Darier disease is a rare genodermatosis clinically characterized by multiple hyperkeratotic papules and plaques predominantly on seborrheic areas. It has characteristic histopathological features; corps-ronds, grains, suprabasal clefts or lacunae, villi projections etc. We present a case of Darier disease in a 36-year-old woman. She had suffered from skin lesions which bore a great resemblance to those of verruca plana or eruptive syringoma. We could confirm the diagnosis as Darier disease because she had the characteristic nail changes.
Adult ; Edible Grain ; Darier Disease* ; Diagnosis ; Female ; Humans ; Skin ; Syringoma ; Warts

Sertoli-Leydig cell tumors of the ovary are rare, accounting for no more than 0.5% of all ovarian neoplasms. These tumors occur predominantly in women 10 to 30 years of age. Heterologous elements are found in over 20% of the moderately and poorly differentiated Sertoli- Leydig cell tumors. The most common element is gastrointestinal mucin-secreting epithelium, found in approximately 20% of all cases. Approximately 4% of Sertoli-Leydig cell tumors contain immature skeletal muscle and/or cartilaginous elements. The origin of these neoplasms is regarded as neometaplasia of the mesodermal neoplastic element. While the degree of differentiation of Sertoli-Leydig cell tumors is probably of greater importance in determining prognosis than the presence of heterologous components, recent evidence does point to an unfavorable effect on clinical outcome when heterologous mesenchymal components such as cartilage and striated muscle are present. We experienced two cases of ovarian heterologous Sertoli-Leydig cell tumors with gastrointestinal type epithelium and striated skeletal muscle in 20 and 21 year-old females, respectively. One case involved a patient who had complained of amenorrhea for 3 years while the other case involved a patient who had complained of left lower abdominal tenderness and amenorrhea for one and one half years. It was determined by ultrasonography of the pelvis that the two patients suffered from right and left ovarian masses. Both patients underwent adnexectomy. These tumors were large with partly solid and partly cystic areas. Based on light microscopic examination one case was classified as an intermediate Sertoli-Leydig cell tumor with heterologous gastrointestinal type epithelium, while the other case was classified as a poorly differentiated Sertoli-Leydig cell tumor with heterologous striated skeletal muscle.
Female ; Humans

BACKGROUND: Dobutamine and amrinone, phosphodiesterase-III inhibitor, are known to have both inotropic and vasodilatory properties. We evaluated the effects of both drugs on systemic and pulmonary hemodynamics in patients with pulmonary hypertension (PH). METHODS: With Institutional Review Board approval, 45 patients whose mean pulmonary arterial pressure was greater than 30 mmHg were studied. After sternotomy under the steady state of anesthesia and controlled ventilation (30 mmHg < PaCO2 < 40 mmHg), patients recieved one of following drugs for 30minutes (min); dobutamine 5.0ug/kg/min (Group I), low dose amrinone (loading dose 1.0 mg/kg, followed by infusion 7.5 g/kg/min, Group II) or high dose amrinone (loading dose 2.0 mg/kg, followed by infusion 10 g/kg/min, Group III). Hemodynamic variables were measured at 10 min and 30 min after start of infusion. RESULTS: Dobutamine didn't decrease pulmonary arterial pressure (PAP) and cause no hemodynamic change while low and high dose amrinone reduced PAP and especcially decrease of PAP in low dose amrinone group was statistically significnat. High dose amrinone increased cardiac index (CI) and decreased both systemic vascular resistance index (SVRI) and central venous pressure (CVP) more significantly than control value. CONCLUSIONS: In patients with chronic right ventricular failure associated with PH, amrinone may decrease the PAP and improve cardiac performance more effectively than dobutamin does. Increment of dosage of amrinone may not result in significant reduction of PAP.
Amrinone* ; Anesthesia ; Arterial Pressure ; Central Venous Pressure ; Dobutamine* ; Ethics Committees, Research ; Hemodynamics* ; Humans ; Hydrogen-Ion Concentration ; Hypertension, Pulmonary* ; Sternotomy ; Vascular Resistance ; Ventilation

