PURPOSE: To report a patient with Freeman-Sheldon syndrome with blepharophimosis. METHODS: A 4-year-old girl with congenital facial abnormalities consistent with Freeman-Sheldon syndrome presented with complaints of blepharophimosis. The characteristic features of microstomia, down-slanting palpebral fissure, blepharoptosis, and telecanthus were also found. Y-V epicanthoplasty and levator aponeurosis resection were performed. RESULTS: Surgical intervention to correct ptosis and telecanthus led to initially fair cosmetic results, but one month later an unexpected decrease in interpalpebral fissure height was noted. CONCLUSIONS: Freeman-Sheldon syndrome with blepharophimosis is very rare. It was necessary to correct blepharoptosis, telecanthus, and blepharophimosis in the oculoplastic service in this case.
Blepharophimosis ; Blepharoptosis ; Child, Preschool ; Female ; Humans ; Microstomia

BACKGROUND AND OBJECTIVES: Interleukin (IL)-10 is a major regulator of innate immunity. It interferes with the production of inflammatory mediators by reacting with polymorphonuclear neutrophils, monocytes and macrophages. In addition, it also up-regulates the ex-pression of molecules that amplify the anti-inflammatory effect of IL-10. This study tested the hypothesis that injection of various concentrations of exogenous IL-10 in the middle ear cleft can inhibit the acute stage of otitis media induced by lipopolysaccharide (LPS). And if so, the level of concentration for inhibition can be determined. MATERIALS AND METHOD: IL-10 and/or LPS were injected transtympanically into the middle ear cavity of normal mice. Histopathological measurement of submucosal thickness and num-ber of inflammatory cell infiltration was performed at 24 hours after inoculation of IL-10 and/or LPS. RESULTS: At 24 hours after inoculation, every group of IL-10 inoculation reduced both thickening of the mucous and submucosal infiltration of inflammatory cells in a dose-dependent manner. As the concentrations of IL-10 inoculated increased, mucosal thickness and submucosal infiltration of inflammatory cells were more reduced. CONCLUSION: This study demonstrated that exogenous IL-10 injection into the middle ear can directly reduce LPS-induced otitis media in acute stage in a dose-dependent manner. These findings also indicate that IL-10 may be a candidate for local modulator of acute otitis media.
Animals ; Ear, Middle ; Immunity, Innate ; Interleukin-10 ; Interleukins ; Macrophages ; Mice ; Monocytes ; Neutrophils ; Otitis ; Otitis Media

BACKGROUND: An oral glucose tolerance test (OGTT) is the current method used for screening and diagnosis of gestational diabetes mellitus (GDM). OGTT is a relatively complicated procedure and is expensive. Thus, new strategies that do not require fasting or more than a single blood draw may improve the diagnosis of GDM and increase the rate of GDM testing. We investigated the utility of monitoring glycosylated hemoglobin (HbA1c) levels for the diagnosis of GDM. METHODS: The data from 992 pregnant women with estimated gestational ages ranging from 24 to 28 weeks were retrospectively reviewed. There were 367 women with plasma glucose levels > or =140 mg/dL 1 hour after a 50-g OGTT. GDM was diagnosed according to the Carpenter-Coustan criteria for a 3-hour 100 g OGTT. A HbA1c assessment was performed at the same time. RESULTS: We enrolled 343 women in this study, and there were 109 women with GDM. The area under the curve the receiver operating characteristic curve for HbA1c detection of GDM was 0.852 (95% confidence interval, 0.808 to 0.897). A HbA1c cutoff value > or =5.35% had maximal points on the Youden index (0.581). The sensitivity was 87.2% and the specificity was 70.9% for diagnosing GDM. A threshold value > or =5.35% indicated that 163 patients had GDM and that 68 (41.7%) were false positive. The positive predictive value was 58.3% at this threshold value. CONCLUSION: Despite substantial progress in methodology, HbA1c values cannot replace OGTT for the diagnosis of GDM.
Blood Glucose ; Diabetes, Gestational* ; Diagnosis* ; Fasting ; Female ; Gestational Age ; Glucose Tolerance Test ; Hemoglobin A, Glycosylated* ; Humans ; Mass Screening ; Pregnancy ; Pregnant Women ; Retrospective Studies ; ROC Curve ; Sensitivity and Specificity

