Diurnally fluctuating hereditary progessive dystonia is a rare movement disorder characterized by marked diurnal fluctuation of symptoms and dramatic response to levodopa. We report two advanced female patients of two families who presented with progressive dystonia and gait disturbance. Both patients have a family history and showed excellent response to levodopa.
Dystonia* ; Female ; Gait ; Humans ; Levodopa ; Movement Disorders

Lymphangioma is an uncommon malformation of the lymphatic system that involves a benign proliferation of the lymphatics, with no established treatment method. Multiple colonic lymphangioma, or colonic lymphangiomatosis, is an extremely rare condition. We report a case of colonic lymphangiomatosis that was detected during a colonoscopic examination conducted as part of a general health check-up. The lesion completely resolved after excisional biopsy.
Biopsy* ; Colon* ; Lymphangioma ; Lymphatic System

Nocardiosis is a rare opportunistic infection and only sporadic cases were reported in systemic lupus erythematosus (SLE). We report a case of a nocardial infection in a patient with SLE. A 37-year-old woman was admitted to our hospital because of general weakness and edema. She had been diagnosed as SLE in 1995 and was diagnosed as lupus nephritis (class IV, diffuse proliferative glomerulonephritis) in 1999, but had refused the treatment for SLE. After admission, she was diagnosed as active SLE with lupus nephritis based on clinical and laboratory findings. She was treated with methylprednisolone pulse (1 gx3 days) and cyclophosphamide pulse (750 mg) therapy followed by daily high dose oral prednisone (60 mg) and plasmapheresis (7 times). On 32 days after admission, chest radiograph revealed a massive pleural effusion. The pleural fluid was exudate and cultures for bacteria, fungi and tubercle bacilli were negative and pleural biopsy showed mesothelial thickening. The thoracentesis was done (1 L), but the effusion recurred within 3 days. Further four thoracentesis and thoracostomy were performed due to recurrent massive pleural effusion. On 60 days after admission, thoracostomy tube was removed, but 4 days later, fever occurred and Nocardia asteroides was cultured in pleural fluid. Treatment with oral trimethoprim/sulfamethoxazole (TMP-SMX, 160~800 mg) was started. After treatment with TMP-SMX, the pleural effusion decreased. On 76 days after admision, she was discharged and maintained on TMP-SMX (80~400 mg) for another 12 months. At present, her SLE activity is on control and has no pleural effusion.
Adult ; Bacteria ; Biopsy ; Cyclophosphamide ; Edema ; Exudates and Transudates ; Female ; Fever ; Fungi ; Humans ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Methylprednisolone ; Nocardia asteroides ; Nocardia Infections ; Opportunistic Infections ; Plasmapheresis ; Pleural Effusion ; Prednisone ; Radiography, Thoracic ; Thoracostomy ; Trimethoprim, Sulfamethoxazole Drug Combination

PURPOSE: The five-year survival rates of patients with stage III colorectal cancer have been reported widely ranging from 22 to 69 percent. Hence, reliable substaging is important for the management of stage III colorectal cancer patients. Therefore, we tried to assess the substages and investigate the possibility of other discriminating numbers for nodal substaging. METHODS: The 381 patients with node-positive colorectal cancer who had undergone surgery, were retrospectively categorized by the number of positive nodes. The patients were grouped in five ways, and each grouping was divided into two subgroups according to the number of positive nodes. The subgroups of each grouping were as follows; in LN1 group, N1=1, N2>1; in LN2 group, N1=2, N2>2; in LN3 group, N1=3, N2>3; in LN4 group, N1=4, N2>4; in LN5 group, N1=5, N2>5. We compared the survival rate of each groups. RESULTS: Node-positive patients had a five-year survival rate of 55.2 percent. The statistical differences between the N1 and N2 subgroups of each grouping were as follows: LN1 group (P=0.0128), LN2 group (P=0.0052), LN3 group (P=0.6268), LN4 group (P=0.1480), and LN5 group (P=0.6875). CONCLUSION: There were significant differences in the five-year survival rates between N1 and N2 in the LN1 group and LN2 group, but there were no differences between N1 and N2 in the other groupings. These data raise the possibility that a novel N1~N2 substaging (N1: 1~2; N2: >2) is superior to the current N1~N2 substaging (N1: 1~3; N2: >3).
Colorectal Neoplasms ; Humans ; Lymph Nodes ; Neoplasm Staging ; Retrospective Studies ; Survival Rate

