No abstract available.
Bronchopulmonary Sequestration*

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.
Foot* ; Neuroma*

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

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

Intrathecal administration of methotrexate(IT-MTX) has constituted the standard approach to prophylaxis and treatment of central nevous system(CNS) leukemia. We experienced a quadriplegia and motor aphasia in a 14-year-old boy following repeated IT-MTX for the prophylaxis of meningeal leukemia. He was diagnosed as ALL without CNS involvement and treated by CCG- 1882 protocol. IT-MTX was administered for CNS prophylaxis. The patient began complaining of urinary incontinence, motor aphasia and weakness in his right leg from 12 days after the 5th dose of the IT-MTX therapy. Even though the IT-MTX was discontinued, loss of muscle power progressed upward resulting in quadriplegia. The patient showed slow and partial recovery on right extremities over 3 months. We report this case with brief review of literature.
Adolescent ; Aphasia, Broca* ; Extremities ; Humans ; Leg ; Leukemia ; Male ; Methotrexate* ; Quadriplegia* ; Urinary Incontinence

No abstract available.
Pregnancy* ; Pyelonephritis*

No abstract available.
Pilomatrixoma* ; Vaccination*

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