The Sturge Weber syndrome is an uncommon disorder characterized by port-wine nevus of the face with angiomatosis of the ipsilateral cerebral leptomeninges and extensive calcification in the underlying cerebral cortex. Associated with this syndrome are mental retardation, choroidal angioma, buphthalmus or glaucoma, seizure and hemiplegia. We studied a case of incomplete form of Sturage Weber syndrome in a 12 year-old boy, who had generalized seizure, a homonymous hemianopsia, intracranial calcification and leptomeningeal angiomatosis. There are no characteristic facial nevus, mental retardation, occular changes and hemiplegia.

Porokeratosis is a disorder of epidermal keratinization that is histologically characterized by the presence of cornoid lamella. Many treatment modalities have been used, such as, surgical excision, cryosurgery, CO2 laser, oral etretinate, topical 5-fluorouracil and keratolytics. However, universal treatment guidelines have never been established. We tried 5% imiquimod cream on three patients who have porokeratosis of Mibelli which are intractable to other previous treatment modalities. Two of them showed a marked and moderate improvement, respectively. But the third patient exhibited only slight response. Herein we report our treatment experience and review the related literature.
Acitretin ; Cryosurgery ; Etretinate ; Fluorouracil ; Humans ; Lasers, Gas ; Porokeratosis*

OBJECTIVE: To evaluate the efficacy of clinical use of a intrauterine Sengstaken-Blakemore tube (S-B tube) in postpartum hemorrhage not controlled with medication and conservative treatment. METHODS: Retrospective review was done in 18 women with insertion of S-B tube into intrauterine cavity who did not controlled with medication and conservative treatment for postpartum hemorrhage. After third stage of labor, women were treated with prophylactic intramuscular Methylergonovine 0.2 mg and intravenous infusions of oxytocin routinely. If the postpartum bleeding continued, Misoprostol 800 microgram (per rectal) and intravenous infusions of Dinoprost 2 mg mixed with 5% D/S 500 cc were employed. And then women were examined for retained placenta and laceration of birth canal. Where necessary, retained placenta was removed and lacerations were sutured. If the postpartum bleeding did not controlled despite all of procedures, we decided use of intrauterine S-B tube. RESULTS: The mean age of the patients was 30.9+/-4.4 (27-39) years, their mean body weights and parities were 67.8+/-8.2 (56.2-85.7) kg, 1.8+/-0.8 (1-4) and mean gestational age was 38.5+/-2.9 (37-42) weeks respectively. The mean time from delivery to insertion of S-B tube was 107.6+/-94.0 (24-360) minutes. The mean filled normal saline amount was 190.5+/-35.2 (120-230) cc. Of 18 who was inserted of S B tube, 15 cases needed not additional surgical therapy. So success rate was 83.3%. CONCLUSION: Insertion of intrauterine S-B tube appears as a simple and effective means of treating postpartum hemorrhage not controlled with medication and conservative treatment.
Body Weight ; Dinoprost ; Female ; Gestational Age ; Hemorrhage ; Humans ; Infusions, Intravenous ; Lacerations ; Methylergonovine ; Misoprostol ; Oxytocin ; Parturition ; Placenta, Retained ; Postpartum Hemorrhage* ; Postpartum Period* ; Retrospective Studies

OBJECTIVES: The current Korean Mental Health Act (KMHA) indicates that a patient, who voluntarily gave their permission for admission into a mental health facility, has the right to be discharged upon personal request. However, there is no clause in the KMHA that allows a change in a patient's voluntary status under special circumstances. The purpose of this study was to investigate problems that may arise from the lack of such a clause ; problems that can result in misinterpretation and lead to the prohibition of voluntary admission status conversion. METHODS: Previous cases presented to the National Human Rights Commission of Korea were investigated in order to determine the current state in Korean psychiatric practice regarding the conversion from voluntary to involuntary admission status. In addition, examples of similar mental health legislation in use by the United Nations (UN), World Health Organization (WHO), and several advanced countries pertaining to such conversions were investigated. These examples were used as models for making recommendations for possible changes to the KMHA. RESULTS: From 2010 to 2014, more than 220 petitions were filed with the National Human Right Commission of Korea. The petitions involved voluntarily institutionalized patients who had their requests for discharge rejected. Based on mental health regulations of the UN, WHO, and such countries as the United States, England, Canada, Australia, and Japan, the KMHA should include a provision that, upon receiving a discharge request, allows for discharge refusal if the voluntarily admitted patient is considered not mentally fit. CONCLUSION: The results suggest that the absence of a regulation allowing admission status conversion in the current KMHA is inappropriate. Rectification of this absence is urgently needed.
Australia ; Canada ; England ; Human Rights ; Humans ; Japan ; Korea ; Mental Health ; Patient Admission ; Social Control, Formal ; United Nations ; United States ; World Health Organization

