before 1979, unilateral orchiectomy of the involved site and testicular irradiation of the opposite site were performed. Probability estimates of disease free survival (DFS) were calculated by the method of Kaplan and Meier, and the relationship of prognostic factors to DFS was compared using the chi-square test in survival analysis. RESULTS: In 410 boys with ALL, testicular leukemia occurred in 33 patients(8%). Of 33 patients, 6 patients presented with testicular involvement at initial diagnosis, 16 patients had testicular relapse while still receiving chemotherapy and 11 patients had testicular relapse 3 to 57 months(median : 15 months) after cessation of chemotherapy. The median age of 33 patients was 7.4 yrs(9 months~18 yrs) and median WBC count 7,600/ L(2,700~270,000/L). All patients presented with painless testicular enlargement and testicular leukemia was confirmed by testicular biopsy. Among 33 patients, 2 had prior CNS relapse and 11 had concomitant bone marrow and/or CNS relapse. Twenty nine patients were treated with combined local testicular irradiation and systemic chemotherapy. Eleven had second relapse(6 bone marrow, 3 CNS, 2 opposite testis). Seventeen have been followed until now: 6 patients on chemotherapy and 11 patients(37.9%) in complete remission for 48.5+/-22.3 months(19~86 months). The 3 year DFS for 29 patients was 55.3%+/-10.1%. The following prognostic factors showed no significant association with DFS in testicular relapse : age and WBC count at initial diagnosis, age at testicular relapse, and concomitant relapse. Whether testicular relapse occurred on initial therapy or off initial therapy has prognostic value in predicting DFS. The 3 year DFS for boys with testicular relapse on and off initial therapy were 40.0%+/-12.9% and 78.8%+/-13.4%, respectively(P: 0.046). CONCLUSION: With the use of chemotherapy and testicular irradiation, prolonged second re mission can be achieved in many patients with testicular leukemia. The patients with testicular relapse off initial therapy fared significantly better than patients on therapy. So, to improve the DFS for boys with testicular leukemia, a better understanding of its biology and prognostic factors is needed.
Biology ; Biopsy ; Bone Marrow ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Leukemia ; Missions and Missionaries ; Orchiectomy ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence ; Seoul ; Survival Rate ; Testis

PURPOSE: Various methods of scleral fixation for the cases of posterior chamber intraocular lens (PCL) dislocation or subluxation into the vitreous cavity had been developed, and in the recent year a modification of internal scleral fixation using a hollow-bore needle has been introduced. The authors endeavored to evaluate clinical usefulness of the method. METHODS: The study was conducted for four cases of PCL dislocation or subluxation. A 10-0 polypropylene suture was threaded up the internal shaft of a 30 G straight needle and retrieved. The needle containing this suture was then inserted through the bed of the partial-thickness scleral flap 1.5 mm posterior to the limbus. The loop of suture was hooked with haphic of PCL. After the needle was retracted, the haptic was captured and the suture was tied to the sclera. The same maneuver was performed for the haptic in the opposite side. RESULTS: Except for pars plana vitrectomy, time taken for the above procedure was 10~15 minutes. Postoperatively induced astigmatism of all 4 cases were less than 3 diopters on the second postoperative day. No specific complication associated with this procedure was noted. IOL remained stable without tilting or rotation 18 months after procedure. CONCLUSIONS: This modified internal scleral fixation technique must be a convenient and effective procedure for repositioning posteriorly dislocated PCL.
Astigmatism ; Dislocations* ; Lenses, Intraocular* ; Needles* ; Polypropylenes ; Sclera ; Sutures ; Vitrectomy

OBJECTIVE: This study evaluated the effects of nitric oxide (NO) on the proliferation and differentiation of human periodontal ligament cells involved in orthodontic tooth movement. METHODS: A range of concentrations of sodium nitroprusside (SNP), a NO donor, were administered to samples of human periodontal ligament cells, followed by measurement of cell viability, alkaline phosphatase (ALP) activity, and expression of osteonectin and bone sialoprotein. RESULTS: Cell viability, ALP activity and the expression of osteonectin and bone sialoprotein were increased in human periodontal ligament cells treated with SNP concentrations of < 0.2 mM compared with controls, but were decreased with SNP concentrations of > 1.0 mM. CONCLUSION: NO has a biphasic effect on proliferation and differentiation in human periodontal ligament cells, with a stimulatory effect at low concentrations and an inhibitory effect at high concentrations.
Alkaline Phosphatase ; Cell Survival ; Humans* ; Integrin-Binding Sialoprotein ; Nitric Oxide* ; Nitroprusside ; Osteonectin ; Periodontal Ligament* ; Tissue Donors ; Tooth Movement

No abstract available.