Although various combinations of chemotherapy regimens have been tried for patients with esophageal cancer, their duration of survival is extremely poor. In this study, we investigated the safety and clinical efficacy of paclitaxel and cisplatin chemotherapy in metastatic or recurrent esophageal cancer. 32 patients enrolled in this study and the median age was 60 yr. Of all the 32, 28 patients (88%) had been treated previously, 22 of them with chemotherapy or radiation therapy. All patients in the study received biweekly paclitaxel (90 mg/m2) followed by cisplatin (50 mg/m2). One patient (3%) responded completely, and 12 patients (38%) showed a partial response; in 9 patients (28%) the disease remained stable, and in 10 patients (31%) it progressed. The objective response rate was 41%. The median duration of response was 4.8 months, and the median overall survival in all patients was 7 months. The 1-yr and 2-yr survival rates were 28.1% and 7.1%, respectively. Grade 3 or 4 of neutropenia and anemia were observed in 6 (19%) and 5 (16%) patients, respectively. The major non-hematologic toxicity was fatigue, but most of them could manageable. In conclusion, biweekly paclitaxel and cisplatin is effective in patients with metastatic or recurrent esophageal cancer.
Aged ; Anemia/chemically induced ; Antineoplastic Combined Chemotherapy Protocols/adverse effects/*therapeutic use ; Bone Neoplasms/drug therapy/secondary ; Cisplatin/administration & dosage/adverse effects ; Diarrhea/chemically induced ; Esophageal Neoplasms/*drug therapy/pathology ; Fatigue/chemically induced ; Humans ; Liver Neoplasms/drug therapy/secondary ; Lung Neoplasms/drug therapy/secondary ; Lymphatic Metastasis ; Male ; Middle Aged ; Nausea/chemically induced ; Neoplasm Recurrence, Local ; Paclitaxel/administration & dosage/adverse effects ; Survival Analysis ; Thrombocytopenia/chemically induced ; Time Factors ; Treatment Outcome ; Vomiting/chemically induced

Ileovesical fistula is a very rare clinical entity, the most frequent cause of which is Crohn's disease. Furthermore, it is an exceptionally rare complication of malignancies. We experienced one case of ileovesical fistula which had been caused by hepatocellular carcinoma (HCC) arising from the noncirrhotic liver. A 27-year-old man was diagnosed with HCC in a noncirrhotic liver. Despite treatment with transarterial chemoembolization (TACE), the disease status became more aggravated. The patient complained of dysuria, fecaluria, and intermittent lower abdominal pain. Pelvic CT scan showed a soft tissue mass of 6 cm abutting on the distal ileum which was downwardly displaced. Barium study of the small bowel showed a fistula between the small bowel loop and the urinary bladder. Upon operation, adhesion and fistula were found between the ileum and the urinary bladder. The microscopic findings of the surgical specimen were compatible with metastatic HCC. We confirmed that ileovesical fistula had been caused by metastatic HCC.
Adult ; Bladder Fistula/*etiology ; Carcinoma, Hepatocellular/*complications ; Humans ; Ileal Diseases/*etiology ; Intestinal Fistula/*etiology ; Liver Neoplasms/*complications ; Male

Pancreatic fistulas are usually caused by the disruption of pancreatic duct. The majority of pancreatic fistulas are external fistulas and common causes of external and internal pancreatic fistulas are trauma and surgery. Internal pancreatic fistulas due to pancreatitis are rare. Internal pancreatic fistulas may communicate with peritoneal cavity, colon, small bowel, biliary system or pleural cavity. Among them, fistula between pancreatic duct and portal vein due to acute pancreatitis is rare. We report a case of 32-year-old male with fistula between pancreatic duct and portal vein as a complication of acute pancreatitis. Pancreaticoportal fistula was diagnosed by endoscopic retrograde cholangiopancreatography. He recovered after distal pancreatectomy with splenectomy and supportive care.
Acute Disease ; Adult ; Cholangiopancreatography, Endoscopic Retrograde ; English Abstract ; Humans ; Male ; Pancreatic Fistula/diagnosis/*etiology ; Pancreatitis/*complications ; *Portal Vein ; Vascular Fistula/diagnosis/*etiology

BACKGROUND/AIMS: This study was performed to compare the mucosal findings after esophagogastroduodenoscopy in two groups before and after the use of alendronate only and following administration of the enteric-coated alendronate (5 mg) and calcitriol (0.5 microg) combined drug (Maxmarvil, Yuyu Co.). METHODS: The study population consisted of 33 postmenopausal healthy female volunteers, aged 50 to 70 years (mean age, 58 +/- 5) without gastrointestinal symptoms and with normal baseline endoscopic findings. Esophagogastroduodenoscopy was performed at baseline and was repeated 2 weeks later after daily intake of Maxmarvil (n = 17 subjects) or alendronate only (n = 16 subjects). Mucosal injury scores were reported by an endoscopist after 2 weeks of treatment with each medication schedule. RESULTS: Esophageal mucosal injuries developed in two of 16 subjects in the alendronate only group and 0 of 17 in the Maxmarvil group. Gastric mucosal injuries developed in eight subjects in the alendronate group and four subjects in the Maxmarvil group; this difference was statistically significant. CONCLUSIONS: The mucosal damage scores for the alendronate group (total score 24) were significantly higher than those for the Maxmarvil group (total score 9) in the esophagus and stomach. Therefore, this study suggested that enteric-coated Maxmarvil is less harmful to gastrointestinal mucosa than alendronate, and may improve the tolerability of osteoporosis medication in clinical practice.
Administration, Oral ; Age Factors ; Aged ; Alendronate/administration & dosage/*adverse effects ; Bone Density Conservation Agents/administration & dosage/*adverse effects ; Calcitriol/administration & dosage/*adverse effects ; Drug Combinations ; *Endoscopy, Digestive System ; Esophagus/*drug effects/pathology ; Female ; Gastric Mucosa/*drug effects/pathology ; Humans ; Middle Aged ; *Postmenopause ; Predictive Value of Tests ; Republic of Korea ; Sex Factors ; Tablets, Enteric-Coated ; Time Factors ; Treatment Outcome ; Vitamins/administration & dosage/*adverse effects