Erlotinib (Tarceva(R)) is a new anti-cancer agent which acts by inhibiting epidermal growth factor receptor (EGFR) signal transduction. It is currently used in the treatment of advanced stage non-small cell lung cancer and pancreatic cancer. We report a case of acneiform eruption and paronychia induced by erlotinib in a 69-year-old man. The patient visited our clinic with multiple erythematous papules and pustules on the face, periungual erythema and pus discharge, xerosis, fissures on the sole. He had taken erlotinib for the treatment of recurred lung cancer for 4 weeks. The skin lesions were partially improved with oral pyridoxine, corticosteroid and topical antibiotics.
Acneiform Eruptions* ; Aged ; Anti-Bacterial Agents ; Carcinoma, Non-Small-Cell Lung ; Erythema ; Humans ; Lung Neoplasms ; Pancreatic Neoplasms ; Paronychia* ; Pyridoxine ; Receptor, Epidermal Growth Factor ; Signal Transduction ; Skin ; Suppuration ; Erlotinib Hydrochloride

Abdominal lymphangiomas are uncommon angiomatous tumor occurring mainly in childhood. This is a retrospective clinicopathologic study of 17 cases of abdominal lymphangioma. The patients included are five children and 12 adults, with a mean age at initial presentation of 30.7 years (age ranges 3-63). The locations of the tumors were mesentery (5), retroperitoneum (4), colon (3), omentum (3), mesocolon (1) and gallbladder (1). Infiltrative growth was more common pattern than entirely circumscribed pattern. Masses were mostly multilocular cysts and contained chyle or serous fluid. On immunohistochemical staining, 16 cases were reactive for either CD31 or factor VIII-related antigen. These fact would suggest that intra-abdominal lymphangiomas simulate the immunohistochemical features of collecting lymphatics. Follow up was possible in 12 cases for 3-50 months (mean 19 months) and only one patient showed local recurrence. Although abdominal lymphangiomas are rare in adulthood and correct preoperative diagnosis is difficult, awareness of such a possibility in adulthood will contribute to make a correct preoperative diagnosis.
Abdominal Neoplasms/physiopathology ; Abdominal Neoplasms/pathology* ; Abdominal Neoplasms/metabolism ; Adult ; Antigens, CD31/biosynthesis ; Child ; Child, Preschool ; Factor VIII/biosynthesis ; Female ; Human ; Lymphangioma/physiopathology ; Lymphangioma/pathology* ; Lymphangioma/metabolism ; Male ; Middle Age ; Retrospective Studies

No abstract available.
Caroli Disease*

Cutaneous polyarteritis nodosa (PAN) is a benign form of vasculitis of small and medium-sized arteries with chronic benign course without systemic involvement. Corticosteroids are the mainstay of treatment for cutaneous PAN but aspirin and non steroidal anti-inflammatory drugs (NSAIDs) are also used for conservative treatment. We treated five patients with cutaneous PAN using oral dapsone. All patients initially showed good response to dapsone, but exacerbation occurred once the dose of dapsone was tapered or stopped. Patients had mild side effects of dapsone which were hemolytic anemia (4 cases) and abnormal liver function (2 cases). No patient has developed major organ system involvement during 1 to 13 years of follow-up. Instead of using oral Corticosteroid, oral dapsone may be an effective and relatively safe alternative therapy for chronic benign cutaneous PAN.
Adrenal Cortex Hormones ; Anemia, Hemolytic ; Arteries ; Aspirin ; Dapsone ; Follow-Up Studies ; Humans ; Liver ; Polyarteritis Nodosa ; Vasculitis

No abstract available.
Humans

No abstract available.
Protein-Losing Enteropathies