Familial dysautonomia, first described by Riley and co-workers in 1949, is a congenital, heritable syndrome. It is transmitted by a recessive autosomal gene which is generally limited to persons of Jewish extraction; however, rare occurrences among non-Jewish Caucasians and in a black girl have been reported. An eight-year old Korean boy was admitted to Severance Hospital with bulbar conjunctival injection OU for 1 month and visual disturbance OS for 10 days. Examination revealed exfoliated epithelium in an area of about 3mm in diameter in the center of the left cornea, multiple punctate erosions and edema of the corneal epithelium. Yet, he experienced no discomfort, blepharospasm, nor photophobia. He had decreased corneal sensation, decreased lacrimation by Schirmer test and miosis was induced biJaterally by 0.25% pilocarpine (equivalent to 2.5% methacholine). The patient had decreased deep tendon reflexes and postural hypotension, and showed emotional lability, indifference to pain, and abnormal temperature control. He also had marked anorexia, swallowing difficulty, cyclic vomiting, abdominal pain, headache, intermittent vascular hypertension and one episode of convulsive seizure. He was positive to histamine intradermal injection test and had abnormal EEG. EMG was suggestive of some form of neuropathy. He was treated with toplca 0.5% chloramphenicol solution and 10% dextran solution alternatively q.2 hrs., terramycin ophthalmic ointment q.h.s. and bilateral patching; he also received 50,000 units of vitamin A for 10 days. He showed marked improvement of his ocular symptoms in 6 days. The above patient shows many of the essential features of the familial dysautonomia syndrome as outlined by Riley; however, he also lacks some of the most important features. Therefore, we feel that the patient has a case of some other type of autonomic dysfunction simulating Riley-Day svndrome.
Abdominal Pain ; Anorexia ; Blepharospasm ; Chloramphenicol ; Cornea ; Deglutition ; Dextrans ; Dysautonomia, Familial* ; Edema ; Electroencephalography ; Epithelium ; Epithelium, Corneal ; Female ; Headache ; Histamine ; Humans ; Hypertension ; Hypotension, Orthostatic ; Injections ; Injections, Intradermal ; Male ; Miosis ; Oxytetracycline ; Photophobia ; Pilocarpine ; Primary Dysautonomias* ; Reflex, Stretch ; Seizures ; Sensation ; Vitamin A ; Vomiting

In 1952, Dr. Collier observed first a characteristic lesion of marginal corneal furrow in the patient who had rheumatoid arthritis with Sjogren's syndrome. The furrows are usually bilateral, but one may be more advanced than the other. This corneal thining may be superficial and nonprogressive or it may progress to epithelial breakdown, lamellar collagen dissolution with marked thining and perforation. Almost absence of inflammatory reaction in this furrow may be differentiated from polyarteritis nodosa. Terrien's marginal degeneration may be differentiated from this furrow by age, vascularization in the thining bed, and encircling the cornea. The case reported in the following was a 42 year old woman who had bilateral marginal furrows in the cornea with rheumatoid arthritis Which was documented by positive C-reactive protein, positive RA factor, increased ESR and hand X-ray finding suggestive of rheumatoid arthritis.
Adult ; Arthritis, Rheumatoid* ; C-Reactive Protein ; Collagen ; Cornea ; Female ; Hand ; Humans ; Polyarteritis Nodosa ; Sjogren's Syndrome