BACKGROUND: Skin organ culture is widely used as a tool to investigate skin biology or skin disease. OBJECTIVE: The objective of the present study was to develop an ideal skin organ culture model for evaluation of melanin pigmentation. METHODS: An air-liquid interface and submerged method were used. The histology of the cultured skin was studied with H&E stain. To examine the epidermal pigmentation, Fontana-Masson stain and NKI/beteb stain were performed. Pigment modifiers (arbutin, LY294002) were applied to the culture medium for 3 days as an air-liquid interface culture. RESULTS: The general architecture of the skin was well maintained for 5 days. The melanin pigment decreased during culture without change of the number of melanocytes. As expected from previous reports, the effect of pigment modifiers (arbutin, LY294002) on cultured skin was demonstrated. CONCLUSION: The results indicate that this skin organ culture model is useful in evaluating the melanin pigmentation
Biology ; Melanins* ; Melanocytes ; Organ Culture Techniques* ; Pigmentation* ; Skin Diseases ; Skin*

Aldosterone-producing adrenal adenoma can induce various clinical manifestations as a result of chronic exposure to aldosterone. We report a rare case of a 37-year-old man who complained of general weakness and polyuria. He was diagnosed with aldosterone-producing adrenal adenoma and nephrogenic diabetes insipidus. Aldosterone enhances the secretion of potassium in the collecting duct, which can lead to hypokalemia. By contrast, nephrogenic diabetes insipidus, which manifests as polyuria and polydipsia, can occur in several clinical conditions such as acquired tubular disease and those attributed to toxins and congenital causes. Among them, hypokalemia can also damage tubular structures in response to vasopressin. The patient's urine output was > 3 L/d and was diluted. Owing to the ineffectiveness of vasopressin, we eventually made a diagnosis of nephrogenic diabetes insipidus. Laparoscopic adrenalectomy and intraoperative kidney biopsy were subsequently performed. The pathologic finding of kidney biopsy revealed a decrease in aquaporin-2 on immunohistochemical stain.
Adenoma ; Adrenalectomy ; Adult ; Aldosterone ; Aquaporin 2 ; Biopsy ; Diabetes Insipidus ; Diabetes Insipidus, Nephrogenic* ; Diagnosis ; Humans ; Hyperaldosteronism* ; Hypokalemia ; Kidney ; Polydipsia ; Polyuria ; Potassium ; Vasopressins

Backgroud/Aims: Endoscopic stent placement is widely used to treat an unresectable malignant gastric outlet obstruction. The covered stent has the disadvantage of an increased risk of migration, and the uncovered stent has an increased risk of ingrowth. This study examined the technical and clinical efficiency of stent placement of a double-layered combination pyloric stent that was newly designed to reduce tumor ingrowth and stent migration. METHODS: Fifteen patients with a gastric outlet obstruction caused by unresectable stomach cancer were treated with the endoscopic placement of a double-layered combination pyloric stent (an outer uncovered stent to reduce migration and an inner PTEF-covered stent to prevent tumor ingrowth). The technical success, clinical success, and complication especially tumor ingrowth and stent migration were analyzed. RESULTS: Technical success was achieved in 15 out of 15 (100%) patients. Among the 15 patients in whom endoscopic stenting was placed successfully, the clinical success rate was 93.3%, the incidence of tumor ingrowth was 0%, the rate of migration was 6.7%, and tumor overgrowth was observed in 13.3%. The median stent patency period was 105 days. CONCLUSIONS: The placement of a double- layered pyloric combination stent appears to be effective in overcoming the disadvantage of the increased migration observed for a covered stent and the increased ingrowth observed for the uncovered stent.
Gastric Outlet Obstruction* ; Humans ; Incidence ; Stents* ; Stomach Neoplasms* ; Stomach*

PURPOSE: We have undertaken this study to evaluate the effects of induction chemotherapy involving BH-AC, idarubicin, and 6-thioguanine (6-TG). METHODS: BH-AC 300mg/m2/day was administered intravenously over three hours for seven consecutive days. Idarubicin 12mg/m2/day was administered intravenously for three days. 6-TG 100 mg/m2/day was administered orally for seven days. Intrathecal ara-C was administered on the first day of treatment. RESULTS: Complete remission (CR) was achieved in 18 cases (66.7%), partial remission (PR) was achieved in 2 cases (7.4%). In previously untreated patients, complete reimission rate was 92.9% (13/ 14), in relapsed patients, 40% (2/5) and in the refractory patients, 37.5% (3/8). The remission duration until December 1996 was 45 to 630 days (median 133). Duration of the neutropenia (ANC<500/microliter) was 0 to 38 days (median 24). Side effects were nausea, vomiting (7/27, 25.9%), liver dysfunction (1/27, 3.7%), skin eruption (1/27, 3.7%), and mucositis (1/27, 3.7%). In all cases, fever developed in the neutropenic state (culture proven sepsis in 5 cases). Death occurred in 5 cases who achieved CR due to sepsis after chemotherapy (4 cases), intracerebral hemorrhage after bone marrow relapse (1 case). CONCLUSION: BH-AC, idarubicin, and 6-TG induction chemotherapy could be a useful induction chemotherapy treatment that combines supportive care for infection and bleeding.
Bone Marrow ; Cerebral Hemorrhage ; Cytarabine ; Drug Therapy ; Fever ; Hemorrhage ; Humans ; Idarubicin* ; Induction Chemotherapy* ; Leukemia, Myeloid, Acute* ; Liver Diseases ; Mucositis ; Nausea ; Neutropenia ; Recurrence ; Sepsis ; Skin ; Thioguanine* ; Vomiting