BACKGROUND: Recent evidence indicates that elevated COX-2 expression is associated with the carcinogenesis of numerous neoplasms. In this study, we investigated COX-2 expression in various thyroid specimens in order to elucidate its physiological role in pathologic conditions, and to evaluate the efficiency of COX-2 protein expression as a molecular marker of malignancy in the thyroid gland. METHODS: COX-2 expression was studied immunohistochemically in 19 papillary carcinomas, 8 follicular carcinomas, 14 follicular adenomas, 2 H rthle cell carcinomas, 4 H rthle cell adenomas, 8 nodular hyperplasias, 3 Graves' diseases, 3 Hashimoto's thyroiditis, 2 medullary carcinomas, 1 anaplastic carcinoma, and 20 normal thyroid tissues. RESULTS: COX-2 staining was not seen in any of the normal thyroid, Graves' disease, or nodular hyperplasia specimens. In contrast, COX-2 staining was observed in all of papillary carcinomas, Hashimoto's thyroiditis, H rthle cell carcinomas, and H rthle cell adenomas tissues. Moreover, 7 of 8 follicular carcinomas and 11 of 14 follicular adenomas showed COX-2 staining. CONCLUSION: These results indicate that COX-2 is not useful as a marker of malignancy. Since COX-2 expression was evident in follicular adenomas and in papillary and follicular carcinomas. Thus, the enzyme may be involved in the early process of thyroid tumorigenesis.
Human ; *Immunohistochemistry ; Isoenzymes/*analysis ; Prostaglandin-Endoperoxide Synthase/*analysis ; Thyroid Nodule/*enzymology/*pathology ; Tumor Markers, Biological/*analysis

BACKGROUND/AIMS: The relationship between Runt-related transcription factor 3 (RUNX3) gene inactivation and various solid tumors has been reported; however, little information is available about RUNX3 in thyroid cancers. METHODS: We evaluated the DNA methylation of RUNX3 in 13 papillary thyroid cancer tissues and four thyroid cancer cell lines. Additionally, using reverse transcriptase-polymerase chain reaction, we analyzed RUNX3 gene expression in several thyroid cancer cell lines after treating with the demethylating agent 5-aza-2'-deoxycytidine (DAC). RESULTS: RUNX3 was hypermethylated in many thyroid cancer cell lines and in 10 of the 12 papillary thyroid cancer tissues. Treatment with DAC increased the expression of RUNX3 in some thyroid cancer cell lines. CONCLUSIONS: We suggest that RUNX3 is associated with thyroid carcinogenesis, and RUNX3 methylation is a potentially useful diagnostic marker for papillary thyroid cancer.
Azacitidine/analogs & derivatives/pharmacology ; Carcinoma/*genetics ; Cell Line, Tumor ; Core Binding Factor Alpha 3 Subunit/*genetics ; DNA Methylation/drug effects ; Gene Expression/drug effects ; Humans ; Thyroid Neoplasms/*genetics ; Tumor Markers, Biological/genetics

Human chorionic gonadotropin (HCG) is one of the glycoproteins families synthesized by the placenta, and consists of 2 noncovalently joined subunits, namely, alpha and beta. The alpha and beta-subunits have a structural homology with the alpha and beta-subunits of TSH and LH. The thyrotropic action of HCG results from its structural similarity to TSH, so beta-HCG can bind to the TSH receptor in the thyroid gland. A high level of HCG, accompanied by an increased thyroid hormone level, can be observed in gestational trophoblastic diseases (GTD), such as a hydatidiform mole or a choriocarcinoma. However, the clinical symptoms of hyperthyroidism in GTD are rarely observed. A 27-years-old woman, admitted due to an amenorrhea of 11 weeks duration, with thyrotoxic symptoms, such as weight loss, palpitation, sweating, tremor, heat intolerance and anxiety, was evaluated. Her serum free T4 level was 8 times higher than normal, and her serum beta-HCG level was over 1,000,000IU/L. She had a curettage operation, with the pathological findings of a complete hydatidiform mole. These thyrotoxic symptoms developed due to a hydatidiform mole, and were accompanied with a highly increased serum beta-HCG level. After evacuation of the molar tissue, the thyroid hormone and thyrotoxic symptoms normalized. Here, this case is reported, with brief review of the literature.
Amenorrhea ; Anxiety ; Choriocarcinoma ; Chorionic Gonadotropin ; Curettage ; Female ; Gestational Trophoblastic Disease ; Glycoproteins ; Hot Temperature ; Humans ; Hydatidiform Mole* ; Hyperthyroidism ; Molar ; Placenta ; Pregnancy ; Receptors, Thyrotropin ; Sweat ; Sweating ; Thyroid Gland ; Thyrotoxicosis* ; Tremor ; Weight Loss