OBJECTIVE: Persistent infection of HPV increases the chance of carcinoma in situ of cervix through stages of cervical intraepithelial neoplasia (CIN) 1, 2, and 3, and finally progresses into cervical cancer. We aimed to explore the safety and efficacy of BLS-M07 which is orally administered agent expressing human papillomavirus (HPV) 16 E7 antigen on the surface of Lactobacillus casei in patients with CIN 3. METHODS: Patients with CIN 3 were recruited in our clinical trial. Reid Colposcopic Index (RCI) grading and serum HPV16 E7 specific antibody production were used to evaluate efficacy of BLS-M07. In phase 1, BLS-M07 was administered orally, 5 times a week, on weeks 1, 2, 4, and 8 with dosages of 500 mg, 1,000 mg, and 1,500 mg. In phase 2a, patients were treated with 1,000 mg. The primary endpoints were the safety and the pathologic regression on colposcopic biopsy. RESULTS: Nineteen patients were enrolled in the CIN 3 cohort. In phase 1, no patients experienced dose limiting toxicity. No grade 3 or 4 treatment-related adverse events or deaths were observed. At 16 weeks after treatment, RCI grading was improved and serum HPV16 E7 specific antibody production increased (p<0.05). Six of 8 (75%) patients with CIN 3 were cured in phase 2a. CONCLUSIONS: Oral immunization with BLS-M07 increases production of serum HPV16 E7 specific antibody which induces protective humoral immunity. The safety of this oral vaccine was proved and could be a competitive non-surgical therapeutic agent of CIN 3. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02195089
Antibody Formation ; Biopsy ; Carcinoma in Situ ; Cervical Intraepithelial Neoplasia ; Cervix Uteri ; Cohort Studies ; Female ; Humans ; Immunity, Humoral ; Immunization ; Lactobacillus casei ; Papillomavirus E7 Proteins ; Papillomavirus Vaccines ; Uterine Cervical Neoplasms

The purpose of this study was to identify the composition and organization of lingual tissues underlying the histo-structural change of developing tongue in Korean native goats by light microscopy (LM). Tongues of the fetuses on days 60, 90, 120 and neonate were examined for the morphological development. In the 60-day-old fetuses, the tongue tissues were differentiated into epithelium, lamina propria and muscle layer. Primordia of filiform, conical, lentiform, fungiform and vallate papillae appeared and rudiments of taste bud were observed in the epithelia of the primordia of the gustatory papillae. The dorsal surface of the lingual epithelia showed a weak PAS positive reaction. Collagenous fibers and small blood vessels were shown in the connective tissues. In the 90-day-old fetuses, Von Ebner's glands were moderately PAS positive while the muscle fibers and connective tissue were strongly positive for PAS. The collagenous fibers increased and came to have a more complex arrangement in the tongue. The muscle fibers were spread out at various directions and developed in striated muscle bundles. In the 120-day-old fetuses, taste buds were observed in the epithelia of the gustatory papillae, and several well-developed tissues visible such as blood vessels, collagenous fibers, muscle fiber bundles and Von Ebner's glands. In the neonates, many taste buds were found in a transverse section of the vallate papilla. The muscle layers, Von Ebner's glands, collagenous fibers and blood vessels were more developed than those of the 120-day old fetuses. These findings indicate that goat tongues have a variety of different shapes during prenatal development.
Blood Vessels ; Collagen ; Connective Tissue ; Epithelium ; Fetus ; Goats* ; Humans ; Infant, Newborn ; Microscopy ; Morphogenesis ; Mucous Membrane ; Muscle, Striated ; Muscles ; Taste Buds ; Tongue* ; von Ebner Glands

