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

Anaphylaxis is a life - threatening syndrome elicited in a hypersensitive individual on subsequent exposure to a sensitizing antigen with, reactions ranging widely in severity from mild pruritus and criteria to shock and death. Anaphylaxis occurs in the United States at rates estimated to be as high as one in every 3000 inpatients and may account for more than 500 deaths annually. Preparations of kelp Laminaria digitata are used as tents in gynecologic procedures to dilate the cervix, and the kelp consists principally of a glucagon carbohydrate called laminarin. Laminarin is thought to function as the active ingredient in dilatation by changing osmotic pressure or uterine prostaglandin metabolism. The tent is inserted into the cervix several hours before the procedure. As the cervix desiccates and dilates, the tent develops an hourglass shape. Recently, we experienced one patient with anaphylaxis to laminaria diagnosed by skin test and showing good response to treatment.
Anaphylaxis* ; Cervix Uteri ; Dilatation ; Female ; Glucagon ; Humans ; Inpatients ; Kelp ; Laminaria* ; Metabolism ; Osmotic Pressure ; Pruritus ; Shock ; Skin Tests ; United States

No abstract available.
Bronchopulmonary Sequestration*

No abstract available.
Pilomatrixoma* ; Vaccination*

No abstract available.
Erythema Multiforme* ; Erythema* ; Pemphigoid, Bullous*

No abstract available.
Pregnancy* ; Pyelonephritis*

Diaper dermatitis is an infant dermatosis associated with the wearing of diapers, Despite of its high frequency in clinical pediatrics the clinical characteristics are not well described in the korean literatvre. We investigated the clinical and mycological characteritics of diaper dermatitis in 50 serial patients. Clinically diaper dermatitis was classified. into five categories,irritant contact derniatitis(ID), candidiasis(CD). combined rash of ID and CD(CR.), intertrigo, and miliari. Of 50 patients with diaper dermatitis, 18 had ID; 16, CI3; 10, CR; 5, intertrigo, and 1, miliaria, The mean age of patients with ID (11.8 months) was higher than that of patients with CD (4.0 months). Candida albicans(C. albicans) w is isolated from 25 patients with diaper dermatitis. The rate of positive cultures by clinical diagnosis was 130% in CD, 11.1% in ID, 60, 0% in CR, and 20.0% in intertrigo. C. albicons was isolated from 23 patients(82.1%) of patients with clinically suspected Ci") and 2 of 22 patients without the characteristic erythematous papulesThe presence of eryhematous papules was the most characteristic clinical feature of infection, Other candida species isolated from cut,aneous lesions were C. tropicalsis, C.'. parapsiolosis, and C. stellatoidea.
Candida ; Candidiasis ; Dermatitis* ; Diagnosis ; Exanthema ; Humans ; Infant ; Intertrigo ; Miliaria ; Pediatrics ; Skin Diseases

No abstract available.
Humans ; Mycosis Fungoides* ; Spondylitis, Ankylosing* ; Infliximab

Choroideremia is a rare hereditary disease with characteristic fundus that causes night blindness and peripheral visual field loss. The authors encounter choroideremia accompanied by recurrent uveitis. This paper is designed to give a description of the condition, along with an investigation of the literature. Ophthalmological tests and treatments were performed. Characteristic fundus, night blindness, peripheral visual field loss, electroretinography and other manifestations led us to a diagnosis of choroideremia. The anterior uveitis was managed with medication.
Adult ; Choroideremia/*complications/diagnosis ; Electroretinography ; Fluorescein Angiography ; Fundus Oculi ; Human ; Male ; Night Blindness/etiology ; Recurrence ; Uveitis, Anterior/*complications ; Vision Disorders/etiology ; Visual Fields

No abstract available.
Foot* ; Neuroma*