No abstract available.
Spinal Muscular Atrophies of Childhood*

No abstract available.
Optic Nerve Injuries* ; Optic Nerve*

A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

No abstract available.
Animals ; Clone Cells* ; Cloning, Organism* ; Mast Cells* ; Mice*

No abstract available.
Autopsy* ; Cardiomyopathies* ; Humans ; Infant, Newborn*

Many oral medications have been attempted to treat erectile impotence, but the results have not been satisfactory. Trazodone has alpha adrenergic antagonist effect locally and 5-HTlc agonist effect centrally. Therefore oral trazodone was tried to 44 impotent patients in this study. Age distribution of patients was between 25 and 63 years, with median age of 45. All men underwent a detailed history-taking, physical examination, laboratory studies and artificial penile erection test with prostaglandin E1 intracorporal injection. If vasculogenic impotence was suspected at artificial penile erection test, duplex ultrasound sonography and/or dynamic infusion cavernosometry and cavernosography were carried out. In case of suspected neurogenic impotence, BCRL and Somatosensory evoked potential tests were conducted, and confirmed neurogenic impotence were excluded in this study. Forty four patients taking trazodone were divided into 31 psychogenic impotence and 13 mild vasculogenic impotence with psychogenic components. Trazodone was administered orally 50mg each 3 times a day for 2-8 weeks. Based on the response to medication, increase in erection quality and frequency was regarded as positive, and no change in quality or deterioration as negative response. Among 31 patients with psychogenic impotence, 15 patients ( 48%) showed positive response and 16 patients (52% ) negative response. Of 13 patients( 30%) with vasculogenic impotence with psychogenic components, 2 patients( 15%) showed positive and 11 patients (85%) negative response. As side effect of drug, 15 patients complained mild drowsiness. Taking account into placebo effect, these results indicate that a prospective, randomizes and placebo controlled drug trials will be needed.
Adrenergic Antagonists ; Age Distribution ; Alprostadil ; Erectile Dysfunction* ; Evoked Potentials, Somatosensory ; Female ; Humans ; Impotence, Vasculogenic ; Male ; Penile Erection ; Physical Examination ; Placebo Effect ; Sleep Stages ; Trazodone* ; Ultrasonography

Cutaneous horn is a clinical term for a circumscribed concial hyperkeratotic mass amounts to at least half of its largest diameter. The number of lesion may be single or multiple. We report herein a cases of penile cutaneous horn in a 22-year-old male. He had dark brownish colored conical shaped protruded mass on the penile shaft for about 2 months. We performed excisional biopsy and curettage. Histopathologic findings showed verruca vulgaris.
Animals ; Biopsy ; Curettage ; Horns* ; Humans ; Male ; Warts ; Young Adult

No abstract available.
Ciliary Motility Disorders*

No abstract available.
Lung* ; Tracheoesophageal Fistula*

Primary cutaneous aspergillosis is rare. In this report we describe primary skin infection by Aspergillus in 9 children with leukemia. The skin lesion was characterized clinically by erythematous macule and papule associated with pain and itching, followed by a rapid progression to ulcer and central black eschars with raised erythematous border at the site of venipuncture, insertion of intravenous cannula, or where arm boards had been taped to extremities. Diagnosis was confirmed by skin biopsy and wound culture. Positive revealed in 6 patients, including A. glaucus in 2 cases, A. flavus in 1 case. Treatment consisted of temporary withdrawl of anticancer chemotherapy, intravenous amphotericin B, oral flucytosine, itraconazole and/or rifampin. One patient recovered completely without antifungal medication with resolution of leukopenia. Six of eight treated patients recovered. One patient discharged against medical advice, while cutaneous aspergillosis was improving. One patient died with persistent skin lesion and neutropenia. We conclude that primary cutaneous aspergillosis is increasingly recognized in association with intravenous cannula, intravenous puncture or prolonged contact with arm boards in immunocompromised patients, and that this serious disease can be treated successfully with appropriate management.
Amphotericin B ; Arm ; Aspergillosis* ; Aspergillus ; Biopsy ; Catheters ; Child* ; Diagnosis ; Drug Therapy ; Extremities ; Flucytosine ; Humans ; Immunocompromised Host ; Itraconazole ; Leukemia ; Leukopenia ; Neutropenia ; Phlebotomy ; Pruritus ; Punctures ; Rifampin ; Skin ; Ulcer ; Wounds and Injuries