We present the therapeutic effects of an aromatic retinoid (Ro 10-9359) in two cases of sex-linked ichthyosis. Case 1, Uncle of Case 2, was a 24-year-old male who had had dark brown scales on the trunk and upper extremities for 15 years. Case 2 was a 10-year-old boy who had had dirty brown scales on the trunk and lower extremities since 3 years of age. The diagnosis of sex-linked ichthyosis was made by characteristic scales and histopathologic findings. Initially, both patients received an oral aromatic retinoid (Ro 10-9359) with a daily dose of 1.5mg/kg body weight for the first two weeks. Thereafter the drug was tapered gradually to the dose of 0.5mg/kg body weight according to the therapeutic response. There was near complete clearing of the skin lesions in both patients after 10 weeks treatment. Side effects were of mild intensity and included dryness of mouth and nose, chelitis, paronychia and arthralgia of knee joint.
Arthralgia ; Body Weight ; Child ; Diagnosis ; Humans ; Ichthyosis* ; Knee Joint ; Lower Extremity ; Male ; Mouth ; Nose ; Paronychia ; Skin ; Upper Extremity ; Weights and Measures ; Young Adult

No abstract available.
Adolescent* ; Humans ; Intention* ; Smoke* ; Smoking Cessation* ; Smoking*

It is believed that the photopatch test is a valuable screening procedure for the determination of responsible antigenic substances in photoallergic contact dermatitis. But the technigues used for this procedure are cumbersome and require expensive equipment. In the present study, we adapted an easy and inexpensive photopatch test met- hod to our need. We report the results of this test method which was performed. in 21 patients with photodermatitis and 9 patients with polymorphic light eruption. 1. The UVB sensitivity determined by minimal erytherna dose (mean+-standard deviation) was I1618mW sec/cm in photodermatitis group, 108+ROmW sec/cm in polymorphic light eruption group and 126-+32mW-sec/cm in control group. But there was no significant difference among them (p>0. 05). 2. The reaction to UVA were negative in all groups. R. The photopatch test using IO potential photosensitizers revealed 12 positive responses in 9 patients; including 7 patients in photodermatitis group and 2 patients in polymorphic light eruption group. And the number of positive photopatch responses obtaied with each photosensitizer in RO patients with photodermatoses was 5 in chlorhexidine, 2 in paraaminobenzoic acid, musk ambrette and bithionol and 1 in chlorpromazine, respectively.
Bithionol ; Chlorhexidine ; Chlorpromazine ; Dermatitis, Photoallergic ; Humans ; Mass Screening ; Photosensitivity Disorders ; Photosensitizing Agents

BACKGROUND: The high concentration of plasma total homocysteine is recently considered an independent risk factor for atherosclerosis. The purpose of this study was to provide reference ranges for plasma homocysteine levels and to investigate the relationship between plasma homocysteine and cardiovascular risk factors in healthy Korean men. METHODS: Anthropometric parameters, alcohol intake, cigarette use and nutrient intake were determined in 166 healthy men within a wide age range(30-69 yr). Serum levels of lipids, glucose and insulin levels during oral glucose tolerance test(OGTT), plasma amino acid concentrations and levels of antioxidant nutrients and enzymes were also measured. Hyperhomocysteinemia was defined as plasma homocysteine levels above the 90th percentile(> or =15micromol/L) of respective plasma homocysteine distribution in study subjects. Characteristics of hyperhomocysteinemic men(n=16) were compared to normohomocys- teinemic men(n=16) matched for age and body mass index. RESULTS: Plasma total homocysteine values ranged from 2.4 to 38.1micromol/L, a skewed, right-tailed distribution. The homocysteine levels of 25th, 50th and 75th percentile were 7.02, 9.61 and 12.4micromol/L, respectively. The mean concentration of plasma total homocysteine was 10.7micromol/L. Plasma total homocysteine level was positively correlated to body mass index, serum cholesterol and triglyceride levels and alcohol intake, but negatively correlated to serum bata-carotene concentration. In multivariate analysis, serum triglyceride level was the strongest determinant of plasma total homocysteine concentration. There were no significant differences between two groups in waist to hip ratio, alcohol intake, cigarette use, blood pressure and serum levels of glucose and insulin during OGTT. Hyperhomocysteinemic men had significantly higher mean values of serum triglyceride(258mg/dl), total cholesterol(226mg/dl), and LDL-cholesterol(140mg/dl) than normohomocysteinemic men. Hyperhomocysteinemic men showed a decrease in lipid corrected values of serum bata-carotene and alpha-tocopherol and plasma concentrations of serine and taurine, when compared to normohomocysteinemic men. The mean intakes of vitamin B6, folate, vitamin B12 and bata-carotene tended to decline by 25-30% in hyper- homocysteinemic group, when compared to normohomocysteinemic group. CONCLUSION: Our results indicate that healthy Korean men with hyperhomocys- teinemia show signs of hyperlipidemia and decreased antioxidants nutrients and these factors increase risk for coronary artery disease. In addition, hyperhomocysteinemia may affect amino acid metabolism related to homocysteine.
alpha-Tocopherol ; Antioxidants ; Atherosclerosis ; Blood Pressure ; Body Mass Index ; Cholesterol ; Coronary Artery Disease ; Folic Acid ; Glucose ; Glucose Tolerance Test ; Homocysteine* ; Humans ; Hyperhomocysteinemia ; Hyperlipidemias ; Insulin ; Male ; Metabolism ; Multivariate Analysis ; Plasma* ; Reference Values ; Risk Factors* ; Serine ; Taurine ; Tobacco Products ; Triglycerides ; Vitamin B 12 ; Vitamin B 6 ; Waist-Hip Ratio