Cytokeratin (CK) comprises the intermediate filament cytoskeleton of epithelial cells. Patterns of CK expression can be regarded as a specific marker for epithelial differentiation status. The aim of this study was to identify CK expression on tongues of Korean native goats ranging from 60-day-old fetuses to newborns during prenatal development using immunohistochemistry. The tongues of fetuses were removed from 2- to 4-year-old female Korean native goats by caesarean section performed under general anesthesia. Immunohistochemistry was performed to assess CK expression patterns on developing goat tongues using serial paraffin-embedded sections. Light zones signifying CK immunoreactivity in dorsal lingual epithelia were weakly positive in 60-day-old fetuses. In 90-day-old fetuses, deep areas in dorsal lingual epithelia were strongly positive for CK expression and superficial areas were moderately positive. In 120-day-old fetuses, light zones of lingual epithelia in the vallate papilla were strongly positive for CK expression, whereas ducts of von Ebner's glands were moderately positive. In neonates, taste buds were positive for CK expression, whereas non-taste epithelial cells and von Ebner's glands were negative. These findings indicate that goat tongues have different patterns of CK expression during development and provide a morphological basis for studies on the biological mechanism of epithelial differentiation.
Anesthesia, General ; Cesarean Section ; Child, Preschool ; Cytoskeleton ; Epithelial Cells ; Epithelium ; Female ; Fetus ; Goats* ; Humans ; Immunohistochemistry ; Infant, Newborn ; Intermediate Filaments ; Keratins* ; Pregnancy ; Taste Buds ; Tongue* ; von Ebner Glands

Mallory-Weiss syndrome is a laceration in the region of the gastroesophageal junction due to vomiting, retching, coughing preceding hematemesis in alcoholic patient. Bleeding from Malloly-Weise tears stop spontaneously without specific therapy in 80-90% of patient, but rebleeding occurs in 2 to 5% of patients. Thus most patients require only supportive care. Rarely endoscopic therapy or operative therapy may be required. We performed endoscopic mucosal ligation using intraluminal negative pressure with band ligation for uncontrolled Mallory-Weiss syndrome. In conclusion, Endoscopic O-ring band ligation in uncontrolled intractable Mallory-Weiss syndrome is safe and effective method, but its important that accurate endoscopic O-ring band ligation an bleeding site in laceration area. We experienced endoscopic O-ring band ligation in 6 cases of Mallory-Weiss syndrome.
Alcoholics ; Cough ; Esophagogastric Junction ; Hematemesis ; Hemorrhage ; Humans ; Lacerations ; Ligation* ; Mallory-Weiss Syndrome ; Vomiting

Mallory-Weiss syndrome is a laceration in the region of the gastroesophageal junction due to vomiting, retching, coughing preceding hematemesis in alcoholic patient. Bleeding from Mallory-Weiss tears stop spontaneously without specific therapy in 80-90 percent of patient, but rebleeding is uncommonly occurring in 2 to 5 percent of patients. Thus most patients require only supportive care. Rarely endoscopic therapy or operative therapy may be required. We conducted an uncontrolled study to evaluate an improved metallic clip(Olympus hemoclip) for the endoscopic treatment of Mallory-Weiss syndrome. Initial hemostasis was achieved in all patients with active bleeding. No complications resulted from this treatment. Clips did not impair healing of teared mucosa. We conclude that endoscopic hemoclip placement is a highly effective and safe method for treating uncontrolled Mallory-Weis syndrome.
Alcoholics ; Cough ; Esophagogastric Junction ; Hematemesis ; Hemorrhage ; Hemostasis ; Humans ; Lacerations ; Mallory-Weiss Syndrome ; Mucous Membrane ; Vomiting

Endoscopic esophageal variceal ligation(EVL) was first introduced by Stiegmann and colleagues in 1986, and it has since grown to he became an extremely popular modality throughout the world as well as Korea. Endoseopic variceal ligation(EVL), which consists of mechanical ligation and thrombosis of varices using elastic O-rings, has been recently developed as a non-operative alternative to endapic injection sclerotherapy(EIS). EVL is minimally operator-dependent and is also associated with fewer local and systemic complications than sclerotherapy. However, the conventional device has only one O ring, and thus the inner cylinder has to be exchanged after each ligation, So, it is a time-consurning procedure that requires the use of an overtube which has somtimes caused tearing of the esophageal mucosa. To save time and control variceal bleeding, multi-band ligation(MBL) was developed. These ligators have five or six O rings, and serial ligation is now possible without exchanging the cylinder or withdrawing the endoscope.(continue...)
Esophageal and Gastric Varices* ; Korea ; Ligation* ; Mucous Membrane ; Sclerotherapy ; Thrombosis ; Varicose Veins