These days, the computer network has become the most popular and widely used source of information. There are two kinds of computer networks, the first one is multimedia internet world wide web site and the second one is text based commercial network (etc. Hitel, Chollian. Nownuri, Unitel). With the rapid development of internet, various kinds of medical-related sites are newly appearing on the internet. Even in Korea, several medical-related information sites are on service, however, there are very few compared with foreign sites. World wide web is popular, and creating the homepage for each hospital is becoming more important. In Korea, the number of homepage of about medicine and medical institutes, is increasing. For the construction of Soonchunhyang University Hospital's Computer Information Network, the authors first investigate Korean medical-related information sites available in internet and commercial network. After researching the authors then construct the Computer Information Network of Soonchunhyang University Hospital. This article is written with the intent of assisting those interested in creating and managing their own computer information network system by means of providing relevant data obtained during this process.
Academies and Institutes ; Information Services* ; Internet ; Korea ; Multimedia

Three rare tumors of the oral cavity are presented. Lingual chondromas, approximately twenty to thirty cases appear to have been reported since the lesion was first described by Berry in 1892, which are usually seen on the lateral borders in the anterior two thirds of the tongue, while the ventral surface and the posterior third are rarely involved. Chondromas of the tonsil are of very rare occurrence, which are thought to arise as a result of metaplasia, rather than from embryonic rests, with chronic fibrosing inflammation being the inciting factor. Because of its rarity, the following cases of chondroma of the tongue and tonsil are being added to small list of such case reported in the literature.

The term Down's syndrome is preferred to mongolism. The most constant characteristic of disease is mental retardation. The incidence of Down's syndrome is about 1/600 in the general population, being roughly the same in various parts of world and in all races. The patient's mother's age at the same time of conception as well as the nature of chromosomalmalformation are in genetic counseling. We reported a case of Down's syndrome in 5 year old female who was bone in 38 year old mother, and she was noted epicanthus, nystagmus, esotropia, Brush-field spots. Other systemic manifestation-, Simian crease, short extrimites, inward curved 5th finger, Sandal gap foot, congenital heart disease, prominent abdomen-, was also noted.
Adult ; Child, Preschool ; Continental Population Groups ; Down Syndrome* ; Esotropia ; Female ; Fertilization ; Fingers ; Foot ; Genetic Counseling ; Heart Defects, Congenital ; Humans ; Incidence ; Intellectual Disability ; Mothers

Ocular vaccinia usually is seen as a disease of the lids but primary involvement of other portions of the eye, such as conjunctiva and cornea, is uncommon. The disease can result from autoinoculation and contamination after contact with the vaccination vesicle. Ocular vaccinia is most likely to occur in patients who have not had previous successful vaccinations and in those previously vaccinated who do not have adequate antibody levels against vacclIua. The case reported in the following was a 2 year old girl who noted ocular vaccinia in her right upper and lower eyelids in 5 days after smallpox vaccination.
Child, Preschool ; Conjunctiva ; Cornea ; Eyelids ; Female ; Humans ; Smallpox ; Vaccination ; Vaccinia*

The Bain circuit system was first used by bain and spoerel in 1972. It is a type of non-rebreathing system which is a variation of the Mapleson D or E system. Its total length, diameter of the inner tube and the outer tube are 1.8 m, 8 mm and 22 mm respectively. In this study the children and infants who weight 5~20 kg were selected, and during open heart surgery using bypass technique the respiration was controlled mechanically by using the bain circuit system with a fresh gas flow of 200 ml/kg/min. The minimum fresh fas flow was 2 L/min and the maximum fresh gas flow 3.5 L/min. The patients were divided into three groups according to body weight; group 1(5~10 kg of B.W.), group 2(10.1~15 kg of B.W.)and group 3(15.1~20 kg of B.W.). Each group was subdivided into the cyanotic heart disease group and the acyanotic. The results of our study showed that CO2 retention was not seen in either group. We observed that the Bain circuit system was very satisfactory and valuable in all groups during controlled ventilation, and this technique is also suitable for prolonged surgery(4~10 hrs) in infants and children.
Body Weight ; Child ; Heart Diseases ; Humans ; Infant ; Respiration ; Thoracic Surgery ; Ventilation