No abstract available.
Mucocutaneous Lymph Node Syndrome

Trabeculectomy, as a surgical intervention in the treatment of glaucoma, has become most popular. The most common cause of filtration failure is obstruction of aqueous outflow due to bleb fibrosis. Adjunctive intraoperative mitomycin-C has been reported to improve the success of filtration surgery in eyes of high risk for failure. But its use increases the rate of complications after filtering surgery: especially postoperative hypotony, wound leakage, and scleral necrosis. We have experienced two patients whose scleral flaps were necrotized after trabeculectomy with mitomycin-C in uncomplicated glaucoma. We report successful results in revision of filtering bleb by using Gore-Tex as a patch graft.
Blister* ; Fibrosis ; Filtering Surgery ; Filtration ; Glaucoma ; Humans ; Mitomycin* ; Necrosis ; Polytetrafluoroethylene* ; Trabeculectomy* ; Transplants ; Wounds and Injuries

No abstract available.
Hydronephrosis*

Epstein's syndrome is a rare disease whish is characterized by the association of thrombocytopenia, macrothrombocytopathia, nephritis and deafness. We experienced a case of Epstein's syndrome in a 12 years old male patient who was presented with a life long history of bleeding, usually as epistaxis, bilateral sensorineural deafness and hematuria with proteinuria starting in late childhood. Hematologic studies showed thrombocytopenia with giant platelets and anemia. A bone marrow aspirate revealed the megakaryocytes to be adequate in number and many giant size platelets. Platelet do not respond to addition of A and epinephrine; collagen and ristocetin induced agglutination response is decreased. It is difficult to be certain the association of thrombocytopenia with giant platelets, nephritis and deafness constitutes a new hereditary disease with a distinct pathogenesis or if it is an expansion of the well recognized Alport's syndrome of hereditary nephritis deafness. We report a case of Epstein's syndrome syndrome with brief review of related literatures.
Agglutination ; Anemia ; Blood Platelets ; Bone Marrow ; Child ; Collagen ; Deafness ; Epinephrine ; Epistaxis ; Genetic Diseases, Inborn ; Hematuria ; Hemorrhage ; Humans ; Male ; Megakaryocytes ; Nephritis ; Nephritis, Hereditary ; Proteinuria ; Rare Diseases ; Ristocetin ; Thrombocytopenia

No abstract available.
Trichothiodystrophy Syndromes*

A 29 kDa cysteine protease of Taenia solium metacestodes was purified by Mono Q anion-exchanger and Superose 6 HR gel filtration chromatography. The enzyme was effectively inhibited by cysteine protease inhibitors, such as iodoacetic acid (IAA) and trans-epoxy-succinyl-L-leucyl-amido (4-guanidino) butane (E-64) while inhibitors acting on serine- or metallo-proteases did not affect the enzyme activity. The purified enzyme degraded human immunoglobulin G (IgG), collagen and bovine serum albumin (BSA), but human IgG was more susceptible for proteolysis by the enzyme. To define the precise biological roles of the enzyme, more detailed biochemical and functional studies would be required.
Taenia solium/*enzymology ; Serum Albumin, Bovine/metabolism ; Leucine/analogs & derivatives/pharmacology ; Iodoacetic Acid/pharmacology ; Immunoglobulin G/metabolism ; Humans ; Cysteine Proteinase Inhibitors/pharmacology ; Cysteine Endopeptidases/chemistry/*isolation & purification/metabolism ; Collagen/metabolism ; Chromatography, Ion Exchange ; Chromatography, Gel ; Animals

To assess the change of nitric oxide(NO) concentration according to photorefractive keratectomy(PRK) depth and the role of NO in cataractohenesis after excimer laser PRK. The treated 36 rabbits were divided into 12 groups according to PRK depth(-3.0, -7.0, -11.0 diopter [0]), the dates of sampling(the 1st day and the 7th day after PRK), and reatment of NOS inhibitor (NG-nitro-L-arginine methylester, L-NAME). NO concentration and superoxide dismutase(SOD) activity in the aqueous humor and malondialdehyde(MDA) concentration in the lens were measured. Changes of lens epithelium were examined by eletron microscopy on the 3rd day and the 6th day after -11.0D of PRK. The NO and MDA concentration in the group without L-NAME treatment on the 1st day after -11.0D of PRK were significantly higher than those in the L-NAME treated group and control group. The activity of SOD increased in all of the PRK treated group on the 1st day and was even higher on the 7th day. The histopathological changes of lens epithelium on the 3rd day after PRK were compatible with cellular necrosis and those of the 6th day after PRK were compatible with cellular reparing. The results suggested that the toxicity of NO after PRK correcting over -11.0D may a role in cataractogenesis, but the progress could be suppressed by treatment of NOS inhibitor.
Aqueous Humor ; Epithelium ; Lasers, Excimer ; Malondialdehyde ; Microscopy ; Necrosis ; NG-Nitroarginine Methyl Ester ; Nitric Oxide* ; Photorefractive Keratectomy* ; Rabbits ; Superoxide Dismutase ; Superoxides

No abstract available.
Carcinoma, Basal Cell* ; Nevus*

No abstract available.
Child* ; Humans ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pneumonia* ; Pneumonia, Mycoplasma*

Chronic sclerosing sialadenitis, Mikulicz disease or Kuttner tumor has been recently recognized as a spectrum of IgG4-related sclerosing disease. IgG4-related disease is characterized by a high serum IgG4 level and tissue infiltration of IgG4-positive plasmacytes. We report three cases of chronic sclerosing sialadenitis with variably associated systemic involvement. All patients presented with a submandibular mass or swelling, and all the resected submandibular glands showed diffuse lymphocytic infiltration, lymphoid follicles, and septal fibrosis. Two of the specimens revealed numerous IgG-positive plasma cells, most of which were IgG4-positive on immunohistochemical staining. One of them was associated with dacryoadenitis and hypophysitis. The other patient had ureterorenal lesions. Immunohistochemical study was unavailable in remaining one case, but the histologic features along with elevated IgG level and associated pancreatitis supported the diagnosis. All patients received steroid therapy postoperatively and are doing well. Salivary gland involvement in IgG4-related fibrosclerosis should be recognized in systemic medical pathology.
Dacryocystitis ; Fibrosis ; Humans ; Immunoglobulin G ; Mikulicz' Disease ; Pancreatitis ; Plasma Cells ; Salivary Glands ; Sialadenitis ; Submandibular Gland