No abstract available.
Hemophilia A* ; Humans

Dyke-Davidoff-Masson syndrome is a rare clinical syndrome consisting of hemiatrophy of cerebral hemisphere, enlargement of ventricles and prominent pneumatization of petrous pyramid of the temporal bone. Its clinical manifestations are hemiparesis, several types of seizure, mental retardation and other neurologic deficits. We experienced a case of Dyke-Davidoff-Masson syndrome in a four months old boy who visited to our department due to right side upper extremity weakness and infantile spasm. The brain magnetic resonance imaging showed marked hemiatrophy of left cerebral hemisphere and enlargement of left lateral ventricle. So with the review of literatures, we report a case of Dyke-Davidoff-Masson syndrome with infantile spasm.
Brain ; Cerebrum ; Humans ; Infant ; Infant, Newborn ; Intellectual Disability ; Lateral Ventricles ; Magnetic Resonance Imaging ; Male ; Neurologic Manifestations ; Paresis ; Petrous Bone ; Seizures ; Spasms, Infantile* ; Temporal Bone ; Upper Extremity

No abstract available.
Osteoma, Osteoid* ; Tetracycline*

No abstract available.
Endocrinology* ; Metabolism*

We studied thirteen, all male, patients who had trans-scaphoid perilunar dislocation(stage I, 5 cases) and dislocation of lunate(stage IV, 8 cases). Age of patient was ranged from 25 years to 49 years(avr. 36 years)and the most common cause of injury was fall down(6 cases, 46%). Seven acute cases, diagnosed and treated within 3 weeks from injury, were treated by open reduction and K-wire fixation(5 cases), and K-wire fixation and bone graft(1 case) and Herbert screw for fracture of scaphoid(1 case). Six chronic cases diagnosed and treated after 3 weeks from injury were treated by open reduction and K-wire fixation(1 case), K-wire fixation and bone graft(1 case), removal of lunate(2 cases), and arthroplasty using muscle after removal of lunate(2 cases). Thirteen cases were followed up for average 33 months(from 13 months to 81 months). Grip power was evaluated as excellent or good in 8 cases and fair in 5 cases. Range of motion of wrist joint was decreased 21 degrees in flexion, 22 degrees in extension, 4 degrees in ulnar deviation, and 5 degrees in radial deviation of wrist joint, compared with uninjured side. Carpal height and carpal height ratio were decreased to 33.1 and 0.51, compared with normal 35.8(p=0.001) and 0.55(P < 0.001), respectively. Between 2 groups of acute and chronic cases, no significant differences were noted in carpal height or carpal height ratio. The surgical treatment of perilunar fracture-dislocation has not simple option, therefore, we would recommend the early diagnosis and exact anatomical reduction.
Arthroplasty ; Dislocations ; Early Diagnosis ; Hand Strength ; Humans ; Male ; Range of Motion, Articular ; Wrist Joint

This erratum is being published to revise the website address of the Korean Reference Genome Database (KRGDB) and correct two typographical errors in the article.