Wilson's disease is an inborn error of copper metabolism which may be associated with hepatic cirrhosis and progressive degeneration of the central nervous system. The most common ophthalmologic finding in Wilson's disease is the Kayser-Fleischer ring. Other much less common physical signs include neurologic signs, endocrinologic abnormality. The Kayser-Fleischer ring occurs in the corneal periphery and is usually yellow- brown color. The Kayser-Fleischer ring.copper deposition at the level of the posteior position of Descemet's membran. The authours have recently experienced two cases of wilson's disease. One case. a 20-year-old girl, has Kayser-Fleischer rings in both eyes, amenorrhea, chronic active hepatitis and the other case, a 21-years-old girl, has dense yellow-green colored Kayser-Fleischer rings in both eyes, palilalia, and family relationship. Both cases have been treated with D-penicillaine and low copper diet. After treatment, clinical manifestation have been improved markedly at former case and the other cae is steady stage, but the Kayse-Fleischer rings have not disappeared yet in both cases.
Amenorrhea ; Central Nervous System ; Copper ; Diet ; Family Relations ; Female ; Hepatitis, Chronic ; Hepatolenticular Degeneration* ; Humans ; Liver Cirrhosis ; Metabolism ; Neurologic Manifestations ; Penicillamine ; Young Adult

Hemangiopericytoma is a rare tumor which originate from the soft tissue. The main sites are lower extremity, pelvic retroperitoneum and especially it is rare to occur primarily in the orbit. It is characterized that hemangiopericytoma possesses the potential of malignancy, but prediction of ultimate behavior is difficult because the clinical course often dose not coincide with histopathologic indicators of malignancy. We experienced a case of primary orbital hemagiopericytoma in a 31-yearold man who had complained diplopia and decreased vision, proptosis, downward and outward deviation of right eye. These symptoms had been occured 7 years ago. MRI examination demonstrated a well-defined tissue mass in superomedial extraconal space in the right orbit. We excised the tumor completely and found it was originated from the vessels with immunohistochemical study utilizing CD34 and Factor VIII-R-Ag as diagnostic marker. Also we confirmed the tumor is a hemangiopericytoma in the Gomoris reticulin stain and EM findings. No recurrence occured for more than 12 months after the initial complete excision. So we present our case with a brief review of the lietrature.
Diplopia ; Exophthalmos ; Hemangiopericytoma ; Lower Extremity ; Magnetic Resonance Imaging ; Orbit* ; Recurrence ; Reticulin

PURPOSE: To evaluate long-term effectiveness of lateral tarsal strip procedure combined with Quickert sutures in correcting involutional entropion. METHODS: The retrospective study reviewed 82 eyes of 71 patients with involutional entropion whose follow-up period was more than 6 months among patients who underwent lateral tarsal strip procedure combined with Quickert sutures between February 2003 and February 2010. RESULTS: The postoperative follow-up period was 6 months to 78 months (average 40.2 months) and the average age was 63.5 years. Out of 82 eyes, 3 eyes recurred and the recurrence rate in the lateral tarsal strip procedure combined with Quickert sutures was 3.6%; postoperative overcorrection was not observed. Out of the 3 recurrence cases, 2 eyes both recurred 6 months after the operation and were treated by Quickert sutures alone with no recurrence. The other case recurred 1 year and 4 months after lateral tarsal strip procedure combined with Quickert sutures and was corrected by this operation again. There was no recurrence for a follow-up period of 2 years. CONCLUSIONS: An effective approach was obtained during long-term follow-up when lateral tarsal strip procedure combined with Quickert sutures was conducted as repairing operations in involutional entropion. The approach showed lower recurrence rate than the existing reports of operations such as lateral tarsal strip procedure or Quickert sutures when conducted alone and provided long-lasting effectiveness in the correction of involutional entropion.
Entropion ; Eye ; Follow-Up Studies ; Humans ; Recurrence ; Retrospective Studies ; Sutures

PURPOSE: Atopic dermatitis (AD) is an inflammatory skin disease, significantly affecting the quality of life. Using AD as a model system, we tested a successive identification of AD-associated microbes, followed by a culture-independent serum detection of the identified microbe. METHODS: A total of 43 genomic DNA preparations from washing fluid of the cubital fossa of 6 healthy controls, skin lesions of 27 AD patients, 10 of which later received treatment (post-treatment), were subjected to high-throughput pyrosequencing on a Roche 454 GS-FLX platform. RESULTS: Microbial diversity was decreased in AD, and was restored following treatment. AD was characterized by the domination of Staphylococcus, Pseudomonas, and Streptococcus, whereas Alcaligenaceae (f), Sediminibacterium, and Lactococcus were characteristic of healthy skin. An enzyme-linked immunosorbent assay (ELISA) showed that serum could be used as a source for the detection of Staphylococcus aureus extracellular vesicles (EVs). S. aureus EV-specific immunoglobulin G (IgG) and immunoglobulin E (IgE) were quantified in the serum. CONCLUSIONS: A metagenomic analysis together with a serum detection of pathogen-specific EVs provides a model for successive identification and diagnosis of pathogens of AD.
Alcaligenaceae ; Dermatitis, Atopic* ; Diagnosis ; DNA ; Enzyme-Linked Immunosorbent Assay ; Extracellular Vesicles ; Humans ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulins ; Lactococcus ; Metagenomics* ; Pseudomonas ; Quality of Life ; Skin ; Skin Diseases ; Staphylococcus ; Staphylococcus aureus ; Streptococcus